Paediatric
Paediatric
Paediatric
A 2-month old baby is admitted to the Paediatric Ward with persistent vomiting and failure to gain weight.
Bloods taken on admission show the following:
Na+
136 mmol/l
K+
3.1 mmol/l
Cl81 mmol/l
HCO3-30 mmol/l
An ultrasound of the stomach and duodenum is performed:
Pyloric stenosis
Malrotation
Coeliac disease
Next question
The ultrasound demonstrates a thickened and elongated pylorus. The bloods also show a hypochloraemic, hypokalaemic
alkalosis in keeping with the diagnosis.
Pyloric stenosis
Pyloric stenosis typically presents in the second to fourth weeks of life with vomiting, although rarely may present later at up to
four months. It is caused by hypertrophy of the circular muscles of the pylorus
Epidemiology
Features
Question 2 of 257
A mother brings her 14-month-old son into surgery. Since yesterday he seems to be straining whilst passing stools. She describes
him screaming, appearing to be in pain and pulling his knees up towards his chest. These episodes are now occurring every 1520 minutes. This morning she noted a small amount of blood in his nappy. He is taking around 50% of his normal feeds and
vomited once this morning. On examination he appears irritable and lethargic but is well hydrated and apyrexial. Abdominal
examination is unremarkable. What is the most likely diagnosis?
Constipation
Intussusception
Gastroenteritis
Meckel's diverticulum
Volvulus
Next question
Intussusception
Intussusception describes the invagination of one portion of bowel into the lumen of the adjacent bowel, most commonly around
the ileo-caecal region.
Intussusception is usually affects infants between 6-18 months old. Boys are affected twice as often as girls
Features
during paroxysm the infant will characteristically draw their knees up and turn pale
vomiting
blood stained stool - 'red-currant jelly'
sausage-shaped mass in the abdomen
Investigation
ultrasound is now the investigation of choice and may show a target -like mass
Management
the majority of children can be treated with reduction by air insufflation under radiological control, which is now widely
used first-line compared to the traditional barium enema
if this fails, or the child has signs of peritonitis, surgery is performed
Question 3 of 257
A 2-month-old boy is brought to the afternoon surgery by his mother. Since the morning he has been taking reduced feeds and
has been 'not his usual self'. On examination the baby appears well but has a temperature of 38.7C. What is the most
appropriate management?
Advise regarding antipyretics, to see if not settling
IM benzylpenicillin
Admit to hospital
Next question
Any child less than 3 months old with a temperature > 38C is regarded as a 'red' feature in the new NICE guidelines, warranting
urgent referral to a paediatrician. Although many experienced GPs may choose not to strictly follow such advice it is important to
be aware of recent guidelines for the exam
temperature
heart rate
respiratory rate
capillary refill time
Signs of dehydration (reduced skin turgor, cool extremities etc) should also be looked for
Measuring temperature should be done with an electronic thermometer in the axilla if the child is < 4 weeks or with an
electronic/chemical dot thermometer in the axilla or an infra-red tympanic thermometer.
Risk stratification
Please see the link for the complete table, below is a modified version
Green - low risk
Normal colour
Responds normally to
social cues
Content/smiles
Stays awake or awakens
quickly
Strong normal cry/not
crying
Colour
Activity
Nasal flaring
Tachypnoea: respiratory rate
Respiratory
Circulation and
hydration
Other
Management
If green:
Child can be managed at home with appropriate care advice, including when to seek further help
If amber:
provide parents with a safety net or refer to a paediatric specialist for further assessment
a safety net includes verbal or written information on warning symptoms and how further healthcare can be accessed, a
follow-up appointment, liaison with other healthcare professionals, e.g. out-of-hours providers, for further follow-up
If red:
oral antibiotics should not be prescribed to children with fever without apparent source
if a pneumonia is suspected but the child is not going to be referred to hospital then a chest x-ray does not need to be
routinely performed
Recommendations
1.1 Thermometers and the detection of fever
1.2 Clinical assessment of children with fever
1.3 Management by remote assessment
1.4 Management by the non-paediatric practitioner
1.5 Management by the paediatric specialist
1.6 Antipyretic interventions
1.7 Advice for home care
1 Recommendations
The following guidance is based on the best available evidence. The full guideline gives
details of the methods and the evidence used to develop the guidance.
The wording used in the recommendations in this guideline denotes the certainty with
which the recommendation is made (the strength of the recommendation). See About
this guideline for details.
This guideline is intended for use by healthcare professionals for the assessment and
initial management in young children with feverish illness. The guideline should be
followed until a clinical diagnosis of the underlying condition has been made. Once a
diagnosis has been made, the child should be treated according to national or local
guidance for that condition.
Parents or carers of a child with fever may approach a range of different healthcare
professionals as their first point of contact, for example, a GP, a pharmacist or an
emergency care practitioner. The training and experience of the healthcare
professionals involved in the child's care will vary and each should interpret the
guidance according to the scope of their own practice.
For the purposes of this guideline, fever was defined as 'an elevation of body
temperature above the normal daily variation'.
This guideline should be read in conjunction with:
Bacterial meningitis and meningococcal septicaemia (NICE clinical guideline 102).
Urinary tract infection in children (NICE clinical guideline 54).
Diarrhoea and vomiting in children under 5 (NICE clinical guideline 84).
1.1 Thermometers and the detection of fever
1.1.1 Oral and rectal temperature measurements
1.1.1.1 Do not routinely use the oral and rectal routes to measure the body temperature
of children aged 05 years. [2007]
1.1.2 Measurement of body temperature at other sites
1.1.2.1 In infants under the age of 4 weeks, measure body temperature with an
electronic thermometer in the axilla. [2007]
1.1.2.2 In children aged 4 weeks to 5 years, measure body temperature by one of the
following methods:
electronic thermometer in the axilla
chemical dot thermometer in the axilla
infra-red tympanic thermometer. [2007]
1.1.2.3 Healthcare professionals who routinely use disposable chemical dot
thermometers should consider using an alternative type of thermometer when multiple
temperature measurements are required. [2007]
1.1.2.4 Forehead chemical thermometers are unreliable and should not be used by
healthcare professionals. [2007]
1.1.3 Subjective detection of fever by parents and carers
1.1.3.1 Reported parental perception of a fever should be considered valid and taken
seriously by healthcare professionals. [2007]
1.2 Clinical assessment of children with fever
1.2.1 Life-threatening features of illness in children
1.2.1.1 First, healthcare professionals should identify any immediately life-threatening
features, including compromise of the airway, breathing or circulation, and decreased
level of consciousness. [2007]
1.2.2 Assessment of risk of serious illness
1.2.2.1 Assess children with feverish illness for the presence or absence of symptoms
and signs that can be used to predict the risk of serious illness using the traffic light
system (see table 1). [2013]
1.2.2.2 When assessing children with learning disabilities, take the individual child's
learning disability into account when interpreting the traffic light table. [new 2013]
1.2.2.3 Recognise that children with any of the following symptoms or signs are in a
high-risk group for serious illness:
pale/mottled/ashen/blue skin, lips or tongue
no response to social cues[3]
appearing ill to a healthcare professional
does not wake or if roused does not stay awake
weak, high-pitched or continuous cry
grunting
respiratory rate greater than 60 breaths per minute
moderate or severe chest indrawing
reduced skin turgor
bulging fontanelle. [new 2013]
1.2.2.4 Recognise that children with any of the following symptoms or signs are in at
least an intermediate-risk group for serious illness:
pallor of skin, lips or tongue reported by parent or carer
not responding normally to social cues[3]
no smile
wakes only with prolonged stimulation
decreased activity
nasal flaring
dry mucous membranes
poor feeding in infants
reduced urine output
rigors. [new 2013]
1.2.2.5 Recognise that children who have all of the following features, and none of the
high- or intermediate-risk features, are in a low-risk group for serious illness:
normal colour of skin, lips and tongue
responds normally to social cues[3]
content/smiles
stays awake or awakens quickly
strong normal cry or not crying
normal skin and eyes
moist mucous membranes. [new 2013]
1.2.2.6 Measure and record temperature, heart rate, respiratory rate and capillary refill
time as part of the routine assessment of a child with fever. [2007]
1.2.2.7 Recognise that a capillary refill time of 3 seconds or longer is an intermediaterisk group marker for serious illness ('amber' sign). [2013]
1.2.2.8 Measure the blood pressure of children with fever if the heart rate or capillary
refill time is abnormal and the facilities to measure blood pressure are available. [2007]
1.2.2.9 In children older than 6 months do not use height of body temperature alone to
identify those with serious illness. [2013]
1.2.2.10 Recognise that children younger than 3 months with a temperature of 38C or
higher are in a high-risk group for serious illness. [2013]
1.2.2.11 Recognise that children aged 36 months with a temperature of 39C or higher
are in at least an intermediate-risk group for serious illness. [new 2013]
1.2.2.12 Do not use duration of fever to predict the likelihood of serious illness.
However, children with a fever lasting more than 5 days should be assessed for
Kawasaki disease (see recommendation 1.2.3.10). [new 2013]
1.2.2.13 Recognise that children with tachycardia are in at least an intermediate-risk
group for serious illness. Use the Advanced Paediatric Life Support (APLS)[4] criteria
below to define tachycardia: [new 2013]
Age
<12 months
>160
>140
1.2.2.14 Assess children with fever for signs of dehydration. Look for:
lethargy
irritability
abdominal pain or tenderness
urinary frequency or dysuria. [new 2013]
1.2.3.9 Consider septic arthritis/osteomyelitis in children with fever and any of the
following signs:
swelling of a limb or joint
not using an extremity
non-weight bearing. [2007]
1.2.3.10 Consider Kawasaki disease in children with fever that has lasted longer than
5 days and who have 4 of the following 5 features:
bilateral conjunctival injection
change in mucous membranes in the upper respiratory tract (for example, injected
pharynx, dry cracked lips or strawberry tongue)
change in the extremities (for example, oedema, erythema or desquamation)
polymorphous rash
cervical lymphadenopathy
Be aware that, in rare cases, incomplete/atypical Kawasaki disease may be diagnosed
with fewer features. [2007]
1.2.4 Imported infections
1.2.4.1 When assessing a child with feverish illness, enquire about recent travel abroad
and consider the possibility of imported infections according to the region visited.
[2007]
Table 1 Traffic light system for identifying risk of serious illness
[new 2013]
Children with fever and any of the symptoms or signs in the red column should be
recognised as being at high risk. Similarly, children with fever and any of the symptoms
or signs in the amber column and none in the red column should be recognised as being
at intermediate risk. Children with symptoms and signs in the green column and none in
the amber or red columns are at low risk. The management of children with fever
should be directed by the level of risk.
This traffic light table should be used in conjunction with the recommendations in this
guideline on investigations and initial management in children with fever.
A colour version of this table is available.
Colour
(of skin, lips or
tongue)
Normal colour
Pallor reported by
parent/carer
Pale/mottled/ashen/blue
Activity
No smile
Stays awake or
awakens quickly
Weak, high-pitched or
continuous cry
Nasal flaring
Grunting
Tachypnoea: respiratory
rate
Other
Status epilepticus
Focal neurological signs
Focal seizures
Table 2 Summary table for symptoms and signs suggestive of specific diseases
[2013]
Diagnosis to be
considered
Meningococcal
disease
Pneumonia
Vomiting
Poor feeding
Lethargy
Irritability
Abdominal pain or tenderness
Urinary frequency or dysuria
Septic arthritis
Kawasaki disease
providing the parent or carer with verbal and/or written information on warning
symptoms and how further healthcare can be accessed (see section 1.7.2)
arranging further follow-up at a specified time and place
liaising with other healthcare professionals, including out-of-hours providers, to ensure
direct access for the child if further assessment is required. [2007]
1.4.2.4 Children with 'green' features and none of the 'amber' or 'red' features can be
cared for at home with appropriate advice for parents and carers, including advice on
when to seek further attention from the healthcare services (see section 1.7). [2007,
amended 2013]
1.4.3 Tests by the non-paediatric practitioner
1.4.3.1 Children with symptoms and signs suggesting pneumonia who are not admitted
to hospital should not routinely have a chest X-ray. [2007]
1.4.3.2 Test urine in children with fever as recommended in Urinary tract infection in
children (NICE clinical guideline 54). [2007]
1.4.4 Use of antibiotics by the non-paediatric practitioner
1.4.4.1 Do not prescribe oral antibiotics to children with fever without apparent source.
[2007]
1.4.4.2 Give parenteral antibiotics to children with suspected meningococcal disease at
the earliest opportunity (either benzylpenicillin or a third-generation cephalosporin)[5].
[2007]
1.5 Management by the paediatric specialist
In this guideline, the term paediatric specialist refers to a healthcare professional who
has had specific training or has recognised expertise in the assessment and treatment of
children and their illnesses. Examples include paediatricians, or healthcare professionals
working in children's emergency departments.
1.5.1 Children younger than 5 years
1.5.1.1 Management by the paediatric specialist should start with a clinical assessment
as described in section 1.2. The healthcare professional should attempt to identify
symptoms and signs of serious illness and specific diseases as summarised in tables 1
and 2. [2007]
1.5.2 Children younger than 3 months
1.5.2.1 Infants younger than 3 months with fever should be observed and have the
following vital signs measured and recorded:
temperature
heart rate
respiratory rate. [2007]
1.5.2.2 Perform the following investigations in infants younger than 3 months with
fever:
full blood count
blood culture
C-reactive protein
urine testing for urinary tract infection[6]
should have urine tested for urinary tract infection[6] and be assessed for symptoms
and signs of pneumonia (see table 2). [2007]
1.5.3.5 Do not routinely perform blood tests and chest X-rays in children with fever who
have no features of serious illness (that is, the 'green' group). [2007]
1.5.4 Viral co-infection
1.5.4.1 Febrile children with proven respiratory syncytial virus or influenza infection
should be assessed for features of serious illness. Consideration should be given to urine
testing for urinary tract infection[6]. [2007]
1.5.5 Observation in hospital
1.5.5.1 In children aged 3 months or older with fever without apparent source, a period
of observation in hospital (with or without investigations) should be considered as part
of the assessment to help differentiate non-serious from serious illness. [2007]
1.5.5.2 When a child has been given antipyretics, do not rely on a decrease or lack of
decrease in temperature at 12 hours to differentiate between serious and non-serious
illness. Nevertheless, in order to detect possible clinical deterioration, all children in
hospital with 'amber' or 'red' features should still be reassessed after 12 hours. [new
2013]
1.5.6 Immediate treatment by the paediatric specialist (for children of all ages)
1.5.6.1 Children with fever and shock presenting to specialist paediatric care or an
emergency department should be:
given an immediate intravenous fluid bolus of 20 ml/kg; the initial fluid should normally
be 0.9% sodium chloride
actively monitored and given further fluid boluses as necessary. [2007]
1.5.6.2 Give immediate parenteral antibiotics to children with fever presenting to
specialist paediatric care or an emergency department if they are:
shocked
unrousable
showing signs of meningococcal disease. [2007]
1.5.6.3 Immediate parenteral antibiotics should be considered for children with fever
and reduced levels of consciousness. In these cases symptoms and signs of meningitis
and herpes simplex encephalitis should be sought (see table 2 and Bacterial meningitis
and meningococcal septicaemia [NICE clinical guideline 102]). [2007]
1.5.6.4 When parenteral antibiotics are indicated, a third-generation cephalosporin (for
example, cefotaxime or ceftriaxone) should be given, until culture results are available.
For children younger than 3 months, an antibiotic active against listeria (for example,
ampicillin or amoxicillin) should also be given. [2007]
1.5.6.5 Give intravenous aciclovir to children with fever and symptoms and signs
suggestive of herpes simplex encephalitis (see recommendation 1.2.3.5). [2007]
1.5.6.6 Oxygen should be given to children with fever who have signs of shock or oxygen
saturation (SpO2) of less than 92% when breathing air. Treatment with oxygen should
also be considered for children with an SpO2 of greater than 92%, as clinically indicated.
[2007]
to keep their child away from nursery or school while the child's fever persists but to
notify the school or nursery of the illness. [2007]
1.7.2 When to seek further help
1.7.2.1 Following contact with a healthcare professional, parents and carers who are
looking after their feverish child at home should seek further advice if:
the child has a fit
the child develops a non-blanching rash
the parent or carer feels that the child is less well than when they previously sought
advice
the parent or carer is more worried than when they previously sought advice
the fever lasts longer than 5 days
the parent or carer is distressed, or concerned that they are unable to look after their
child. [2007]
Question 4 of 257
A 3-year-old girl is brought in by her mother. Her mother reports that she has been eating less and refusing food for the past few
weeks. Despite this her mother has noticed that her abdomen is distended and she has developed a 'beer belly'. For the past y ear
she has opened her bowels around once every other day, passing a stool of 'normal' consistency. There are no urinary symptoms.
On examination she is on the 50th centile for height and weight. Her abdomen is soft but slightly distended and a non -tender
ballotable mass can be felt on the left side. Her mother has tried lactulose but there has no significant improvement. What is the
most appropriate next step in management?
Switch to polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) and review in two weeks
Reassure normal findings and advise Health Visitor review to improve oral intake
Next question
The history of constipation is not particularly convincing. A child passing a stool of normal consistency every other day is within the
boundaries of normal. The key point to this question is recognising the abnormal examination finding - a ballotable mass
associated with abdominal distension. Whilst an adult with such a 'red flag' symptom/sign would be fast-tracked it is more
appropriate to speak to a paediatrician to determine the best referral pathway, which would probably be clinic review the sam e
week.
Wilms' tumour
Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age,
with a median age of 3 years old.
Features
Associations
Beckwith-Wiedemann syndrome
as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
hemihypertrophy
around one-third of cases are associated with a mutation in the WT1 gene on chromosome 11
Management
nephrectomy
chemotherapy
radiotherapy if advanced disease
prognosis: good, 80% cure rate
Histological features include epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells and small cell
blastomatous tissues resembling the metanephric blastema
Question 5 of 257
A 6-month-old baby who was born in Bangladesh is brought to surgery. Around one week ago he started with coryzal symptoms.
His mother reports he has not been feeding well for the past two days and has started to vomit today. Her main concern is a
cough which occurs in bouts and is so severe he often turns red. No inspiratory or expiratory noises are noted. Clinical
examination reveals an apyrexial child with a clear chest. What is the most likely diagnosis?
Bronchiolitis
Mycoplasma pneumonia
Pertussis
Tuberculosis
Next question
coughing bouts: usually worse at night and after feeding, may be ended by vomiting & associated central cyanosis
inspiratory whoop: not always present (caused by forced inspiration against a closed glottis)
persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures
symptoms may last 10-14 weeks* and tend to be more severe in infants
marked lymphocytosis
Diagnosis
per nasal swab culture for Bordetella pertussis - may take several days or weeks to come back
PCR and serology are now increasingly used as their availability becomes more widespread
Management
Complications
subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures
Women who are between 28-38 weeks pregnant will be offered the vaccine.
*weeks, not days
Question 6 of 257
At what age would the average child acquire the ability to walk unsupported?
6-7 months
8-9 months
10-11 months
13-15 months
2 years
Next question
Milestone
Little or no head lag on being pulled to sit
3 months
Lying on abdomen, good head control
Held sitting, lumbar curve
Lying on abdomen, arms extended
Lying on back, lifts and grasps feet
6 months
Pulls self to sitting
Held sitting, back straight
Rolls front to back
7-8 months Sits without support (Refer at 12 months)
Pulls to standing
9 months
Crawls
Cruises
12 months
Walks with one hand held
13-15 monthsWalks unsupported (Refer at 18 months)
18 months Squats to pick up a toy
Runs
2 years
Walks upstairs and downstairs holding on to rail
Rides a tricycle using pedals
3 years
Walks up stairs without holding on to rail
4 years
Hops on one leg
Notes
the majority of children crawl on all fours before walking but some children 'bottom -shuffle'. This is a normal variant and
runs in families
Question 7 of 257
A male child from a travelling community is diagnosed with measles. Which one of the following complications is he at risk from in
the immediate aftermath of the initial infection?
Arthritis
Pancreatitis
Infertility
Pneumonia
Next question
Subacute sclerosing panencephalitis is seen but develops 5-10 years following the illness. Pancreatitis and infertility may follow
mumps infection
Measles
Overview
RNA paramyxovirus
spread by droplets
infective from prodrome until 4 days after rash starts
incubation period = 10-14 days
Features
Koplik spots
Complications
encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness
febrile convulsions
The rash typically starts behind the ears and then spreads to the whole body
Management of contacts
if a child not immunized against measles comes into contact with measles then MMR should be offered (vaccine-induced
measles antibody develops more rapidly than that following natural infection)
this should be given within 72 hours
Question 8 of 257
Which one of the following is the most common cause of nephrotic syndrome in children?
Minimal change disease
IgA nephropathy
Chronic pyelonephritis
Next question
Minimal change glomerulonephritis nearly always presents as nephrotic syndrome, accounting for 80% of cases in children and
25% in adults. The majority of cases are idiopathic and respond well to steroids
In children the peak incidence is between 2 and 5 years of age. Around 80% of cases in children are due to a condition called
minimal change glomerulonephritis. The condition generally carries a good prognosis with around 90% of cases responding to
high-dose oral steroids.
Other features include hyperlipidaemia, a hypercoagulable state (due to loss of antithrombin III) and a predisposition to infection
(due to loss of immunoglobulins)
Question 9 of 257
A 5-year-old boy from a travelling community presents to the Emergency Department with breathing difficulties. On examination
he has a temperature of 38.2C, stridor and a toxic looking appearance. A diagnosis of acute epiglottitis is suspected. Which one
of the following organisms is most likely to be responsible?
Epstein Barr Virus
Streptococcus pneumoniae
Neisseria meningitidis
Haemophilus influenzae
Staphylococcus aureus
Next question
Patients from travelling communities may not always receive a full course of immunisation
Acute epiglottitis
Acute epiglottitis is rare but serious infection caused by Haemophilus influenzae type B. Prompt recognition and treatment is
essential as airway obstruction may develop. Epiglottitis was generally considered a disease of childhood but in the UK it is now
more common in adults due to the immunisation programme. The incidence of epiglottitis has decreased since the introduction o f
the Hib vaccine
Features
rapid onset
high temperature, generally unwell
stridor
drooling of saliva
Birth
Question 10 of 257
At what age would the average child start to smile?
2 weeks
6 weeks
3 months
4 months
Next question
Age
Milestone
6 weeks Smiles (Refer at 10 weeks)
Laughs
3 months
Enjoys friendly handling
6 monthsNot shy
Shy
9 months
Takes everything to mouth
Feeding
Age
Milestone
May put hand on bottle when being fed
6 months
Drinks from cup + uses spoon, develops over 3 month period 12 -15 months
Competent with spoon, doesn't spill with cup
2 years
Uses spoon and fork
3 years
Uses knife and fork
5 years
Dressing
Age
Milestone
Helps getting dressed/undressed
12-15 months
Takes off shoes, hat but unable to replace
18 months
Puts on hat and shoes
2 years
Can dress and undress independently except for laces and buttons 4 years
Play
Age
Milestone
Plays 'peek-a-boo'
9 months
Waves 'bye-bye'
12 months
Plays 'pat-a-cake'
Plays contentedly alone
18 months
Plays near others, not with them 2 years
Plays with other children
4 years
Question 11 of 257
A 7-year-old boy is brought in to the GP surgery with an exacerbation of asthma. On examination he has a bilateral expiratory
wheeze but there are no signs of respiratory distress. His respiratory rate is 36 / min and PEF around 60% of normal. What is the
most appropriate action with regards to steroid therapy?
Oral prednisolone for 3 days
Next question
Severe attack
Life-threatening attack
SpO2 <92%
SpO2 < 92%
Silent chest
Too breathless to talk or feed
SpO2 > 92%
Poor respiratory effort
Heart rate > 140/min
No clinical features of severe asthma
Agitation
Respiratory rate > 40/min
Altered consciousness
Use of accessory neck muscles
Cyanosis
Children greater than 5 years of age
Attempt to measure PEF in all children aged > 5 years.
Moderate attack
Severe attack
Life-threatening attack
SpO2 < 92%
SpO2 < 92%
SpO2 > 92%
PEF 33-50% best or predicted
PEF < 33% best or predicted
PEF > 50% best or predicted Can't complete sentences in one breath or too breathless to talk or feed Silent chest
No clinical features of
Heart rate > 125/min
Poor respiratory effort
severe asthma
Respiratory rate > 30/min
Altered consciousness
Use of accessory neck muscles
Cyanosis
For children with mild to moderate acute asthma:
Bronchodilator therapy
give a beta-2 agonist via a spacer (for a child < 3 years use a close-fitting mask)
give 1 puff every 15-30 seconds up to a maximum of 10 puffs; repeat dose after 10-20 minutes if necessary
if symptoms are not controlled repeat beta-2 agonist and refer to hospital
Steroid therapy
Question 12 of 257
A 9-year-old boy is brought to surgery with recurrent headaches. What is the most common cause of headaches in children?
Migraine
Depression
Refractive errors
Tension-type headache
Cluster headache
Next question
Migraine is the most common cause of headache in children
Headache in children
Some of the following is based on an excellent review article on the Great Ormond Street Hospital website. Please see the link for
more details.
Epidemiology
up to 50 per cent of 7-year-olds and up to 80 per cent of 15-year-old have experienced at least one headache
equally as common in boys/girls until puberty then strong (3:1) female preponderance
Migraine
Migraine without aura is the most common cause of primary headache in children. The International Headache Society (IHS) have
produced criteria for paediatric migraine without aura:
A>= 5 attacks fulfilling features B to D
B Headache attack lasting 4-72 hours
Headache has at least two of the following four features:
Acute management
Prophylaxis
no nausea or vomiting
photophobia and phonophobia, or one, but not the other is present
Question 13 of 257
The parents of a 14-month-old girl present to their GP. They have noticed that in some photos there is no 'red eye' on the left hand
side. When you examine the girl you notice an esotropic strabismus and a loss of the red-reflex in the left eye. There is a family
history of a grandparent having an enucleation as a child. What is the most likely diagnosis?
Congenital hypertrophy of the retinal pigment epithelium
Neuroblastoma
Retinoblastoma
Congenital cataract
Next question
A congenital cataract may cause a loss of the red-reflex but is likely to have been detected at birth or during the routine babychecks. It would also not explain the family history of enucleation.
Retinoblastoma
Retinoblastoma is a the most common ocular malignancy found in children. The average age of diagnosis is 18 months.
Pathophysiology
Possible features
absence of red-reflex, repalced by a white pupil (leukocoria) - the most common presenting symptom
strabismus
visual problems
Management
Prognosis
Question 14 of 257
Which one of the following is an example of a purely secondary accident prevention strategy?
Speed limits
Stair guards
Cycling helmets
Next question
Speed limits are an example of both a primary and secondary accident prevention strategy, whereas cycling helmets are purely a
secondary strategy.
Accidents in children
Around 15-20% of children attend Emergency Departments in the course of a year due to an accident. Accidents account for a
third of all childhood deaths and are the single most common cause of death in children aged between 1 - 15 years of age.
Key points
road traffic accidents are the most common cause of fatal accidents
boys and children from lower social classes are more likely to have an accident
Accident prevention
Accident prevention can be divided up into primary (preventing the accident from happening), secondary (prevent injury from t he
accident) and tertiary (limit the impact of the injury) prevention strategies
The table below gives examples of accident prevention strategies
Question 15 of 257
A mother is concerned about the risk of her son developing influenza. Her son is fit and otherwise well. Following NHS
immunisation guidance, at what age should the child first be offered the influenza vaccine?
3 months
4 months
12-13 months
2-3 years
65 years
Next question
Influenza vaccination
Seasonal influenza still accounts for a significant morbidity and mortality in the UK each winter, with the influenza season typically
starting in the middle of November. This may vary year from year so it is recommended that vaccination occurs between
September and early November. There are three types of influenza virus; A, B and C. Types A and B account for the majority of
clinical disease.
Prior to 2013 flu vaccination was only offered to the elderly and at risk groups.
Remember that the type of vaccine given routinely to children and the one given to the elderly and at risk groups is different (live
vs. inactivated) - this explains the different contraindications
Children
A new NHS influenza vaccination programme for children was announced in 2013. There are three key things to remember about
the children's vaccine:
it is given intranasally
the first dose is given at 2-3 years, then annually after that
it is a live vaccine (cf. injectable vaccine below)
children who were traditionally offered the flu vaccine (e.g. asthmatics) will now be given intranasal vaccine unless this is
inappropriate, for example if they are immunosuppressed. In this situation the inactivated, injectable vaccine should be
given
only children aged 2-9 years who have not received an influenza vaccine before need 2 doses
it is more effective than the injectable vaccine
Contraindications
immunocompromised
aged < 2 years
current febrile illness or blocked nose/rhinorrhoea
current wheeze (e.g. ongoing viral-induced wheeze/asthma) or history of severe asthma (BTS step 4)
egg allergy
pregnancy/breastfeeding
if the child is taking aspirin (e.g. for Kawasaki disease) due to a risk of Reye's syndrome
Side-effects
blocked-nose/rhinorrhoea
headache
anorexia
health and social care staff directly involved in patient care (e.g. NHS staff)
those living in long-stay residential care homes
carers of the elderly or disabled person whose welfare may be at risk if the carer becomes ill (at the GP's discretion)
it is an inactivated vaccine, so cannot cause influenza. A minority of patients however develop fever and malaise which
may last 1-2 days
should be stored between +2 and +8C and shielded from light
contraindications include hypersensitivity to egg protein.
in adults the vaccination is around 75% effective, although this figure decreases in the elderly
it takes around 10-14 days after immunisation before antibody levels are at protective levels
Question 16 of 257
Which one of the following statements regarding croup is true?
Symptoms are typically worse during the day
Next question
Croup
Croup is a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a
combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases.
Epidemiology
Features
stridor
barking cough (worse at night)
fever
coryzal symptoms
Clinical Knowledge Summaries (CKS) suggest using the following criteria to grade the severity*:
Mild
Moderate
Severe
Frequent barking cough
Frequent barking cough
Occasional barking cough
Prominent inspiratory (and occasionally, expiratory)
Easily audible stridor at rest
No audible stridor at rest
stridor at rest
Suprasternal and sternal wall retraction
No or mild suprasternal and/or
Marked sternal wall retractions
at rest
intercostal recession
Significant distress and agitation, or lethargy or
No or little distress or agitation
The child is happy and is prepared to
restlessness (a sign of hypoxaemia)
The child can be placated and is
eat, drink, and play
Tachycardia occurs with more severe obstructive
interested in its surroundings
symptoms and hypoxaemia
CKS suggest admitting any child with moderate or severe croup. Other features which should prompt admission include:
Management
CKS recommend giving a single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity
prednisolone is an alternative if dexamethasone is not available
Emergency treatment
high-flow oxygen
nebulised adrenaline
*these in turn are based partly on the Alberta Medical Association (2008) Guideline for the diagnosis and management of croup.
Croup - Summary
Question 17 of 257
A newborn baby is transferred to the neonatal intensive care unit shortly after birth due to respiratory distress. An x-ray
taken on arrival is shown below:
Left pneumothorax
Next question
Bowel loops can be seen in the left side of the thoracic cavity.
Question 18 of 257
Which one of the following is responsible for causing scarlet fever?
Group A haemolytic streptococci
Staphylococcus aureus
Parvovirus B19
Coxsackie A16
Next question
Scarlet fever
Scarlet fever is a reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes).
It is more common in children aged 2 - 6 years with the peak incidence being at 4 years.
Scarlet fever has an incubation period of 2-4 days and typically presents with:
fever
malaise
tonsillitis
'strawberry' tongue
rash - fine punctate erythema ('pinhead') which generally appears first on the torso and spares the face although children
often have a flushed appearance with perioral pallor. The rash often has a rough 'sandpaper' texture. Desquamination
occurs later in the course of the illness, particularly around the fingers and toes
Diagnosis
a throat swab is normally taken but antibiotic treatment should be commenced immediately, rather than waiting for the
results
Management
oral penicillin V
patients who have a penicillin allergy should be given azithromycin
children can return to school 24 hours after commencing antibiotics
scarlet fever is a notifiable disease
Complications
Question 19 of 257
Which of the following conditions is inherited in an autosomal recessive fashion?
Homocystinuria
Huntington's disease
Hereditary spherocytosis
Next question
Autosomal recessive conditions are 'metabolic' - exceptions: inherited ataxias
Autosomal dominant conditions are 'structural' - exceptions: Gilbert's, hyperlipidaemia type II
Huntington's disease, hereditary non-polyposis colorectal carcinoma, hereditary spherocytosis and hypokalaemic periodic
paralysis are all inherited in an autosomal dominant fashion
some 'metabolic' conditions such as Hunter's and G6PD are X-linked recessive whilst others such as hyperlipidemia type
II and hypokalemic periodic paralysis are autosomal dominant
some 'structural' conditions such as ataxia telangiectasia and Friedreich's ataxia are autosomal recessive
Albinism
Ataxia telangiectasia
Congenital adrenal hyperplasia
Cystic fibrosis
Cystinuria
Familial Mediterranean Fever
Fanconi anaemia
Friedreich's ataxia
Gilbert's syndrome*
Glycogen storage disease
Haemochromatosis
Homocystinuria
Lipid storage disease: Tay-Sach's, Gaucher, Niemann-Pick
Mucopolysaccharidoses: Hurler's
PKU
Sickle cell anaemia
Thalassaemias
Wilson's disease
*this is still a matter of debate and many textbooks will list Gilbert's as autosomal dominant
Question 20 of 257
A mother brings her 6-year-old son into surgery. He is not doing well at school and she worries he may be 'hyperactive'. Which one of the
following features is not consistent with a diagnosis of Attention Deficit Hyperactivity Disorder (ADHD)?
Poor concentration
Impulsiveness
Uncontrolled activity
Repetitive behaviour
Extreme restlessness
Next question
Repetitive behaviour is not part of the diagnostic criteria for ADHD and may suggest a disorder on the autistic spectrum.
extreme restlessness
poor concentration
uncontrolled activity
impulsiveness
ADHD is diagnosed in about 5% of American children, In the UK, where the term hyperkinetic syndrome is preferred, only 0.1% of children
are diagnosed with the condition. The male:female ratio is 5:1
Management
1 Guidance
1.1 Prerequisites of treatment and care for all people with ADHD
1.2 Identification, pre-diagnostic intervention in the community and referral to secondary
services
1.3 Diagnosis of ADHD
1.4 Post-diagnostic advice
1.5 Treatment for children and young people
1.6 Transition to adult services
1.7 Treatment of adults with ADHD
1.8 How to use drugs for the treatment of ADHD
1 Guidance
March 2013: In treatment for children and young people footnote 6 has been
updated. Recommendations 1.5.1.7 and 1.5.1.9 have been removed and are
replaced by recommendations 1.5.2 and 1.5.4 in 'Antisocial behaviour and
conduct disorders in children and young people' (NICE clinical guideline 158).
Recommendation 1.5.1.10 has been removed.
The following guidance is based on the best available evidence. The full
guideline gives details of the methods and the evidence used to develop the
guidance.
1.1 Prerequisites of treatment and care for all people with ADHD
People with ADHD require integrated care that addresses a wide range of
personal, social, educational and occupational needs. Care should be provided
by adequately trained healthcare and education professionals.
1.1.1 Organisation and planning of services
1.1.1.1 People with ADHD would benefit from improved organisation of care and
better integration of paediatric, child and adolescent mental health services
(CAMHS) and adult mental health services
1.1.1.2 Mental health trusts, and children's trusts that provide mental health/child
development services, should form multidisciplinary specialist ADHD teams
and/or clinics for children and young people and separate teams and/or clinics for
adults. These teams and clinics should have expertise in the diagnosis and
management of ADHD, and should:
produce local protocols for shared care arrangements with primary care
providers, and ensure that clear lines of communication between primary
and secondary care are maintained
1.1.2 Information, consent, the law and support for people with ADHD and their carers
Many people with ADHD, and their parents or carers, experience stigma and
other difficulties because of the symptoms and impairment associated with
ADHD and current practice within healthcare and education. The following
recommendations have been developed based on the experiences of people with
ADHD and their families.
respecting the person and their family's knowledge and experience of ADHD
1.1.2.2 Healthcare professionals should provide people with ADHD and their
families or carers with relevant, age-appropriate information (including written
information) about ADHD at every stage of their care. The information should
cover diagnosis and assessment, support and self-help, psychological treatment,
and the use and possible side effects of drug treatment.
1.1.2.3 When assessing a child or young person with ADHD, and throughout
their care, healthcare professionals should:
allow the child or young person to give their own account of how they feel,
and record this in the notes
involve the child or young person and the family or carer in treatment
decisions
1.1.2.4 Healthcare professionals working with children and young people with
ADHD should be:
familiar with local and national guidelines on confidentiality and the rights of
the child
1.1.2.5 Healthcare professionals should work with children and young people
with ADHD and their parents or carers to anticipate major life changes (such as
puberty, starting or changing schools, the birth of a sibling) and make appropriate
arrangements for adequate personal and social support during times of
increased need. The need for psychological treatment at these times should be
considered.
1.1.2.6 Adults with ADHD should be given written information about local and
national support groups and voluntary organisations.
1.1.2.7 Healthcare professionals should ask families or carers about the impact
of ADHD on themselves and other family members, and discuss any concerns
they may have. Healthcare professionals should:
offer general advice to parents and carers about positive parent and carer
child contact, clear and appropriate rules about behaviour, and the
importance of structure in the child or young person's day
1.1.3 Training
Children and young people with behavioural problems suggestive of ADHD can
be referred by their school or primary care practitioner for parenttraining/education programmes without a formal diagnosis of ADHD. The
diagnosis of ADHD in children, young people and adults should take place in
secondary care.
1.2.1 Identification and referral in children and young people with ADHD
1.2.1.6 If the child or young person's behavioural and/or attention problems are
associated with severe impairment, referral should be made directly to secondary
care (that is, a child psychiatrist, paediatrician, or specialist ADHD CAMHS) for
assessment.
1.2.1.7 Group-based parent-training/education programmes are recommended in
the management of children with conduct disorders [4].
1.2.1.8 Primary care practitioners should not make the initial diagnosis or start
drug treatment in children or young people with suspected ADHD.
1.2.1.9 A child or young person who is currently treated in primary care with
methylphenidate, atomoxetine, dexamfetamine, or any other psychotropic drug
for a presumptive diagnosis of ADHD, but has not yet been assessed by a
specialist in ADHD in secondary care, should be referred for assessment to a
child psychiatrist, paediatrician, or specialist ADHD CAMHS as a matter of
clinical priority.
1.2.2.2 Adults who have previously been treated for ADHD as children or young
people and present with symptoms suggestive of continuing ADHD should be
referred to general adult psychiatric services for assessment. The symptoms
should be associated with at least moderate or severe psychological and/or
social or educational or occupational impairment.
1.3 Diagnosis of ADHD
a full clinical and psychosocial assessment of the person; this should include
discussion about behaviour and symptoms in the different domains and
settings of the person's everyday life, and
1.3.1.2 A diagnosis of ADHD should not be made solely on the basis of rating
scale or observational data. However rating scales such as the Conners' rating
scales and the Strengths and Difficulties questionnaire are valuable adjuncts,
and observations (for example, at school) are useful when there is doubt about
symptoms.
1.3.1.3 For a diagnosis of ADHD, symptoms of hyperactivity/impulsivity and/or
inattention should:
1.3.1.4 ADHD should be considered in all age groups, with symptom criteria
adjusted for age-appropriate changes in behaviour.
1.3.1.5 In determining the clinical significance of impairment resulting from the
symptoms of ADHD in children and young people, their views should be taken
into account wherever possible.
1.4 Post-diagnostic advice
After diagnosis people with ADHD and their parents or carers may benefit from
advice about diet, behaviour and general care.
1.4.1 General advice
1.4.2.1 Healthcare professionals should stress the value of a balanced diet, good
nutrition and regular exercise for children, young people and adults with ADHD.
1.4.2.2 The elimination of artificial colouring and additives from the diet is not
recommended as a generally applicable treatment for children and young people
with ADHD.
1.4.2.3 Clinical assessment of ADHD in children and young people should
include asking about foods or drinks that appear to influence their hyperactive
behaviour. If there is a clear link, healthcare professionals should advise parents
or carers to keep a diary of food and drinks taken and ADHD behaviour. If the
diary supports a relationship between specific foods and drinks and behaviour,
then referral to a dietitian should be offered. Further management (for example,
specific dietary elimination) should be jointly undertaken by the dietitian, mental
health specialist or paediatrician, and the parent or carer and child or young
person.
1.4.2.4 Dietary fatty acid supplementation is not recommended for the treatment
of ADHD in children and young people.
there are particular difficulties for families in attending group sessions (for
example, because of disability, needs related to diversity such as language
differences, parental ill-health, problems with transport, or where other
factors suggest poor prospects for therapeutic engagement)
1.5.1.6 When individual-based parent-training/education programmes for preschool children with ADHD are undertaken, the skills training stages should
involve both the parents or carers and the child.
1.5.1.7 This recommendation has been replaced by recommendations 1.5.2 and
1.5.4 in 'Antisocial behaviour and conduct disorders in children and young
people' (NICE clinical guideline 158).
1.5.1.8 Consideration should be given to involving both of the parents or all
carers of children or young people with ADHD in parent-training/education
programmes wherever this is feasible.
review the child, with their parents or carers and siblings, for any residual
coexisting conditions and develop a treatment plan for these if needed
1.5.2.3 Teachers who have received training about ADHD and its management
should provide behavioural interventions in the classroom to help children and
young people with ADHD.
1.5.2.4 If the child or young person with ADHD has moderate levels of
impairment, the parents or carers should be offered referral to a group parenttraining/education programme, either on its own or together with a group
treatment programme (CBT and/or social skills training) for the child or young
person.
1.5.2.5 When using group treatment (CBT and/or social skills training) for the
child or young person in conjunction with a parent-training/education programme,
particular emphasis should be given to targeting a range of areas, including
social skills with peers, problem solving, self-control, listening skills and dealing
with and expressing feelings. Active learning strategies should be used, and
rewards given for achieving key elements of learning.
1.5.2.6 For older adolescents with ADHD and moderate impairment, individual
psychological interventions (such as CBT or social skills training) may be
considered as they may be more effective and acceptable than group parenttraining/education programmes or group CBT and/or social skills training.
1.5.2.7 For children and young people (including older age groups) with ADHD
and a learning disability, a parent-training/education programme should be
offered on either a group or individual basis, whichever is preferred following
discussion with the parents or carers and the child or young person.
1.5.2.8 When parents or carers of children or young people with ADHD
undertake parent-training/education programmes, the professional delivering the
sessions should consider contacting the school and providing the child or young
person's teacher with written information on the areas of behavioural
management covered in these sessions. This should only be done with parental
consent.
1.5.2.9 Following successful treatment with a parent-training/education
programme and before considering discharge from secondary care, the child or
young person should be reviewed, with their parents or carers and siblings, for
any residual problems such as anxiety, aggression or learning difficulties.
Treatment plans should be developed for any coexisting conditions.
1.5.2.10 Following treatment with a parent-training/education programme,
children and young people with ADHD and persisting significant impairment
should be offered drug treatment.
1.5.3 Treatment for school-age children and young people with severe ADHD (hyperkinetic disorder) and severe
impairment
The first-line treatment for school-age children and young people with severe
ADHD (hyperkinetic disorder) and severe impairment is drug treatment. If the
child or young person wishes to refuse medication and/or the parents or carers
reject it, a psychological intervention may be tried but drug treatment has more
benefits and is superior to other treatments for this group.
1.5.3.1 In school-age children and young people with severe ADHD, drug
treatment should be offered as the first-line treatment. Parents should also be
offered a group-based parent-training/education programme.
1.5.3.2 Drug treatment should only be initiated by an appropriately qualified
healthcare professional with expertise in ADHD and should be based on a
comprehensive assessment and diagnosis. Continued prescribing and
monitoring of drug therapy may be performed by general practitioners, under
shared care arrangements[7].
1.5.3.3 If drug treatment is not accepted by the child or young person with severe
ADHD, or their parents or carers, healthcare professionals should advise parents
or carers and the child or young person about the benefits and superiority of drug
treatment in this group. If drug treatment is still not accepted, a group parenttraining/education programme should be offered.
1.5.3.4 If a group parent-training/education programme is effective in children
and young people with severe ADHD who have refused drug treatment,
healthcare professionals should assess the child or young person for possible
coexisting conditions and develop a longer-term care plan.
1.5.3.5 If a group parent-training/education programme is not effective for a child
or young person with severe ADHD, and if drug treatment has not been
accepted, discuss the possibility of drug treatment again or other psychological
treatment (group CBT and/or social skills training), highlighting the clear benefits
and superiority of drug treatment in children or young people with severe ADHD.
1.5.3.6 Following a diagnosis of severe ADHD in a school-age child or young
person healthcare professionals should, with the parents' or carers' consent,
contact the child or young person's teacher to explain:
1.5.3.7 Teachers who have received training about ADHD and its management
should provide behavioural interventions in the classroom to help children and
young people with ADHD.
1.5.4 Pre-drug treatment assessment
risk assessment for substance misuse and drug diversion (where the drug is
passed on to others for non-prescription use).
1.5.4.2 Drug treatment for children and young people with ADHD should always
form part of a comprehensive treatment plan that includes psychological,
behavioural and educational advice and interventions.
1.5.5 Choice of drug for children and young people with ADHD
1.5.5.3 When a decision has been made to treat children or young people with
ADHD with drugs, healthcare professionals should consider:
convenience
improving adherence
reducing stigma (because the child or young person does not need to take
medication at school)
1.5.5.5 When starting drug treatment children and young people should be
monitored for side effects. In particular, those treated with atomoxetine should be
closely observed for agitation, irritability, suicidal thinking and self-harming
behaviour, and unusual changes in behaviour, particularly during the initial
months of treatment, or after a change in dose. Parents and/or carers should be
warned about the potential for suicidal thinking and self-harming behaviour with
atomoxetine and asked to report these to their healthcare professionals. Parents
or carers should also be warned about the potential for liver damage in rare
cases with atomoxetine (usually presenting as abdominal pain, unexplained
nausea, malaise, darkening of the urine or jaundice).
1.5.5.6 If there is a choice of more than one appropriate drug, the product with
the lowest cost (taking into account the cost per dose and number of daily doses)
should be prescribed[7].
1.5.5.7 Antipsychotics are not recommended for the treatment of ADHD in
children and young people.
1.5.6 Poor response to treatment
and atomoxetine in a child or young person with ADHD, there should be a further
review of:
the diagnosis
uptake and use of psychological interventions for the child or young person
and their parents or carers
1.5.6.2 Following review of poor response to treatment, a dose higher than that
licensed for methylphenidate or atomoxetine should be considered following
consultation with a tertiary or regional centre. This may exceed 'British national
formulary' (BNF) recommendations: methylphenidate can be increased to
0.7 mg/kg per dose up to three times a day or a total daily dose of 2.1 mg/kg/day
(up to a total maximum dose of 90 mg/day for immediate release; or an
equivalent dose of modified-release methylphenidate)[8]; atomoxetine may be
increased to 1.8 mg/kg/day (up to a total maximum dose of 120 mg/day). The
prescriber should closely monitor the child or young person for side effects.
1.5.6.3 Dexamfetamine should be considered in children and young people
whose ADHD is unresponsive to a maximum tolerated dose of methylphenidate
or atomoxetine.
1.5.6.4 In children and young people whose ADHD is unresponsive to
methylphenidate, atomoxetine and dexamfetamine, further treatment should only
follow after referral to tertiary services. Further treatment may include the use of
medication unlicensed for the treatment of ADHD (such as bupropion, clonidine,
modafinil and imipramine)[9] or combination treatments (including psychological
treatments for the parent or carer and the child or young person). The use of
medication unlicensed for ADHD should only be considered in the context of
tertiary services.
1.5.6.5 A cardiovascular examination and ECG should be carried out before
starting treatment with clonidine in children or young people with ADHD.
1.6 Transition to adult services
Young people with ADHD receiving treatment and care from CAMHS or
paediatric services should normally be transferred to adult services if they
continue to have significant symptoms of ADHD or other coexisting conditions
that require treatment. Transition should be planned in advance by both referring
and receiving services. If needs are severe and/or complex, use of the care
programme approach should be considered.
1.6.1.1 A young person with ADHD receiving treatment and care from CAMHS or
paediatric services should be reassessed at school-leaving age to establish the
need for continuing treatment into adulthood. If treatment is necessary,
arrangements should be made for a smooth transition to adult services with
details of the anticipated treatment and services that the young person will
require. Precise timing of arrangements may vary locally but should usually be
completed by the time the young person is 18 years.
1.6.1.2 During the transition to adult services, a formal meeting involving CAMHS
and/or paediatrics and adult psychiatric services should be considered, and full
information provided to the young person about adult services. For young people
aged 16 years and older, the care programme approach (CPA) should be used
as an aid to transfer between services. The young person, and when appropriate
the parent or carer, should be involved in the planning.
1.6.1.3 After transition to adult services, adult healthcare professionals should
carry out a comprehensive assessment of the person with ADHD that includes
personal, educational, occupational and social functioning, and assessment of
any coexisting conditions, especially drug misuse, personality disorders,
emotional problems and learning difficulties.
1.7 Treatment of adults with ADHD
Drug treatment is the first-line treatment for adults with ADHD with either
moderate or severe levels of impairment. Methylphenidate is the first-line drug.
Psychological interventions without medication may be effective for some adults
with moderate impairment, but there are insufficient data to support this
recommendation. If methylphenidate is ineffective or unacceptable, atomoxetine
or dexamfetamine can be tried. If there is residual impairment despite some
benefit from drug treatment, or there is no response to drug treatment, CBT may
be considered. There is the potential for drug misuse and diversion in adults with
ADHD, especially in some settings, such as prison, although there is no strong
evidence that this is a significant problem.
1.7.1.1 For adults with ADHD, drug treatment[10] should be the first-line treatment
unless the person would prefer a psychological approach.
1.7.1.2 Drug treatment for adults with ADHD should be started only under the
guidance of a psychiatrist, nurse prescriber specialising in ADHD, or other clinical
prescriber with training in the diagnosis and management of ADHD.
1.7.1.3 Before starting drug treatment for adults with ADHD a full assessment
should be completed, which should include:
weight
1.7.1.4 Drug treatment for adults with ADHD should always form part of a
comprehensive treatment programme that addresses psychological, behavioural
and educational or occupational needs.
1.7.1.5 Following a decision to start drug treatment in adults with ADHD,
methylphenidate should normally be tried first.
1.7.1.6 Atomoxetine or dexamfetamine should be considered in adults
unresponsive or intolerant to an adequate trial of methylphenidate (this should
usually be about 6 weeks)[10]. Caution should be exercised when prescribing
dexamfetamine to those likely to be at risk of stimulant misuse or diversion.
1.7.1.7 When starting drug treatment, adults should be monitored for side effects.
In particular, people treated with atomoxetine should be observed for agitation,
irritability, suicidal thinking and self-harming behaviour, and unusual changes in
behaviour, particularly during the initial months of treatment, or after a change in
dose. They should also be warned of potential liver damage in rare cases
(usually presenting as abdominal pain, unexplained nausea, malaise, darkening
of the urine or jaundice). Younger adults aged 30 years or younger should also
be warned of the potential of atomoxetine to increase agitation, anxiety, suicidal
thinking and self-harming behaviour in some people, especially during the first
few weeks of treatment.
1.7.1.8 For adults with ADHD stabilised on medication but with persisting
functional impairment associated with the disorder, or where there has been no
response to drug treatment, a course of either group or individual CBT to address
the person's functional impairment should be considered. Group therapy is
recommended as the first-line psychological treatment because it is the most
cost effective.
1.7.1.9 For adults with ADHD, CBT may be considered when:
the person has made an informed choice not to have drug treatment
people have difficulty accepting the diagnosis of ADHD and accepting and
adhering to drug treatment
1.7.1.10 Where there may be concern about the potential for drug misuse and
diversion (for example, in prison services), atomoxetine may be considered as
the first-line drug treatment for ADHD in adults[10].
1.7.1.11 Drug treatment for adults with ADHD who also misuse substances
should only be prescribed by an appropriately qualified healthcare professional
with expertise in managing both ADHD and substance misuse. For adults with
ADHD and drug or alcohol addiction disorders there should be close liaison
between the professional treating the person's ADHD and an addiction specialist.
1.7.1.12 Antipsychotics are not recommended for the treatment of ADHD in
adults.
1.8 How to use drugs for the treatment of ADHD
Good knowledge of the drugs used in the treatment of ADHD and their different
preparations is essential (refer to the BNF and summaries of product
characteristics). It is important to start with low doses and titrate upwards,
monitoring effects and side effects carefully. Higher doses may need to be
prescribed to some adults. The recommendations on improving adherence in
children and young people may also be of use in adults.
1.8.1 General principles
1.8.1.1 Prescribers should be familiar with the pharmacokinetic profiles of all the
modified-release and immediate-release preparations available for ADHD to
ensure that treatment is tailored effectively to the individual needs of the child,
young person or adult.
1.8.1.2 Prescribers should be familiar with the requirements of controlled drug
legislation governing the prescription and supply of stimulants.
1.8.1.3 During the titration phase, doses should be gradually increased until there
is no further clinical improvement in ADHD (that is, symptom reduction,
behaviour change, improvements in education and/or relationships) and side
effects are tolerable.
1.8.1.4 Following titration and dose stabilisation, prescribing and monitoring
should be carried out under locally agreed shared care arrangements with
primary care.
1.8.1.5 Side effects resulting from drug treatment for ADHD should be routinely
monitored and documented in the person's notes.
1.8.1.6 If side effects become troublesome in people receiving drug treatment for
ADHD, a reduction in dose should be considered.
1.8.1.7 Healthcare professionals should be aware that dose titration should be
slower if tics or seizures are present in people with ADHD.
1.8.2 Initiation and titration of methylphenidate, atomoxetine and dexamfetamine in children and young people
1.8.2.1 During the titration phase, symptoms and side effects should be recorded
at each dose change on standard scales (for example, Conners' 10-item scale)
by parents and teachers, and progress reviewed regularly (for example, by
weekly telephone contact and at each dose change) with a specialist clinician.
1.8.2.2 If using methylphenidate in children and young people with ADHD aged
6 years and older:
the dose should be titrated against symptoms and side effects over 4
6 weeks until dose optimisation is achieved
1.8.2.3 If using atomoxetine in children and young people with ADHD aged
6 years and older:
for those weighing up to 70 kg, the initial total daily dose should be
approximately 0.5 mg/kg; the dose should be increased after 7 days to
approximately 1.2 mg/kg/day
for those weighing more than 70 kg, the initial total daily dose should be
40 mg; the dose should be increased after 7 days up to a maintenance dose
of 80 mg/day
a single daily dose can be given; two divided doses may be prescribed to
minimise side effects.
initial treatment should begin with low doses consistent with starting doses in
the BNF
the dose should be titrated against symptoms and side effects over 4
6 weeks
1.8.3.1 In order to optimise drug treatment, the initial dose should be titrated
against symptoms and side effects over 46 weeks.
1.8.3.2 During the titration phase, symptoms and side effects should be recorded
at each dose change by the prescriber after discussion with the person with
ADHD and, wherever possible, a carer (for example, a spouse, parent or close
initial treatment should begin with low doses (5 mg three times daily for
immediate-release preparations; the equivalent dose for modified-release
preparations)
the dose should be titrated against symptoms and side effects over 4
6 weeks
for people with ADHD weighing up to 70 kg, the initial total daily dose should
be approximately 0.5 mg/kg; the dose should be increased after 7 days to
approximately 1.2 mg/kg/day
for people with ADHD weighing more than 70 kg, the initial total daily dose
should be 40 mg; the dose should be increased after 7 days up to a
maintenance dose of 100 mg/day
the usual maintenance dose is either 80 or 100 mg, which may be taken in
divided doses
the dose should be titrated against symptoms and side effects over 4
6 weeks
the dose should usually be given between two and four times daily.
1.8.4 Monitoring side effects and the potential for misuse in children, young people and adults
weight should be measured 3 and 6 months after drug treatment has started
and every 6 months thereafter in children, young people and adults
1.8.4.3 If there is evidence of weight loss associated with drug treatment in adults
with ADHD, healthcare professionals should consider monitoring body mass
index and changing the drug if weight loss persists.
1.8.4.4 Strategies to reduce weight loss in people with ADHD, or manage
decreased weight gain in children, include:
taking medication either with or after food, rather than before meals
taking additional meals or snacks early in the morning or late in the evening
when the stimulant effects of the drug have worn off
1.8.4.5 If growth is significantly affected by drug treatment (that is, the child or
young person has not met the height expected for their age), the option of a
planned break in treatment over school holidays should be considered to allow
'catch-up' growth to occur.
1.8.4.6 In people with ADHD, heart rate and blood pressure should be monitored
and recorded on a centile chart before and after each dose change and routinely
every 3 months.
1.8.4.7 For people taking methylphenidate, dexamfetamine and atomoxetine,
routine blood tests and ECGs are not recommended unless there is a clinical
indication.
1.8.4.8 Liver damage is a rare and idiosyncratic adverse effect of atomoxetine
and routine liver function tests are not recommended.
1.8.4.9 For children and young people taking methylphenidate and
dexamfetamine, healthcare professionals and parents or carers should monitor
changes in the potential for drug misuse and diversion, which may come with
changes in circumstances and age. In these situations, modified-release
methylphenidate or atomoxetine may be preferred.
1.8.4.10 In young people and adults, sexual dysfunction (that is, erectile and
ejaculatory dysfunction) and dysmenorrhoea should be monitored as potential
side effects of atomoxetine.
For children and young people with ADHD, the strategies outlined in the
recommendations below should be considered to improve treatment adherence.
Similar strategies, adapted for age, may be considered for adults.
1.8.5.1 Communication between the prescriber and the child or young person
should be improved by educating parents or carers and ensuring there are
regular three-way conversations between prescriber, parent or carer and the
child or young person. For adults with ADHD, and with their permission, a
spouse, partner, parent, close friend or carer wherever possible should be part of
these conversations. Clear instructions about how to take the drug should be
offered in picture or written format, which may include information on dose,
duration, side effects, dosage schedule, the need for supervision and how this
should be done.
1.8.5.2 Healthcare professionals should consider suggesting peer-support
groups for the child or young person with ADHD and their parents or carers if
adherence to drug treatment is difficult or uncertain.
It is advisable to review each year whether the child or young person needs to
continue drug treatment and to ensure that the long-term pattern of use is
tailored to the person's needs, preferences and circumstances.
1.8.6.1 Following an adequate treatment response, drug treatment for ADHD
should be continued for as long as it remains clinically effective. This should be
reviewed at least annually. The review should include a comprehensive
assessment of clinical need, benefits and side effects, taking into account the
views of the child or young person, as well as those of parents, carers and
teachers, and how these views may differ. The effect of missed doses, planned
dose reductions and brief periods of no treatment should be taken into account
and the preferred pattern of use should also be reviewed. Coexisting conditions
should be reviewed, and the child or young person treated or referred if
necessary. The need for psychological and social support for the child or young
person and for the parents or other carers should be assessed.
1.8.6.2 Drug holidays are not routinely recommended; however, consideration
should be given to the parent or carer and child or young person with ADHD
working with their healthcare professional to find the best pattern of use, which
may include periods without drug treatment.
1.8.7 Duration, discontinuation and continuity of treatment in adults
be reviewed, and the person treated or referred if necessary. The need for
psychological, social and occupational support for the person and their carers
should be assessed.
1.8.7.2 An individual treatment approach is important for adults, and healthcare
professionals should regularly review (at least annually) the need to adapt
patterns of use, including the effect of drug treatment on coexisting conditions
and mood changes.
Question 21 of 257
A 3-year-old boy is brought to surgery by his father. He is currently being managed with paracetamol for a middle ear infection but his father
is concerned his heart is racing. What is the normal heart rate of a 3-year-old?
90 - 140 bpm
50 - 70 bpm
60 - 100 bpm
70 - 120 bpm
Next question
110 - 160 30 - 40
1 - 2 100 - 150 25 - 35
2 - 5 90 - 140
25 - 30
5 - 12 80 - 120
20 - 25
> 12 60 - 100
15 - 20
Question 22 of 257
A 2-year-old boy is seen in the Emergency Department with watery diarrhoea for the past two day. What is the most likely causative agent?
Norovirus
Rotavirus
E. coli
Norwalk virus
Adenovirus
Next question
diarrhoea usually lasts for 5-7 days and stops within 2 weeks
vomiting usually lasts for 1-2 days and stops within 3 days
When assessing hydration status NICE advocate using normal, dehydrated or shocked categories rather than the traditional normal, mild,
moderate or severe categories.
Clinical dehydration
Clinical shock
Tachycardia
Tachypnoea
Weak peripheral pulses
Prolonged capillary refill time
Hypotension
jittery movements
increased muscle tone
hyperreflexia
convulsions
drowsiness or coma
Diagnosis
NICE suggest doing a stool culture in the following situations:
Management
If clinical shock is suspected children should be admitted for intravenous rehydration.
For children with no evidence of dehydration
If dehydration is suspected:
give 50 ml/kg low osmolarity oral rehydration solution (ORS) solution over 4 hours, plus ORS solution for maintenance, often
and in small amounts
continue breastfeeding
consider supplementing with usual fluids (including milk feeds or water, but not fruit juices or carbonated drinks)
Question 23 of 257
You are asked to give a talk to local district nurses about childhood diseases. In the UK, what is the most common cause of death in
children greater than one year old?
Cancer
Non-accidental injury
Congenital disorders
Infection
Accidents
Next question
Accidents in children
Around 15-20% of children attend Emergency Departments in the course of a year due to an accident. Accidents account for a third of all
childhood deaths and are the single most common cause of death in children aged between 1 - 15 years of age.
Key points
road traffic accidents are the most common cause of fatal accidents
boys and children from lower social classes are more likely to have an accident
Accident prevention
Accident prevention can be divided up into primary (preventing the accident from happening), secondary (prevent injury from the accident)
and tertiary (limit the impact of the injury) prevention strategies
The table below gives examples of accident prevention strategies
Primary prevention
Stair guards
Wearing seat belts
Speed limits*
Cycling helmets
Teaching parents first aid
Teaching road safety Smoke alarms
Window safety catchesLaminated safety glass
*some strategies such as reducing driving speed may have a role in both primary and secondary accident prevention
Question 24 of 257
A 16-month-old girl is reviewed by her GP. She has a 3 day history of fever and coryzal symptoms. Overnight she has developed a harsh
cough. On examination she has a temperature of 38C and inspiratory stridor is noticed although there are no signs of intercostal
recession. What is the most likely diagnosis?
Bronchiolitis
Croup
Bacterial tracheitis
Pertussis
Acute epiglottitis
Next question
Croup
Croup is a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a
combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases.
Epidemiology
Features
stridor
barking cough (worse at night)
fever
coryzal symptoms
Clinical Knowledge Summaries (CKS) suggest using the following criteria to grade the severity*:
Mild
Moderate
Severe
CKS suggest admitting any child with moderate or severe croup. Other features which should prompt admission include:
Management
CKS recommend giving a single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity
prednisolone is an alternative if dexamethasone is not available
Emergency treatment
high-flow oxygen
nebulised adrenaline
*these in turn are based partly on the Alberta Medical Association (2008) Guideline for the diagnosis and management of croup.
Question 25 of 257
Next question
Infantile colic
Infantile colic describes a relatively common and benign set of symptoms seen in young infants. It typically occurs in infants less than 3
months old and is characterised by bouts of excessive crying and pulling-up of the legs, often worse in the evening
Infantile colic occurs in up to 20% of infants. The cause of infantile colic is unknown
Infantile colic is defined for clinical purposes as repeated episodes of excessive and
inconsolable crying in an infant that otherwise appears to be healthy and thriving.
The underlying cause is unknown.
Estimates of prevalence range from 520% of infants, depending on the definition used.
Infantile colic can cause significant distress and suffering to the parents.
Typically:
o Colic starts in the first weeks of life and resolves by around 4 months of age.
o Crying most often occurs in the late afternoon or evening.
o The baby draws its knees up to its abdomen, or arches its back when crying.
The history and examination should include the:
o General health of the baby.
o Antenatal and perinatal history.
o Onset and length of crying.
o Nature of the stools.
o Feeding assessment.
o Mother's diet if breastfeeding.
o Family history of allergy.
o Parent's response to the baby's crying.
o Factors which lessen or worsen the crying.
The most useful intervention is support for parents and reassurance that infantile colic
will resolve.
Holding the baby through the crying episode may be helpful. Other strategies include:
o Gentle motion (e.g. pushing the pram, rocking the crib).
o 'White noise' (e.g. vacuum cleaner, hairdryer, running water).
o Bathing in a warm bath.
Parents should be encouraged to look after their own well-being by:
o Resting when possible.
o Asking family and friends for support. CRY-SIS is a support group for families
(www.cry-sis.org.uk).
o Meeting other parents with babies of the same age.
Health visitors will also provide advice and support.
Medical treatments should only be tried if parents feel unable to cope despite advice
and reassurance. The options for medical treatments are:
o A 1-week trial of simeticone drops (breastfed or bottle-fed).
o A 1-week trial of diet modification to exclude cow's milk protein breastfed babies:
dairy-free diet for the mother; bottle-fed babies: hypoallergenic formula.
o A 1-week trial of lactase drops (breastfed or bottle-fed).
Treatment should only be continued if there is a response (i.e. the duration of crying
shortens). If no response, another treatment can be considered.
Breastfeeding mothers should take a calcium supplement if they are on a dairy-free diet
long term.
If the baby responds to lactase or a hypoallergenic diet, the parents should be
reassured that this does not necessarily mean that they are lactose intolerant or
allergic to cow's milk.
If there is a response to treatment: after the age of 3 months (and by 6 months of age at
the latest), treatment can be weaned off over a period of about 1 week.
Advice from a paediatrician is required if:
o The parents are not coping.
o There is diagnostic doubt.
o It is not possible to wean the baby off treatment by the age of 6 months.
Question 26 of 257
A mother brings her 4-year-old daughter into surgery as she thinks she may have 'nits'. Which one of the following statements regarding
head lice is incorrect?
Malathion is an option for first-line treatment
Fine-toothed combing of wet or dry hair is the standard way of diagnosing head lice
Children should be excluded from school until treatment has been started
Next question
Head lice
Head lice (also known as pediculosis capitis or 'nits') is a common condition in children caused by the parasitic insect Pediculus capitis,
which lives on and among the hair of the scalp of humans
Diagnosis
Management
Question 1 of 231
The chance of a 35-year-old mother giving birth to a child with Down's syndrome is approximately:
1 in 125
1 in 350
1 in 550
1 in 1,000
1 in 2,000
Next question
Down's syndrome risk - 1/1,000 at 30 years then divide by 3 for every 5 years
One way of remembering this is by starting at 1/1,000 at 30 years and then dividing the denominator by 3 (i.e. 3 times more common) for
every extra 5 years of age
Cytogenetics
Mode
Non-disjunction
94%
Robertsonian translocation
5%
(usually onto 14)
Mosaicism
1%
The chance of a further child with Down's syndrome is approximately 1 in 100 if the mother is less than 35 years old. If the trisomy 21 is a
result of a translocation the risk is much higher
Question 2 of 231
Next question
Autism
Epidemiology
Other features
Associated conditions
Fragile X
Rett's syndrome
Question 3 of 231
A 14-month-old boy is brought to surgery. Mum says he has been off his food for the past few days and is a bit 'niggly'. Clinical examination
reveals the following:
Erythema multiforme
Kawasaki disease
Measles
Next question
Management
*The HPA recommends that children who are unwell should be kept off school until they feel better. They also advise that you contact them
if you suspect that there may be a large outbreak.
Question 4 of 231
A 6-year-old boy is brought for review. You can see from his records that he has been treated for constipation in the past but is otherwise fit
and well. His mother reports that he is currently passing only one hard stool every 4-5 days. The stool is described as being like 'rabbit
droppings'. There is no history of overflow soiling or diarrhoea. Examination of the abdomen is unremarkable. What is the most appropriate
first-line intervention?
Advice on diet/fluid intake + lactulose
Next question
Constipation in children
The frequency at which children open their bowels varies widely, but generally decreases with age from a mean of 3 times per day for
infants under 6 months old to once a day after 3 years of age.
NICE produced guidelines in 2010 on the diagnosis and management of constipation in children. A diagnosis of constipation is suggested
by 2 or more of the following:
Child < 1 year
Stool pattern
History
The vast majority of children have no identifiable cause - idiopathic constipation. Other causes of constipation in children include:
dehydration
low-fibre diet
medications: e.g. Opiates
anal fissure
over-enthusiastic potty training
hypothyroidism
Hirschsprung's disease
hypercalcaemia
learning disabilities
After making a diagnosis of constipation NICE then suggesting excluding secondary causes. If no red or amber flags are present then a
diagnosis of idiopathic constipation can be made:
Timing
medicines
Passage of
meconium
< 48 hours
Stool pattern
Growth
> 48 hours
'Ribbon' stools
Generally well, weight and height within normal limits, fit and active
Previously unknown or
undiagnosed weakness in legs,
locomotor delay
Abdomen
Distension
Diet
Other
Prior to starting treatment the child needs to be assessed for faecal impaction. Factors which suggest faecal impaction include:
polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) using an escalating dose regimen as the first-line treatment
add a stimulant laxative if Movicol Paediatric Plain does not lead to disimpaction after 2 weeks
substitute a stimulant laxative singly or in combination with an osmotic laxative such as lactulose if Movicol Paediatric Plain is
not tolerated
inform families that disimpaction treatment can initially increase symptoms of soiling and abdominal pain
Maintenance therapy
very similar to the above regime, with obvious adjustments to the starting dose, i.e.
first-line: Movicol Paediatric Plain
add a stimulant laxative if no response
substitute a stimulant laxative if Movicol Paediatric Plain is not tolerated. Add another laxative such as lactulose or docusate if
stools are hard
continue medication at maintenance dose for several weeks after regular bowel habit is established, then reduce dose gradually
General points
do not use dietary interventions alone as first-line treatment although ensure child is having adequate fluid and fibre intake
consider regular toileting and non-punitive behavioural interventions
for all children consider asking the Health Visitor or Paediatric Continence Advisor to help support the parents.
The NICE guidelines do not specifically discuss the management of very young child. The following recommendations are largely based on
bottle-fed infants: give extra water in between feeds. Try gentle abdominal massage and bicycling the infant's legs
breast-fed infants: constipation is unusual and organic causes should be considered
An adequate fluid intake and a balanced diet with sufficient fibre (unless exclusively
breastfed) should be recommended.
o Faecal impaction should be treated using the recommended disimpaction regimen.
o If impaction is not present or has been treated, the child should be promptly treated
with a laxative (even if the history of constipation is very short).
o Polyethylene glycol 3350 plus electrolytes (Movicol Paediatric Plain) is preferred
as first-line management. If this approach does not work, a stimulant laxative,
lactulose, or docusate should be considered.
Advice should be offered on behavioural interventions such as scheduled toileting, use
of a bowel diary, and reward systems.
Follow up should be arranged regularly to assess adherence and response to treatment
o
Guidance
1 Guidance
The following guidance is based on the best available evidence. The full
guideline gives details of the methods and the evidence used to develop the
guidance.
1.1 History-taking and physical examination
1.1.1 Establish during history-taking whether the child or young person has
constipation. Two or more findings from table 1 indicate constipation.
Table 1 Key components of history-taking to diagnose constipation
Key
components
Stool patterns
Potential findings in a
child/young person older than
1 year
exclusively breastfed
babies after 6 weeks of
age)
Symptoms
associated with
defecation
History
Distress on stooling
Evidence of retentive
posturing: typical straight
legged, tiptoed, back
arching posture
Straining
Anal pain
Straining
Previous episode(s) of
constipation
Previous episode(s) of
constipation
1.1.2 If the child or young person has constipation take a history using table 2 to
establish a positive diagnosis of idiopathic constipation by excluding underlying
causes. If a child or young person has any 'red flag' symptoms, do not treat them
for constipation. Instead, refer them urgently to a healthcare professional with
experience in the specific aspect of child health that is causing concern.
Table 2 Key components of history-taking to diagnose idiopathic
constipation
Key
components
Timing of onset
of constipation
and potential
precipitating
factors
Passage of
meconium
life
Failure to pass
meconium/delay
(more than 48
hours after birth [in
term baby])
'Ribbon stools'
(more likely in a
child younger than
1 year)
Previously
unknown or
undiagnosed
weakness in legs,
locomotor delay
Stool patterns
Growth and
general wellbeing
Symptoms in
legs/locomotor
development
of life
Abdomen
Abdominal
distension with
vomiting
Findings and
'Red flag' findings and
diagnostic clues that diagnostic clues that indicate an
indicate idiopathic underlying disorder or
constipation
condition: not idiopathic
constipation
Inspection of perianal
area: appearance,
position, patency, etc
Normal
appearance of
anus and
surrounding area
Abdominal
examination
Soft abdomen.
Flat or distension
that can be
explained
because of age or
excess weight
Spine/lumbosacral
Normal
Abnormal
appearance/position/patency
of anus: fistulae, bruising,
multiple fissures, tight or
patulous anus, anteriorly
placed anus, absent anal wink
Gross abdominal distension
Abnormal: asymmetry or
region/gluteal
examination
appearance of the
skin and
anatomical
structures of
lumbosacral/glute
al regions
Lower limb
neuromuscular
examination including
tone and strength
Lower limb
neuromuscular
examination: reflexes
(perform only if 'red
flags' in history or
physical examination
suggest new onset
neurological
impairment)
Normal gait.
Normal tone and
strength in lower
limbs
Abnormal neuromuscular
signs unexplained by any
existing condition, such as
cerebral palsy
Reflexes present
and of normal
amplitude
Abnormal reflexes
privacy
informed consent is given by the child or young person, or the parent or legal
guardian if the child is not able to give it, and is documented
a chaperone is present
Endoscopy
1.3.1 Do not use gastrointestinal endoscopy to investigate idiopathic
constipation.
Coeliac disease and hypothyroidism
1.3.2 Test for coeliac disease[2] and hypothyroidism in the ongoing management
of intractable constipation in children and young people if requested by specialist
services.
Manometry
1.3.3 Do not use anorectal manometry to exclude Hirschsprung's disease in
children and young people with chronic constipation.
Radiography
1.3.4 Do not use a plain abdominal radiograph to make a diagnosis of idiopathic
constipation.
1.3.5 Consider using a plain abdominal radiograph only if requested by specialist
services in the ongoing management of intractable idiopathic constipation.
Rectal biopsy
1.3.6 Do not perform rectal biopsy unless any of the following clinical features of
Hirschsprung's disease are or have been present:
Transit studies
1.3.7 Do not use transit studies to make a diagnosis of idiopathic constipation.
1.3.8 Consider using transit studies in the ongoing management of intractable
idiopathic constipation only if requested by specialist services.
Ultrasound
1.3.9 Do not use abdominal ultrasound to make a diagnosis of idiopathic
constipation.
1.3.10 Consider using abdominal ultrasound in the ongoing management of
intractable idiopathic constipation only if requested by specialist services.
1.4 Clinical management
Disimpaction
1.4.1 Assess all children and young people with idiopathic constipation for faecal
impaction, including children and young people who were originally referred to
the relevant services because of 'red flags' but in whom there were no significant
findings following further investigations (see tables 2 and 3). Use a combination
of history-taking and physical examination to diagnose faecal impaction look for
overflow soiling and/or faecal mass palpable abdominally and/or rectally if
indicated.
1.4.2 Start maintenance therapy if the child or young person is not faecally
impacted.
1.4.3 Offer the following oral medication regimen for disimpaction if indicated:
Recommended doses
Macrogols
Polyethylene glycol Paediatric formula: Oral powder: macrogol 3350
Osmotic laxatives
Lactulose
Stimulant laxatives
Sodium picosulfateb Non-BNFC recommended doses
Elixir (5 mg/5 ml)
Bisacodyl
By rectum (suppository)
Sennad
Docusate sodiume
All drugs listed above are given by mouth unless stated otherwise.
Unless stated otherwise, doses are those recommended by the British National
Formulary for Children (BNFC) 2009. Informed consent should be obtained and
documented whenever medications/doses are prescribed that are different from
those recommended by the BNFC.
At the time of publication (May 2010) Movicol Paediatric Plain is the only
macrogol licensed for children under 12 years that includes electrolytes. It does
not have UK marketing authorisation for use in faecal impaction in children under
5 years, or for chronic constipation in children under 2 years. Informed consent
should be obtained and documented. Movicol Paediatric Plain is the only
macrogol licensed for children under 12 years that is also unflavoured.
b
Elixir, licensed for use in children (age range not specified by manufacturer).
Perles not licensed for use in children under 4 years. Informed consent should be
obtained and documented.
c
Syrup not licensed for use in children under 2 years. Informed consent should
be obtained and documented.
e
Adult oral solution and capsules not licensed for use in children under 12 years.
Informed consent should be obtained and documented.
1.4.4 Do not use rectal medications for disimpaction unless all oral medications
have failed and only if the child or young person and their family consent.
1.4.5 Administer sodium citrate enemas only if all oral medications for
disimpaction have failed.
1.4.6 Do not administer phosphate enemas for disimpaction unless under
specialist supervision in hospital/health centre/clinic, and only if all oral
medications and sodium citrate enemas have failed.
1.4.7 Do not perform manual evacuation of the bowel under anaesthesia unless
optimum treatment with oral and rectal medications has failed.
1.4.8 Review children and young people undergoing disimpaction within 1 week.
Maintenance therapy
1.4.9 Start maintenance therapy as soon as the child or young person's bowel is
disimpacted.
1.4.10 Reassess children frequently during maintenance treatment to ensure
they do not become reimpacted and assess issues in maintaining treatment such
as taking medicine and toileting. Tailor the frequency of assessment to the
individual needs of the child and their families (this could range from daily contact
to contact every few weeks). Where possible, reassessment should be provided
by the same person/team.
1.4.11 Offer the following regimen for ongoing treatment or maintenance therapy:
1.5.1 Do not use dietary interventions alone as first-line treatment for idiopathic
constipation.
1.5.2 Treat constipation with laxatives and a combination of:
1.5.3 Advise parents and children and young people (if appropriate) that a
balanced diet should include:
Adequate fibre. Recommend including foods with a high fibre content (such
as fruit, vegetables, high-fibre bread, baked beans and wholegrain breakfast
cereals) (not applicable to exclusively breastfed infants). Do not recommend
unprocessed bran, which can cause bloating and flatulence and reduce the
absorption of micronutrients.
700 ml
assumed to be from breast milk
600 ml
1300 ml
900 ml
48 years
1700 ml
1200 ml
Boys 913
years
2400 ml
1800 ml
Girls 913
years
2100 ml
1600 ml
Boys 1418
years
3300 ml
2600 ml
Girls 1418
years
2300 ml
1800 ml
The above recommendations are for adequate intakes and should not be
interpreted as a specific requirement. Higher intakes of total water will be
required for those who are physically active or who are exposed to hot
environments. It should be noted that obese children may also require higher
total intakes of water.
1.5.4 Provide children and young people with idiopathic constipation and their
families with written information about diet and fluid intake.
1.5.5 In children with idiopathic constipation, start a cows' milk exclusion diet only
on the advice of the relevant specialist services.
1.5.6 Advise daily physical activity that is tailored to the child or young person's
stage of development and individual ability as part of ongoing maintenance in
children and young people with idiopathic constipation.
1.6 Psychological interventions
1.6.1 Do not use biofeedback for ongoing treatment in children and young people
with idiopathic constipation.
1.6.2 Do not routinely refer children and young people with idiopathic
constipation to a psychologist or child and adolescent mental health services
unless the child or young person has been identified as likely to benefit from
receiving a psychological intervention.
1.7 Antegrade colonic enema procedure
1.7.1 Refer children and young people with idiopathic constipation who still have
unresolved symptoms on optimum management to a paediatric surgical centre to
assess their suitability for an antegrade colonic enema (ACE) procedure.
1.7.2 Ensure that all children and young people who are referred for an ACE
procedure have access to support, information and follow-up from paediatric
1.8.1 Provide tailored follow-up to children and young people and their parents or
carers according to the child or young person's response to treatment, measured
by frequency, amount and consistency of stools. Use the Bristol Stool Form
Scale to assess this (see appendix D). This could include:
1.8.2 Offer children and young people with idiopathic constipation and their
families a point of contact with specialist healthcare professionals, including
school nurses, who can give ongoing support.
1.8.3 Healthcare professionals should liaise with school nurses to provide
information and support, and to help school nurses raise awareness of the issues
surrounding constipation with children and young people and school staff.
1.8.4 Refer children and young people with idiopathic constipation who do not
respond to initial treatment within 3 months to a practitioner with expertise in the
problem.
Question 5 of 231
1 in 10
1 in 25
1 in 100
1 in 500
Next question
Cystic fibrosis
Cystic fibrosis (CF) is an autosomal recessive disorder causing increased viscosity of secretions (e.g. lungs and pancreas). It is due to a
defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel
In the UK 80% of CF cases are due to delta F508 on the long arm of chromosome 7. Cystic fibrosis affects 1 per 2500 births, and the
carrier rate is c. 1 in 25
Organisms which may colonise CF patients
Staphylococcus aureus
Pseudomonas aeruginosa
Burkholderia cepacia*
Aspergillus
Question 6 of 231
A 10-month-old boy is brought to surgery as his mother has noticed some noisy breathing. For the past 2-3 days he has had a runny nose
and has felt hot. There is no past medical history of note and he is currently feeding satisfactorily. On examination temperature is 37.6C,
respiratory rate is 36 / min and there is no intercostal recession noted. Chest auscultation reveals a mild expiratory wheeze bilaterally with
the occasional fine crackle. What is the most appropriate management?
Amoxicillin + chest x-ray + review
Paracetamol + review
Admit
Next question
SIGN guidelines do not support the use of bronchodilators in children with bronchiolitis.
Bronchiolitis
Bronchiolitis is a condition characterised by acute bronchiolar inflammation. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of
cases. SIGN released guidelines on bronchiolitis in 2006. Please see the link for more details.
Epidemiology
most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 36 months). Maternal IgG provides protection to newborns against RSV
higher incidence in winter
Basics
Features
Investigation
Question 7 of 231
An emergency call is put out as a 9-year-old boy has collapsed in the waiting room. The receptionists have already dialled 999. He does not
respond to stimulation and no signs of respiration can be found after 10 seconds. There is no obvious foreign body in the mouth. What is
the most appropriate next step?
Start chest compressions/ventilations at a ratio of 15:2
Next question
Current paediatric basic life support guidelines do not yet reflect the change in emphasis away from rescue breaths in adults. This partly
reflects the fact that the majority of paediatric arrests are secondary to airway or breathing problems.
compression:ventilation ratio: lay rescuers should use a ratio of 30:2. If there are two or more rescuers with a duty to respond
then a ratio of 15:2 should be used
age definitions: an infant is a child under 1 year, a child is between 1 year and puberty
Key points of algorithm (please see link attached for more details)
unresponsive?
shout for help
open airway
look, listen, feel for breathing
give 5 rescue breaths
check for signs of circulation
15 chest compressions:2 rescue breaths (see above)
Question 8 of 231
A mother comes to surgery with her 6-year-old son. During the MMR scare she decided not to have her son immunised. However, due to a
recent measles outbreak she asks if he can still receive the MMR vaccine. What is the most appropriate action?
Arrange for measles immunoglobulin to be given
Next question
The Green Book recommends allowing 3 months between doses to maximise the response rate. A period of 1 month is considered
adequate if the child is greater than 10 years of age. In an urgent situation (e.g. an outbreak at the child's school) then a shorter period of 1
month can be used in younger children.
MMR vaccine
Children in the UK receive two doses of the Measles, Mumps and Rubella (MMR) vaccine before entry to primary school. This currently
occurs at 12-15 months and 3-4 years as part of the routine immunisation schedule
Contraindications to MMR
severe immunosuppression
allergy to neomycin
children who have received another live vaccine by injection within 4 weeks
pregnancy should be avoided for at least 1 month following vaccination
immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are
present)
Adverse effects
malaise, fever and rash may occur after the first dose of MMR. This typically occurs after 5-10 days and lasts around 2-3 days
Question 9 of 231
A 3-month-old boy is brought to surgery as his mother is concerned he is 'floppy'. Examination confirms a greater degree of hypotonia than
would be expected at this age. Which one of the following is least likely to be responsible?
Cystic fibrosis
Prader-Willi syndrome
Down's syndrome
Cerebral palsy
Next question
Hypotonia
Hypotonia, or floppiness, may be central in origin or related to nerve and muscle problems. An acutely ill child (e.g. septicaemic) may be
hypotonic on examination. Hypotonia associated with encephalopathy in the newborn period is most likely caused by hypoxic ischaemic
encephalopathy
Central causes
Down's syndrome
Prader-Willi syndrome
hypothyroidism
cerebral palsy (hypotonia may precede the development of spasticity)
Question 10 of 231
Typically affects the fingers, interdigital webs and flexor aspects of the wrist in adults
Next question
Scabies
Scabies is caused by the mite Sarcoptes scabiei and is spread by prolonged skin contact. It typically affects children and young adults.
The scabies mite burrows into the skin, laying its eggs in the stratum corneum. The intense pruritus associated with scabies is due to a
delayed type IV hypersensitivity reaction to mites/eggs which occurs about 30 days after the initial infection.
Features
widespread pruritus
linear burrows on the side of fingers, interdigital webs and flexor aspects of the wrist
in infants the face and scalp may also be affected
secondary features are seen due to scratching: excoriation, infection
Management
permethrin 5% is first-line
malathion 0.5% is second-line
give appropriate guidance on use (see below)
pruritus persists for up to 4-6 weeks post eradication
launder, iron or tumble dry clothing, bedding, towels, etc., on the first day of treatment to kill off mites.
The BNF advises to apply the insecticide to all areas, including the face and scalp, contrary to the manufacturer's recommendation.
Patients should be given the following instructions:
Question 11 of 231
Which one of the following is a risk factor for developmental dysplasia of the hip?
Vertex presentation
Oligohydramnios
Male sex
Polyhydramnios
Next question
DDH is slightly more common in the left hip. Around 20% of cases are bilateral.
Clinical examination is made using the Barlow and Ortolani tests:
Question 12 of 231
Which one of the following vaccines is not given routinely in the first 6 months of life?
MMR
Tetanus vaccine
Meningitis C vaccine
Next question
Immunisation schedule
The current UK immunisation schedule is as follows. Please note that this table includes the changes announced in 2010 which merged the
12 and 13 month visits into one.
Age
Recommended immunisations
At birth
2 months
DTaP/IPV/Hib + PCV
Oral rotavirus vaccine
3 months
DTaP/IPV/Hib + Men C
Oral rotavirus vaccine
4 months
DTaP/IPV/Hib + PCV
3-4 years
MMR + DTaP/IPV
At birth the BCG vaccine should be given if the baby is deemed at risk of tuberculosis (e.g. Tuberculosis in the family in the past 6 months).
Hepatitis B vaccine should be given at birth if the mother is HBsAg +ve.
Key
Question 13 of 231
A 10-month-old boy is brought to surgery. Around 4 days ago he developed a fever after being irritable the previous day. The fever settled
after around 3 days but following this he developed a rash, which prompted his mother to bring him to surgery. He is taking around 75% of
his normal feeds, is producing wet nappies and has had two episodes of loose stools. On examination he is alert, temperature is 37.0C,
chest is clear, ears/throat unremarkable. There are a number of blanching, rose pink macules present on his trunk. What is the most likely
diagnosis?
Rubella
Chickenpox
Roseola infantum
Pityriasis rosea
Measles
Next question
Roseola infantum
Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human
herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years.
Features
aseptic meningitis
hepatitis
Question 14 of 231
A 3-year-old child is brought to surgery as her mother has noticed that she is 'cross-eyed'. The corneal light reflection test confirms this.
What is the most appropriate management?
Advise that referral to secondary care should be delayed until 5 years of age, when surgery may be
contemplated
Refer to ophthalmology
Next question
Squint
Squint (strabismus) is characterised by misalignment of the visual axes. Squints may be divided into concomitant (common) and paralytic
(rare)
Concomitant
Paralytic
Detection of a squint may be made by the corneal light reflection test - holding a light source 30cm from the child's face to see if the light
reflects symmetrically on the pupils
The cover test is used to identify the nature of the squint
Management
Question 15 of 231
A 2-month-old girl is brought to surgery with poor feeding and vomiting. Mother reports that her urine has a strong smell. A urinary tract
infection is suspected. What is the most appropriate management?
Urine culture + empirical oral antibiotic therapy + ultrasound as soon as possible
Urine culture + oral antibiotics based upon results + ultrasound within 6 weeks
Next question
Management
Urinary tract infection (UTI) is illness caused by micro-organisms in the urinary tract.
Most UTIs are caused by a single species of bacteria from the gastrointestinal tract.
Common organisms causing UTI in children include Escherichia coli (about 75% or more
of cases), Klebsiella species, and Staphylococcus saprophyticus.
Around 1 in 10 girls and 1 in 30 boys will have had a UTI by the time they turn 16 years
of age.
Most UTIs are not associated with any risk factor.
The prognosis after childhood UTI is generally excellent and a course of antibiotics
cures UTI in most infants and children. However, UTI may recur and rarely, long-term
complications (such as renal scarring and hypertension) may occur.
In a child less than 3 months old, UTI should be suspected if there is a combination of:
o Fever (without obvious cause), vomiting, irritability, lethargy.
o Poor feeding, failure to thrive.
o Failure to respond adequately to appropriate treatment of another presumed cause
of the illness.
o Abdominal pain, jaundice, haematuria, offensive urine.
An urgent admission should be made in all infants less than 3 months of age with
suspected UTI. Urine testing is not necessary in primary care.
In a child over 3 months of age, UTI should be suspected if there is a combination of:
o Frequency, dysuria.
o Fever without an obvious cause (if the temperature is greater than 38C urine
should be tested within 24 hours).
o Fever with a presumed cause (other than UTI) but poor response to treatment (if
there is doubt about the cause of fever urine should be tested within 24 hours).
o Abdominal pain, loin tenderness.
o Voluntary withholding or incontinence of urine or faeces.
o Vomiting, poor feeding.
o Malaise, lethargy, irritability.
o Haematuria, offensive urine, cloudy urine.
o Failure to thrive.
Management of suspected UTI in a child over 3 months of age involves:
o Assessing the risk of serious illness and arranging a referral (or admission) with the
appropriate urgency. This should include taking a full history; measurement of blood
pressure; abdominal palpation; inspection of external genitalia, anus and urethral
meatus; assessment of lower back and limbs, and examination of urine.
o Considering the need for antibiotics. A urine specimen should be sent for culture
and sensitivities prior to starting antibiotics.
o Arranging a review (e.g. after 48 hours) to ensure response to treatment and to
reassess the antibiotic choice, if applicable.
o Offering appropriate advice to parents or carers (such as to return for assessment if
the child is still unwell after 2448 hours of treatment or if they suspect a repeat
UTI).
Infants/children aged over 3 months with suspected UTI are at:
o High risk of serious illness if they are systemically unwell, dehydrated, or vomiting
and cannot tolerate oral fluids and medication or have a history or clinical features
suggesting urinary tract obstruction.
o Low risk of serious illness if temperature is less than 38C with no history of fever
and there is no loin pain/tenderness.
o Intermediate risk of serious illness if they do not satisfy the criteria for being at high
or low risk.
Question 16 of 231
A 4-week-old child is brought to clinic with a red rash on her scalp associated with yellow flakes. What is the most likely diagnosis?
Irritant contact dermatitis
Seborrhoeic dermatitis
Psoriasis
Atopic eczema
Candidiasis
Next question
Question 17 of 231
Wasp venom
Medication
Food
Blood products
Next question
Anaphylaxis
Anaphylaxis may be defined as a severe, life-threatening, generalised or systemic
hypersensitivity reaction.
Anaphylaxis is one of the few times when you would not have time to look up the dose of a medication. The Resuscitation Council
guidelines on anaphylaxis have recently been updated. Adrenaline is by far the most important drug in anaphylaxis and should be given as
soon as possible. The recommended doses for adrenaline, hydrocortisone and chlorphenamine are as follows:
Adrenaline
Hydrocortisone Chlorphenamine
Adrenaline
Hydrocortisone Chlorphenamine
< 6 months
250 mcg/kg
6 months - 6 years
2.5 mg
6-12 years
5 mg
Adult and child > 12 years 500 mcg (0.5ml 1 in 1,000) 200 mg
10 mg
Adrenaline can be repeated every 5 minutes if necessary. The best site for IM injection is the anterolateral aspect of the middle third of the
thigh.
Common identified causes of anaphylaxis
Question 18 of 231
A 2-month-old girl is brought in by her mother. She was breastfed for the first two weeks of life before being switched to formula. For the
past six weeks she has experienced a number of problems including regurgitation, vomiting, diarrhoea and eczema. Despite these
problems she has kept to the 50th centile for weight. Clinical examination is unremarkable other than some dry skin on her torso. What is
the most likely diagnosis?
Coeliac disease
Lactose intolerance
Cystic fibrosis
Next question
The emergence of symptoms following the introduction of formula is very suggestive of cow's milk protein intolerance.
Diagnosis is often clinical (e.g. improvement with cow's milk protein elimination). Investigations include:
Management
If the symptoms are severe (e.g. failure to thrive) refer to a paediatrician.
Management if formula-fed
extensive hydrolysed formula (eHF) milk is the first-line replacement formula for infants with mild-moderate symptoms
amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF
around 10% of infants are also intolerant to soya milk
Management if breast-fed
continue breastfeeding
eliminate cow's milk protein from maternal diet
use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months
CMPI usually resolves by 1-2 years of age. A challenge is often performed in the hospital setting as anaphylaxis can occur.
Question 19 of 231
A 9-year-old boy who is having an asthma attack is brought to surgery. Which one of the following findings would be categorise the asthma
attack as life-threatening, rather than just severe, according to the British Thoracic Society guidelines?
Heart rate 140 bpm
Sats 93%
Next question
The 2014 BTS/SIGN guidelines suggest the following criteria are used to assess the severity of asthma in general practice:
Children between 2 and 5 years of age
Moderate attack
Severe attack
Life-threatening attack
SpO2 <92%
SpO2 < 92%
Silent chest
Too breathless to talk or feed
SpO2 > 92%
Poor respiratory effort
Heart rate > 140/min
No clinical features of severe asthma
Agitation
Respiratory rate > 40/min
Altered consciousness
Use of accessory neck muscles
Cyanosis
Severe attack
Life-threatening attack
Question 20 of 231
A 6-year-old boy is brought to surgery by his mother. For the past 2 months he has been complaining of pain in his shins and ankles at
night-time. His symptoms are bilateral he is otherwise well. There is no family history of note. Clinical examination is unremarkable. What is
the most likely diagnosis?
Legg-Calve-Perthes disease
Osteoid osteoma
Growing pains
Talipes equinovarus
Osteochondritis dissecans
Next question
Growing pains
A common presentation in General Practice is a child complaining of pain in the legs with no obvious cause. Such presentations, in the
absence of any worrying features, are often attributed to 'growing pains'. This is a misnomer as the pains are often not related to growth the current term used in rheumatology is 'benign idiopathic nocturnal limb pains of childhood'
Growing pains are equally common in boys and girls and occur in the age range of 3-12 years.
never present at the start of the day after the child has woken
no limp
no limitation of physical activity
systemically well
normal physical examination
motor milestones normal
symptoms are often intermittent and worse after a day of vigorous activity
Question 21 of 231
A 7-year-old child is brought to surgery by her father after he collected her from school. She was unwell in the morning with a fever and
cough but has become gradually worse during the day. His father is panicked because he has noticed a red rash on her legs which doesn't
blanch following the 'tumbler test'. The girl is now also complaining of a headache and cool hands and feet. On examination she has a
petechial rash on her lower legs and is pyrexial with a temperature of 38.4C. Her father reports she is allergic to penicillin. On reviewing
the records you note she developed a rash and vomiting after been given amoxicillin for otitis media 4 years ago. What is the most
appropriate next step?
Phone 999 + administer intramuscular benzylpenicillin
Reassure petechiae are likely secondary to cough but admit to paediatrics for further tests.=
Phone 999
Next question
This girl has meningococcal septicaemia until proven otherwise. The question looks at the urgency of your response and also how you
would deal with a history of 'allergy' to penicillin. NICE state the following:
Withhold benzylpenicillin only in children and young people who have a clear history of anaphylaxis after a
previous dose; a history of a rash following penicillin is not a contraindication.
A cough would not explain petechiae in the legs.
Meningococcal septicaemia
Meningococcal septicaemia is a frightening condition for patients, parents and doctors. It is associated with a high morbidity and mortality
unless treated early - meningococcal disease is the leading infectious cause of death in early childhood. A high index of suspicion is
therefore needed. Much of the following is based on the 2010 NICE guidelines (please see link).
Presentation of meningococcal disease:
15% - meningitis
25% - septicaemia
60% - a combination of meningitis and septicaemia
give intramuscular or intravenous benzylpenicillin unless there is a history of anaphylaxis (do not give if this will delay hospital
transfer)
NICE recommend phoning 999
Guidance
1.1 Bacterial meningitis and meningococcal septicaemia in children and young people
symptoms, signs and initial assessment
1.2 Pre-hospital management of suspected bacterial meningitis and meningococcal
septicaemia
1.3 Diagnosis in secondary care
1.4 Management in secondary care
1.5 Long-term management
1 Guidance
The following guidance is based on the best available evidence. The full
guideline gives details of the methods and the evidence used to develop the
guidance.
This guideline assumes that fever in children younger than 5 years will be
managed according to Feverish illness in children (NICE clinical guideline 47)
until bacterial meningitis or meningococcal septicaemia is suspected.
1.1 Bacterial meningitis and meningococcal septicaemia in children and young
people symptoms, signs and initial assessment
Be aware that:
o
some children and young people will present with mostly non-specific
symptoms or signs, and the conditions may be difficult to distinguish
from other less important (viral) infections presenting in this way
children and young people with the more specific symptoms and signs
are more likely to have bacterial meningitis or meningococcal
septicaemia, and the symptoms and signs may become more severe
and more specific over time.
Vomiting/nausea
Irritable/unsettle
d
Ill appearance
Refusing
food/drink
Headache
Muscle
ache/joint pain
Respiratory
symptoms/signs
or breathing
difficulty
Fever
Lethargy
Diarrhoea,
abdominal
pain/distension
NK
Sore
throat/coryza or
other ear, nose
NK
Not always
present,
especially in
neonates
and throat
symptoms/signs
More specific symptoms/signs
Non-blanching
rash
Stiff neck
NK
Altered mental
state
Capillary refill
time more than
2 seconds
NK
Unusual skin
colour
NK
Shock
Hypotension
NK
Leg pain
NK
Cold hands/feet NK
Back rigidity
NK
Bulging
fontanelle
NK
Photophobia
Kernig's sign
Brudzinski's sign
Be aware that
a rash may
be less visible
in darker skin
tones check
soles of feet,
palms of
hands and
conjunctivae
Includes
confusion,
delirium and
drowsiness,
and impaired
consciousnes
s
Only relevant
in children
aged under
2 years
Unconsciousnes
s
Toxic/moribund
state
Focal
neurological
deficit including
cranial nerve
involvement and
abnormal pupils
Paresis
Seizures
Signs of shock
Leg pain
Cold hands/feet
Toxic/moribund state
symptom/sign present
X symptom/sign not present
NK not known if a symptom/sign is present (not reported in the evidence)
1.1.2 Be alert to the possibility of bacterial meningitis or meningococcal
septicaemia when assessing children or young people with acute febrile illness.
1.1.3 Healthcare professionals should be aware that classical signs of meningitis
(neck stiffness, bulging fontanelle, high-pitched cry) are often absent in infants
with bacterial meningitis[3].
1.1.4 Be aware that children and young people with bacterial meningitis
commonly present with non-specific symptoms and signs, including fever,
vomiting, irritability, and upper respiratory tract symptoms. Some children with
bacterial meningitis present with seizures[4].
1.1.5 Consider other non-specific features of the child's or young person's
presentation, such as:
1.2.1 Primary care healthcare professionals should transfer children and young
people with suspected bacterial meningitis or suspected meningococcal
septicaemia to secondary care as an emergency by telephoning 999.
Suspected bacterial meningitis without non-blanching rash
1.2.2 Transfer children and young people with suspected bacterial meningitis
without non-blanching rash directly to secondary care without giving parenteral
antibiotics.
1.2.3 If urgent transfer to hospital is not possible (for example, in remote
locations or adverse weather conditions), administer antibiotics to children and
young people with suspected bacterial meningitis.
Suspected meningococcal disease (meningitis with non-blanching rash or
meningococcal septicaemia)
1.2.4 Give parenteral antibiotics (intramuscular or intravenous benzylpenicillin) at
the earliest opportunity, either in primary or secondary care, but do not delay
urgent transfer to hospital to give the parenteral antibiotics.
1.2.5 Withhold benzylpenicillin only in children and young people who have a
clear history of anaphylaxis after a previous dose; a history of a rash following
penicillin is not a contraindication.
1.3 Diagnosis in secondary care
1.3.3 If a child or young person has an unexplained petechial rash and fever (or
history of fever) carry out the following investigations:
coagulation screen
blood culture
blood glucose
blood gas.
1.3.4 In a child or young person with an unexplained petechial rash and fever (or
history of fever) but none of the high-risk clinical manifestations (see table 1
above):
Treat with intravenous ceftriaxone immediately if the CRP and/or white blood
cell count (especially neutrophil count) is raised, as this indicates an
increased risk of having meningococcal disease.
Be aware that while a normal CRP and normal white blood cell count mean
meningococcal disease is less likely, they do not rule it out. The CRP may
be normal and the white blood cell count normal or low even in severe
meningococcal disease.
1.3.6 Be aware that in children and young people who present with a nonspreading petechial rash without fever (or history of fever) who do not appear ill
to a healthcare professional, meningococcal disease is unlikely, especially if the
rash has been present for more than 24 hours. In such cases consider:
If the CRP and/or white blood cell count is raised and there is a
non-specifically abnormal cerebrospinal fluid (CSF) (for example consistent
with viral meningitis), treat as bacterial meningitis.
Be aware that a normal CRP and white blood cell count does not rule out
bacterial meningitis.
Regardless of the CRP and white blood cell count, if no CSF is available for
examination or if the CSF findings are uninterpretable, manage as if the
diagnosis of meningitis is confirmed.
papilloedema
coagulation abnormalities
o
1.3.20 CSF white blood cell counts, total protein and glucose concentrations
should be made available within 4 hours to support the decision regarding
adjunctive steroid therapy.
1.3.21 Start antibiotic treatment for bacterial meningitis if the CSF white blood
cell count is abnormal:
in older children and young people more than 5 cells/microlitre or more than
1 neutrophil/microlitre, regardless of other CSF variables.
1.3.22 In children and young people with suspected bacterial meningitis, consider
alternative diagnoses if the child or young person is significantly ill and has CSF
variables within the accepted normal ranges.
1.3.23 Consider herpes simplex encephalitis as an alternative diagnosis.
1.3.24 If CSF white cell count is increased and there is a history suggesting a risk
of tuberculous meningitis, evaluate for the diagnosis of tuberculous meningitis in
line with Tuberculosis: clinical diagnosis and management of tuberculosis, and
measures for its prevention and control (NICE clinical guideline 33).
1.3.25 Perform a repeat lumbar puncture in neonates with:
hypoglycaemia
acidosis
hypokalaemia
hypocalcaemia
hypomagnesaemia
anaemia
coagulopathy.
Seizures
1.4.19 Use local or national protocols for management of seizures in children and
young people with suspected bacterial meningitis or meningococcal septicaemia.
Raised intracranial pressure
1.4.20 Use local or national protocols to treat raised intracranial pressure.
Fluid management in suspected or confirmed bacterial meningitis
1.4.21 Assess for all of the following:
signs of dehydration.
Refer to Diarrhoea and vomiting in children (NICE clinical guideline 84) for
assessment of shock and dehydration.
1.4.22 If present, correct dehydration using enteral fluids or feeds, or intravenous
isotonic fluids (for example, sodium chloride 0.9% with glucose 5% or sodium
chloride 0.9% with dextrose 5%).
1.4.23 Do not restrict fluids unless there is evidence of:
If there are signs of shock, give an immediate fluid bolus of 20 ml/kg sodium
chloride 0.9% over 510 minutes. Give the fluid intravenously or via an
be aware that some children and young people may require large
volumes of fluid over a short period of time to restore their circulating
volume
1.4.37 Undertake tracheal intubation and mechanical ventilation for the following
indications:
threatened (for example, loss of gag reflex), or actual loss of airway patency
hypoventilation or apnoea
moribund state
raised CSF white blood cell count with protein concentration greater than
1 g/litre
1.5.2 Offer children and young people and their parents or carers:
hearing loss (with the child or young person having undergone an urgent
assessment for cochlear implants as soon as they are fit)
psychosocial problems
renal failure.
1.5.6 Inform the child's or young person's GP, health visitor and school nurse (for
school-age children and young people) about their bacterial meningitis or
meningococcal septicaemia.
1.5.7 Healthcare professionals with responsibility for monitoring the child's or
young person's health should be alert to possible late-onset sensory,
A 4-year-old girl is reviewed by her GP due to poor control of asthma. She currently takes a regular steroid inhaler and a salbutamol inhaler
as required. What is the next step in management?
Trial of a leukotriene receptor antagonist
Referral to a paediatrician
Oral theophylline
Next question
Children aged 2-5 years: trial of a leukotriene receptor antagonist. If already taking leukotriene receptor antagonist reconsider inhaled
corticosteroids
Children aged under 2 years: refer to respiratory paediatrician
Question 23 of 231
Which one of the following is not a risk factor for sudden infant death syndrome?
Female sex
Hyperthermia
Prematurity
Bottle feeding
Next question
Male rather than female sex is a risk factor for sudden infant death syndrome
prematurity
parental smoking
hyperthermia (e.g. over-wrapping)
putting the baby to sleep prone
male sex
multiple births
bottle feeding
social classes IV and V
maternal drug use
incidence increases in winter
Following a cot death siblings should be screened for potential sepsis and inborn errors of metabolism
Question 24 of 231
A 4-year-old boy is reviewed in the Paediatric Admissions Unit. He has had a fever for the past week. On examination he has red, sore lips
and conjunctival injection. He also has swollen, red hands. Blood tests show:
Hb
13.1 g/dl
WBC
12.7 *109/l
Platelets
520 *109/l
CRP
96 mg/L
Dermatomyositis
Lyme disease
Still's disease
Kawasaki disease
Next question
Kawasaki disease
Kawasaki disease is a type of vasculitis which is predominately seen in children. Whilst Kawasaki disease is uncommon it is important to
recognise as it may cause potentially serious complications, including coronary artery aneurysms
Features
high-grade fever which lasts for > 5 days. Fever is characteristically resistant to antipyretics
conjunctival injection
bright red, cracked lips
strawberry tongue
cervical lymphadenopathy
red palms of the hands and the soles of the feet which later peel
high-dose aspirin*
intravenous immunoglobulin
echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms
Complications
*Kawasaki disease is one of the few indications for the use of aspirin in children. Due to the risk of Reye's syndrome aspirin is normally
contraindicated in children.
Question 25 of 231
A 14-year-old girl requests to start the combined oral contraceptive pill. Which one of the following is not an integral part of the Fraser
guidelines?
Unless they receive contraceptive treatment their health is likely to suffer
They cannot be persuaded to inform their parents or allow the professional to contact them on their behalf
They are likely to begin, or continue having, sexual intercourse with or without contraceptive treatment
They do not have, or have had in the past, a mental illness or learning disability
Next question
Fraser guidelines
The Fraser guidelines are used to assess if patient who has not yet reached 16 years of age is competent to consent to treatment, for
example with respect to contraception
The following points should be fulfilled:
Question 26 of 231
A 6-year-old boy is reviewed in clinic due to nocturnal enuresis. His mother has tried using a star-chart but unfortunately this has not
resulted in any significant improvement. Of the following options, what is the most appropriate initial management strategy?
Enuresis alarm
Trial of imipramine
Next question
Restricting fluids is not recommended advice - Clinical Knowledge Summaries suggest: 'Do not restrict fluids. The child should have about
eight drinks a day, spaced out throughout the day, the last one about 1 hour before bed.'
Nocturnal enuresis
The majority of children achieve day and night time continence by 3 or 4 years of age. Enuresis may be defined as the 'involuntary
discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous
system or urinary tract'
Nocturnal enuresis can be defined as either primary (the child has never achieved continence) or secondary (the child has been dry for at
least 6 months before)
NICE issued guidance in 2010. Management:
look for possible underlying causes/triggers (e.g. Constipation, diabetes mellitus, UTI if recent onset)
advise on fluid intake, diet and toileting behaviour
reward systems (e.g. Star charts). NICE recommend these 'should be given for agreed behaviour rather than dry nights' e.g.
Using the toilet to pass urine before sleep
NICE advise: 'Consider whether alarm or drug treatment is appropriate, depending on the age, maturity and abilities of the child
or young person, the frequency of bedwetting and the motivation and needs of the family'. Generally:
an enuresis alarm is first-line for children under the age of 7 years
desmopressin may be used first-line for children over the ago 7 years, particularly if short-term control is needed or an enuresis
alarm has been ineffective/is not acceptable to the family
please see the link for more details
Question 27 of 231
A 2-month-old baby girl is admitted to hospital with suspected meningitis. Her parents describe her becoming pyrexial and drowsy over the
past 24 hours. On examination her temperature is 39.2C, heart rate is 160/min and respiratory rate is 50.min. Her anterior fontanelle is
bulging. No petechial rash is seen. In addition to cefotaxime, what antibiotic should be given intravenously?
Rifampicin
Flucloxacillin
Amoxicillin
Clarithromycin
Gentamicin
Next question
Meningitis in children < 3 months: give IV amoxicillin in addition to cefotaxime to cover for Listeria
For patients with meningococcal septicaemia lumbar puncture is contraindicated - blood cultures and PCR for meningococcus should be
obtained
Management
1. Antibiotics
2. Steroids
3. Fluids
4. Cerebral monitoring
rifampicin
Question 28 of 231
A 4-month-old girl is brought to surgery for her routine immunisations. She has had all the previous immunisations as per the routine
schedule and there is no past medical history. What should she be given at this visit?
Key
DTaP/IPV/Hib + Men C
DTaP/IPV/Hib + PCV
Next question
The Men C booster has recently been dropped from the 4 month visit - please see the updated UK immunisation schedule.
Immunisation schedule
The current UK immunisation schedule is as follows. Please note that this table includes the changes announced in 2010 which merged the
12 and 13 month visits into one.
Age
Recommended immunisations
At birth
2 months
DTaP/IPV/Hib + PCV
Oral rotavirus vaccine
3 months
DTaP/IPV/Hib + Men C
Oral rotavirus vaccine
4 months
DTaP/IPV/Hib + PCV
3-4 years
MMR + DTaP/IPV
At birth the BCG vaccine should be given if the baby is deemed at risk of tuberculosis (e.g. Tuberculosis in the family in the past 6 months).
Hepatitis B vaccine should be given at birth if the mother is HBsAg +ve.
Key
Question 29 of 231
Next question
Cerebral palsy
Cerebral palsy may be defined as a disorder of movement and posture due to a non-progressive lesion of the motor pathways in the
developing brain. It affects 2 in 1,000 live births and is the most common cause of major motor impairment
Possible manifestations include:
Children with cerebral palsy often have associated non-motor problems such as:
Causes
antenatal (80%): e.g. cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
intrapartum (10%): birth asphyxia/trauma
postnatal (10%): intraventricular haemorrhage, meningitis, head-trauma
Classification
Management
Question 30 of 231
A mother brings her 8-month-old infant to the GP due to the development of a nappy rash. On examination an erythematous rash with
flexural sparing is seen. What is the most likely cause?
Irritant dermatitis
Psoriasis
Seborrhoeic dermatitis
Candida infection
Atopic eczema
Next question
Napkin rashes
Causes of a napkin ('nappy') rash include the following:
Irritant dermatitis
The most common cause, due to irritant effect of urinary ammonia and faeces
Creases are characteristically spared
Candida dermatitis
Typically an erythematous rash which involve the flexures and has characteristic satellite lesions
Seborrhoeic dermatitis Erythematous rash with flakes. May be coexistent scalp rash
Irritant dermatitis
The most common cause, due to irritant effect of urinary ammonia and faeces
Creases are characteristically spared
Psoriasis
A less common cause characterised by an erythematous scaly rash also present elsewhere on the skin
Atopic eczema
Question 31 of 231
A 1-year-old girl is investigated for recurrent urinary tract infections. A micturating cystourethrogram is ordered:
Paediatric urolithiasis
Bilateral hydronephrosis
Next question
This image demonstrates grade V vesicoureteric reflux - gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity. A DMSA
scan is needed to identify renal scarring.
Vesicoureteric reflux
Vesicoureteric reflux (VUR) is the abnormal backflow of urine from the bladder into the ureter and kidney. It is relatively common
abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI), being found in around 30% of children who
present with a UTI. As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI
Pathophysiology of VUR
ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle
therefore shortened intramural course of ureter
vesicoureteric junction cannot therefore function adequately
II
III
IV
Dilation of the renal pelvis and calyces with moderate ureteral tortuosity
Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity
Investigation
Question 32 of 231
A 4-year-old boy is brought to the surgery as his mother is concerned that he has had an intermittent fever for 48 hours. Which one of the
following is least relevant when examining the child?
Blood pressure
Respiratory rate
Temperature
Heart rate
Next question
temperature
heart rate
respiratory rate
capillary refill time
Signs of dehydration (reduced skin turgor, cool extremities etc) should also be looked for
Measuring temperature should be done with an electronic thermometer in the axilla if the child is < 4 weeks or with an electronic/chemical
dot thermometer in the axilla or an infra-red tympanic thermometer.
Risk stratification
Please see the link for the complete table, below is a modified version
Green - low risk
Colour
Normal colour
Pale/mottled/ashen/blue
Activity
Responds normally to
social cues
Content/smiles
Stays awake or awakens
quickly
Strong normal cry/not
crying
Nasal flaring
Tachypnoea: respiratory rate
Respiratory
Grunting
Tachypnoea: respiratory rate >60
breaths/minute
Moderate or severe chest indrawing
Circulation and
hydration
Other
Management
If green:
Child can be managed at home with appropriate care advice, including when to seek further help
If amber:
provide parents with a safety net or refer to a paediatric specialist for further assessment
a safety net includes verbal or written information on warning symptoms and how further healthcare can be accessed, a followup appointment, liaison with other healthcare professionals, e.g. out-of-hours providers, for further follow-up
If red:
oral antibiotics should not be prescribed to children with fever without apparent source
if a pneumonia is suspected but the child is not going to be referred to hospital then a chest x-ray does not need to be routinely
performed
Question 33 of 231
A 3-year-old child is diagnosed with whooping cough. What is the most appropriate antibiotic therapy?
Oral amoxicillin
Oral levofloxacin
Oral erythromycin
Oral penicillin
Next question
coughing bouts: usually worse at night and after feeding, may be ended by vomiting & associated central cyanosis
inspiratory whoop: not always present (caused by forced inspiration against a closed glottis)
persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures
symptoms may last 10-14 weeks* and tend to be more severe in infants
marked lymphocytosis
Diagnosis
per nasal swab culture for Bordetella pertussis - may take several days or weeks to come back
PCR and serology are now increasingly used as their availability becomes more widespread
Management
Complications
subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures
Question 34 of 231
You are reviewing a 6-month-old child with suspected bronchiolitis. Which one of the following should prompt a referral to hospital?
Oxygen saturations of 96%
Lethargy
Crackles on auscultation
Next question
Bronchiolitis
Bronchiolitis is a condition characterised by acute bronchiolar inflammation. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of
cases. SIGN released guidelines on bronchiolitis in 2006. Please see the link for more details.
Epidemiology
most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 36 months). Maternal IgG provides protection to newborns against RSV
higher incidence in winter
Basics
Features
Investigation
A 6-year-old boy is noted to have pectus excavatum and pulmonary stenosis during a cardiorespiratory exam. What is the most
likely diagnosis?
Noonan syndrome
Pierre-Robin syndrome
Edward's syndrome
William's syndrome
Patau syndrome
Childhood syndromes
Next question
Below is a list of common features of selected childhood syndromes
Syndrome
Key features
Micrognathia
Low-set ears
Edward's syndrome (trisomy 18)
Rocker bottom feet
Overlapping of fingers
Fragile X
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
Noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
Pierre-Robin syndrome*
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
Prader-Willi syndrome
Hypotonia
Hypogonadism
Obesity
William's syndrome
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
*this condition has many similarities with Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome
is autosomal dominant so there is usually a family history of similar problems
Question 36 of 231
When is the neonatal blood spot screening test typically performed in the United Kingdom
At birth
Next question
congenital hypothyroidism
cystic fibrosis
phenylketonuria
sickle cell disease
medium chain acyl-CoA dehydrogenase deficiency (MCADD)
Question 37 of 231
A mother brings her 2-week-old baby girl into the surgery for review. She has noted a bright red, well-circumscribed and lobulated lesion
developing on her right temple. This wasn't noted at birth but is now 5 mm in diameter. What is the most appropriate management?
Silver nitrate cautery
Next question
This baby has a strawberry naevus. Treatment is only usually required if the lesion is causing a mechanical problem or bleeding.
Strawberry naevus
Strawberry naevi (capillary haemangioma) are usually not present at birth but may develop rapidly in the first month of life. They appear as
erythematous, raised and multilobed tumours.
Typically they increase in size until around 6-9 months before regressing over the next few years (around 95% resolve before 10 years of
age).
Common sites include the face, scalp and back. Rarely they may be present in the upper respiratory tract leading to potential airway
obstruction
Capillary haemangiomas are present in around 10% of white infants. Female infants, premature infants and those of mothers who have
undergone chorionic villous sampling are more likely to be affected
Potential complications
thrombocytopaenia
If treatment is required (e.g. Visual field obstruction) then systemic steroids are used
Cavernous haemangioma is a deep capillary haemangioma
Question 38 of 231
You are called to the treatment room of a GP surgery as a 12-month-old boy has developed a rash and breathing difficulties following a
routine vaccination. On examination he is developing swelling around the mouth and neck. What is the most appropriate initial action?
Phone 999 and reassure mother
Next question
Anaphylaxis
Anaphylaxis may be defined as a severe, life-threatening, generalised or systemic
hypersensitivity reaction.
Anaphylaxis is one of the few times when you would not have time to look up the dose of a medication. The Resuscitation Council
guidelines on anaphylaxis have recently been updated. Adrenaline is by far the most important drug in anaphylaxis and should be given as
soon as possible. The recommended doses for adrenaline, hydrocortisone and chlorphenamine are as follows:
Adrenaline
Hydrocortisone Chlorphenamine
< 6 months
250 mcg/kg
6 months - 6 years
2.5 mg
6-12 years
5 mg
Adult and child > 12 years 500 mcg (0.5ml 1 in 1,000) 200 mg
10 mg
Adrenaline can be repeated every 5 minutes if necessary. The best site for IM injection is the anterolateral aspect of the middle third of the
thigh.
Common identified causes of anaphylaxis
Question 39 of 231
Cycling helmets
Smoke alarms
Next question
Accidents in children
Around 15-20% of children attend Emergency Departments in the course of a year due to an accident. Accidents account for a third of all
childhood deaths and are the single most common cause of death in children aged between 1 - 15 years of age.
Key points
road traffic accidents are the most common cause of fatal accidents
boys and children from lower social classes are more likely to have an accident
Accident prevention
Accident prevention can be divided up into primary (preventing the accident from happening), secondary (prevent injury from the accident)
and tertiary (limit the impact of the injury) prevention strategies
The table below gives examples of accident prevention strategies
Primary prevention
Stair guards
Wearing seat belts
Speed limits*
Cycling helmets
Teaching parents first aid
Teaching road safety Smoke alarms
Window safety catchesLaminated safety glass
*some strategies such as reducing driving speed may have a role in both primary and secondary accident prevention
Question 40 of 231
A 2-year-old boy is brought to the surgery by his mother with earache and pyrexia. On examination of the precordium a murmur is heard.
Which one of the following characteristics is not consistent with an innocent murmur?
Diastolic murmur
Next question
Innocent murmurs
Innocent murmurs heard in children include
Ejection
murmurs
Venous hums
Due to the turbulent blood flow in the great veins returning to the heart. Heard as a continuous blowing noise heard just
below the clavicles
Still's murmur
soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area
may vary with posture
localised with no radiation
no diastolic component
no thrill
no added sounds (e.g. clicks)
asymptomatic child
no other abnormality
Question 41 of 231
Next question
Perthes disease
Perthes disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to
avascular necrosis of the femoral head
Perthes disease is 5 times more common in boys. Around 10% of cases are bilateral
Features
Complications
osteoarthritis
premature fusion of the growth plates
Perthes disease - both femoral epiphyses show extensive destruction, the acetabula are deformed
Question 42 of 231
Which one of the following statements regarding child health surveillance in the UK is incorrect?
The health visitor distraction test is the first screening test done on infants hearing
The midwife should visit the mother for at least the first 2 weeks following birth
Cystic fibrosis is one of the conditions the heel-prick test is used to screen for
Next question
The Newborn Hearing Screening Programme is gradually replacing distraction testing as the major screening test of infant hearing. In
everyday practice it is uncommon for midwives to visit up to 4 weeks
Antenatal
Newborn
First month
Heel-prick test day 5-9 - hypothyroidism, PKU, metabolic diseases, cystic fibrosis, medium-chain acyl Co-A
dehydrogenase deficiency (MCADD)
Midwife visit up to 4 weeks*
Following
months
Pre school
Ongoing
*this doesn't seem to happen in practice with health visitors usually taking over at 2 weeks
Question 43 of 231
Which one of the following types of glomerulonephritis is most characteristically associated with streptococcal infection in children?
Focal segmental glomerulosclerosis
Membranous glomerulonephritis
Mesangiocapillary glomerulonephritis
Next question
Glomerulonephritides
Knowing a few key facts is the best way to approach the difficult subject of glomerulonephritis:
Membranous glomerulonephritis
Question 44 of 231
Which one of the following statements regarding hand, foot and mouth disease is incorrect?
May be caused by enterovirus 71
Next question
In hand, foot and mouth disease oral lesion usually occur before palm and sole lesions
Management
*The HPA recommends that children who are unwell should be kept off school until they feel better. They also advise that you contact them
if you suspect that there may be a large outbreak.
Question 45 of 231
Lithium
Penicillin
Warfarin
Carbamazepine
Next question
galactosaemia
viral infections - this is controversial with respect to HIV in the developing world. This is because there is such an increased
infant mortality and morbidity associated with bottle feeding that some doctors think the benefits outweigh the risk of HIV
transmission
Drug contraindications
The following drugs can be given to mothers who are breast feeding:
*the BNF advises that the amount is too small to affect neonatal hypothyroidism screening
**clozapine should be avoided
Question 46 of 231
A 5-year-old boy is diagnosed as having absence seizures. What is the chance he will be seizure free by the age of 16-years-old?
5-10%
20-25%
40-45%
65-70%
90-95%
Next question
Absence seizures
Absence seizures (petit mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old
and girls are affected twice as commonly as boys
Features
absences last a few seconds and are associated with a quick recovery
seizures may be provoked by hyperventilation or stress
the child is usually unaware of the seizure
they may occur many times a day
EEG: bilateral, symmetrical 3Hz spike and wave pattern
Management
Question 47 of 231
Which one of the following statements regarding cleft lip and palate is incorrect?
Cleft lip is repaired earlier than cleft palate
Cleft palate results from failure of the palatine processes and the nasal septum to fuse
Polygenic inheritance
Next question
polygenic inheritance
maternal antiepileptic use increases risk
cleft lip results from failure of the fronto-nasal and maxillary processes to fuse
cleft palate results from failure of the palatine processes and the nasal septum to fuse
Problems
Management
cleft lip is repaired earlier than cleft palate, with practices varying from repair in the first week of life to three months
cleft palates are typically repaired between 6-12 months of age
Question 48 of 231
Mumps
Hepatitis B
Meningococcus
Diphtheria
Next question
BCG
MMR
oral polio
yellow fever
oral typhoid
Vaccinations
It is important to be aware of vaccines which are of the live-attenuated type as these may pose a risk to immunocompromised patients. The
main types of vaccine are as follows:
Live attenuated
BCG
measles, mumps, rubella (MMR)
influenza (intranasal)
oral rotavirus
oral polio
yellow fever
oral typhoid*
Inactivated preparations
rabies
influenza (intramuscular)
Detoxified exotoxins
tetanus
diphtheria
pertussis ('acellular' vaccine)
hepatitis B
meningococcus, pneumococcus, haemophilus
Notes
influenza: different types are available, including whole inactivated virus, split virion (virus particles disrupted by detergent
treatment) and sub-unit (mainly haemagglutinin and neuraminidase)
cholera: contains inactivated Inaba and Ogawa strains of Vibrio cholerae together with recombinant B-subunit of the cholera
toxin
hepatitis B: contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant
DNA technology
Question 49 of 231
A mother brings her 3-year-old child in to receive the DTP booster. Which one of the following would make it inappropriate to give the
vaccination today?
Being below the 2nd centile for weight
Next question
Immunisation
The Department of Health published guidance in 2006 on the safe administration of vaccines in its publication 'Immunisation against
infectious disease'
General contraindications to immunisation
confirmed anaphylactic reaction to a previous dose of a vaccine containing the same antigens
confirmed anaphylactic reaction to another component contained in the relevant vaccine (e.g. egg protein)
pregnancy
immunosuppression
Specific vaccines
DTP: vaccination should be deferred in children with an evolving or unstable neurological condition
asthma or eczema
history of seizures (if associated with fever then advice should be given regarding antipyretics)
breastfed child
previous history of natural pertussis, measles, mumps or rubella infection
history of neonatal jaundice
family history of autism
neurological conditions such as Down's or cerebral palsy
low birth weight or prematurity
patients on replacement steroids e.g. (CAH)
Question 50 of 231
A 18-month-old boy is brought to surgery as his mother is concerned about his hearing. Which one of the following conditions is least
associated with hearing problems in children?
Cerebral palsy
Pendred syndrome
Tuberous sclerosis
Next question
Sensorineural
Question 51 of 231
Which one of the following is not a notifiable disease in the United Kingdom?
Tuberculosis
HIV
Measles
Whooping cough
Meningococcal septicaemia
Next question
Notifiable diseases
Below is a list of notifiable diseases in the UK. The 'proper officer' of the Local Authority (usually a consultant in communicable disease)
needs to be notified. They in turn will notify the Health Protection Agency on a weekly basis.
Notable exceptions include:
HIV
In April 2010 the following diseases were removed from the list:
Dysentery
Ophthalmia neonatorum
Leptospirosis
Relapsing fever
Acute encephalitis
Acute infectious hepatitis
Acute meningitis
Acute poliomyelitis
Anthrax
Botulism
Brucellosis
Cholera
Diphtheria
Enteric fever (typhoid or paratyphoid fever)
Food poisoning
Haemolytic uraemic syndrome (HUS)
Infectious bloody diarrhoea
Invasive group A streptococcal disease
Legionnaires Disease
Leprosy
Malaria
Measles
Meningococcal septicaemia
Mumps
Plague
Rabies
Rubella
SARS
Scarlet fever
Smallpox
Tetanus
Tuberculosis
Typhus
A 3-year-old girl is taken to the GP due to a rash on her upper arm. On examination multiple raised lesions of about 2 mm in
diameter are seen. On close inspection a central dimple is present in the majority of lesions. What is the likely diagnosis?
Roseola infantum
Molluscum contagiosum
Kawasaki disease
Viral warts
Pityriasis rosea
Next question
Molluscum contagiosum
Molluscum contagiosum is caused by a pox DNA virus infection. It is typically seen in younger children and results in characteristic
small, pearly, umbilicated lesions
Molluscum contagiosum is highly infectious.
Lesions may be present for up to 12 months and usually resolve spontaneously. Whilst various treatments may be effective in
removing the lesions (e.g. surgery, cryotherapy, topical agents) no treatment is recommend in the initial phase due to the benign
nature of the condition
Image used on license from DermNet NZ and with the kind permission of Prof Raimo Suhonen
Question 2 of 180
At what age do the majority of children achieve day and night time urinary continence?
2-3 years old
Next question
Nocturnal enuresis
The majority of children achieve day and night time continence by 3 or 4 years of age. Enuresis may be defined as the 'involuntary
discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous
system or urinary tract'
Nocturnal enuresis can be defined as either primary (the child has never achieved continence) or secondary (the child has been dry for at
least 6 months before)
NICE issued guidance in 2010. Management:
look for possible underlying causes/triggers (e.g. Constipation, diabetes mellitus, UTI if recent onset)
advise on fluid intake, diet and toileting behaviour
reward systems (e.g. Star charts). NICE recommend these 'should be given for agreed behaviour rather than dry nights' e.g.
Using the toilet to pass urine before sleep
NICE advise: 'Consider whether alarm or drug treatment is appropriate, depending on the age, maturity and abilities of the child
or young person, the frequency of bedwetting and the motivation and needs of the family'. Generally:
an enuresis alarm is first-line for children under the age of 7 years
desmopressin may be used first-line for children over the ago 7 years, particularly if short-term control is needed or an enuresis
alarm has been ineffective/is not acceptable to the family
please see the link for more details
In children > 5 years of age, if bedwetting is infrequent (less than twice a week),
reassurance that bedwetting may resolve without treatment and offering the option
of a wait-and-see approach. If long-term treatment is required, treatment with an
enuresis alarm (first-line treatment) should be offered in combination with positive
reward systems desmopressin may be considered if the alarm is unsuitable. If
rapid or short-term control of bedwetting is required, treatment with desmopressin
is an option.
Managing primary bedwetting (with daytime symptoms) involves:
o Referring the child to secondary care or an enuresis clinic for further investigations
and assessment.
o Considering referral for younger children (older than 2 years of age) who are
struggling to not wet themselves during the day as well as during the night, despite
awareness of toileting needs and showing appropriate toileting behaviour.
Managing secondary enuresis involves:
o Treatment of UTIs and constipation in primary care.
o Referral to a paediatrician or an enuresis clinic if children have other underlying
causes which have been identified but are generally not managed in primary care
(e.g. diabetes and learning difficulties).
o
Question 3 of 180
A 23-year-old female with Down's syndrome is reviewed in clinic. Which one of the following features is least associated with her condition?
Infertility
Hypothyroidism
Alzheimer's disease
Short stature
Next question
As this patient is female she is likely to be subfertile rather than infertile - please see the notes below
face: upslanting palpebral fissures, epicanthic folds, Brushfield spots in iris, protruding tongue, small ears, round/flat face
flat occiput
single palmar crease, pronounced 'sandal gap' between big and first toe
hypotonia
congenital heart defects (40-50%, see below)
duodenal atresia
Hirschsprung's disease
Cardiac complications
Later complications
subfertility: males are almost always infertile due to impaired spermatogenesis. Females are usually subfertile, and have an
increased incidence of problems with pregnancy and labour
learning difficulties
short stature
repeated respiratory infections (+hearing impairment from glue ear)
acute lymphoblastic leukaemia
hypothyroidism
Alzheimer's
atlantoaxial instability
Question 4 of 180
A 9-year-old girl is brought to surgery as her mother is concerned that she is too fat. This has now been a problem for over two years and
mum feels this is holding her back at school. What is the most appropriate method to ascertain how obese she is?
Body mass index
Mother's perception
Waist circumference
Next question
Obesity in children
Defining obesity is more difficult in children than adults as body mass index (BMI) varies with age. BMI percentile charts are therefore
needed to make an accurate assessment. Recent NICE guidelines suggest to use 'UK 1990 BMI charts to give age- and gender-specific
information'
NICE recommend
By far the most common cause of obesity in childhood is lifestyle factors. Other associations of obesity in children include:
Asian children: four times more likely to be obese than white children
female children
taller children: children with obesity are often above the 50th percentile in height
orthopaedic problems: slipped upper femoral epiphyses, Blount's disease (a development abnormality of the tibia resulting in
bowing of the legs), musculoskeletal pains
psychological consequences: poor self-esteem, bullying
sleep apnoea
benign intracranial hypertension
long-term consequences: increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease
Question 5 of 180
Next question
Acne vulgaris
Acne vulgaris is a common skin disorder which usually occurs in adolescence. It typically affects the face, neck and upper trunk and is
characterised by the obstruction of the pilosebaceous follicle with keratin plugs which results in comedones, inflammation and pustules.
Epidemiology
Pathophysiology is multifactorial
follicular epidermal hyperproliferation resulting in the formation of a keratin plug. This in turn causes obstruction of the
pilosebaceous follicle. Activity of sebaceous glands may be controlled by androgen, although levels are often normal in patients
with acne
colonisation by the anaerobic bacterium Propionibacterium acnes
inflammation
Assessment
Management
Scenario: Mild acne : covers the management of acne that consists mainly of noninflammatory comedones. It is usually limited in its extent.
Scenario: Moderate acne : covers the management of acne that consists of a mixture of
non-inflammatory comedones and inflammatory papules and pustules. It may extend to
the shoulders and back.
Scenario: Severe acne : covers the management of acne that is characterized by nodules
and cysts (nodulocystic acne), as well as a preponderance of inflammatory papules and
pustules. It may be extensive.
Scenario: Mild acne
Scenario: Moderate acne
Scenario: Severe acne
Prescribing information
Important aspects of prescribing information relevant to primary healthcare are covered
in this section specifically for the drugs recommended in this CKS topic. For further
information on contraindications, cautions, drug interactions, and adverse effects, see
the electronic Medicines Compendium (eMC) (http://medicines.org.uk/emc), or the
British National Formulary (BNF) (www.bnf.org).
Benzoyl peroxide
Topical retinoids
Topical antibiotics
Azelaic acid
Oral antibiotics
Combined oral contraceptives
Evidence
Supporting evidence
Search strategy
References
ABPI Medicines Compendium (2012) Summary of product characteristics for Dianette.
Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013a) Summary of product characteristics for Skinoren
cream. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013b) Summary of product characteristics for Finacea
15% gel. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013c) Summary of product characteristics for
Isotretinoin 5mg capsules. Electronic Medicines CompendiumDatapharm
Communications Ltd. www.medicines.org.uk [Free Full-text]
DTB (2006) Is minocycline overused in acne? Drug and Therapeutics Bulletin 44(8), 6064. [Abstract]
DTB (2009) Minocycline for acne - update. Drug and Therapeutics Bulletin 47(1), 7-8.
[Abstract]
Farmery, M.R., Jones, C.E., Eady, E.A. et al. (1994) In vitro activity of azelaic acid, benzoyl
peroxide and zinc acetate against antibiotic-resistant propionibacteria from acne
patients. Journal of Dermatological Treatment 5(2), 63-65.
FFPRHC (2007) First prescription of combined oral contraception. Faculty of Sexual &
Reproductive Healthcare. www.ffprhc.org.uk [Free Full-text]
Franks, S., Layton, A. and Glasier, A. (2008) Cyproterone acetate/ethinyl estradiol for
acne and hirsutism: time to revise prescribing policy. Human Reproduction 23(2), 231232. [Abstract] [Free Full-text]
FSRH (2009) UK medical eligibility criteria for contraceptive use. Faculty of Sexual and
Reproductive Healthcare. www.fsrh.org [Free Full-text]
FSRH (2011) Drug interactions with hormonal contraception. Faculty of Sexual and
Reproductive Healthcare. www.fsrh.org [Free Full-text]
Garner, S.E. (2003) Acne vulgaris. In: Williams, H., Bigby, M., Diepgen, T. et al. (Eds.)
Evidence-based dermatology. London: BMJ Publishing Group. Chapter 13. 87-114.
Garner, S.E., Eady, E.A., Popescu, C. et al. (2003) Minocycline for acne vulgaris: efficacy
and safety (Cochrane Review). The Cochrane Library. Issue 1. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Garner, S.E., Eady, A., Bennett, C. et al. (2012) Minocycline for acne vulgaris: efficacy
and safety (Cochrane review). The Cochrane Library. Issue 8. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Gold, L.S., Colon, L.E., Johnson, L.A. and Gottschalk, R.W. (2008) Is switching retinoids a
sound strategy for the treatment of acne vulgaris? Journal of Drugs in Dermatology 7(6
Suppl), s11-s17. [Abstract]
Gollnick, H., Cunliffe, W., Berson, D. et al. (2003) Management of acne: a report from a
global alliance to improve outcomes in acne. Journal of the American Academy of
Dermatology 49(Suppl 1), S1-S38.
Habbema, L., Koopmans, B., Menke, H.E. et al. (1989) A 4% erythromycin and zinc
combination (Zineryt) versus 2% erythromycin (Eryderm) in acne vulgaris: a randomized,
double-blind comparative study. British Journal of Dermatology 121(4), 497-502.
[Abstract]
Haider, A. and Shaw, J.C. (2004) Treatment of acne vulgaris. Journal of the American
Medical Association 292(6), 726-735. [Abstract] [Free Full-text]
Hamilton, F.L., Car, J., Lyons, C. et al. (2009) Laser and other light therapies for the
treatment of acne vulgaris: systematic review. British Journal of Dermatology 160(6),
1273-1285. [Abstract]
Healy, E. and Simpson, N. (1994) Acne vulgaris. British Medical Journal 308(6932), 831833. [Free Full-text]
Holland, K.T., Bojar, R.A., Cunliffe, W.J. et al. (1992) The effect of zinc and erythromycin
on the growth of erythromycin-resistant and erythromycin-sensitive isolates of
Propionibacterium acnes: an in-vitro study. British Journal of Dermatology 126(5), 505509. [Abstract]
James, W.D. (2005) Acne. New England Journal of Medicine 352(14), 1463-1472.
Jordan, R.E., Cummins, C.L., Burls, A.J.E. and Seukeran, D.C. (2000) Laser resurfacing for
facial acne scars (Cochrane Review). The Cochrane Library. Issue 3. John Wiley & Sons,
Ltd. www.thecochranelibrary.com [Free Full-text]
Ko, H.C., Song, M., Seo, S.H. et al. (2009) Prospective, open-label, comparative study of
clindamycin 1%/benzoyl peroxide 5% gel with adapalene 0.1% gel in Asian acne
patients: Efficacy and tolerability. Journal of The European Academy of Dermatology and
Venereology 23(3), 245-250. [Abstract]
Koltun, W., Lucky, A.W., Thiboutot, D. et al. (2008) Efficacy and safety of 3 mg
drospirenone/20 mcg ethinylestradiol oral contraceptive administered in 24/4 regimen
in the treatment of acne vulgaris: a randomized, double-blind, placebo-controlled trial.
Contraception 77(4), 249-256. [Abstract]
Korkut, C. and Piskin, S. (2005) Benzoyl peroxide, adapalene, and their combination in
the treatment of acne vulgaris. Journal of Dermatology 32(3), 169-173. [Abstract]
Kus, S., Yucelten, D. and Aytug, A. (2005) Comparison of efficacy of azithromycin vs.
doxycycline in the treatment of acne vulgaris. Clinical & Experimental Dermatology
30(3), 215-220. [Abstract]
Langner, A., Sheehan-Dare, R. and Layton, A. (2007) A randomized, single-blind
comparison of topical clindamycin + benzoyl peroxide (Duac) and erythromycin + zinc
acetate (Zineryt) in the treatment of mild to moderate facial acne vulgaris. Journal of
the European Academy of Dermatology and Venereology 21(3), 311-319. [Abstract]
Layton, A.M. (2000) Acne vulgaris and similar eruptions. Medicine 28(12), 46-50.
Layton, A.M. (2010) Disorders of the sebaceous glands. In: Burns, T., Breathnach, S., Cox,
N. and Griffiths, C. (Eds.) Rook's textbook of dermatology. 8th edn. Chichester: WileyBlackwell. 42.1-42.89.
Leyden, J.J. (1997) Therapy for acne vulgaris. New England Journal of Medicine 336(16),
1156-1162.
Magin, P., Pond, D., Smith, W. and Watson, A. (2005a) A systematic review of the
evidence for 'myths and misconceptions' in acne management: diet, face-washing and
sunlight. Family Practice 22(1), 1-9. [Abstract] [Free Full-text]
Magin, P., Pond, D. and Smith, W. (2005b) Isotretinoin, depression and suicide: a review
of the evidence. British Journal of General Practice 55(511), 134-138. [Abstract] [Free
Full-text]
Magin, P.J., Adams, J., Pond, C.D. and Smith, W. (2006) Topical and oral CAM in acne: a
review of the empirical evidence and a consideration of its context. Complementary
Therapies in Medicine 14(1), 62-76. [Abstract]
Maloney, J.M., Dietze, P., Watson, D. et al. (2008) Treatment of acne using a 3-milligram
drospirenone/20-microgram ethinyl estradiol oral contraceptive administered in a 24/4
Question 6 of 180
A mother brings her 9-month-old child to surgery. She is concerned he is not developing normally and is 'falling behind' the children of her
friends. Whilst observing the child you notice he sits without support but makes little effort to move around the room. He has a crude pincer
grip, appears shy when you try to interact and says 'mama' but little else. What is the most accurate description of his development?
Normal development
Next question
Development problems
Referral points
hand preference before 12 months is abnormal and may indicate cerebral palsy
most common causes of problems: variant of normal, cerebral palsy and neuromuscular disorders (e.g. Duchenne muscular
dystrophy)
Question 7 of 180
A mother brings her 18-month-old daughter for review following a recent admission after a febrile convulsion. Which one of the following
statements regarding febrile convulsions is not correct?
They are rare in children older than 5 years of age
Carers should place the child in the recovery position and dial 999 if the seizure lasts > 5 minutes
The immunisation schedule should continue and can be safely done in the community
Next question
There is no evidence that giving a pyrexial child antipyretics reduces the chance of a febrile convulsion
Febrile convulsions
Febrile convulsions are seizures provoked by fever in otherwise normal children. They typically occur between the ages of 6 months and 5
years and are seen in 3% of children
Clinical features
Prognosis
Inform parents that seizures of short duration are not harmful to the child and that
about 1 in 3 children will have another febrile seizure in the future.
Advise parents about what to do if further seizures occur, including how to protect
them from injury during the seizure and when to call for medical help.
Advise parents about managing fever, but explain that reducing fever does not
prevent recurrence.
Drugs should not be prescribed to manage or prevent future seizures unless advised
to do so by a specialist.
Follow-up should be arranged, the timing of which will depend on the clinical
condition of the child.
o
o
o
o
o
Question 8 of 180
Hydralazine
Lymecycline
Sodium valproate
Aminophylline
Next question
galactosaemia
viral infections - this is controversial with respect to HIV in the developing world. This is because there is such an increased
infant mortality and morbidity associated with bottle feeding that some doctors think the benefits outweigh the risk of HIV
transmission
Drug contraindications
The following drugs can be given to mothers who are breast feeding:
digoxin
*the BNF advises that the amount is too small to affect neonatal hypothyroidism screening
**clozapine should be avoided
Question 9 of 180
The parents of a 3-year-old boy with cystic fibrosis ask for advice. They are considering having more children. What is the chance that their
next child will be a carrier of the cystic fibrosis gene?
50%
100%
1 in 25
25%
66.6%
Next question
As cystic fibrosis is an autosomal recessive condition there is a 50% chance that their next child will be a carrier of cystic fibrosis (i.e. be
heterozygous for the genetic defect) and a 25% chance that the child will actually have the disease (be homozygous).
Cystic fibrosis
Cystic fibrosis (CF) is an autosomal recessive disorder causing increased viscosity of secretions (e.g. lungs and pancreas). It is due to a
defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel
In the UK 80% of CF cases are due to delta F508 on the long arm of chromosome 7. Cystic fibrosis affects 1 per 2500 births, and the
carrier rate is c. 1 in 25
Organisms which may colonise CF patients
Staphylococcus aureus
Pseudomonas aeruginosa
Burkholderia cepacia*
Aspergillus
Question 10 of 180
Which one of the following best describes the emergency treatment of a child with severe croup?
Oxygen + nebulised saline
Oxygen + IM benzylpenicillin
Next question
Oral dexamethasone should also be given if the child is able to take it.
Croup
Croup is a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a
combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases.
Epidemiology
Features
stridor
barking cough (worse at night)
fever
coryzal symptoms
Clinical Knowledge Summaries (CKS) suggest using the following criteria to grade the severity*:
Mild
Moderate
Severe
CKS suggest admitting any child with moderate or severe croup. Other features which should prompt admission include:
Management
CKS recommend giving a single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity
prednisolone is an alternative if dexamethasone is not available
Emergency treatment
high-flow oxygen
nebulised adrenaline
*these in turn are based partly on the Alberta Medical Association (2008) Guideline for the diagnosis and management of croup.
Croup - Summary
Croup (laryngotracheitis) is a common childhood disease that is usually caused by a virus. It is
characterized by the sudden onset of a seal-like barking cough usually accompanied by stridor
(predominantly inspiratory), hoarse voice, and respiratory distress due to upper airways
obstruction. Symptoms are usually worse at night. There may be a fever up to 40C.
There is often a preceding 14-day history of a non-specific cough, rhinorrhoea, and fever.
Croup most commonly affects children 6 months to 3 years of age, with a peak incidence during
the second year of life.
Mild:
Occasional barking cough and no audible stridor at rest.
No or mild suprasternal and/or intercostal recession.
The child is happy and is prepared to eat, drink, and play.
Moderate:
Frequent barking cough and easily audible stridor at rest.
Suprasternal and sternal wall retraction at rest.
No or little distress or agitation.
Alshehr, M., Almegamsi, T. and Hammdi, A. (2005) Efficacy of a small dose of oral
dexamethasone in croup. Biomedical Research 16(1), 65-72.
Bjornson, C.L. and Johnson, D.W. (2008) Croup. Lancet 371(9609), 329-339. [Abstract]
Bjornson, C.L., Klassen, T.P., Williamson, J. et al. (2004) A randomized trial of a single dose of
oral dexamethasone for mild croup. New England Journal of Medicine 351(13), 1306-1313.
[Abstract] [Free Full-text]
BNF 64 (2012) British National Formulary. 64th edn. London: British Medical Association and
Royal Pharmaceutical Society of Great Britain.
Brown, J.C. (2002) The management of croup. British Medical Bulletin 61(1), 189-202. [Abstract]
[Free Full-text]
Brown, S. (2006b) GP guide to the assessment and management of croup. Prescriber 17(16), 2528. [Free Full-text]
Brown, J.C. and Klassen, T.P. (2000) Croup. In: Moyer, V.A., Elliott, E.J., Davis, R.L. et al. (Eds.)
Evidence based pediatrics and child health. London: BMJ Books. 215-227.
Cherry, J.D. (2005) State of the evidence for standard-of-care treatments for croup: are we
where we need to be? Pediatric Infectious Disease Journal 24(11 Suppl), S198-S201. [Abstract]
Chub-Uppakarn, S. and Sangsupawanich, P. (2007) A randomized comparison of dexamethasone
0.15 mg/kg versus 0.6 mg/kg for the treatment of moderate to severe croup. International
Journal of Pediatric Otorhinolaryngology 71(3), 473-477. [Abstract]
Fifoot, A.A. and Ting, J.Y. (2007) Comparison between single-dose oral prednisolone and oral
dexamethasone in the treatment of croup: a randomized, double-blinded clinical trial.
Emergency Medicine Australasia 19(1), 51-58. [Abstract]
Fitzgerald, D.A. (2006) The assessment and management of croup. Paediatric Respiratory
Reviews 7(1), 73-81. [Abstract]
Geelhoed, G.C. and Macdonald, W.B. (1995) Oral dexamethasone in the treatment of croup:
0.15 mg/kg versus 0.3 mg/kg. Pediatric Pulmonology 20(6), 362-368. [Abstract]
Geelhoed, G.C., Turner, J. and Macdonald, W.B.G. (1996) Efficacy of a small single dose of oral
dexamethasone for outpatient croup: a double blind placebo controlled trial. British Medical
Journal 313(7050), 140-142. [Abstract] [Free Full-text]
Greally, P., Cheng, K., Tanner, M.S. and Field, D.J. (1990) Children with croup presenting with
scalds. British Medical Journal 301(6743), 113. [Free Full-text]
Helms, P. and Henderson, J. (2003b) Infections causing upper airway obstruction. In: McIntosh,
N., Helms, P. and Smyth, R. (Eds.) Forfar & Arneil's textbook of pediatrics. 6th edn. Edinburgh:
Churchill Livingstone. 776-777.
Kairys, S.W., Olmstead, E.M. and O'Connor, G.T. (1989) Steroid treatment of laryngotracheitis: a
meta-analysis of the evidence from randomized trials. Pediatrics 83(5), 683-693. [Abstract]
MHRA (2011g) Press release: more exact paracetamol dosing for children to be introduced.
Medicines and Healthcare products Regulatory Agency. www.mhra.gov.uk [Free Full-text]
Moore, M. and Little, P. (2006) Humidified air inhalation for treating croup (Cochrane Review)
[Withdrawn]. The Cochrane Library. Issue 3. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Moore, M. and Little, P. (2007) Humidified air inhalation for treating croup: a systematic review
and meta-analysis. Family Practice 24(4), 295-301. [Abstract] [Free Full-text]
NICE (2007o) Feverish illness in children. Assessment and initial management in children
younger than 5 years (NICE guideline). . Clinical guideline 47. National Institute for Health and
Care Excellence. www.nice.org.uk
Russell, K.F., Liang, Y., O'Gorman, K. et al. (2011) Glucocorticoids for croup (Cochrane Review).
The Cochrane Library. Issue 1. John Wiley & Sons, Ltd. www.thecochranelibrary.com [Free Fulltext]
Scolnik, D., Coates, A.L., Stephens, D. et al. (2006) Controlled delivery of high vs low humidity vs
mist therapy for croup in emergency departments: a randomized controlled trial. Journal of the
American Medical Association 295(11), 1274-1280. [Abstract] [Free Full-text]
Sparrow, A. and Geelhoed, G. (2006) Prednisolone versus dexamethasone in croup: a
randomised equivalence trial. Archives of Disease in Childhood 91(7), 580-583. [Abstract] [Free
Full-text]
Sydney West Area Health Service (2004) Nurse practitioner clinical practice guidelines for the
management of croup. NSW Health. www.health.nsw.gov.au [Free Full-text]
Vyas, J. (2007) Croup. New Zealand Family Physician 34(4), 266-269. [Free Full-text]
Wagener, J.S., Landau, L.I., Olinsky, A. and Phelan, P.D. (1986) Management of children
hospitalized for laryngotracheobronchitis. Pediatric Pulmonology 2(3), 159-162. [Abstract]
Question 11 of 180
An 18-month-old girl is admitted as she has been vomiting and unwell for the past two days. Her parents think that she has some
abdominal pain as she is pointing to her abdomen. An abdominal x-ray and subsequent ultrasound are taken:
Intussception
Pyloric stenosis
Incarcerated hernia
Meckel's diverticulum
Next question
The abdominal film demonstrates a large soft tissue opacity ('sausage shaped') in the left upper quadrant. Ultrasound confirms an
intussusception with a nice example of a target sign.
Intussusception
Intussusception describes the invagination of one portion of bowel into the lumen of the adjacent bowel, most commonly around the ileocaecal region.
Intussusception is usually affects infants between 6-18 months old. Boys are affected twice as often as girls
Features
Investigation
ultrasound is now the investigation of choice and may show a target-like mass
Management
the majority of children can be treated with reduction by air insufflation under radiological control, which is now widely used firstline compared to the traditional barium enema
if this fails, or the child has signs of peritonitis, surgery is performed
Question 12 of 180
What is the average age that the first signs of puberty appear in girls?
10 years
11.5 years
12.5 years
9 years
8 years
Next question
Puberty
Males
first sign is testicular growth at around 12 years of age (range = 10-15 years)
testicular volume > 4 ml indicates onset of puberty
maximum height spurt at 14
Females
first sign is breast development at around 11.5 years of age (range = 9-13 years)
height spurt reaches its maximum early in puberty (at 12) , before menarche
menarche at 13 (11-15)
there is an increase of only about 4% of height following menarche
Question 13 of 180
You are asked to organise a tutorial on child protection for medical students attached to the surgery. When discussing patterns of behaviour
which may point towards child abuse, which one of the following is least likely to be relevant?
Frequent attendances to see the GP
Torn frenulum
Next question
Frequent attendance to the A&E department, rather than GP, may point towards child abuse as parents presume they will see a different
doctor each time, making it less likely suspicions will be aroused
bruising
fractures: particularly metaphyseal, posterior rib fractures or multiple fractures at different stages of healing
torn frenulum: e.g. from forcing a bottle into a child's mouth
burns or scalds
failure to thrive
sexually transmitted infections e.g. Chlamydia, Gonorrhoea, Trichomonas
Question 14 of 180
A 15-year-old girl presents with a palpable purpuric rash over her lower limbs accompanied by polyarthralgia following a recent sore throat.
What is the most likely diagnosis?
Rubella
Measles
Erythema multiforme
Henoch-Schonlein purpura
Next question
Henoch-Schonlein purpura
Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis. There is a degree of overlap with IgA nephropathy (Berger's
disease). HSP is usually seen in children following an infection.
Features
palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
abdominal pain
polyarthritis
features of IgA nephropathy may occur e.g. haematuria, renal failure
Treatment
Prognosis
usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
around 1/3rd of patients have a relapse
Question 15 of 180
A 4-week-old baby girl is diagnosed as having developmental dysplasia of the left hip following an ultrasound examination. Clinical
examination of the hip was abnormal at birth. What treatment is she most likely to be given?
Double nappies
Pavlik harness
Re-scan at 3 months
Surgery
Next question
DDH is slightly more common in the left hip. Around 20% of cases are bilateral.
Clinical examination is made using the Barlow and Ortolani tests:
Question 16 of 180
You review a 3-year-old girl who is being treated for idiopathic constipation with Movicol Paediatric Plain. Her mother has increased the
dose but unfortunately there has been no response. She remains well and examination of the abdomen is normal. What is the most
appropriate next step?
Add senna
Add lactulose
Prescribe a one-off dose of sodium picosulfate to be given whilst the health visitor is present
Next question
Constipation in children
The frequency at which children open their bowels varies widely, but generally decreases with age from a mean of 3 times per day for
infants under 6 months old to once a day after 3 years of age.
NICE produced guidelines in 2010 on the diagnosis and management of constipation in children. A diagnosis of constipation is suggested
by 2 or more of the following:
Child < 1 year
Stool pattern
History
The vast majority of children have no identifiable cause - idiopathic constipation. Other causes of constipation in children include:
dehydration
low-fibre diet
medications: e.g. Opiates
anal fissure
over-enthusiastic potty training
hypothyroidism
Hirschsprung's disease
hypercalcaemia
learning disabilities
After making a diagnosis of constipation NICE then suggesting excluding secondary causes. If no red or amber flags are present then a
diagnosis of idiopathic constipation can be made:
Timing
Passage of
meconium
< 48 hours
Stool pattern
Growth
> 48 hours
'Ribbon' stools
Generally well, weight and height within normal limits, fit and active
Previously unknown or
undiagnosed weakness in legs,
locomotor delay
Abdomen
Distension
Diet
Other
Prior to starting treatment the child needs to be assessed for faecal impaction. Factors which suggest faecal impaction include:
polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) using an escalating dose regimen as the first-line treatment
add a stimulant laxative if Movicol Paediatric Plain does not lead to disimpaction after 2 weeks
substitute a stimulant laxative singly or in combination with an osmotic laxative such as lactulose if Movicol Paediatric Plain is
not tolerated
inform families that disimpaction treatment can initially increase symptoms of soiling and abdominal pain
Maintenance therapy
very similar to the above regime, with obvious adjustments to the starting dose, i.e.
first-line: Movicol Paediatric Plain
add a stimulant laxative if no response
substitute a stimulant laxative if Movicol Paediatric Plain is not tolerated. Add another laxative such as lactulose or docusate if
stools are hard
continue medication at maintenance dose for several weeks after regular bowel habit is established, then reduce dose gradually
General points
do not use dietary interventions alone as first-line treatment although ensure child is having adequate fluid and fibre intake
consider regular toileting and non-punitive behavioural interventions
for all children consider asking the Health Visitor or Paediatric Continence Advisor to help support the parents.
The NICE guidelines do not specifically discuss the management of very young child. The following recommendations are largely based on
the old Clinical Knowledge Summaries recommendations.
Infants not yet weaned (usually < 6 months)
bottle-fed infants: give extra water in between feeds. Try gentle abdominal massage and bicycling the infant's legs
breast-fed infants: constipation is unusual and organic causes should be considered
Changes
Update
Goals
QIPP - Options for local implementation
Background information
Definition
Causes
Prevalence
Diagnosis
Management
Scenario: Management
Prescribing information
Important aspects of prescribing information relevant to primary healthcare are
covered in this section specifically for the drugs recommended in this CKS topic.
For further information on contraindications, cautions, drug interactions, and
adverse effects, see the electronic Medicines Compendium (eMC)
(http://medicines.org.uk/emc), or the British National Formulary (BNF)
(www.bnf.org).
Types of laxatives
Choice of stimulant laxative
Palatability
Contraindications
Dose and titration
Adverse effects
Evidence
Supporting evidence
Search strategy
References
ABPI Medicines Compendium (2010a) Summary of product characteristics for
Dulcolax tablets, 5mg. Electronic Medicines CompendiumDatapharm
Communications Ltd. www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2010b) Summary of product characteristics for
Dulcolax liquid. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2010c) Summary of product characteristics for
Senokot syrup. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
BNF 59 (2010) British National Formulary. 59th edn. London: British Medical
Association and Royal Pharmaceutical Society of Great Britain.
Clayden, G.S., Keshtgar, A.S., Carcani-Rathwell, I. and Abhyankar, A. (2005) The
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National Collaborating Centre for Women's and Children's Health (2010)
Constipation in children and young people: diagnosis and management of idiopathic
childhood constipation in primary and secondary care (full NICE guideline). . Clinical
guideline 99. Royal College of Obstetricians and Gynaecologists. www.nice.org.uk
[Free Full-text]
NICE (2010) Constipation in children and young people: diagnosis and management of
idiopathic childhood constipation in primary and secondary care (NICE guideline). .
Clinical guideline 99. National Institute for Health and Care Excellence.
www.nice.org.uk [Free Full-text]
NICE (2013) Key therapeutic topics - medicines management options for local
implementation. National Institute for Health and Care Excellence. www.nice.org.uk
[Free Full-text]
NPC (2012) Key therapeutic topics - medicines management options for local
implementation. National Prescribing Centre. www.npc.nhs.uk [Free Full-text]
van den Berg, M.M., Benninga, M.A. and Di Lorenzo, C. (2006) Epidemiology of
childhood constipation: a systematic review. American Journal of Gastroenterology
101(10), 2401-2409. [Abstract]
Question 17 of 180
At what age would the average child start to say 'mama' and 'dada'?
3 months
4-5 months
6-7 months
9-10 months
13-14 months
Next question
Milestone
3 months
6 months
9 months
12 months
12-15 months
2 years
2 years
3 years
4 years
Question 18 of 180
The chance of a 40-year-old mother giving birth to a child with Down's syndrome is approximately:
1 in 5
1 in 10
1 in 30
1 in 100
1 in 500
Next question
Down's syndrome risk - 1/1,000 at 30 years then divide by 3 for every 5 years
One way of remembering this is by starting at 1/1,000 at 30 years and then dividing the denominator by 3 (i.e. 3 times more common) for
every extra 5 years of age
Cytogenetics
Mode
Non-disjunction
94%
Robertsonian translocation
5%
(usually onto 14)
Mosaicism
1%
The chance of a further child with Down's syndrome is approximately 1 in 100 if the mother is less than 35 years old. If the trisomy 21 is a
result of a translocation the risk is much higher
Question 19 of 180
A mother asks for information following a recent admission of her 2-year-old son with a febrile convulsion. What is the chance of her son
having a further febrile convulsion?
< 2%
5%
10%
20%
30%
Next question
Febrile convulsions
Febrile convulsions are seizures provoked by fever in otherwise normal children. They typically occur between the ages of 6 months and 5
years and are seen in 3% of children
Clinical features
Prognosis
Advise parents about what to do if further seizures occur, including how to protect them from
injury during the seizure and when to call for medical help.
Advise parents about managing fever, but explain that reducing fever does not prevent
recurrence.
Drugs should not be prescribed to manage or prevent future seizures unless advised to do so by
a specialist.
Follow-up should be arranged, the timing of which will depend on the clinical condition of the
child.
Have I got the right topic?
Age from 6 months to 6 years
This CKS topic covers the management of a child who presents with, or after, a febrile seizure.
This CKS topic does not cover the management of a child who has epilepsy or any other seizure
disorder.
There are separate CKS topics on Epilepsy, Feverish children - risk assessment, and Feverish
children - management.
The target audience for this CKS topic is healthcare professionals working within the NHS in the
UK, and providing first contact or primary health care.
How up-to-date is this topic?
Changes
Update
Goals and outcome measures
Goals
Background information
Definition
Cause
Prevalence
Risk factors for first seizure
Complications
Prognosis
Diagnosis
Diagnosis of febrile seizure
Diagnosis
Management
Scenario: Management after a seizure : covers the management of a child who has had a febrile
seizure, including assessment, advice, follow up, when to refer, and emergency management.
Scenario: Managing a seizure : covers the management of a child who is currently having a
febrile seizure, including safety advice, airway management, when to use anticonvulsant
medication, when to admit to hospital, and when to measure blood glucose levels.
Scenario: Management after a seizure
Scenario: Managing a seizure
Evidence
Supporting evidence
Search strategy
References
Armon, K., Stephenson, T., Hemingway, P. et al. (2003) An evidence and consensus based
guideline for the management of a child after a seizure. Emergency Medicine Journal 20(1), 1320. [Abstract] [Free Full-text]
Barlow, W.E., Davis, R.L., Glasser, J.W. et al. (2001) The risk of seizures after receipt of whole-cell
pertussis or measles, mumps, and rubella vaccine. New England Journal of Medicine 345(9),
656-661. [Abstract] [Free Full-text]
Baysun, S., Aydin, O.F., Atmaca, E. and Yavuz, G.Y.K. (2005) A comparison of buccal midazolam
and rectal diazepam for the acute treatment of seizures. Clinical Pediatrics 44(9), 771-776.
[Abstract]
Berg, A.T., Shinnar, S., Darefsky, A.S. et al. (1997) Predictors of recurrent febrile seizures. A
prospective cohort study. Archives of Pediatrics & Adolescent Medicine 151(4), 371-378.
[Abstract] [Free Full-text]
Bhattacharyya, M., Kalra, V. and Gulati, S. (2006) Intranasal midazolam vs rectal diazepam in
acute childhood seizures. Pediatric Neurology 34(5), 355-359. [Abstract]
Chungath, M. and Shorvon, S. (2008) The mortality and morbidity of febrile seizures. Nature
Clinical Practice Neurology 4(11), 610-621. [Abstract]
DTB (2005) Drugs for the doctors bag: 2 - children. Drug & Therapeutics Bulletin 43(11), 81-84.
[Abstract]
Epilepsy Action (2005) The role of primary care in epilepsy management 2. Epilepsy Action.
www.epilepsy.org.uk [Free Full-text]
Ferrie, C. (2008) Seizures, epilepsy and other paroxysmal disorders. In: McIntosh, N., Helms, P.J.,
Smyth, R.L. and Logan, S. (Eds.) Forfar and Arneil's textbook of pediatrics. 7th edn. Edinburgh:
Churchill Livingstone. 841-872.
Fisher, R.G. and Boyce, T.G. (Eds.) (2005) Roseola syndrome (exanthem subitum). In: Moffet's
pediatric infectious diseases: a problem-orientated approach. 4th edn. Philadelphia: Lippincott
Williams & Wilkins. 407-408.
Fukuyama, Y., Seki, T., Ohtsuka, C. et al. (1996) Practical guidelines for physicians in the
management of febrile seizures. Brain & Development 18(6), 479-484. [Abstract]
Ganesh, R. and Janakiraman, L. (2008) Serum zinc levels in children with simple febrile seizure.
Clinical Pediatrics 47(2), 164-166. [Abstract]
Graves, R.C., Oehler, K. and Tingle, L.E. (2012) Febrile seizures: risks, evaluation, and prognosis.
American Family Physician 85(2), 149-153. [Abstract] [Free Full-text]
Hartfield, D.S., Tan, J., Yager, J.Y. et al. (2009) The association between iron deficiency and
febrile seizures in childhood. Clinical Pediatrics 48(4), 420-426. [Abstract]
Jones, C. and Isaacs, D. (2008) Exanthemata. In: McIntosh, N., Helms, P.J., Smyth, R.L. and Logan,
S. (Eds.) Forfar and Arneil's textbook of pediatrics. 7th edn. Edinburgh: Churchill Livingstone.
1274-1279.
Jones, T. and Jacobsen, S.J. (2007) Childhood febrile seizures: overview and implications.
International Journal of Medical Sciences 4(2), 110-114. [Abstract] [Free Full-text]
Knudsen, F.U. (1996) Febrile seizures - treatment and outcome. Brain & Development 18(6),
438-449. [Abstract]
Koren, G. (2000) Intranasal midazolam for febrile seizures. A step forward in treating a common
and distressing conditions. British Medical Journal 321(7253), 64-65. [Free Full-text]
Lahat, E., Goldman, M., Barr, J. et al. (2000) Comparison of intranasal midazolam with
intravenous diazepam for treating febrile seizures in children: prospective randomised study.
British Medical Journal 321(7253), 83-86. [Abstract] [Free Full-text]
McIntyre, J., Robertson, S., Norris, E. et al. (2005) Safety and efficacy of buccal midazolam versus
rectal diazepam for emergency treatment of seizures in children: a randomised controlled trial.
Lancet 336(9481), 205-210. [Abstract]
Mewasingh, L.D. (2010) Febrile seizures. Clinical EvidenceBMJ Publishing Group Ltd.
www.clinicalevidence.com [Free Full-text]
MHRA (2011) Press release: more exact paracetamol dosing for children to be introduced.
Medicines and Healthcare products Regulatory Agency. www.mhra.gov.uk [Free Full-text]
National Collaborating Centre for Women's and Children's Health (2013) Feverish illness in
children: assessment and initial management in children younger than 5 years (full NICE
guideline). Clinical guidance 160National Centre for Health and Care Excellence.
www.nice.org.uk [Free Full-text]
Offringa, M. and Moyer, V.A. (2001) Evidence based paediatrics: evidence based management of
seizures associated with fever. British Medical Journal 323(7321), 1111-1114. [Free Full-text]
Offringa, M. and Newton, R. (2012) Prophylactic drug management for febrile seizures in
children (Cochrane Review). The Cochrane Library. Issue 4. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Rosenbloom, E., Finkelstein, Y., Adams-Webber, T. and Kozer, E. (2013) Do antipyretics prevent
the recurrence of febrile seizures in children? A systematic review of randomized controlled
trials and meta-analysis. European Journal of Paediatric Neurology 17(6), 585-588. [Abstract]
Royal College of Physicians and the British Paediatric Association (1991) Guidelines for the
management of convulsions with fever. British Medical Journal 303(6803), 634-636. [Free Fulltext]
Sadleir, L.G. and Scheffer, I.E. (2007) Febrile seizures. British Medical Journal 334(7588), 307311. [Free Full-text]
Siqueira, L.F. (2010) Febrile seizures: update on diagnosis and management. Revista Da
Associacao Medica Brasileira 56(4), 489-492. [Abstract] [Free Full-text]
Strengell, T., Uhari, M., Tarkka, R. et al. (2009) Antipyretic agents for preventing recurrences of
febrile seizures: randomized controlled trial. Archives of Pediatrics & Adolescent Medicine
163(9), 799-804. [Abstract] [Free Full-text]
van Stuijvenberg, M., Derksen-Lubsen, G., Steyerberg, E.W. et al. (1998) Randomized, controlled
trial of ibuprofen sysrup administered during febrile illnesses to prevent febrile seizure
recurrences. Pediatrics 102(5), E51. [Abstract]
Verity, C.M., Greenwood, R. and Golding, J. (1998) Long-term intellectual and behavioral
outcomes of children with febrile convulsions. New England Journal of Medicine 338(24), 17231728. [Abstract] [Free Full-text]
Vestergaard, M., Hviid, A., Madsen, K.M. et al. (2004) MMR vaccination and febrile seizures:
evaluation of susceptible subgroups and long-term prognosis. Journal of the American Medical
Association 292(3), 351-357. [Abstract] [Free Full-text]
Vestergaard, M., Pedersen, M.G., Ostergaard, J.R. et al. (2008) Death in children with febrile
seizures: a population-based cohort study. Lancet 372(9637), 457-463. [Abstract]
Warden, C.R., Zibulewsky, J., Mace, S. et al. (2003) Evaluation and management of febrile
seizures in the out-of-hospital and emergency department settings. Annals of Emergency
Medicine 41(2), 215-222. [Abstract]
Waruiru, C. and Appleton, R. (2004) Febrile seizures: an update. Archives of Disease in Childhood
89(8), 751-756. [Abstract] [Free Full-text]
Question 20 of 180
Mortality is 15-25%
Next question
Reye's syndrome
Reye's syndrome is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys
and pancreas. The aetiology of Reye's syndrome is not fully understood although there is a known association with aspirin use and a viral
cause has been postulated
The peak incidence is 2 years of age, features include:
Management is supportive
Although the prognosis has improved over recent years there is still a mortality rate of 15-25%.
Question 21 of 180
An 11-year-old girl presents with a productive cough and fever. A chest x-ray is taken:
Next question
The loss of the left heart border is a classic sign of left lingual consolidation. There is no left middle lobe!
consolidation
pleural effusion
collapse
pneumonectomy
specific lesions e.g. tumours
fluid e.g. pulmonary oedema
For more information check out the link to the superb Radiology Masterclass website.
Question 22 of 180
A 10-year-old girl is admitted with shortness-of-breath and fatigue. A chest x-ray is performed on admission:
Heart failure
Asthma
Cystic fibrosis
Pneumonia
Next question
The x-ray shows dilated cardiomyopathy and features of pulmonary oedema including fluid in the horizontal fissure.
interstitial oedema
bat's wing appearance
upper lobe diversion (increased blood flow to the superior parts of the lung)
Kerley B lines
pleural effusion
cardiomegaly may be seen if there is cardiogenic cause
Question 23 of 180
Tonsillitis
Obesity
Kallman's syndrome
Down's syndrome
Next question
Kallman's syndrome is a cause of delayed puberty secondary to hypogonadotrophic hypogonadism. It is not associated with snoring
Snoring in children
Causes
obesity
nasal problems: polyps, deviated septum, hypertrophic nasal turbinates
recurrent tonsillitis
Down's syndrome
hypothyroidism
Question 24 of 180
For which one of the following indications is carbamazepine least likely to be a useful management option?
Trigeminal neuralgia
Absence seizures
Bipolar disorder
Next question
Carbamazepine
Carbamazepine is chemically similar to the tricyclic antidepressant drugs. It is most commonly used in the treatment of epilepsy, particularly
partial seizures, where carbamazepine remains a first-line medication. Other uses include
Mechanism of action
sodium channel blocker, decreasing the sodium influx into neurons which in turn decreases excitability
Adverse effects
Question 25 of 180
Carbamazepine
Sodium valproate
Methyldopa
Amiodarone
Next question
galactosaemia
viral infections - this is controversial with respect to HIV in the developing world. This is because there is such an increased
infant mortality and morbidity associated with bottle feeding that some doctors think the benefits outweigh the risk of HIV
transmission
Drug contraindications
The following drugs can be given to mothers who are breast feeding:
*the BNF advises that the amount is too small to affect neonatal hypothyroidism screening
**clozapine should be avoided
Question 26 of 180
Menstrual bleeding
Breast development
Height spurt
Next question
Puberty
Males
first sign is testicular growth at around 12 years of age (range = 10-15 years)
testicular volume > 4 ml indicates onset of puberty
maximum height spurt at 14
Females
first sign is breast development at around 11.5 years of age (range = 9-13 years)
height spurt reaches its maximum early in puberty (at 12) , before menarche
menarche at 13 (11-15)
there is an increase of only about 4% of height following menarche
Question 27 of 180
A 9-year-old boy who has recently arrived from India presents with fever. On examination a grey coating is seen surrounding the tonsils and
there is extensive cervical lymphadenopathy. What is the most likely diagnosis?
Dengue fever
Typhoid
Paratyphoid
Actinomycosis
Diphtheria
Next question
Diphtheria
Diphtheria is caused by the Gram positive bacterium Corynebacterium diphtheriae
Pathophysiology
Diphtheria toxin commonly causes a 'diphtheric membrane' on tonsils caused by necrotic mucosal cells. Systemic distribution may produce
necrosis of myocardial, neural and renal tissue
Possible presentations
Question 28 of 180
A 2-year-old boy presents with a harsh cough and pyrexia. His symptoms worsened overnight and on examination stridor is noted. Which
one of the following interventions may improve his symptoms?
Codeine linctus
Humidified oxygen
Nebulised salbutamol
Oral erythromycin
Oral dexamethasone
Next question
Croup
Croup is a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a
combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases.
Epidemiology
Features
stridor
barking cough (worse at night)
fever
coryzal symptoms
Clinical Knowledge Summaries (CKS) suggest using the following criteria to grade the severity*:
Mild
Moderate
Severe
CKS suggest admitting any child with moderate or severe croup. Other features which should prompt admission include:
Management
CKS recommend giving a single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity
prednisolone is an alternative if dexamethasone is not available
Emergency treatment
high-flow oxygen
nebulised adrenaline
*these in turn are based partly on the Alberta Medical Association (2008) Guideline for the diagnosis and management of croup.
Question 29 of 180
Next question
Hypospadias
Hypospadias is a congenital abnormality of the penis which occurs in approximately 3/1,000 male infants
Hypospadias is characterised by
Corrective surgery is performed before 2 years of age. It is essential that the child is not circumcised prior to the surgery as the foreskin
may be used in the corrective procedure
Question 30 of 180
A 4-year-old boy is brought in to surgery by his mother. He has reportedly been drowsy and pyrexial for the past 2 hours. Whilst waiting to
be seen he becomes unresponsive. One of your GP colleagues performs an urgent assessment whilst the practice nurse calls 999. He is
making no respiratory effort so 5 rescue breaths are given. As you arrive your colleague cannot detect a brachial or carotid pulse. What is
the most appropriate course of action?
Start chest compressions/ventilations at a ratio of 5:1
Next question
As two trained members of staff are present the ratio of chest compressions to ventilations should be 15:2
compression:ventilation ratio: lay rescuers should use a ratio of 30:2. If there are two or more rescuers with a duty to respond
then a ratio of 15:2 should be used
age definitions: an infant is a child under 1 year, a child is between 1 year and puberty
Key points of algorithm (please see link attached for more details)
unresponsive?
shout for help
open airway
look, listen, feel for breathing
give 5 rescue breaths
check for signs of circulation
15 chest compressions:2 rescue breaths (see above)
Question 31 of 180
A newborn female baby is diagnosed with cystic fibrosis following an episode of meconium ileus shortly after birth. Which one of the
following is least likely to occur as a consequence of her underlying diagnosis?
Delayed puberty
Nasal polyps
Diabetes mellitus
Rectal prolapse
Arthropathy
Next question
neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice
recurrent chest infections (40%)
malabsorption (30%): steatorrhoea, failure to thrive
other features (10%): liver disease
short stature
diabetes mellitus
delayed puberty
rectal prolapse (due to bulky stools)
nasal polyps
male infertility, female subfertility
Question 32 of 180
At what age would the average child acquire the ability to build a tower of three blocks?
18 months
2 years
2 years
3 years
4 years
Next question
Milestone
3 months
Age
Milestone
9 months
12 months
Bricks
Age
Milestone
15 monthsTower of 2
18 monthsTower of 3
2 years
Tower of 6
3 years
Tower of 9
Drawing
Age
Milestone
18 monthsCircular scribble
2 years
3 years
Copies circle
4 years
Copies cross
5 years
Book
Age
Milestone
Notes
hand preference before 12 months is abnormal and may indicate cerebral palsy
Question 33 of 180
Genu valgum
Harrison's sulcus
Next question
Rickets
Rickets is a term which describes inadequately mineralised bone in developing and growing bones. This results in soft and easily deformed
bones. It is usually due to vitamin D deficiency. In adults the equivalent condition is termed osteomalacia
Predisposing factors
Features
in toddlers - genu varum (bow legs), in older children - genu valgum (knock knees)
'rickety rosary' - swelling at the costochondral junction
kyphoscoliosis
craniotabes - soft skull bones in early life
Harrison's sulcus
reduced serum calcium - symptoms may results from hypocalcaemia
raised alkaline phosphatase
Management
oral vitamin D
Question 34 of 180
A 12-year-old girl presents with a two-day history of an itchy rash over her whole body associated with a low-grade pyrexia:
Encephalitis
Dilated cardiomyopathy
Pneumonia
Next question
Dilated cardiomyopathy is not an established complication of chickenpox, unlike the other four options.
Chickenpox
Chickenpox is caused by primary infection with varicella zoster virus. Shingles is reactivation of dormant virus in dorsal root ganglion
Chickenpox is highly infectious
fever initially
itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
systemic upset is usually mild
Management is supportive
A common complication is secondary bacterial infection of the lesions. Rare complications include
pneumonia
encephalitis (cerebellar involvement may be seen)
disseminated haemorrhagic chickenpox
arthritis, nephritis and pancreatitis may very rarely be seen
*it was traditionally taught that patients were infective until all lesions had scabbed over
Question 35 of 180
A mother brings her 3-year-old boy who is known to have Down's syndrome to surgery, as she is concerned about his vision. Which one of
the following eye problems is least associated with Down's syndrome?
Refractive errors
Cataracts
Retinal detachment
Strabismus
Recurrent blepharitis
Next question
Hearing
otitis media and glue ear are very common resulting in hearing problems
Question 36 of 180
At what age would the average child acquire the ability to sit without support?
12 months
4-6 months
10-11 months
7-8 months
3 months
Next question
Milestone
3 months
6 months
7-8 months
9 months
Pulls to standing
Crawls
12 months
Cruises
Walks with one hand held
Age
Milestone
18 months
2 years
Runs
Walks upstairs and downstairs holding on to rail
3 years
4 years
Notes
the majority of children crawl on all fours before walking but some children 'bottom-shuffle'. This is a normal variant and runs in
families
Question 37 of 180
Which one of the following statements regarding the MMR vaccine is incorrect?
Malaise, fever and rash may occur after around one week
Children who received another live vaccine 2 weeks ago can safely have MMR
Next question
MMR vaccine
Children in the UK receive two doses of the Measles, Mumps and Rubella (MMR) vaccine before entry to primary school. This currently
occurs at 12-15 months and 3-4 years as part of the routine immunisation schedule
Contraindications to MMR
severe immunosuppression
allergy to neomycin
children who have received another live vaccine by injection within 4 weeks
pregnancy should be avoided for at least 1 month following vaccination
immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are
present)
Adverse effects
malaise, fever and rash may occur after the first dose of MMR. This typically occurs after 5-10 days and lasts around 2-3 days
Question 38 of 180
Next question
Retinoblastoma
Retinoblastoma is a the most common ocular malignancy found in children. The average age of diagnosis is 18 months.
Pathophysiology
Possible features
absence of red-reflex, repalced by a white pupil (leukocoria) - the most common presenting symptom
strabismus
visual problems
Management
Prognosis
Question 39 of 180
A 2-year-old child with a history of atopic eczema is brought to the local GP surgery. Her eczema is usually well controlled with emollients
but her parents are concerned as the facial eczema has got significantly worse overnight. She now has painful clustered blisters on both
cheeks, around her mouth on her neck. Her temperature is 37.9C. What is the most appropriate management?
Advise paracetamol + emollients and reassure
Admit to hospital
Add hydrocortisone 1%
Oral flucloxacillin
Next question
Eczema herpeticum
Eczema herpeticum describes a severe primary infection of the skin by herpes simplex virus 1 or 2. It is more commonly seen in children
with atopic eczema. As it is potentially life threatening children should be admitted for IV aciclovir
Question 40 of 180
A 5-year-old girl is brought to surgery due to a high temperature. On examination she is noted to have an evolving purpuric rash. What is
the most appropriate course of action?
IM benzylpenicillin 150mg
IM benzylpenicillin 300mg
IM benzylpenicillin 600mg
IM benzylpenicillin 900mg
IM benzylpenicillin 1200mg
Next question
The RCGP have previously fed back that doctors are expected to be familiar with emergency drug doses, and have mentioned suspected
meningococcal septicaemia in particular
Age
Dose
< 1 year
300 mg
1 - 10 years600 mg
> 10 years 1200 mg
Question 41 of 180
What is the most useful investigation to screen for the complications of Kawasaki disease?
Echocardiogram
Lumbar puncture
Coronary angiogram
ECG
Next question
Kawasaki disease
Kawasaki disease is a type of vasculitis which is predominately seen in children. Whilst Kawasaki disease is uncommon it is important to
recognise as it may cause potentially serious complications, including coronary artery aneurysms
Features
high-grade fever which lasts for > 5 days. Fever is characteristically resistant to antipyretics
conjunctival injection
bright red, cracked lips
strawberry tongue
cervical lymphadenopathy
red palms of the hands and the soles of the feet which later peel
high-dose aspirin*
intravenous immunoglobulin
echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms
Complications
*Kawasaki disease is one of the few indications for the use of aspirin in children. Due to the risk of Reye's syndrome aspirin is normally
contraindicated in children.
Question 42 of 180
Precocious puberty in females may be defined as the development of secondary sexual characteristics before:
8 years of age
9 years of age
10 years of age
11 years of age
12 years of age
Next question
Precocious puberty
Definition
'development of secondary sexual characteristics before 8 years in females and 9 years in males'
more common in females
Testes
are rare, associated with rapid onset, neurological symptoms and signs and dissonance
e.g. McCune Albright syndrome
Question 43 of 180
Which one of the following vaccinations should be avoided in patients who are HIV positive?
Rabies
BCG
Hepatitis B
Pertussis
Diphtheria
Next question
BCG
MMR
oral polio
yellow fever
oral typhoid
Vaccinations
It is important to be aware of vaccines which are of the live-attenuated type as these may pose a risk to immunocompromised patients. The
main types of vaccine are as follows:
Live attenuated
BCG
measles, mumps, rubella (MMR)
influenza (intranasal)
oral rotavirus
oral polio
yellow fever
oral typhoid*
Inactivated preparations
rabies
influenza (intramuscular)
Detoxified exotoxins
tetanus
diphtheria
pertussis ('acellular' vaccine)
hepatitis B
meningococcus, pneumococcus, haemophilus
Notes
influenza: different types are available, including whole inactivated virus, split virion (virus particles disrupted by detergent
treatment) and sub-unit (mainly haemagglutinin and neuraminidase)
cholera: contains inactivated Inaba and Ogawa strains of Vibrio cholerae together with recombinant B-subunit of the cholera
toxin
hepatitis B: contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant
DNA technology
Question 44 of 180
A mother brings her 9-year-old daughter into surgery. She has been having recurrent headaches. Which one of the following features of
migraine is more common in children?
Prolonged migraines (e.g. 24-48 hours)
Hemiplegia
Gastrointestinal disturbance
Next question
Nausea, vomiting and abdominal pain are common in children with migraine.
1. unilateral location*
2. pulsating quality (i.e., varying with the heartbeat)
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs)
Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out
by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)
*In children, attacks may be shorter-lasting, headache is more commonly bilateral, and gastrointestinal disturbance is more prominent.
Migraine with aura (seen in around 25% of migraine patients) tends to be easier to diagnose with a typical aura being progressive in nature
and may occur hours prior to the headache. Typical aura include a transient hemianopic disturbance or a spreading scintillating scotoma
('jagged crescent'). Sensory symptoms may also occur
If we compare these guidelines to the NICE criteria the following points are noted:
The following aura symptoms are atypical and may prompt further investigation/referral;
motor weakness
double vision
visual symptoms affecting only one eye
poor balance
110 - 160 30 - 40
1 - 2 100 - 150 25 - 35
2 - 5 90 - 140
25 - 30
5 - 12 80 - 120
20 - 25
> 12 60 - 100
15 - 20
Question 46 of 180
Which booster vaccines do young people usually receive between the ages of 13 - 18 years?
Key
Next question
Hib + Men C
DT + Hib
DT + IPV + Men C
IPV + Hib
DT + Men C
Next question
Immunisation schedule
The current UK immunisation schedule is as follows. Please note that this table includes the changes announced in 2010 which merged the
12 and 13 month visits into one.
Age
Recommended immunisations
At birth
2 months
DTaP/IPV/Hib + PCV
Oral rotavirus vaccine
3 months
DTaP/IPV/Hib + Men C
Oral rotavirus vaccine
4 months
DTaP/IPV/Hib + PCV
3-4 years
MMR + DTaP/IPV
At birth the BCG vaccine should be given if the baby is deemed at risk of tuberculosis (e.g. Tuberculosis in the family in the past 6 months).
Hepatitis B vaccine should be given at birth if the mother is HBsAg +ve.
Key
Question 47 of 180
Your next patient is a 2-year-old boy. His mother is very concerned as he has had a high temperature all day and is not taking fluids. On
review he is drowsy with a toxic appearance but no rash is seen. Which one of the following features is most supportive of a diagnosis of
meningococcal septicaemia?
Photophobia
Temperature of 39.4C
Neck stiffness
Bulging fontanelle
Cold peripheries
Next question
Whilst the typical non-blanching rash (petechial or purpuric) develops in over 80% of patients with meningococcal septicaemia it is
important to be aware of other symptoms/signs. A high temperature can be seen with any childhood illness and is non-specific. Neck
stiffness, photophobia and a bulging fontanelle suggest meninigits rather than septicaemia. Of course you would not be able to assess the
fontanelle in a patient of this age anyway. Please see the link for a comprehensive list of symptoms/signs.
Meningococcal septicaemia
Meningococcal septicaemia is a frightening condition for patients, parents and doctors. It is associated with a high morbidity and mortality
unless treated early - meningococcal disease is the leading infectious cause of death in early childhood. A high index of suspicion is
therefore needed. Much of the following is based on the 2010 NICE guidelines (please see link).
Presentation of meningococcal disease:
15% - meningitis
25% - septicaemia
60% - a combination of meningitis and septicaemia
give intramuscular or intravenous benzylpenicillin unless there is a history of anaphylaxis (do not give if this will delay hospital
transfer)
NICE recommend phoning 999
Question 48 of 180
Abdominal mass
Fever
Loin pain
Haematuria
Next question
Wilms' tumour
Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a
median age of 3 years old.
Features
Associations
Beckwith-Wiedemann syndrome
as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
hemihypertrophy
around one-third of cases are associated with a mutation in the WT1 gene on chromosome 11
Management
nephrectomy
chemotherapy
radiotherapy if advanced disease
prognosis: good, 80% cure rate
Histological features include epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells and small cell blastomatous tissues
resembling the metanephric blastema
Question 49 of 180
Hirsutism
Diplopia
Ataxia
Next question
Carbamazepine
Carbamazepine is chemically similar to the tricyclic antidepressant drugs. It is most commonly used in the treatment of epilepsy, particularly
partial seizures, where carbamazepine remains a first-line medication. Other uses include
Mechanism of action
sodium channel blocker, decreasing the sodium influx into neurons which in turn decreases excitability
Adverse effects
Question 50 of 180
Which one of the following statements regarding the vaccine used to routinely immunise children against influenza is correct?
The most common side-effect is pyrexia
It is a live vaccine
Should be given at 12 months if the child was born before 35 weeks gestation
Next question
Influenza vaccination
Seasonal influenza still accounts for a significant morbidity and mortality in the UK each winter, with the influenza season typically starting
in the middle of November. This may vary year from year so it is recommended that vaccination occurs between September and early
November. There are three types of influenza virus; A, B and C. Types A and B account for the majority of clinical disease.
Prior to 2013 flu vaccination was only offered to the elderly and at risk groups.
Remember that the type of vaccine given routinely to children and the one given to the elderly and at risk groups is different (live vs.
inactivated) - this explains the different contraindications
Children
A new NHS influenza vaccination programme for children was announced in 2013. There are three key things to remember about the
children's vaccine:
it is given intranasally
the first dose is given at 2-3 years, then annually after that
children who were traditionally offered the flu vaccine (e.g. asthmatics) will now be given intranasal vaccine unless this is
inappropriate, for example if they are immunosuppressed. In this situation the inactivated, injectable vaccine should be given
only children aged 2-9 years who have not received an influenza vaccine before need 2 doses
it is more effective than the injectable vaccine
Contraindications
immunocompromised
aged < 2 years
current febrile illness or blocked nose/rhinorrhoea
current wheeze (e.g. ongoing viral-induced wheeze/asthma) or history of severe asthma (BTS step 4)
egg allergy
pregnancy/breastfeeding
if the child is taking aspirin (e.g. for Kawasaki disease) due to a risk of Reye's syndrome
Side-effects
blocked-nose/rhinorrhoea
headache
anorexia
health and social care staff directly involved in patient care (e.g. NHS staff)
those living in long-stay residential care homes
carers of the elderly or disabled person whose welfare may be at risk if the carer becomes ill (at the GP's discretion)
it is an inactivated vaccine, so cannot cause influenza. A minority of patients however develop fever and malaise which may last
1-2 days
should be stored between +2 and +8C and shielded from light
contraindications include hypersensitivity to egg protein.
in adults the vaccination is around 75% effective, although this figure decreases in the elderly
it takes around 10-14 days after immunisation before antibody levels are at protective levels
Question 51 of 180
Hurler's syndrome
Phenylketonuria
Haemochromatosis
Next question
Autosomal recessive conditions are often thought to be 'metabolic' as opposed to autosomal dominant conditions being 'structural', notable
exceptions:
some 'metabolic' conditions such as Hunter's and G6PD are X-linked recessive whilst others such as hyperlipidaemia type II
and hypokalaemic periodic paralysis are autosomal dominant
some 'structural' conditions such as ataxia telangiectasia and Friedreich's ataxia are autosomal recessive
Achondroplasia
Acute intermittent porphyria
Adult polycystic disease
Antithrombin III deficiency
Ehlers-Danlos syndrome
Familial adenomatous polyposis
Hereditary haemorrhagic telangiectasia
Hereditary spherocytosis
Hereditary non-polyposis colorectal carcinoma
Huntington's disease
Hyperlipidaemia type II
Hypokalaemic periodic paralysis
Malignant hyperthermia
Marfan's syndromes
Myotonic dystrophy
Neurofibromatosis
Noonan syndrome
Osteogenesis imperfecta
Peutz-Jeghers syndrome
Retinoblastoma
Romano-Ward syndrome
Tuberose sclerosis
Von Hippel-Lindau syndrome
Von Willebrand's disease*
*type 3 von Willebrand's disease (most severe form) is inherited as an autosomal recessive trait. Around 80% of patients have type 1
disease
Question 52 of 180
A mother brings her 14-month-old daughter into surgery as she is concerned about her hearing. For a child born in the United Kingdom, at
what age would their hearing first be formally assessed?
Newborn - otoacoustic emission
Next question
Test
Comments
Newborn
All newborns should be tested as part of the Newborn Hearing Screening Programme. A computer
Otoacoustic emission
generated click is played through a small earpiece. The presence of a soft echo indicates a healthy
test
cochlea
Newborn &
infants
Auditory Brainstem
Response test
Age
Test
Comments
6-9 months
Distraction test
Recognition of familiar
Uses familiar objects e.g. teddy, cup. Ask child simple questions - e.g. 'where is the teddy?'
objects
Performance testing
Speech discrimination
Uses similar sounding objects e.g. Kendall Toy test, McCormick Toy Test
tests
> 3 years
As well as the above test there is a questionnaire for parents in the Personal Child Health Records - 'Can your baby hear you?'
Question 53 of 180
A 14-year-old male being investigated for iron-deficiency anaemia is found to have numerous polyps in his jejunum. On examination he is
also noted to have pigmented lesions on his palms and soles. What is the likely diagnosis?
Hereditary non-polyposis colorectal carcinoma
Gardner's syndrome
Peutz-Jeghers syndrome
Next question
Hereditary haemorrhagic telangiectasia is associated with mucocutaneous lesions and iron-deficiency anaemia but intestinal polyps are not
a feature
Peutz-Jeghers syndrome
Peutz-Jeghers syndrome is an autosomal dominant condition characterised by numerous hamartomatous polyps in the gastrointestinal
tract. It is also associated with pigmented freckles on the lips, face, palms and soles. Around 50% of patients will have died from a
gastrointestinal tract cancer by the age of 60 years.
Genetics
autosomal dominant
responsible gene encodes serine threonine kinase LKB1 or STK11
Features
gastrointestinal bleeding
Management
Question 54 of 180
A 14-year-old boy is brought to surgery by his father who is concerned he is shorter than the other boys at school, despite having started
puberty. His father is 1.70 m tall and his mother is 1.50 m tall. Given his parents height, what is his adult height potential?
1.67 m
1.72 m
1.53 m
1.65 m
1.70 m
Next question
Short stature
Short stature may be caused by:
The adult height potential may be calculated for a male child by (father's height in cm + mother's height in cm) / 2 then add 7 cm
For a female child by (father's height in cm + mother's height in cm) / 2 then minus 7 cm
This can then be plotted on a height centile chart to find the mid-parental centile
Question 55 of 180
Which one of the following conditions does the neonatal blood spot screening test not screen for?
Phenylketonuria
Cystic fibrosis
Galactosaemia
Hypothyroidism
Next question
congenital hypothyroidism
cystic fibrosis
phenylketonuria
sickle cell disease
medium chain acyl-CoA dehydrogenase deficiency (MCADD)
Question 56 of 180
Which one of the following neonatal complications is least commonly seen in diabetic pregnancies?
Stillbirth
Microsomia
Hypoglycaemia
Sacral agenesis
Next question
Neonatal complications
macrosomia (although diabetes may also cause small for gestational age babies)
hypoglycaemia (secondary to beta cell hyperplasia)
respiratory distress syndrome: surfactant production is delayed
polycythaemia: therefore more neonatal jaundice
malformation rates increase 3-4 fold e.g. sacral agenesis, CNS and CVS malformations (hypertrophic cardiomyopathy)
stillbirth
hypomagnesaemia
hypocalcaemia
shoulder dystocia (may cause Erb's palsy)
Question 57 of 180
A 14-month-old girl is diagnosed as having roseola infantum. What is the most common complication of this disease?
Febrile convulsions
Transient synovitis
Pneumonia
Orchitis
Next question
Roseola infantum
Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human
herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years.
Features
aseptic meningitis
hepatitis
Question 58 of 180
Anal fissure
Addison's disease
Hirschsprung's disease
Hypercalcaemia
Next question
Constipation in children
The frequency at which children open their bowels varies widely, but generally decreases with age from a mean of 3 times per day for
infants under 6 months old to once a day after 3 years of age.
NICE produced guidelines in 2010 on the diagnosis and management of constipation in children. A diagnosis of constipation is suggested
by 2 or more of the following:
Child < 1 year
Stool pattern
History
The vast majority of children have no identifiable cause - idiopathic constipation. Other causes of constipation in children include:
dehydration
low-fibre diet
medications: e.g. Opiates
anal fissure
over-enthusiastic potty training
hypothyroidism
Hirschsprung's disease
hypercalcaemia
learning disabilities
After making a diagnosis of constipation NICE then suggesting excluding secondary causes. If no red or amber flags are present then a
diagnosis of idiopathic constipation can be made:
Timing
Passage of
meconium
< 48 hours
Stool pattern
Growth
> 48 hours
'Ribbon' stools
Generally well, weight and height within normal limits, fit and active
Previously unknown or
undiagnosed weakness in legs,
locomotor delay
Abdomen
Distension
Diet
Other
Prior to starting treatment the child needs to be assessed for faecal impaction. Factors which suggest faecal impaction include:
polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) using an escalating dose regimen as the first-line treatment
add a stimulant laxative if Movicol Paediatric Plain does not lead to disimpaction after 2 weeks
substitute a stimulant laxative singly or in combination with an osmotic laxative such as lactulose if Movicol Paediatric Plain is
not tolerated
inform families that disimpaction treatment can initially increase symptoms of soiling and abdominal pain
Maintenance therapy
very similar to the above regime, with obvious adjustments to the starting dose, i.e.
first-line: Movicol Paediatric Plain
add a stimulant laxative if no response
substitute a stimulant laxative if Movicol Paediatric Plain is not tolerated. Add another laxative such as lactulose or docusate if
stools are hard
continue medication at maintenance dose for several weeks after regular bowel habit is established, then reduce dose gradually
General points
do not use dietary interventions alone as first-line treatment although ensure child is having adequate fluid and fibre intake
consider regular toileting and non-punitive behavioural interventions
for all children consider asking the Health Visitor or Paediatric Continence Advisor to help support the parents.
The NICE guidelines do not specifically discuss the management of very young child. The following recommendations are largely based on
the old Clinical Knowledge Summaries recommendations.
Infants not yet weaned (usually < 6 months)
bottle-fed infants: give extra water in between feeds. Try gentle abdominal massage and bicycling the infant's legs
breast-fed infants: constipation is unusual and organic causes should be considered
Question 59 of 180
You are doing the six week check on a baby girl. Which one of the following best describes the Barlow test for developmental dysplasia of
the hip?
Attempts to relocate a dislocated femoral head
Observation of the relative height of the knees with the hips flexed at 90 degrees
Observation for buttock crease asymmetry with the hips flexed at 90 degrees
Next question
Developmental dysplasia of the hip (DDH) is gradually replacing the old term 'congenital dislocation of the hip' (CDH). It affects around 13% of newborns.
Risk factors
DDH is slightly more common in the left hip. Around 20% of cases are bilateral.
Clinical examination is made using the Barlow and Ortolani tests:
Question 60 of 180
A 30-year-old woman presents for the 6 week check of her son who was diagnosed with Down's syndrome shortly after birth. A letter from
genetics has confirmed non-disjunction as the cause of the trisomy. She asks you about future pregnancies. What is the chance that her
next child will also have Down's syndrome?
1 in 10
1 in 100
1 in 25
1 in 250
Next question
One way of remembering this is by starting at 1/1,000 at 30 years and then dividing the denominator by 3 (i.e. 3 times more common) for
every extra 5 years of age
Cytogenetics
Mode
Non-disjunction
94%
Robertsonian translocation
5%
(usually onto 14)
Mosaicism
1%
The chance of a further child with Down's syndrome is approximately 1 in 100 if the mother is less than 35 years old. If the trisomy 21 is a
result of a translocation the risk is much higher
Question 61 of 180
The mother of a 6-week-old baby girl born at 32 weeks gestation asks for advice about immunisation. What should happen regarding the
first set of vaccines?
Give first set of vaccinations at 3 months (i.e. delay for 1 month)
Give first set of vaccinations at 4 months (i.e. correct for gestational age)
Give first set of vaccinations as per normal timetable but within hospital environment
Next question
Immunisation schedule
The current UK immunisation schedule is as follows. Please note that this table includes the changes announced in 2010 which merged the
12 and 13 month visits into one.
Age
Recommended immunisations
At birth
2 months
DTaP/IPV/Hib + PCV
Oral rotavirus vaccine
3 months
DTaP/IPV/Hib + Men C
Oral rotavirus vaccine
4 months
DTaP/IPV/Hib + PCV
3-4 years
MMR + DTaP/IPV
At birth the BCG vaccine should be given if the baby is deemed at risk of tuberculosis (e.g. Tuberculosis in the family in the past 6 months).
Hepatitis B vaccine should be given at birth if the mother is HBsAg +ve.
Key
Question 62 of 180
Which one of the following drugs is not contra-indicated whilst breast feeding?
Tetracycline
Ciprofloxacin
Chloramphenicol
Doxycycline
Ceftriaxone
Next question
The major breastfeeding contraindications tested in exams relate to drugs (see below). Other contraindications of note include:
galactosaemia
viral infections - this is controversial with respect to HIV in the developing world. This is because there is such an increased
infant mortality and morbidity associated with bottle feeding that some doctors think the benefits outweigh the risk of HIV
transmission
Drug contraindications
The following drugs can be given to mothers who are breast feeding:
*the BNF advises that the amount is too small to affect neonatal hypothyroidism screening
**clozapine should be avoided
Question 63 of 180
You are asked to by the Primary Care Trust to design a program to improve the health of infants in the local community. What is the most
common cause of death of infants greater than one month but less than one year old?
Accidents
Congenital disorders
Cancer
Infection
Next question
After the age of 1 year accidents are the most common cause of death in children
prematurity
parental smoking
hyperthermia (e.g. over-wrapping)
putting the baby to sleep prone
male sex
multiple births
bottle feeding
social classes IV and V
maternal drug use
incidence increases in winter
Following a cot death siblings should be screened for potential sepsis and inborn errors of metabolism
Question 64 of 180
A 12-year-old female from Bulgaria presents to the surgery. She reports being unwell for the past 2 weeks. Initially she had a sore throat
but she is now experiencing joint pains intermittently in her knees, hips and ankles. On examination there are some pink, ring shaped
lesions on the trunk and occasional jerking movements of the face and hands. What is the most likely diagnosis?
Lyme disease
Infective endocarditis
Rheumatic fever
Still's disease
Next question
2 major criteria
Major criteria
erythema marginatum
Sydenham's chorea
polyarthritis
carditis (endo-, myo- or peri-)
subcutaneous nodules
Minor criteria
Erythema marginatum is seen in around 10% of children with rheumatic fever. It is rare in adults
Question 65 of 180
Infertility
Keratoconjunctivitis
Encephalitis
Pneumonia
Next question
Measles
Overview
RNA paramyxovirus
spread by droplets
infective from prodrome until 4 days after rash starts
incubation period = 10-14 days
Features
Koplik spots
Complications
encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness
febrile convulsions
giant cell pneumonia
keratoconjunctivitis, corneal ulceration
diarrhoea
increased incidence of appendicitis
myocarditis
The rash typically starts behind the ears and then spreads to the whole body
Management of contacts
if a child not immunized against measles comes into contact with measles then MMR should be offered (vaccine-induced
measles antibody develops more rapidly than that following natural infection)
this should be given within 72 hours
Reference ranges
End and review
Question 66 of 180
You are reviewing a 9-month-old child with suspected bronchiolitis. Which one of the following features should make you consider other
possible diagnoses?
Rhinitis
Feeding difficulties
Temperature of 39.7C
Expiratory wheeze
Next question
A low-grade fever is typical in bronchiolitis. SIGN guidelines advise that the presence of high fever should make the clinician carefully
consider other causes before making the diagnosis.
Bronchiolitis
Bronchiolitis is a condition characterised by acute bronchiolar inflammation. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of
cases. SIGN released guidelines on bronchiolitis in 2006. Please see the link for more details.
Epidemiology
most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 36 months). Maternal IgG provides protection to newborns against RSV
higher incidence in winter
Basics
Features
Investigation
A newborn baby is noted to have low-set ears, rocker bottom feet and overlapping of her fingers. What is the most likely
diagnosis?
Patau syndrome
Edward's syndrome
William's syndrome
Fragile X
Pierre-Robin syndrome
Childhood syndromes
Key features
Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers
Fragile X
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
Noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Next question
Syndrome
Key features
Pulmonary stenosis
Pierre-Robin syndrome*
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
Prader-Willi syndrome
Hypotonia
Hypogonadism
Obesity
William's syndrome
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
*this condition has many similarities with Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome
is autosomal dominant so there is usually a family history of similar problems
Question 68 of 180
An 8-year-old boy who is known to have asthma is reviewed. His current treatment is a salbutamol inhaler as required and beclometasone
inhaler 200mcg bd. Despite this he regular requires salbutamol for exacerbations and suffers with a night time cough. What is the most
appropriate next step in management?
Increase beclometasone to 400mcg bd
Referral to a paediatrician
Next question
The British Thoracic Society guidelines advise adding a long-acting beta2-agonist before maximising the inhaled steroid dose.
StepTherapy
Children aged 2-5 years: trial of a leukotriene receptor antagonist. If already taking leukotriene receptor antagonist reconsider inhaled
corticosteroids
Children aged under 2 years: refer to respiratory paediatrician
Question 69 of 180
2-5%
15-20%
11-12%
5-10%
Next question
Eczema in children
Eczema occurs in around 15-20% of children and is becoming more common. It typically presents before 6 months but clears in around
50% of children by 5 years of age and in 75% of children by 10 years of age
Features
Management
avoid irritants
simple emollients: large quantities should be prescribed (e.g. 250g / week), roughly in a ratio of with topical steroids of 10:1. If a
topical steroid is also being used the emollient should be applied first followed by waiting at least 30 minutes before applying the
topical steroid. Creams soak into the skin faster than ointments. Emollients can become contaminated with bacteria - fingers
should not be inserted into pots (many brands have pump dispensers)
topical steroids
in severe cases wet wraps and oral ciclosporin may be used
Question 70 of 180
Which one of the following congenital infections is most characteristically associated with sensorineural deafness?
Toxoplasma gondii
Parvovirus B19
Rubella
Treponema pallidum
Cytomegalovirus
Next question
Congenital rubella
sensorineural deafness
congenital cataracts
Congenital infections
The major congenital infections encountered in examinations are rubella, toxoplasmosis and cytomegalovirus
Cytomegalovirus is the most common congenital infection in the UK. Maternal infection is usually asymptomatic
Rubella
Toxoplasmosis
Cytomegalovirus
Rubella
Toxoplasmosis
Cytomegalovirus
Characteristic features
Sensorineural deafness
Cerebral calcification
Congenital cataracts
Growth retardation
Chorioretinitis
Congenital heart disease (e.g. patent ductus arteriosus)
Purpuric skin lesions
Hydrocephalus
Glaucoma
Other features
Growth retardation
Hepatosplenomegaly
Purpuric skin lesions
'Salt and pepper' chorioretinitis
Microphthalmia
Cerebral palsy
Sensorineural deafness
Encephalitis/seizures
Anaemia
Pneumonitis
HepatosplenomegalyHepatosplenomegaly
Cerebral palsy
Anaemia
Jaundice
Cerebral palsy
Question 71 of 180
Overriding aorta
Next question
Tetralogy of Fallot
Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease*. It typically presents at around 1-2 months,
although may not be picked up until the baby is 6 months old
TOF is a result of anterior malalignment of the aorticopulmonary septum. The four characteristic features are:
The severity of the right ventricular outflow tract obstruction determines the degree of cyanosis and clinical severity
Other features
cyanosis
causes a right-to-left shunt
ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually cause a murmur)
a right-sided aortic arch is seen in 25% of patients
chest x-ray shows a 'boot-shaped' heart, ECG shows right ventricular hypertrophy
Management
*however, at birth transposition of the great arteries is the more common lesion as patients with TOF generally present at around 1-2
months
Question 72 of 180
Next question
Squint
Squint (strabismus) is characterised by misalignment of the visual axes. Squints may be divided into concomitant (common) and paralytic
(rare)
Concomitant
Paralytic
Detection of a squint may be made by the corneal light reflection test - holding a light source 30cm from the child's face to see if the light
reflects symmetrically on the pupils
The cover test is used to identify the nature of the squint
Management
Question 73 of 180
Which one of the following is not associated with the development of obesity in children?
Male sex
Asian ethnicity
Down's syndrome
Next question
Obesity in children
Defining obesity is more difficult in children than adults as body mass index (BMI) varies with age. BMI percentile charts are therefore
needed to make an accurate assessment. Recent NICE guidelines suggest to use 'UK 1990 BMI charts to give age- and gender-specific
information'
NICE recommend
By far the most common cause of obesity in childhood is lifestyle factors. Other associations of obesity in children include:
Asian children: four times more likely to be obese than white children
female children
taller children: children with obesity are often above the 50th percentile in height
orthopaedic problems: slipped upper femoral epiphyses, Blount's disease (a development abnormality of the tibia resulting in
bowing of the legs), musculoskeletal pains
psychological consequences: poor self-esteem, bullying
sleep apnoea
benign intracranial hypertension
long-term consequences: increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease
Question 74 of 180
Phaeochromocytoma
Next question
Hypertension in children
Measuring blood pressure in children
correct cuff size is approximately 2/3 the length of the upper arm
the 4th Korotkoff sound is used to measure the diastolic blood pressure until adolescence, when the 5th Korotkoff sound can be
used
results should be compared with a graph of normal values for age
In younger children secondary hypertension is the most common cause, with renal parenchymal disease accounting for up to 80%
Causes of hypertension in children
You are asked to teach a 14-year-old boy how to use his metered dose salbutamol inhaler. After removing the cap and shaking,
what is the next step?
Next question
Inhaler technique
The following inhaler technique guideline is for metered dose inhalers (source: Asthma.org.uk, a resource recommended to
patients by the British Thoracic Society)
1. Remove cap and shake
Next question
VSDs are more common than ASDs. However, in adult patients ASDs are the more common new diagnosis as they generally presents
later
Cyanotic - most common causes
tetralogy of Fallot
transposition of the great arteries (TGA)
tricuspid atresia
pulmonary valve stenosis
Fallot's is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot's generally presenting at
around 1-2 months
Question 77 of 180
What is the investigation of choice to look for renal scarring in a child with vesicoureteric reflux?
Abdominal x-ray
Ultrasound
DMSA
CT
Micturating cystourethrogram
Next question
Vesicoureteric reflux
Vesicoureteric reflux (VUR) is the abnormal backflow of urine from the bladder into the ureter
and kidney. It is relatively common abnormality of the urinary tract in children and predisposes
to urinary tract infection (UTI), being found in around 30% of children who present with a UTI.
As around 35% of children develop renal scarring it is important to investigate for VUR in
children following a UTI
Pathophysiology of VUR
ureters are displaced laterally, entering the bladder in a more perpendicular fashion
than at an angle
therefore shortened intramural course of ureter
vesicoureteric junction cannot therefore function adequately
Grade
II
III
IV
Dilation of the renal pelvis and calyces with moderate ureteral tortuosity
Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity
Investigation
Question 78 of 180
Which one of the following congenital infections is most characteristically associated with chorioretinitis?
Cytomegalovirus
Treponema pallidum
Rubella
Toxoplasma gondii
Parvovirus B19
Next question
Congenital toxoplasmosis
cerebral calcification
chorioretinitis
A form of 'salt and pepper' chorioretinitis is also seen in congenital rubella but this is not a common feature.
Chorioretinitis is found in around 75% of patients with congenital toxoplasmosis.
Congenital infections
The major congenital infections encountered in examinations are rubella, toxoplasmosis and cytomegalovirus
Cytomegalovirus is the most common congenital infection in the UK. Maternal infection is usually asymptomatic
Rubella
Toxoplasmosis
Cytomegalovirus
Characteristic features
Sensorineural deafness
Cerebral calcification
Congenital cataracts
Growth retardation
Chorioretinitis
Congenital heart disease (e.g. patent ductus arteriosus)
Purpuric skin lesions
Hydrocephalus
Glaucoma
Other features
Growth retardation
Hepatosplenomegaly
Purpuric skin lesions
'Salt and pepper' chorioretinitis
Microphthalmia
Cerebral palsy
Sensorineural deafness
Encephalitis/seizures
Anaemia
Pneumonitis
HepatosplenomegalyHepatosplenomegaly
Cerebral palsy
Anaemia
Jaundice
Cerebral palsy
Question 79 of 180
Which one of the following is not a risk factor for sudden infant death syndrome?
Bottle feeding
Male sex
Summer months
Being a twin
Next question
The incidence of sudden infant death syndrome increases in the winter months
prematurity
parental smoking
hyperthermia (e.g. over-wrapping)
putting the baby to sleep prone
male sex
multiple births
bottle feeding
social classes IV and V
maternal drug use
incidence increases in winter
Following a cot death siblings should be screened for potential sepsis and inborn errors of metabolism
Question 80 of 180
Which one of the following statements regarding benign rolandic epilepsy is incorrect?
Seizures are usually partial in nature
Next question
Question 81 of 180
You review a 7-year-old girl who has cerebral palsy. She is having ongoing problems with spasticity in her legs which is causing pain and
contractures. On speaking to her mother you ascertain that she is having regular physiotherapy, using the appropriate orthoses and has
tried oral diazepam in the past. Which one of the following treatments may she be offered to try and improve her symptoms?
Baclofen
Clozapine
Clonidine
Dantrolene
Glyceryl trinitrate
Next question
Cerebral palsy
Cerebral palsy may be defined as a disorder of movement and posture due to a non-progressive lesion of the motor pathways in the
developing brain. It affects 2 in 1,000 live births and is the most common cause of major motor impairment
Possible manifestations include:
Children with cerebral palsy often have associated non-motor problems such as:
Causes
antenatal (80%): e.g. cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
intrapartum (10%): birth asphyxia/trauma
postnatal (10%): intraventricular haemorrhage, meningitis, head-trauma
Classification
Management
Question 82 of 180
Each of the following organisms commonly cause respiratory tract infections in patients with cystic fibrosis, except:
Aspergillus
Pseudomonas aeruginosa
Burkholderia cepacia
Staphylococcal aureus
Strongyloides stercoralis
Next question
Cystic fibrosis
Cystic fibrosis (CF) is an autosomal recessive disorder causing increased viscosity of secretions (e.g. lungs and pancreas). It is due to a
defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel
In the UK 80% of CF cases are due to delta F508 on the long arm of chromosome 7. Cystic fibrosis affects 1 per 2500 births, and the
carrier rate is c. 1 in 25
Organisms which may colonise CF patients
Staphylococcus aureus
Pseudomonas aeruginosa
Burkholderia cepacia*
Aspergillus
Question 83 of 180
A 10-year-old boy who is known to have asthma is reviewed. He currently uses salbutamol prn, beclometasone 100mcg bd and recently
had a trial of salmeterol 50mcg bd. Unfortunately he has noticed no benefit from adding salmeterol. What is the most appropriate next step
in management?
Stop salmeterol + trial of a leukotriene receptor antagonist
Refer to a paediatrician
Next question
As the trial of salmeterol has failed the next step would be to increase the inhaled steroid to 400 mcg / day.
Children aged 2-5 years: trial of a leukotriene receptor antagonist. If already taking leukotriene receptor antagonist reconsider inhaled
corticosteroids
Children aged under 2 years: refer to respiratory paediatrician
Question 84 of 180
A 6-year-old boy is diagnosed as having nephrotic syndrome. A presumptive diagnosis of minimal change glomerulonephritis is made.
What is the most appropriate treatment?
Cyclophosphamide
Albumin infusion
Plasma exchange
Prednisolone
Next question
Pathophysiology
T-cell and cytokine mediated damage to the glomerular basement membrane polyanion loss
the resultant reduction of electrostatic charge increased glomerular permeability to serum albumin
Features
nephrotic syndrome
normotension - hypertension is rare
highly selective proteinuria*
renal biopsy: electron microscopy shows fusion of podocytes
Management
*only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus
Question 85 of 180
A baby is born by elective Caesarean section at 38 weeks performed due to pregnancy-induced hypertension. At one hour the female baby
is noted to be grunting with mild intercostal recession. Oxygen saturations are 95-96% on air. What is the most likely cause of her
respiratory distress?
Surfactant deficient lung disease
Congenital pneumonia
Pulmonary hypoplasia
Next question
Pregnancy
Eczema
Next question
Immunisation
The Department of Health published guidance in 2006 on the safe administration of vaccines in its publication 'Immunisation against
infectious disease'
General contraindications to immunisation
confirmed anaphylactic reaction to a previous dose of a vaccine containing the same antigens
confirmed anaphylactic reaction to another component contained in the relevant vaccine (e.g. egg protein)
pregnancy
immunosuppression
Specific vaccines
DTP: vaccination should be deferred in children with an evolving or unstable neurological condition
asthma or eczema
history of seizures (if associated with fever then advice should be given regarding antipyretics)
breastfed child
previous history of natural pertussis, measles, mumps or rubella infection
history of neonatal jaundice
family history of autism
neurological conditions such as Down's or cerebral palsy
low birth weight or prematurity
patients on replacement steroids e.g. (CAH)
Question 87 of 180
Children from lower social classes are more likely to have an accident
Road traffic accidents are the most common cause of fatal accidents
Next question
Accidents in children
Around 15-20% of children attend Emergency Departments in the course of a year due to an accident. Accidents account for a third of all
childhood deaths and are the single most common cause of death in children aged between 1 - 15 years of age.
Key points
road traffic accidents are the most common cause of fatal accidents
boys and children from lower social classes are more likely to have an accident
Accident prevention
Accident prevention can be divided up into primary (preventing the accident from happening), secondary (prevent injury from the accident)
and tertiary (limit the impact of the injury) prevention strategies
The table below gives examples of accident prevention strategies
Primary prevention
Stair guards
Wearing seat belts
Speed limits*
Cycling helmets
Teaching parents first aid
Teaching road safety Smoke alarms
Window safety catchesLaminated safety glass
*some strategies such as reducing driving speed may have a role in both primary and secondary accident prevention
Question 88 of 180
Tricuspid atresia
Next question
VSDs are more common than ASDs. However, in adult patients ASDs are the more common new diagnosis as they generally presents
later
Cyanotic - most common causes
tetralogy of Fallot
transposition of the great arteries (TGA)
tricuspid atresia
pulmonary valve stenosis
Fallot's is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot's generally presenting at
around 1-2 months
Question 89 of 180
Which one of the following vaccines uses an inactivated preparation of the organism or virus?
Influenza (intramuscular)
Yellow fever
Oral polio
Measles
Diphtheria
Next question
Vaccinations
It is important to be aware of vaccines which are of the live-attenuated type as these may pose a risk to immunocompromised patients. The
main types of vaccine are as follows:
Live attenuated
BCG
Inactivated preparations
rabies
influenza (intramuscular)
Detoxified exotoxins
tetanus
diphtheria
pertussis ('acellular' vaccine)
hepatitis B
meningococcus, pneumococcus, haemophilus
Notes
influenza: different types are available, including whole inactivated virus, split virion (virus particles disrupted by detergent
treatment) and sub-unit (mainly haemagglutinin and neuraminidase)
cholera: contains inactivated Inaba and Ogawa strains of Vibrio cholerae together with recombinant B-subunit of the cholera
toxin
hepatitis B: contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant
DNA technology
Question 90 of 180
Sputum culture
Throat swab
Next question
coughing bouts: usually worse at night and after feeding, may be ended by vomiting & associated central cyanosis
inspiratory whoop: not always present (caused by forced inspiration against a closed glottis)
persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures
symptoms may last 10-14 weeks* and tend to be more severe in infants
marked lymphocytosis
Diagnosis
per nasal swab culture for Bordetella pertussis - may take several days or weeks to come back
PCR and serology are now increasingly used as their availability becomes more widespread
Management
Complications
subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures
Women who are between 28-38 weeks pregnant will be offered the vaccine.
*weeks, not days
Question 91 of 180
A 6-month-old girl is brought to the surgery by her mother. She has a 24 hour history of poor feeding, taking around two-thirds her normal
intake. On examination she is alert and there are no focal signs of infection. She smiles initially then has a strong cry when you try to
examine her chest. She stops crying after being soothed by her mother.
The respiratory rate is 36 / min, capillary refill time is < 2 secs and the temperature is 39.0C. What is the most appropriate management?
Advise to give antipyretics if the child is distressed + discuss red flags + booked appointment for next day
Admit to hospital
Advise to alternate paracetamol and ibuprofen + discuss red flags + see if not improving
IM benzylpenicillin
Next question
The temperature of 39.0C in a 6-month-old child is regarded as a 'amber' feature in the latest 2013 NICE guidelines. All the other features
however are green or low risk. It is therefore reasonable to manage this patient at home with the usual safety-netting advice.
NICE recommend that parents should only give antipyretics if the child is distressed.
temperature
heart rate
respiratory rate
capillary refill time
Signs of dehydration (reduced skin turgor, cool extremities etc) should also be looked for
Measuring temperature should be done with an electronic thermometer in the axilla if the child is < 4 weeks or with an electronic/chemical
dot thermometer in the axilla or an infra-red tympanic thermometer.
Risk stratification
Please see the link for the complete table, below is a modified version
Colour
Normal colour
Pale/mottled/ashen/blue
Activity
Responds normally to
social cues
Content/smiles
Stays awake or awakens
quickly
Strong normal cry/not
crying
Nasal flaring
Tachypnoea: respiratory rate
Respiratory
Grunting
Tachypnoea: respiratory rate >60
breaths/minute
Moderate or severe chest indrawing
Circulation and
hydration
Other
Management
If green:
Child can be managed at home with appropriate care advice, including when to seek further help
If amber:
provide parents with a safety net or refer to a paediatric specialist for further assessment
a safety net includes verbal or written information on warning symptoms and how further healthcare can be accessed, a followup appointment, liaison with other healthcare professionals, e.g. out-of-hours providers, for further follow-up
If red:
oral antibiotics should not be prescribed to children with fever without apparent source
if a pneumonia is suspected but the child is not going to be referred to hospital then a chest x-ray does not need to be routinely
performed
Recommendations
1.1 Thermometers and the detection of fever
1.2 Clinical assessment of children with fever
1.3 Management by remote assessment
1.4 Management by the non-paediatric practitioner
1.5 Management by the paediatric specialist
1.6 Antipyretic interventions
1.7 Advice for home care
1 Recommendations
The following guidance is based on the best available evidence. The full guideline gives details of
the methods and the evidence used to develop the guidance.
The wording used in the recommendations in this guideline denotes the certainty with which
the recommendation is made (the strength of the recommendation). See About this guideline
for details.
This guideline is intended for use by healthcare professionals for the assessment and initial
management in young children with feverish illness. The guideline should be followed until a
clinical diagnosis of the underlying condition has been made. Once a diagnosis has been made,
the child should be treated according to national or local guidance for that condition.
Parents or carers of a child with fever may approach a range of different healthcare
professionals as their first point of contact, for example, a GP, a pharmacist or an emergency
care practitioner. The training and experience of the healthcare professionals involved in the
child's care will vary and each should interpret the guidance according to the scope of their own
practice.
For the purposes of this guideline, fever was defined as 'an elevation of body temperature
above the normal daily variation'.
This guideline should be read in conjunction with:
1.2.2.1 Assess children with feverish illness for the presence or absence of symptoms and signs
that can be used to predict the risk of serious illness using the traffic light system (see table 1).
[2013]
1.2.2.2 When assessing children with learning disabilities, take the individual child's learning
disability into account when interpreting the traffic light table. [new 2013]
1.2.2.3 Recognise that children with any of the following symptoms or signs are in a high-risk
group for serious illness:
pale/mottled/ashen/blue skin, lips or tongue
no response to social cues[3]
appearing ill to a healthcare professional
does not wake or if roused does not stay awake
weak, high-pitched or continuous cry
grunting
respiratory rate greater than 60 breaths per minute
moderate or severe chest indrawing
reduced skin turgor
bulging fontanelle. [new 2013]
1.2.2.4 Recognise that children with any of the following symptoms or signs are in at least an
intermediate-risk group for serious illness:
pallor of skin, lips or tongue reported by parent or carer
not responding normally to social cues[3]
no smile
wakes only with prolonged stimulation
decreased activity
nasal flaring
dry mucous membranes
poor feeding in infants
Age
<12 months
>160
>140
1.2.2.14 Assess children with fever for signs of dehydration. Look for:
prolonged capillary refill time
abnormal skin turgor
abnormal respiratory pattern
weak pulse
cool extremities. [2007]
1.2.3 Symptoms and signs of specific illnesses
1.2.3.1 Look for a source of fever and check for the presence of symptoms and signs that are
associated with specific diseases (see table 2). [2007]
1.2.3.2 Consider meningococcal disease in any child with fever and a non-blanching rash,
particularly if any of the following features are present[5]:
an ill-looking child
lesions larger than 2 mm in diameter (purpura)
a capillary refill time of 3 seconds or longer
neck stiffness. [2007]
1.2.3.3 Consider bacterial meningitis in a child with fever and any of the following features[5]:
neck stiffness
bulging fontanelle
decreased level of consciousness
convulsive status epilepticus. [2007, amended 2013]
1.2.3.4 Be aware that classic signs of meningitis (neck stiffness, bulging fontanelle, high-pitched
cry) are often absent in infants with bacterial meningitis[5]. [2007]
1.2.3.5 Consider herpes simplex encephalitis in children with fever and any of the following
features:
focal neurological signs
focal seizures
decreased level of consciousness. [2007]
1.2.3.6 Consider pneumonia in children with fever and any of the following signs:
tachypnoea (respiratory rate greater than 60 breaths per minute, age 05 months; greater than
50 breaths per minute, age 612 months; greater than 40 breaths per minute, age older than
12 months)
crackles in the chest
nasal flaring
chest indrawing
cyanosis
oxygen saturation of 95% or less when breathing air. [2007]
1.2.3.7 Consider urinary tract infection in any child younger than 3 months with fever[6]. [2007]
1.2.3.8 Consider urinary tract infection in a child aged 3 months or older with fever and 1 or
more of the following[6]:
vomiting
poor feeding
lethargy
irritability
abdominal pain or tenderness
urinary frequency or dysuria. [new 2013]
1.2.3.9 Consider septic arthritis/osteomyelitis in children with fever and any of the following
signs:
swelling of a limb or joint
not using an extremity
Colour
(of skin, lips or
tongue)
Normal colour
Pallor reported by
parent/carer
Pale/mottled/ashen/blue
Activity
No smile
Stays awake or
awakens quickly
Weak, high-pitched or
continuous cry
Nasal flaring
Grunting
Tachypnoea: respiratory
rate
Other
Status epilepticus
Focal neurological signs
Focal seizures
Table 2 Summary table for symptoms and signs suggestive of specific diseases
[2013]
Diagnosis to be
considered
Meningococcal
disease
Pneumonia
Vomiting
Poor feeding
Lethargy
Irritability
Abdominal pain or tenderness
Urinary frequency or dysuria
Septic arthritis
Kawasaki disease
1.4.2.3 If any 'amber' features are present and no diagnosis has been reached, provide parents
or carers with a 'safety net' or refer to specialist paediatric care for further assessment. The
safety net should be 1 or more of the following:
providing the parent or carer with verbal and/or written information on warning symptoms and
how further healthcare can be accessed (see section 1.7.2)
arranging further follow-up at a specified time and place
liaising with other healthcare professionals, including out-of-hours providers, to ensure direct
access for the child if further assessment is required. [2007]
1.4.2.4 Children with 'green' features and none of the 'amber' or 'red' features can be cared for
at home with appropriate advice for parents and carers, including advice on when to seek
further attention from the healthcare services (see section 1.7). [2007, amended 2013]
1.4.3 Tests by the non-paediatric practitioner
1.4.3.1 Children with symptoms and signs suggesting pneumonia who are not admitted to
hospital should not routinely have a chest X-ray. [2007]
1.4.3.2 Test urine in children with fever as recommended in Urinary tract infection in children
(NICE clinical guideline 54). [2007]
1.4.4 Use of antibiotics by the non-paediatric practitioner
1.4.4.1 Do not prescribe oral antibiotics to children with fever without apparent source. [2007]
1.4.4.2 Give parenteral antibiotics to children with suspected meningococcal disease at the
earliest opportunity (either benzylpenicillin or a third-generation cephalosporin)[5]. [2007]
1.5 Management by the paediatric specialist
In this guideline, the term paediatric specialist refers to a healthcare professional who has had
specific training or has recognised expertise in the assessment and treatment of children and
their illnesses. Examples include paediatricians, or healthcare professionals working in children's
emergency departments.
1.5.1 Children younger than 5 years
1.5.1.1 Management by the paediatric specialist should start with a clinical assessment as
described in section 1.2. The healthcare professional should attempt to identify symptoms and
signs of serious illness and specific diseases as summarised in tables 1 and 2. [2007]
1.5.2 Children younger than 3 months
1.5.2.1 Infants younger than 3 months with fever should be observed and have the following
vital signs measured and recorded:
temperature
heart rate
respiratory rate. [2007]
1.5.2.2 Perform the following investigations in infants younger than 3 months with fever:
full blood count
blood culture
C-reactive protein
urine testing for urinary tract infection[6]
chest X-ray only if respiratory signs are present
stool culture, if diarrhoea is present. [2013]
1.5.2.3 Perform lumbar puncture in the following children with fever (unless contraindicated):
infants younger than 1 month
all infants aged 13 months who appear unwell
infants aged 13 months with a white blood cell count (WBC) less than 5 109/litre or greater
than 15 109/litre. [2007, amended 2013]
1.5.2.4 When indicated, perform a lumbar puncture without delay and, whenever possible,
before the administration of antibiotics. [2007]
1.5.2.5 Give parenteral antibiotics to:
infants younger than 1 month with fever
all infants aged 13 months with fever who appear unwell
infants aged 13 months with WBC less than 5 109/litre or greater than 15 109/litre. [2007,
amended 2013]
1.5.2.6 When parenteral antibiotics are indicated for infants younger than 3 months of age, a
third-generation cephalosporin (for example cefotaxime or ceftriaxone) should be given plus an
antibiotic active against listeria (for example, ampicillin or amoxicillin). [2007]
1.5.3 Children aged 3 months or older
1.5.3.1 Perform the following investigations in children with fever without apparent source who
present to paediatric specialists with 1 or more 'red' features:
full blood count
blood culture
C-reactive protein
urine testing for urinary tract infection[6]. [2013]
1.5.3.2 The following investigations should also be considered in children with 'red' features, as
guided by the clinical assessment:
lumbar puncture in children of all ages (if not contraindicated)
chest X-ray irrespective of body temperature and WBC
serum electrolytes and blood gas. [2007]
1.5.3.3 Children with fever without apparent source presenting to paediatric specialists who
have 1 or more 'amber' features, should have the following investigations performed unless
deemed unnecessary by an experienced paediatrician.
urine should be collected and tested for urinary tract infection[6]
blood tests: full blood count, C-reactive protein and blood cultures
lumbar puncture should be considered for children younger than 1 year
chest X-ray in a child with a fever greater than 39C and WBC greater than 20 109/litre. [2007]
1.5.3.4 Children who have been referred to a paediatric specialist with fever without apparent
source and who have no features of serious illness (that is, the 'green' group), should have urine
tested for urinary tract infection[6] and be assessed for symptoms and signs of pneumonia (see
table 2). [2007]
1.5.3.5 Do not routinely perform blood tests and chest X-rays in children with fever who have no
features of serious illness (that is, the 'green' group). [2007]
1.5.4 Viral co-infection
1.5.4.1 Febrile children with proven respiratory syncytial virus or influenza infection should be
assessed for features of serious illness. Consideration should be given to urine testing for urinary
tract infection[6]. [2007]
1.5.5 Observation in hospital
1.5.5.1 In children aged 3 months or older with fever without apparent source, a period of
observation in hospital (with or without investigations) should be considered as part of the
assessment to help differentiate non-serious from serious illness. [2007]
1.5.5.2 When a child has been given antipyretics, do not rely on a decrease or lack of decrease in
temperature at 12 hours to differentiate between serious and non-serious illness.
Nevertheless, in order to detect possible clinical deterioration, all children in hospital with
'amber' or 'red' features should still be reassessed after 12 hours. [new 2013]
1.5.6 Immediate treatment by the paediatric specialist (for children of all ages)
1.5.6.1 Children with fever and shock presenting to specialist paediatric care or an emergency
department should be:
given an immediate intravenous fluid bolus of 20 ml/kg; the initial fluid should normally be 0.9%
sodium chloride
actively monitored and given further fluid boluses as necessary. [2007]
1.5.6.2 Give immediate parenteral antibiotics to children with fever presenting to specialist
paediatric care or an emergency department if they are:
shocked
unrousable
showing signs of meningococcal disease. [2007]
1.5.6.3 Immediate parenteral antibiotics should be considered for children with fever and
reduced levels of consciousness. In these cases symptoms and signs of meningitis and herpes
simplex encephalitis should be sought (see table 2 and Bacterial meningitis and meningococcal
septicaemia [NICE clinical guideline 102]). [2007]
1.5.6.4 When parenteral antibiotics are indicated, a third-generation cephalosporin (for
example, cefotaxime or ceftriaxone) should be given, until culture results are available. For
children younger than 3 months, an antibiotic active against listeria (for example, ampicillin or
amoxicillin) should also be given. [2007]
1.5.6.5 Give intravenous aciclovir to children with fever and symptoms and signs suggestive of
herpes simplex encephalitis (see recommendation 1.2.3.5). [2007]
1.5.6.6 Oxygen should be given to children with fever who have signs of shock or oxygen
saturation (SpO2) of less than 92% when breathing air. Treatment with oxygen should also be
considered for children with an SpO2 of greater than 92%, as clinically indicated. [2007]
1.5.7 Causes and incidence of serious bacterial infection
1.5.7.1 In a child presenting to hospital with a fever and suspected serious bacterial infection,
requiring immediate treatment, antibiotics should be directed against Neisseria meningitidis,
Streptococcus pneumoniae, Escherichia coli, Staphylococcus aureus and Haemophilus influenzae
type b. A third-generation cephalosporin (for example, cefotaxime or ceftriaxone) is
appropriate, until culture results are available. For infants younger than 3 months, an antibiotic
active against listeria (for example, ampicillin or amoxicillin) should be added. [2007]
1.5.7.2 Refer to local treatment guidelines when rates of bacterial antibiotic resistance are
significant. [2007]
1.5.8 Admission to and discharge from hospital
1.5.8.1 In addition to the child's clinical condition, consider the following factors when deciding
whether to admit a child with fever to hospital:
social and family circumstances
other illnesses that affect the child or other family members
parental anxiety and instinct (based on their knowledge of their child)
contacts with other people who have serious infectious diseases
recent travel abroad to tropical/subtropical areas, or areas with a high risk of endemic infectious
disease
when the parent or carer's concern for their child's current illness has caused them to seek
healthcare advice repeatedly
where the family has experienced a previous serious illness or death due to feverish illness
which has increased their anxiety levels
when a feverish illness has no obvious cause, but the child remains ill longer than expected for a
self-limiting illness. [2007]
1.5.8.2 If it is decided that a child does not need to be admitted to hospital, but no diagnosis has
been reached, provide a safety net for parents and carers if any 'red' or 'amber' features are
present. The safety net should be 1 or more of the following:
providing the parent or carer with verbal and/or written information on warning symptoms and
how further healthcare can be accessed (see section 1.7.2)
arranging further follow-up at a specified time and place
liaising with other healthcare professionals, including out-of-hours providers, to ensure direct
access for the child if further assessment is required. [2007]
1.5.8.3 Children with 'green' features and none of the 'amber' or 'red' features can be cared for
at home with appropriate advice for parents and carers, including advice on when to seek
further attention from the healthcare services (see section 1.7). [2007, amended 2013]
1.5.9 Referral to paediatric intensive care
1.5.9.1 Children with fever who are shocked, unrousable or showing signs of meningococcal
disease should be urgently reviewed by an experienced paediatrician and consideration given to
referral to paediatric intensive care. [2007]
1.5.9.2 Give parenteral antibiotics to children with suspected meningococcal disease at the
earliest opportunity (either benzylpenicillin or a third-generation cephalosporin). [2007]
1.5.9.3 Children admitted to hospital with meningococcal disease should be under paediatric
care, supervised by a consultant and have their need for inotropes assessed. [2007]
1.6 Antipyretic interventions
1.6.1 Effects of body temperature reduction
1.6.1.1 Antipyretic agents do not prevent febrile convulsions and should not be used specifically
for this purpose. [2007]
1.6.2 Physical interventions to reduce body temperature
1.6.2.1 Tepid sponging is not recommended for the treatment of fever. [2007]
1.6.2.2 Children with fever should not be underdressed or over-wrapped. [2007]
1.6.3 Drug interventions to reduce body temperature
1.6.3.1 Consider using either paracetamol or ibuprofen in children with fever who appear
distressed. [new 2013]
1.6.3.2 Do not use antipyretic agents with the sole aim of reducing body temperature in children
with fever. [new 2013]
1.6.3.3 When using paracetamol or ibuprofen in children with fever:
continue only as long as the child appears distressed
consider changing to the other agent if the child's distress is not alleviated
do not give both agents simultaneously
only consider alternating these agents if the distress persists or recurs before the next dose is
due. [new 2013]
the parent or carer is distressed, or concerned that they are unable to look after their child.
[2007]
Question 92 of 180
What is the correct dose of intramuscular adrenaline to give a 10-year-old boy who is having an anaphylactic reaction?
IM adrenaline 300 mcg (0.3ml of 1 in 1,000)
Next question
Anaphylaxis
Anaphylaxis may be defined as a severe, life-threatening, generalised or systemic
hypersensitivity reaction.
Anaphylaxis is one of the few times when you would not have time to look up the dose of a medication. The Resuscitation Council
guidelines on anaphylaxis have recently been updated. Adrenaline is by far the most important drug in anaphylaxis and should be given as
soon as possible. The recommended doses for adrenaline, hydrocortisone and chlorphenamine are as follows:
Adrenaline
Hydrocortisone Chlorphenamine
< 6 months
250 mcg/kg
6 months - 6 years
2.5 mg
6-12 years
5 mg
Adult and child > 12 years 500 mcg (0.5ml 1 in 1,000) 200 mg
10 mg
Adrenaline can be repeated every 5 minutes if necessary. The best site for IM injection is the anterolateral aspect of the middle third of the
thigh.
Common identified causes of anaphylaxis
Question 93 of 180
Perihilar lymphadenopathy
No abnormality
Next question
The loss of the right heart border is a common finding in right middle lobe consolidation
consolidation
pleural effusion
collapse
pneumonectomy
specific lesions e.g. tumours
fluid e.g. pulmonary oedema
For more information check out the link to the superb Radiology Masterclass website.
Question 94 of 180
Which one of the following statements regarding cow's milk protein intolerance/allergy in infants is true?
An adrenaline pen should be given to all parents
Next question
Diagnosis is often clinical (e.g. improvement with cow's milk protein elimination). Investigations include:
Management
If the symptoms are severe (e.g. failure to thrive) refer to a paediatrician.
Management if formula-fed
extensive hydrolysed formula (eHF) milk is the first-line replacement formula for infants with mild-moderate symptoms
amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF
around 10% of infants are also intolerant to soya milk
Management if breast-fed
continue breastfeeding
eliminate cow's milk protein from maternal diet
use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months
CMPI usually resolves by 1-2 years of age. A challenge is often performed in the hospital setting as anaphylaxis can occur.
Question 95 of 180
Which one of the following is not part of the Apgar score for assessing the newborn?
Colour
Respiratory effort
Tone
Heart rate
Next question
Apgar score
The Apgar score is used to assess the health of a newborn baby
Score Pulse Respiratory effort Colour
Muscle tone
Reflex irritability
Pink
Grimace
Absent Nil
Muscle tone
Reflex irritability
Nil
Question 96 of 180
Height spurt
Next question
Puberty
Males
first sign is testicular growth at around 12 years of age (range = 10-15 years)
testicular volume > 4 ml indicates onset of puberty
maximum height spurt at 14
Females
first sign is breast development at around 11.5 years of age (range = 9-13 years)
height spurt reaches its maximum early in puberty (at 12) , before menarche
menarche at 13 (11-15)
there is an increase of only about 4% of height following menarche
Question 97 of 180
A 11-week-old boy is reviewed in surgery. As part of a general assessment his head circumference is noted to be between the 0.4th and
2nd centile. Which one of the following would not explain this finding?
Congenital infection
Fragile X syndrome
Normal variant
Craniosynostosis
Next question
Whilst not a classic cause of macrocephaly, children with Fragile X syndrome tend to have a head larger than normal
Microcephaly
Microcephaly may be defined as an occipital-frontal circumference < 2nd centile
Causes include
Question 98 of 180
A mother brings her son in to surgery as she suspects he has a squint. She thinks his right eye is 'turned inwards'. You perform a cover test
to gather further information. Which one of the following findings would be consistent with a right esotropia?
On covering the left eye the right eye moves medially to take up fixation
The cover test could not be used to identify this type of defect
On covering the left eye the right eye moves laterally to take up fixation
On covering the right eye the left eye moves laterally to take up fixation
On covering the right eye the left eye moves medially to take up fixation
Next question
On covering the left eye in this example the right eye moves laterally from the nasal (esotropic) position to take up fixation.
Squint
Squint (strabismus) is characterised by misalignment of the visual axes. Squints may be divided into concomitant (common) and paralytic
(rare)
Concomitant
Paralytic
Detection of a squint may be made by the corneal light reflection test - holding a light source 30cm from the child's face to see if the light
reflects symmetrically on the pupils
The cover test is used to identify the nature of the squint
Management
Question 99 of 180
Oral corticosteroids have been shown to reduce the duration of the illness
Next question
Bronchiolitis
Bronchiolitis is a condition characterised by acute bronchiolar inflammation. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of
cases. SIGN released guidelines on bronchiolitis in 2006. Please see the link for more details.
Epidemiology
most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 36 months). Maternal IgG provides protection to newborns against RSV
higher incidence in winter
Basics
Features
Investigation
A 4-year-old boy is noted to have macrocephaly and learning difficulties. What is the most likely diagnosis?
Patau syndrome
Pierre-Robin syndrome
Edward's syndrome
Fragile X
William's syndrome
Next question
Childhood syndromes
Key features
Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers
Fragile X
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
Noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
Pierre-Robin syndrome*
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
Prader-Willi syndrome
Hypotonia
Hypogonadism
Obesity
William's syndrome
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
*this condition has many similarities with Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome
is autosomal dominant so there is usually a family history of similar problems
Question 1 of 80
Wet combing
Dimeticone
Malathion
Benzyl benzoate
Next question
Head lice
Head lice (also known as pediculosis capitis or 'nits') is a common condition in children caused by the parasitic insect Pediculus capitis,
which lives on and among the hair of the scalp of humans
Diagnosis
Management
Head lice infestation (also known as pediculosis capitis) is the condition caused by the
parasitic insect Pediculus capitis, which lives on, and among, the hair of the scalp and
neck of humans.
o Live lice can be found anywhere on the scalp; the eggs are most commonly found
behind the ears and at the back of the neck.
o The severity of infestation varies from a few lice to thousands of lice, but a typical
infestation might have about 30 lice per head.
If left untreated, infestation with head lice may persist for long periods.
To confirm a person has head lice:
o Detection combing (systematic combing of wet or dry hair with a detection comb)
confirms the presence of lice.
o A diagnosis of active head lice infestation should only be made if a live head louse is
found.
o An itching scalp is not sufficient to diagnose active infestation.
o The presence of louse eggs alone, whether hatched (nits) or unhatched, are not
proof of active infestation.
o A person should only be treated if a live head louse is found.
All affected household members need simultaneous treatment.
Depending on the preference of the individual or parent and on the treatment history,
head lice can be treated with:
o Dimeticone 4% lotion (Hedrin).
o Dimeticone 92% spray (NYDA).
o Wet combing using the Bug Buster comb and method.
o Isopropyl myristate and cyclomethicone solution (Full Marks Solution ).
o Coconut, anise, and ylang ylang spray (Lyclear SprayAway).
o Malathion 0.5% aqueous liquid.
All treatments need more than one treatment session and no treatment can guarantee
success. Treatment has the best chance of success if it is performed correctly and if all
affected household members are treated on the same day.
People should be advised to check whether treatment was successful by detection
combing on day 2 or day 3 after completing a course of treatment, and again after an
interval of 7 days (day 9 or day 10 after completing a course of treatment).
If treatment is unsuccessful:
o The same treatment should be repeated, or a different treatment tried.
o All affected household contacts need to be treated simultaneously again.
Children who are being treated for head lice can still attend school.
Question 2 of 80
A mother brings her 2-year-old son to surgery. For the past two weeks he has been complaining of an itchy bottom. He is otherwise well
and clinical examination including that of the perianal area is unremarkable. What is the most appropriate management?
Hygiene measures + single dose dimeticone for child
Hygiene measures + single dose mebendazole repeated after 2 weeks for all the family
Next question
Threadworms are extremely common and in the absence of any other concern an itchy bottom would not warrant referral to the child
protection officer
Threadworms
Infestation with threadworms (Enterobius vermicularis, sometimes called pinworms) is extremely common amongst children in the UK.
Infestation occurs after swallowing eggs that are present in the environment.
Threadworm infestation is asymptomatic in around 90% of cases, possible features include:
Diagnosis may be made by the applying Sellotape to the perianal area and sending it to the laboratory for microscopy to see the eggs.
However, most patients are treated empirically and this approach is supported in the CKS guidelines.
Management
CKS recommend a combination of anthelmintic with hygiene measures for all members of the household
mebendazole is used first-line for children > 6 months old. A single dose is given unless infestation persists
Threadworm - Summary
The most common symptom is perianal itching, which is worse during the night.
Infestation may be symptomless and only detected when threadworms are seen on the
perianal skin or in the stools.There may be signs of scratching in the perianal area and
sometimes localized secondary bacterial infection. It is unusual to see worms in the
perianal area when the person is examined during the day.
If the diagnosis is uncertain, the adhesive tape test for eggs may be useful. Transparent
tape is applied to the perianal area first thing in the morning and then examined under a
microscope to detect threadworm eggs. Stool examination is not generally
recommended.
Other causes of perineal itch include dermatitis, candidal infection, and haemorrhoids.
Threadworms are rarely confused with other types of worm infestation because of
their different appearance.
Treatment is recommended if threadworms have been seen or eggs detected
treatment. All household members should be treated at the same time.
o For adults and children aged over 6 months, an anthelmintic (mebendazole)
combined with hygiene measures is recommended.
o For children aged 6 months and under, hygiene measures alone are recommended.
o For people who do not wish to take an anthelmintic, physical removal of the eggs,
combined with hygiene measures is recommended.
o For pregnant or breastfeeding women, physical removal of eggs combined with
hygiene methods is the preferred treatment. Treatment with mebendazole is
contraindicated in the first trimester of pregnancy; however, it can be considered in
the second or third trimester, or during breastfeeding if drug treatment is
considered necessary (off-label use).
If infestation persists after hygiene measures have been continued for the
recommended duration, a further course of drug treatment is recommended. It is
important that household members are treated and adhere to hygiene measures.
Question 3 of 80
What is the most likely outcome following the diagnosis of minimal change nephropathy in a 10-year-old male?
Chronic kidney disease requiring renal replacement therapy within 30 years
Next question
As 1/3 of patients have infrequent relapses and 1/3 of patients have frequent relapses a majority (2/3) will have later recurrent episodes. It
is important however to stress to patients that generally speaking the longer term prognosis in minimal change glomerulonephritis is good.
Pathophysiology
T-cell and cytokine mediated damage to the glomerular basement membrane polyanion loss
the resultant reduction of electrostatic charge increased glomerular permeability to serum albumin
Features
nephrotic syndrome
normotension - hypertension is rare
highly selective proteinuria*
renal biopsy: electron microscopy shows fusion of podocytes
Management
*only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus
Question 4 of 80
When assessing an 18-month-old child with diarrhoea and vomiting which one of the following signs is most likely to indicate shock?
Tachycardia
Sunken eyes
Next question
diarrhoea usually lasts for 5-7 days and stops within 2 weeks
vomiting usually lasts for 1-2 days and stops within 3 days
When assessing hydration status NICE advocate using normal, dehydrated or shocked categories rather than the traditional normal, mild,
moderate or severe categories.
Clinical dehydration
Clinical shock
Tachycardia
Tachypnoea
Weak peripheral pulses
Prolonged capillary refill time
Hypotension
jittery movements
increased muscle tone
hyperreflexia
convulsions
drowsiness or coma
Diagnosis
NICE suggest doing a stool culture in the following situations:
Management
If clinical shock is suspected children should be admitted for intravenous rehydration.
For children with no evidence of dehydration
If dehydration is suspected:
give 50 ml/kg low osmolarity oral rehydration solution (ORS) solution over 4 hours, plus ORS solution for maintenance, often
and in small amounts
continue breastfeeding
consider supplementing with usual fluids (including milk feeds or water, but not fruit juices or carbonated drinks)
Question 5 of 80
An 8-year-old boy is reviewed in the Enuresis clinic. He is still wetting the bed at night despite using an enuresis alarm for the past three
months. There are no problems with micturition during the daytime and he passes one soft stool everyday. Which one of the following
treatments is most likely to be offered?
Lactulose
Desmopressin
Imipramine
Duloxetine
Next question
Nocturnal enuresis
The majority of children achieve day and night time continence by 3 or 4 years of age. Enuresis may be defined as the 'involuntary
discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous
system or urinary tract'
Nocturnal enuresis can be defined as either primary (the child has never achieved continence) or secondary (the child has been dry for at
least 6 months before)
NICE issued guidance in 2010. Management:
look for possible underlying causes/triggers (e.g. Constipation, diabetes mellitus, UTI if recent onset)
advise on fluid intake, diet and toileting behaviour
reward systems (e.g. Star charts). NICE recommend these 'should be given for agreed behaviour rather than dry nights' e.g.
Using the toilet to pass urine before sleep
NICE advise: 'Consider whether alarm or drug treatment is appropriate, depending on the age, maturity and abilities of the child
or young person, the frequency of bedwetting and the motivation and needs of the family'. Generally:
an enuresis alarm is first-line for children under the age of 7 years
desmopressin may be used first-line for children over the ago 7 years, particularly if short-term control is needed or an enuresis
alarm has been ineffective/is not acceptable to the family
please see the link for more details
There are separate CKS topics on Constipation in children, Diabetes - type 1, Diabetes type 2, and Urinary tract infection - children.
The target audience for this CKS topic is healthcare professionals working within the
NHS in the UK, and providing first contact or primary health care.
How up-to-date is this topic?
Changes
Update
Goals and outcome measures
Goals
Background information
Definition
Causes
Prevalence
Risk factors
Prognosis
Impact
Assessment
Assessment of bedwetting (enuresis)
Assessment
When to consider child maltreatment
Investigations
Management
Scenario: Primary bedwetting (without daytime symptoms) : covers the management of
children with primary bedwetting without daytime symptoms in primary care.
Scenario: Primary bedwetting (with daytime symptoms) : covers the management of
children with daytime symptoms in primary care.
Scenario: Secondary bedwetting : covers the primary care management of children who
have previously been dry at night for 6 months and who start to wet the bed again.
Scenario: Primary bedwetting (without daytime symptoms)
Scenario: Primary bedwetting (with daytime symptoms)
Scenario: Secondary bedwetting
Prescribing information
Important aspects of prescribing information relevant to primary healthcare are covered
in this section specifically for the drugs recommended in this CKS topic. For further
information on contraindications, cautions, drug interactions, and adverse effects, see
the electronic Medicines Compendium (eMC) (http://medicines.org.uk/emc), or the
British National Formulary (BNF) (www.bnf.org).
Desmopressin prescribing issues
Evidence
Supporting evidence
Search strategy
References
MHRA (2007) Desmopressin nasal spray: removal of the primary nocturnal enuresis
(bedwetting) indication. Medicines and Healthcare products Regulatory Agency.
www.mhra.gov.uk [Free Full-text]
Montaldo, P., Tafuro, L., Rea, M. et al. (2012) Desmopressin and oxybutynin in
monosymptomatic nocturnal enuresis: a randomized, double-blind, placebo-controlled
trial and an assessment of predictive factors. BJU International 110(8(Pt B)), E381-E386.
[Abstract]
National Clinical Guideline Centre (2010) Nocturnal enuresis: the management of
bedwetting in children and young people. National Institute for Health and Care
Excellence. www.nice.org.uk [Free Full-text]
Nevus, T. (2009) Diagnosis and management of nocturnal enuresis. Current Opinion in
Pediatrics 21(2), 199-202. [Abstract]
NICE (2009) When to suspect child maltreatment (NICE guideline). . Clinical guideline 89.
National Institute for Health and Care Excellence. www.nice.org.uk [Free Full-text]
NICE (2012) Nocturnal enuresis: evidence update July 2012. A summary of selected new
evidence relevant to NICE clinical guideline 111 'The management of bedwetting in
children and young people' (2010). Evidence update 21National Institute for Health and
Care Excellence. www.evidence.nhs.uk [Free Full-text]
Perrin, N., Sayer, L. and While, A. (2013) The efficacy of alarm therapy versus
desmopressin therapy in the treatment of primary mono-symptomatic nocturnal
enuresis: a systematic review. Primary Health Care Research and Development (epub
ahead of print), . [Abstract]
Sapi, M.C., Vasconcelos, J.S., Silva, F.G. et al. (2009) Assessment of domestic violence
against children and adolescents with enuresis. Jornal de Pediatria 85(5), 433-437.
[Abstract]
van Dommelen, P., Kamphuis, M., van Leerdam, F. et al. (2009) The short- and long-term
effects of simple behavioral interventions for nocturnal enuresis in young children: a
randomized controlled trial. Journal of Pediatrics 154(5), 662-666. [Abstract]
Question 6 of 80
A 14-year-old attends surgery. She was diagnosed with having migraines three years ago and requests advice about options for treating an
acute attack. Which one of the following medications is it least suitable to recommend?
Aspirin
Paracetamol + prochlorperazine
Paracetamol + codeine
Ibuprofen
Paracetamol
Next question
Migraine: management
It should be noted that as a general rule 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor
antagonists are used in prophylaxis. NICE produced guidelines in 2012 on the management of headache, including migraines.
Acute treatment
first-line: offer combination therapy with an oral triptan and an NSAID, or an oral triptan and paracetamol
for young people aged 12-17 years consider a nasal triptan in preference to an oral triptan
if the above measures are not effective or not tolerated offer a non-oral preparation of metoclopramide* or prochlorperazine and
consider adding a non-oral NSAID or triptan
Prophylaxis
prophylaxis should be given if patients are experiencing 2 or more attacks per month. Modern treatment is effective in about
60% of patients.
NICE advise either topiramate or propranolol 'according to the person's preference, comorbidities and risk of adverse events'.
Propranolol should be used in preference to topiramate in women of child bearing age as it may be teratogenic and it can
reduce the effectiveness of hormonal contraceptives
if these measures fail NICE recommend 'a course of up to 10 sessions of acupuncture over 5-8 weeks' or gabapentin
NICE recommend: 'Advise people with migraine that riboflavin (400 mg once a day) may be effective in reducing migraine
frequency and intensity for some people'
for women with predictable menstrual migraine treatment NICE recommend either frovatriptan (2.5 mg twice a day) or
zolmitriptan (2.5 mg twice or three times a day) as a type of 'mini-prophylaxis'
pizotifen is no longer recommend. Adverse effects such as weight gain & drowsiness are common
*caution should be exercised with young patients as acute dystonic reactions may develop
Migraine - Summary
Migraine is a primary episodic headache disorder. It is characterized by episodic severe
headaches with associated symptoms such as photophobia, phonophobia, and nausea and
vomiting. The most common subtypes of migraine are migraine without aura and migraine with
aura.
Migraine is a complex condition, and the exact pathophysiological cause is not fully understood.
It has a significant genetic component, with about half of people with migraine having a firstdegree relative with the condition.
The prevalence of migraine differs between the sexes, being about three times more common in
women (18%, mean onset of age 18 years) than men (6%, mean onset of age 14 years).
In adults, migraine without aura is diagnosed when at least five attacks fulfil the following
criteria:
Headache lasts 472 hours.
At least two of the following characteristics are present: unilateral location, moderate or severe
pain intensity, pulsating quality, aggravation by routine physical activity.
At least one of the following is present: nausea or vomiting, photophobia or phonophobia.
In adults, migraine with aura (reported by about one third of people with migraine) is diagnosed
when:
There are two or more attacks with one or more symptoms of aura including visual or sensory
symptoms, or dysphasic speech disturbance.
Each individual symptom of aura lasts less than 60 minutes.
Other conditions may present with signs and symptoms similar to migraine. These may be an
alternative form of primary headache disorder (such a cluster headache or tension-type
headache), or a secondary cause, which may be serious and life-threatening.
A headache diary may be useful to identify potential triggers (such as stress, specific foods,
dehydration, missed meals), which can be managed as appropriate.
First-line treatment of acute migraine consists of combination therapy with an oral triptan and
analgesia (paracetamol or a nonsteroidal anti-inflammatory drug). An anti-emetic
(prochlorperazine, domperidone, or metoclopramide) may be added even in the absence of
nausea or vomiting. Monotherapy, if preferred, consists of an oral triptan, NSAID, aspirin, or
paracetamol.
When two or more triptans have been trialled unsuccessfully, or treatment is successful but
attacks are frequent, preventive treatment is often used. First-line preventive treatment
consists of topiramate or propranolol. If both of these have been trialled unsuccessfully or are
inappropriate, gabapentin or acupuncture can be tried.
Hospitalisation or urgent referral is only necessary if a serious cause of headache is suspected,
or if the person is in severe, uncontrolled status migrainosus (migraine lasting for more than 72
hours).
Have I got the right topic?
Age from 12 years onwards
This CKS topic is based on the guidance Headaches - Diagnosis and management of headaches in
young people and adults, published by the National Institute for Health and Clinical Excellence
(NICE) [NICE, 2012], and Guidelines for all healthcare professionals in the diagnosis and
Management
Scenario: Adults : covers the management of people aged 18 years and older with migraine.
Scenario: Young people aged 1217 years : covers the management of young people aged
between 12 and 17 years of age with migraine.
Scenario: Pregnant or breastfeeding women : covers the management of pregnant or
breastfeeding women with migraine.
Scenario: Adults
Scenario: Young people aged 1217 years
Scenario: Pregnant or breastfeeding women
Prescribing information
Important aspects of prescribing information relevant to primary healthcare are covered in this
section specifically for the drugs recommended in this CKS topic. For further information on
contraindications, cautions, drug interactions, and adverse effects, see the electronic Medicines
Compendium (eMC) (http://medicines.org.uk/emc), or the British National Formulary (BNF)
(www.bnf.org).
Standard analgesics, NSAIDs, and anti-emetic drugs
Triptans (5-hydoxytryptamine agonists)
Drugs for the prevention of migraine
Evidence
Supporting evidence
Search strategy
References
ABPI Medicines Compendium (2011a) Summary of product characteristics for Boots Dispersible
Aspirin Tablets BP 75mg. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk
ABPI Medicines Compendium (2011b) Summary of product characteristics for half inderal LA
80mg. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2012) Summary of product characteristics for propranolol tablets
BP 40mg. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013a) Summary of product characteristics for Ponstan Forte
tablets 500mg. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013b) Summary of product characteristics for Zomig tablets
2.5mg. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013c) Summary of product characteristics for Imigran 10mg and
20mg nasal spray. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013d) Summary of product characteristics for Neurontin 100mg
Hard Capsules. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013e) Summary of product characteristics for Imigran tablets
50mg Imigran tablets 100mg. Electronic Medicines CompendiumDatapharm Communications
Ltd. www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2013f) Summary of product characteristics for Topamax 25 mg,
50mg, 100mg, 200mg tablets and sprinkle capsules 15, 25 or 50 mg. Electronic Medicines
CompendiumDatapharm Communications Ltd. www.medicines.org.uk [Free Full-text]
Ashcroft, D.M. and Millson, D. (2004) Naratriptan for the treatment of acute migraine: metaanalysis of randomised controlled trials. Pharmacoepidemiology and Drug Safety 13(2), 73-82.
[Abstract]
Aube, M. (1999) Migraine in pregnancy. Neurology 53(4 Suppl 1), S26-S28. [Abstract]
Azzopardi, T.D. and Brooks, N.A. (2008) Oral metoclopramide as an adjunct to analgesics for the
outpatient treatment of acute migraine. Annals of Pharmacotherapy 42(3), 397-402. [Abstract]
Bandolier (2002) Migraine special issue. Bandolier. www.medicine.ox.ac.uk/bandolier [Free Fulltext]
BASH (2010) Guidelines for all healthcare professionals in the diagnosis and management of
migraine, tension-type headache, cluster headache, medication-overuse headache. . 3rd edn.
British Association for the Study of Headache. www.bash.org.uk [Free Full-text]
Bates, D., Ashford, E., Dawson, R. et al. (1994) Subcutaneous sumatriptan during the migraine
aura. Sumatriptan Aura Study Group. Neurology 44(9), 1587-1592.
Baxter, K. and Preston, C.L. (Eds.) (2013) Stockley's drug interactions 2013: pocket companion.
London: Pharmaceutical Press.
BMA, NHS Employers and DH (2007) 2006/7 UK general practice workload survey. British
Medical Association. www.ic.nhs.uk [Free Full-text]
BNF 65 (2013) British National Formulary. 65th edn. London: British Medical Association and
Royal Pharmaceutical Society of Great Britain.
Brandes, J.L. (2006) The influence of estrogen on migraine: a systematic review. Journal of the
American Medical Association 295(15), 1824-1830. [Abstract] [Free Full-text]
Campbell, J.K., Penzien, D.B. and Wall, E.M. (2000) Evidence-based guidelines for migraine
headache: behavioral and physical treatments. American Academy of Neurology. www.aan.com
[Free Full-text]
Chen, L.C. and Ashcroft, D.M. (2007) Meta-analysis examining the efficacy and safety of
almotriptan in the acute treatment of migraine. Headache 47(8), 1169-1177. [Abstract]
Chen, L.C. and Ashcroft, D.M. (2008) Meta-analysis of the efficacy and safety of zolmitriptan in
the acute treatment of migraine. Headache 48(2), 236-247. [Abstract]
Colman, I., Brown, M.D., Innes, G.D. et al. (2004) Parenteral metoclopramide for acute migraine:
meta-analysis of randomised controlled trials. British Medical Journal 329(7479), 1369-1373.
[Abstract] [Free Full-text]
Colman, I., Friedman, B.W., Brown, M.D. et al. (2008) Parenteral dexamethasone for acute
severe migraine headache: meta-analysis of randomised controlled trials for preventing
recurrence. British Medical Journal 336(7657), 1359-1361. [Abstract] [Free Full-text]
CSM (2005) Updated advice on the safety of selective COX-2 inhibitors. Committee on Safety of
Medicines. www.mhra.gov.uk [Free Full-text]
Cutrer, F.M., Goadsby, P.J., Ferrari, M.D. et al. (2004) Priorities for triptan treatment attributes
and the implications for selecting an oral triptan for acute migraine: a study of US primary care
physicians (the TRIPSTAR project). Clinical Therapeutics 26(9), 1533-1545. [Abstract]
Damen, L., Bruijn, J.K., Verhagen, A.P. et al. (2005) Symptomatic treatment of migraine in
children: a systematic review of medication trials. Pediatrics 116(2), e295-e302. [Abstract] [Free
Full-text]
Derry, C.J., Derry, S. and Moore, R.A. (2012a) Sumatriptan (intranasal route of administration)
for acute migraine attacks in adults (Cochrane review). The Cochrane Library. Issue 2. John Wiley
& Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
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administration) for acute migraine attacks in adults (Cochrane review). The Cochrane Library.
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Question 7 of 80
Parvovirus B19
Cytomegalovirus
Next question
Roseola infantum
Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human
herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years.
Features
aseptic meningitis
hepatitis
Question 8 of 80
Which one of the following is not a risk factor for developmental dysplasia of the hip?
Positive family history
Breech presentation
Female sex
Afro-Caribbean origin
Firstborn child
Next question
DDH is slightly more common in the left hip. Around 20% of cases are bilateral.
Clinical examination is made using the Barlow and Ortolani tests:
Question 9 of 80
What percentage of male term infants are found to have an undescended testicle on newborn examination?
0.1-0.3%
0.5-1%
2-4%
6-8%
10-14%
Next question
Undescended testis
Undescended testis occurs in around 2-4% of term male infants., but is much more common if the baby is preterm. Around 25% of cases
are bilateral
Complications of undescended testis
infertility
torsion
testicular cancer
psychological
Management
orchidopexy: referral should be considered from around 6 months of age. Surgical practices vary although the majority of
procedures are performed at around 1 year of age
Question 10 of 80
You are reviewing a 11-month-old baby with a viral upper respiratory tract infection. She is clinically well but at the end of the consultation
her mother asks you about her development. You notice that she points and babbles 'mama' and 'dada' but has no other words. She is shy
and cries when you try to examine her. There is an early pincer grip and she can roll from front to back but she cannot yet sit without
support. How would you describe her development?
Normal development
Next question
Most babies can sit without support at 7-8 months so this probably represents a delay in gross motor skills. If still present at 12 months she
should be considered for referral to a paediatrician. The other development features are normal for her age.
Milestone
3 months
6 months
7-8 months
9 months
Pulls to standing
Crawls
12 months
Cruises
Walks with one hand held
2 years
Runs
Walks upstairs and downstairs holding on to rail
3 years
4 years
Notes
the majority of children crawl on all fours before walking but some children 'bottom-shuffle'. This is a normal variant and runs in
families
Question 10 of 80
You are reviewing a 11-month-old baby with a viral upper respiratory tract infection. She is clinically well but at the end of the consultation
her mother asks you about her development. You notice that she points and babbles 'mama' and 'dada' but has no other words. She is shy
and cries when you try to examine her. There is an early pincer grip and she can roll from front to back but she cannot yet sit without
support. How would you describe her development?
Normal development
Next question
Most babies can sit without support at 7-8 months so this probably represents a delay in gross motor skills. If still present at 12 months she
should be considered for referral to a paediatrician. The other development features are normal for her age.
Milestone
3 months
6 months
7-8 months
9 months
Pulls to standing
Crawls
12 months
Cruises
Walks with one hand held
2 years
Runs
Walks upstairs and downstairs holding on to rail
Age
Milestone
3 years
4 years
Notes
the majority of children crawl on all fours before walking but some children 'bottom-shuffle'. This is a normal variant and runs in
families
Question 11 of 80
A newborn infant is noted to have posterior displacement of the tongue and a clef palate. What is the most likely diagnosis?
Pierre-Robin syndrome
Patau syndrome
Edward's syndrome
Noonan syndrome
William's syndrome
Childhood syndromes
Key features
Micrognathia
Low-set ears
Edward's syndrome (trisomy 18)
Rocker bottom feet
Overlapping of fingers
Fragile X
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
Noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
Next question
Syndrome
Key features
Pierre-Robin syndrome*
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
Prader-Willi syndrome
Hypotonia
Hypogonadism
Obesity
William's syndrome
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
*this condition has many similarities with Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome
is autosomal dominant so there is usually a family history of similar problems
Question 12 of 80
An oral rotavirus vaccine has recently been introduced into the NHS immunisation schedule. When should it be given?
2 months + 4 months
2 months + 3 months
3 + 4 months
Next question
Rotavirus vaccine
Rotavirus is a major public health problem, accounting for significant morbidity and hospital admissions in the developed world and
childhood mortality in the developing world.
A vaccine was introduced into the NHS immunisation programme in 2013. The key points to remember as as follows:
Other points
the vaccine is around 85-90% effective and is predicted to decrease hospitalisation by 70%
Question 13 of 80
A 9-year-old girl is brought to surgery due to persistent leg pains. Which one of the following would not be consistent with a diagnosis of
'growing pains'?
Present upon waking in the morning
Bilateral symptoms
Intermittent symptoms
Next question
Growing pains
A common presentation in General Practice is a child complaining of pain in the legs with no obvious cause. Such presentations, in the
absence of any worrying features, are often attributed to 'growing pains'. This is a misnomer as the pains are often not related to growth the current term used in rheumatology is 'benign idiopathic nocturnal limb pains of childhood'
Growing pains are equally common in boys and girls and occur in the age range of 3-12 years.
Features of growing pains
never present at the start of the day after the child has woken
no limp
no limitation of physical activity
systemically well
normal physical examination
motor milestones normal
symptoms are often intermittent and worse after a day of vigorous activity
Question 13 of 80
A 9-year-old girl is brought to surgery due to persistent leg pains. Which one of the following would not be consistent with a diagnosis of
'growing pains'?
Present upon waking in the morning
Bilateral symptoms
Intermittent symptoms
Next question
Growing pains
A common presentation in General Practice is a child complaining of pain in the legs with no obvious cause. Such presentations, in the
absence of any worrying features, are often attributed to 'growing pains'. This is a misnomer as the pains are often not related to growth the current term used in rheumatology is 'benign idiopathic nocturnal limb pains of childhood'
Growing pains are equally common in boys and girls and occur in the age range of 3-12 years.
Features of growing pains
never present at the start of the day after the child has woken
no limp
no limitation of physical activity
systemically well
normal physical examination
motor milestones normal
symptoms are often intermittent and worse after a day of vigorous activity
Question 14 of 80
A 9-year-old boy is brought to surgery as his asthma has been getting worse over the past 2 days. His mother is concerned that
his breathing is getting worse and not responding to inhaled salbutamol as normal. Given the overall clinical context, which one of
the following is most consistent with a life-threatening asthma attack in this particular patient?
SpO2 of 94%
Next question
Quiet breath sounds in a child with an asthma exacerbation is a worrying feature. Children with asthma normally have an obvious
bilateral wheeze - the absence of this may suggest a life-threatening asthma attack.
The 2014 BTS/SIGN guidelines suggest the following criteria are used to assess the severity of asthma in general practice:
Children between 2 and 5 years of age
Moderate attack
Severe attack
Life-threatening attack
SpO2 <92%
SpO2 < 92%
Silent chest
Too breathless to talk or feed
SpO2 > 92%
Poor respiratory effort
Heart rate > 140/min
No clinical features of severe asthma
Agitation
Respiratory rate > 40/min
Altered consciousness
Use of accessory neck muscles
Cyanosis
Severe attack
Life-threatening attack
How many doses of tetanus vaccine should a child receive as part of the routine UK immunisation schedule?
None
Next question
Tetanus: vaccination
The tetanus vaccine is a cell-free purified toxin that is normally given as part of a combined vaccine.
Tetanus vaccine is currently given in the UK as part of the routine immunisation schedule at:
2 months
3 months
4 months
3-5 years
13-18 years
This therefore provides 5 doses of tetanus-containing vaccine. Five doses is now considered to provide adequate long-term protection
against tetanus.
Intramuscular human tetanus immunoglobulin should be given to patients with high-risk wounds (e.g. Compound fractures, delayed surgical
intervention, significant degree of devitalised tissue) irrespective of whether 5 doses of tetanus vaccine have previously been given
If vaccination history is incomplete or unknown then a dose of tetanus vaccine should be given combined with intramuscular human tetanus
immunoglobulin for high-risk wounds
Question 16 of 80
Which one of the following conditions is not associated with obesity in children?
Down's syndrome
Hypothyroidism
Cushing's syndrome
Prader-Willi syndrome
Next question
Obesity in children
Defining obesity is more difficult in children than adults as body mass index (BMI) varies with age. BMI percentile charts are therefore
needed to make an accurate assessment. Recent NICE guidelines suggest to use 'UK 1990 BMI charts to give age- and gender-specific
information'
NICE recommend
By far the most common cause of obesity in childhood is lifestyle factors. Other associations of obesity in children include:
Asian children: four times more likely to be obese than white children
female children
taller children: children with obesity are often above the 50th percentile in height
Cushing's syndrome
Prader-Willi syndrome
orthopaedic problems: slipped upper femoral epiphyses, Blount's disease (a development abnormality of the tibia resulting in
bowing of the legs), musculoskeletal pains
psychological consequences: poor self-esteem, bullying
sleep apnoea
benign intracranial hypertension
long-term consequences: increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease
Question 17 of 80
11 years
12 years
13 years
14 years
Next question
Puberty
Males
first sign is testicular growth at around 12 years of age (range = 10-15 years)
testicular volume > 4 ml indicates onset of puberty
maximum height spurt at 14
Females
first sign is breast development at around 11.5 years of age (range = 9-13 years)
height spurt reaches its maximum early in puberty (at 12) , before menarche
menarche at 13 (11-15)
there is an increase of only about 4% of height following menarche
Question 18 of 80
A 3-year-old boy from a Turkish family is referred to the local paediatric unit due to recurrent lethargy and pallor. His parents report no other
symptoms such as fever, pain or poor feeding. He had been treated with a course of ciprofloxacin for otitis externa two weeks ago.
Admission bloods show:
Hb
5.2 g/dl
WBC
10.7 *109/l
Platelets
346 *109/l
Reticulocytes
5%
Beta-thalassaemia major
Next question
G6PD deficiency
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest red blood cell enzyme defect. It is more common in people from
the Mediterranean and Africa and is inherited in a X-linked recessive fashion. Many drugs can precipitate a crisis as well as infections and
broad (fava) beans
Pathophysiology
Features
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
penicillins
cephalosporins
macrolides
tetracyclines
trimethoprim
Hereditary spherocytosis
Gender
Ethnicity
Neonatal jaundice
Neonatal jaundice
Chronic symptoms although haemolytic crises may be precipitated by infection
Typical history Infection/drugs precipitate haemolysis
Gallstones
Gallstones
Splenomegaly is common
Blood film
Heinz bodies
Question 19 of 80
The EEG characteristically shows a bilateral, symmetrical 3Hz spike and wave pattern
Next question
Absence seizures
Absence seizures (petit mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old
and girls are affected twice as commonly as boys
Features
absences last a few seconds and are associated with a quick recovery
seizures may be provoked by hyperventilation or stress
the child is usually unaware of the seizure
they may occur many times a day
EEG: bilateral, symmetrical 3Hz spike and wave pattern
Management
Question 20 of 80
Which one of the following is least associated with fetal varicella syndrome?
Limb hypoplasia
Microphthalmia
Sensorineural deafness
Skin scarring
Microcephaly
Next question
risk of FVS following maternal varicella exposure is around 1% if occurs before 20 weeks gestation
studies have shown a very small number of cases occurring between 20-28 weeks gestation and none following 28 weeks
features of FVS include skin scarring, eye defects (microphthalmia), limb hypoplasia, microcephaly and learning disabilities
if there is any doubt about the mother previously having chickenpox maternal blood should be checked for varicella antibodies
if the pregnant women is not immune to varicella she should be given varicella zoster immunoglobulin (VZIG) as soon as
possible. RCOG and Greenbook guidelines suggest VZIG is effective up to 10 days post exposure
consensus guidelines suggest oral aciclovir should be given if pregnant women with chickenpox present within 24 hours of
onset of the rash
Question 21 of 80
Which one of the following is not part of the core Child Health Promotion Program as outlined in the Children's National Service
Framework?
Next question
The routine surveillance reviews at 8 months, 2 years and 3-4 years have now being stopped. However, if a child is deemed 'at
risk' more frequent reviews are advisable
The following table gives a basic outline of child health surveillance in the UK
Antenatal
Newborn
First month
Heel-prick test day 5-9 - hypothyroidism, PKU, metabolic diseases, cystic fibrosis, medium-chain acyl Co-A
dehydrogenase deficiency (MCADD)
Midwife visit up to 4 weeks*
Following
months
Pre school
Ongoing
*this doesn't seem to happen in practice with health visitors usually taking over at 2 weeks
Question 22 of 80
Which one of the following is not a risk factor for the development of glue ear?
Day care attendance
Parental smoking
Female sex
Bottle feeding
Family history
Next question
Glue ear
Glue ear describes otitis media with an effusion (other terms include serous otitis media). It is common with the majority of children having
at least one episode during childhood
Risk factors
male sex
siblings with glue ear
higher incidence in Winter and Spring
bottle feeding
day care attendance
parental smoking
Features
grommet insertion - to allow air to pass through into the middle ear and hence do the job normally done by the Eustachian tube.
The majority stop functioning after about 10 months
adenoidectomy
Question 23 of 80
Children are infectious once rash begins until all lesions have scabbed over
Next question
Chickenpox
Chickenpox is caused by primary infection with varicella zoster virus. Shingles is reactivation of dormant virus in dorsal root ganglion
Chickenpox is highly infectious
fever initially
itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
systemic upset is usually mild
Management is supportive
A common complication is secondary bacterial infection of the lesions. Rare complications include
pneumonia
encephalitis (cerebellar involvement may be seen)
disseminated haemorrhagic chickenpox
arthritis, nephritis and pancreatitis may very rarely be seen
*it was traditionally taught that patients were infective until all lesions had scabbed over
Question 24 of 80
A 9-month-old girl is generally unwell with a low-grade pyrexia, oral ulcers and the following appearance of her feet:
Which one of the following is most likely to be responsible for this presentation?
Measles
Parvovirus B19
Vasculitis
Coxsackie A16
Next question
Management
*The HPA recommends that children who are unwell should be kept off school until they feel better. They also advise that you contact them
if you suspect that there may be a large outbreak.
Question 25 of 80
Meningococcus
Rabies
Oral polio
Diphtheria
Next question
BCG
MMR
oral polio
yellow fever
oral typhoid
Vaccinations
It is important to be aware of vaccines which are of the live-attenuated type as these may pose a risk to immunocompromised patients. The
main types of vaccine are as follows:
Live attenuated
BCG
measles, mumps, rubella (MMR)
influenza (intranasal)
oral rotavirus
oral polio
yellow fever
oral typhoid*
Inactivated preparations
rabies
influenza (intramuscular)
Detoxified exotoxins
tetanus
diphtheria
pertussis ('acellular' vaccine)
hepatitis B
meningococcus, pneumococcus, haemophilus
Notes
influenza: different types are available, including whole inactivated virus, split virion (virus particles disrupted by detergent
treatment) and sub-unit (mainly haemagglutinin and neuraminidase)
cholera: contains inactivated Inaba and Ogawa strains of Vibrio cholerae together with recombinant B-subunit of the cholera
toxin
hepatitis B: contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant
DNA technology
Which one of the following is the most likely result if a fetus is homozygous for alpha-thalassaemia?
Anencephalic fetus
Normal pregnancy
Prematurity
Hydrops fetalis
Macrosomia
Next question
Alpha-thalassaemia
Alpha-thalassaemia is due to a deficiency of alpha chains in haemoglobin
Overview
Question 27 of 80
Ehler-Danlos syndrome
Homocystinuria
Kallman's syndrome
Next question
Tall stature
Causes of tall stature
constitutional
excessive growth hormone (pituitary gigantism)
hyperthyroidism
Marfan syndrome
homocystinuria
Klinefelter's syndrome
Congenital adrenal hyperplasia may be associated with a premature growth spurt but early fusion of the epiphyses results in short stature
Question 28 of 80
The parents of a 9-year-old child come for advice regarding malaria prophylaxis. They are of Indian origin and are due to go back to their
own country for a wedding. Which one of the following is contraindicated?
Mefloquine
Doxycycline
Proguanil + chloroquine
Next question
Malaria: prophylaxis
There are around 1,500-2,000 cases each year of malaria in patients returning from endemic countries. The majority of these cases
(around 75%) are caused by the potentially fatal Plasmodium falciparum protozoa. The majority of patients who develop malaria did not
take prophylaxis. It should also be remembered that UK citizens who originate from malaria endemic areas quickly lose their innate
immunity.
Up-to-date charts with recommended regimes for malarial zones should be consulted prior to prescribing
Drug
Side-effects + notes
7 days
1 week
4 weeks
1 - 2 days
4 weeks
Headache
Chloroquine
Contraindicated in epilepsy
Taken weekly
Doxycycline
Photosensitivity
Oesophagitis
Mefloquine (Lariam)
Dizziness
Neuropsychiatric disturbance
2 - 3 weeks
Contraindicated in epilepsy
Taken weekly
Proguanil (Paludrine)
Proguanil + chloroquine
See above
4 weeks
1 week
4 weeks
1 week
4 weeks
Pregnant women should be advised to avoid travelling to regions where malaria is endemic. Diagnosis can also be difficult as parasites
may not be detectable in the blood film due to placental sequestration. However, if travel cannot be avoided:
It is again advisable to avoid travel to malaria endemic regions with children if avoidable. However, if travel is essential then children should
take malarial prophylaxis as they are more at risk of serious complications.
Question 29 of 80
A newborn female baby is noted to have a clicky left hip during the routine newborn examination. What is the most appropriate
investigation?
X-ray
Urine dipstick
Ultrasound
MRI
Next question
DDH is slightly more common in the left hip. Around 20% of cases are bilateral.
Clinical examination is made using the Barlow and Ortolani tests:
Question 30 of 80
The mother of a 4-year-old boy comes to surgery as she is concerned he is still wetting the bed at night. This is in contrast to his older
brother who was dry at night by the age of 3 years. She is wondering if there is any treatment you can offer. What is the most appropriate
management?
Trial of oral desmopressin
Enuresis alarm
Next question
Nocturnal bedwetting is still very common at 4 years and the mother should be reassured
Nocturnal enuresis
The majority of children achieve day and night time continence by 3 or 4 years of age. Enuresis may be defined as the 'involuntary
discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous
system or urinary tract'
Nocturnal enuresis can be defined as either primary (the child has never achieved continence) or secondary (the child has been dry for at
least 6 months before)
NICE issued guidance in 2010. Management:
look for possible underlying causes/triggers (e.g. Constipation, diabetes mellitus, UTI if recent onset)
advise on fluid intake, diet and toileting behaviour
reward systems (e.g. Star charts). NICE recommend these 'should be given for agreed behaviour rather than dry nights' e.g.
Using the toilet to pass urine before sleep
NICE advise: 'Consider whether alarm or drug treatment is appropriate, depending on the age, maturity and abilities of the child
or young person, the frequency of bedwetting and the motivation and needs of the family'. Generally:
an enuresis alarm is first-line for children under the age of 7 years
desmopressin may be used first-line for children over the ago 7 years, particularly if short-term control is needed or an enuresis
alarm has been ineffective/is not acceptable to the family
please see the link for more details
Question 31 of 80
A 12-month-old boy is brought to surgery as mum is concerned about his eyesight. On examination the red reflex is present bilaterally and
no squint is seen. Which one of the following is least associated with visual problems in childhood?
Cerebral palsy
Albinism
Hydrocephalus
Prematurity
Prader-Willi syndrome
Next question
Question 32 of 80
A 5-year-old boy is brought to the surgery with chickenpox. His mother wants advice regarding school exclusion. What is the most
appropriate advice to give?
Should be excluded until 2 days after all lesions have scabbed over
Should be excluded until 5 days after all lesions have scabbed over
Next question
Chickenpox
Chickenpox is caused by primary infection with varicella zoster virus. Shingles is reactivation of dormant virus in dorsal root ganglion
fever initially
itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
systemic upset is usually mild
Management is supportive
A common complication is secondary bacterial infection of the lesions. Rare complications include
pneumonia
encephalitis (cerebellar involvement may be seen)
disseminated haemorrhagic chickenpox
arthritis, nephritis and pancreatitis may very rarely be seen
*it was traditionally taught that patients were infective until all lesions had scabbed over
Question 33 of 80
A 2-day-old baby girl is noted to become cyanotic whilst feeding and crying. A diagnosis of congenital heart disease is suspected. What is
the most likely cause?
Transposition of the great arteries
Tetralogy of Fallot
Next question
The key point to this question is that whilst tetralogy of Fallot is more common than transposition of the great arteries (TGA), Fallot's doesn't
usually present until 1-2 months following the identification of a murmur. In the neonate, TGA is the most common presenting cause of
cyanotic congenital heart disease
The other 3 options are causes of acyanotic congenital heart disease
VSDs are more common than ASDs. However, in adult patients ASDs are the more common new diagnosis as they generally presents
later
Cyanotic - most common causes
tetralogy of Fallot
transposition of the great arteries (TGA)
tricuspid atresia
pulmonary valve stenosis
Fallot's is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot's generally presenting at
around 1-2 months
Question 34 of 80
A 3-year-old boy is investigated for lethargy. Examination is unremarkable with a blood pressure of 90/46 mmHg (normal for his age). Blood
tests reveal:
Na+
140 mmol/l
K+
2.6 mmol/l
Bicarbonate
33 mmol/l
Urea
4.2 mmol/l
Creatinine
91 mol/l
Conn's syndrome
Bartter's syndrome
Liddle's syndrome
Next question
Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the
Na+ K+ 2Cl- cotransporter in the ascending loop of Henle
Cushing's syndrome
Conn's syndrome (primary hyperaldosteronism)
Liddle's syndrome
11-beta hydroxylase deficiency*
Carbenoxolone, an anti-ulcer drug, and liquorice excess can potentially cause hypokalaemia associated with hypertension
Hypokalaemia without hypertension
diuretics
GI loss (e.g. Diarrhoea, vomiting)
renal tubular acidosis (type 1 and 2**)
Bartter's syndrome
Gitelman syndrome
*21-hydroxylase deficiency, which accounts for 90% of congenital adrenal hyperplasia cases, is not associated with hypertension
**type 4 renal tubular acidosis is associated with hyperkalaemia
Question 35 of 80
You are contacted by the health visitor who is concerned about the head growth of a 4-month-old boy. His head growth has crossed from
the 50th to the 91st centile in the past 2 months. Which one of the following is least likely to be the cause?
Neurofibromatosis
Subdural effusion
Hydrocephalus
Next question
Macrocephaly
Causes of macrocephaly in children include:
normal variant
chronic hydrocephalus
chronic subdural effusion
neurofibromatosis
gigantism (e.g. Soto's syndrome)
metabolic storage diseases
bone problems e.g. thalassaemia
Question 36 of 80
Seizures
Eczema
Recurrent infections
'Musty' urine
Next question
Phenylketonuria
Phenylketonuria (PKU) is an autosomal recessive condition caused by a disorder of phenylalanine metabolism. This is usually due to defect
in phenylalanine hydroxylase, an enzyme which converts phenylalanine to tyrosine. In a small number of cases the underlying defect is a
deficiency of the tetrahydrobiopterin-deficient cofactor, e.g. secondary to defective dihydrobiopterin reductase. High levels of phenylalanine
lead to problems such as learning difficulties and seizures. The gene for phenylalanine hydroxylase is located on chromosome 12. The
incidence of PKU is around 1 in 10,000 live births.
Features
Diagnosis
Guthrie test: the 'heel-prick' test done at 5-9 days of life - also looks for other biochemical disorders such as hypothyroidism
hyperphenylalaninaemia
phenylpyruvic acid in urine
Management
Question 37 of 80
At what age would the average child acquire the ability to take off their socks?
3 months
6 months
9 months
12 months
18 months
Next question
Milestone
Laughs
Enjoys friendly handling
6 monthsNot shy
9 months
Shy
Takes everything to mouth
Feeding
Age
Milestone
6 months
Drinks from cup + uses spoon, develops over 3 month period 12 -15 months
Competent with spoon, doesn't spill with cup
2 years
3 years
5 years
Dressing
Age
Milestone
12-15 months
18 months
2 years
Can dress and undress independently except for laces and buttons 4 years
Play
Age
Milestone
Plays 'peek-a-boo'
9 months
Waves 'bye-bye'
Plays 'pat-a-cake'
12 months
18 months
4 years
Question 38 of 80
A 1-year-old girl is brought to surgery due to a high fever. She has a past history of febrile convulsions. Whilst examining her she starts to
fit. You carry her to the treatment room and apply oxygen. After 5 minutes she continues to fit. What is the most appropriate next step?
Call 999 for an ambulance
Next question
BNF recommend dose for rectal diazepam, repeated once after 10-15 minutes if necessary
Neonate
1.25 - 2.5 mg
10 - 20 mg (max. 30 mg)
Neonate
1.25 - 2.5 mg
Elderly
10 mg (max. 15 mg)
Question 39 of 80
Which one of the following is the most important factor in determining fetal growth?
Age of mother
Placental factors
Hormonal factors
Genetic factors
Next question
Growth
Factors which affect fetal growth are as follows:
environmental: this is the most important factor affecting fetal growth e.g. maternal nutrition and uterine capacity
placental
hormonal
genetic: predominately maternal
Following birth genetic factors are the most important determinant of final adult height
Monitoring of growth
Question 40 of 80
Which one of the following statements regarding the UK-WHO growth charts is true?
They only include data from UK children
Next question
Growth charts
The UK has recently switched to the new growth charts based on the WHO growth standard for children under the age of 5 years. The new
UK-WHO charts have a separate preterm section and a 0-1 year section.
Key points
based on data from breast fed infants and all ethnic groups
the data matches UK children well for height and length but after 6 months UK children and slightly more heavy and more likel y
to be above the 98% centile
preterm infants born at 32-36 weeks have a separate chart until 2 weeks post-term
Please see the comprehensive review by Wright CM et al. BMJ 2010; 340:c1140 for more information
Question 41 of 80
A mother brings her 13-year-old son in who has developed acne. On both his face and back he has a number of inflammatory lesions
consistent with moderate acne. Which one of the following is the most appropriate guidance when prescribing oral tetracyclines?
Patients must be 11 years or older
Next question
mild: open and closed comedones with or without sparse inflammatory lesions
moderate acne: widespread non-inflammatory lesions and numerous papules and pustules
severe acne: extensive inflammatory lesions, which may include nodules, pitting, and scarring
A simple step-up management scheme often used in the treatment of acne is as follows:
Question 42 of 80
Learning difficulties
Joint laxity
Next question
Fragile X
Fragile X is a trinucleotide repeat disorder
Features in males
learning difficulties
large low set ears, long thin face, high arched palate
macroorchidism
hypotonia
autism is more common
mitral valve prolapse
Features in females (who have one fragile chromosome and one normal X chromosome) range from normal to mild
Diagnosis
Question 43 of 80
At what age would the average child acquire the ability to crawl?
6 months
9 months
12 months
18 months
2 years
Next question
Milestone
3 months
6 months
7-8 months
9 months
Pulls to standing
Crawls
12 months
Cruises
Walks with one hand held
2 years
Runs
Walks upstairs and downstairs holding on to rail
3 years
4 years
Notes
the majority of children crawl on all fours before walking but some children 'bottom-shuffle'. This is a normal variant and runs in
families
Question 43 of 80
At what age would the average child acquire the ability to crawl?
6 months
9 months
12 months
18 months
2 years
Next question
Milestone
3 months
6 months
7-8 months
9 months
Pulls to standing
Crawls
12 months
Cruises
Walks with one hand held
2 years
Runs
Walks upstairs and downstairs holding on to rail
3 years
Age
Milestone
Walks up stairs without holding on to rail
4 years
Notes
the majority of children crawl on all fours before walking but some children 'bottom-shuffle'. This is a normal variant and runs in
families
Question 44 of 80
A 4-year-old boy is admitted after developing a haemarthrosis in his right knee whilst playing in the garden. The following blood results are
obtained:
Platelets
220 * 109/l
PT
12 secs
APTT
78 secs
Factor VIIIc
activity
Normal
Antiphospholipid syndrome
Haemophilia A
Haemophilia B
Next question
A grossly elevated APTT may be caused by heparin therapy, haemophilia or antiphospholipid syndrome. A normal factor VIIIc activity
points to a diagnosis of haemophilia B (lack of factor IX). Antiphospholipid syndrome is a prothrombotic condition
Haemophilia
Haemophilia is a X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition. Haemophilia A is
due to a deficiency of factor VIII whilst in haemophilia B (Christmas disease) there is a lack of factor IX
Features
haemoarthroses, haematomas
prolonged bleeding after surgery or trauma
Blood tests
prolonged APTT
bleeding time, thrombin time, prothrombin time normal
Question 45 of 80
A 4-year-old girl is brought into the Emergency Department after falling down some stairs at home. You have been asked by the Consultant
to make an initial assessment of her. On examination her eyes are open spontaneously but she is crying and is inconsolable at times. She
is repeatedly moaning 'mummy' but does not appear agitated. She is not moving spontaneously but does withdraw her hand when you
touch it saying 'ow'. What is her paediatric Glasgow Coma Scale score?
10
11
12
13
14
Next question
Modality
Options
Modality
Options
2. Inconsolable, agitated.
1. No verbal response.
Eye opening
Glasgow coma scale (GCS) scores are generally expressed in the following format 'GCS = 13, M5 V4 E4 at 21:30'.
Question 46 of 80
Cephalosporins
Aspirin
Tricyclic antidepressants
Chlorpromazine
Next question
galactosaemia
viral infections - this is controversial with respect to HIV in the developing world. This is because there is such an increased
infant mortality and morbidity associated with bottle feeding that some doctors think the benefits outweigh the risk of HIV
transmission
Drug contraindications
The following drugs can be given to mothers who are breast feeding:
*the BNF advises that the amount is too small to affect neonatal hypothyroidism screening
**clozapine should be avoided
Question 47 of 80
A mother requests a home visit for her 12-year-old son who is too poorly to come to surgery, On arrival the boy is noted to be pyrexial, have
cool peripheries and purpura on his legs. What action should be taken?
IM benzylpenicillin 150mg
IM benzylpenicillin 300mg
IM benzylpenicillin 600mg
IM benzylpenicillin 900mg
IM benzylpenicillin 1200mg
Next question
The RCGP have previously fed back that doctors are expected to be familiar with emergency drug doses, and have mentioned suspected
meningococcal septicaemia in particular
Dose
< 1 year
300 mg
1 - 10 years600 mg
> 10 years 1200 mg
Question 48 of 80
Next question
Infantile spasms
Infantile spasms, or West syndrome, is a type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more
common in male infants. They are often associated with a serious underlying condition and carry a poor prognosis
Features
characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms
this lasts only 1-2 seconds but may be repeated up to 50 times
progressive mental handicap
Investigation
Management
poor prognosis
vigabatrin is now considered first-line therapy
ACTH is also used
Question 49 of 80
Next question
Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe hypokalaemia due to defective chloride absorption at the
Na+ K+ 2Cl- cotransporter in the ascending loop of Henle. It should be noted that it is associated with normotension (unlike other endocrine
causes of hypokalaemia such as Conn's, Cushing's and Liddle's syndrome which are associated with hypertension)
Features
Hypertension in children
Measuring blood pressure in children
correct cuff size is approximately 2/3 the length of the upper arm
the 4th Korotkoff sound is used to measure the diastolic blood pressure until adolescence, when the 5th Korotkoff sound can be
used
results should be compared with a graph of normal values for age
In younger children secondary hypertension is the most common cause, with renal parenchymal disease accounting for up to 80%
Causes of hypertension in children
Question 50 of 80
The chance of a 30-year-old mother giving birth to a child with Down's syndrome is approximately:
1 in 125
1 in 350
1 in 550
1 in 1,000
1 in 2,000
Next question
Down's syndrome risk - 1/1,000 at 30 years then divide by 3 for every 5 years
One way of remembering this is by starting at 1/1,000 at 30 years and then dividing the denominator by 3 (i.e. 3 times more common) for
every extra 5 years of age
Cytogenetics
Mode
Non-disjunction
94%
Robertsonian translocation
5%
(usually onto 14)
Mosaicism
1%
The chance of a further child with Down's syndrome is approximately 1 in 100 if the mother is less than 35 years old. If the trisomy 21 is a
result of a translocation the risk is much higher
Question 51 of 80
A 13-year-old girl develops purpura on her lower limbs and buttocks associated with microscopic haematuria. A diagnosis of HenochSchonlein purpura is made. Her urea and electrolytes show mild renal impairment that is still present 4 weeks later, although she does not
require any specific therapy. What is the most likely renal outcome?
Hypertension within 20 years
Persistent proteinuria
Frequent relapses
Next question
Henoch-Schonlein purpura
Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis. There is a degree of overlap with IgA nephropathy (Berger's
disease). HSP is usually seen in children following an infection.
Features
palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
abdominal pain
polyarthritis
features of IgA nephropathy may occur e.g. haematuria, renal failure
Treatment
Prognosis
usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
around 1/3rd of patients have a relapse
Question 52 of 80
Myotonic dystrophy
Ehlers-Danlos syndrome
Huntington's disease
Next question
4 months
6 months
8 months
12 months
Next question
Primitive reflexes
Reflex Description
Moro
Reflex Description
Grasp
Rooting
Assists in breastfeeding
Present from birth to around 4 months of age
Stepping
Question 53 of 80
4 months
6 months
8 months
12 months
Next question
Primitive reflexes
Reflex Description
Moro
Grasp
Rooting
Assists in breastfeeding
Present from birth to around 4 months of age
Stepping
Question 54 of 80
A 12-month-old child is brought into surgery for her next routine immunisations. She has received all the recommended immunisations to
date. What should be given at this stage?
MMR + PCV
MMR + Hib
Hib/Men C
MMR + Men C
Next question
Immunisation schedule
In 2010 the NHS immunisation guidelines changed and it is now recommended that infants aged 12-13 months are given Hib/Men C +
MMR + PCV at the same time, rather than split between two visits at 12 and 13 months.
Immunisation schedule
The current UK immunisation schedule is as follows. Please note that this table includes the changes announced in 2010 which merged the
12 and 13 month visits into one.
Age
Recommended immunisations
At birth
2 months
DTaP/IPV/Hib + PCV
Oral rotavirus vaccine
3 months
DTaP/IPV/Hib + Men C
Oral rotavirus vaccine
4 months
DTaP/IPV/Hib + PCV
3-4 years
MMR + DTaP/IPV
At birth the BCG vaccine should be given if the baby is deemed at risk of tuberculosis (e.g. Tuberculosis in the family in the past 6 months).
Hepatitis B vaccine should be given at birth if the mother is HBsAg +ve.
Key
Question 55 of 80
Which one of the following is a risk factor for the development surfactant deficient lung disease in the newborn?
Maternal diabetes mellitus
Vaginal delivery
Maternal asthma
Female sex
Next question
male sex
diabetic mothers
Caesarean section
second born of premature twins
Clinical features are those common to respiratory distress in the newborn, i.e. tachypnoea, intercostal recession, expiratory grunting and
cyanosis
Chest x-ray characteristically shows 'ground-glass' appearance with an indistinct heart border
Management
Question 56 of 80
Which one of the following features is least associated with roseola infantum?
Tonsillopharyngitis
Cough
Febrile convulsions
High fever
Diarrhoea
Next question
Whilst Nagayama spots (erythematous papules on the soft palate and uvula) may be seen, tonsillopharyngitis is not a common feature
Roseola infantum
Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human
herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years.
Features
aseptic meningitis
hepatitis
Question 56 of 80
Which one of the following features is least associated with roseola infantum?
Tonsillopharyngitis
Cough
Febrile convulsions
High fever
Diarrhoea
Next question
Whilst Nagayama spots (erythematous papules on the soft palate and uvula) may be seen, tonsillopharyngitis is not a common feature
Roseola infantum
Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human
herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years.
Features
aseptic meningitis
hepatitis
Question 57 of 80
Precocious puberty in males may be defined as the development of secondary sexual characteristics before:
8 years of age
9 years of age
10 years of age
11 years of age
12 years of age
Next question
Precocious puberty
Definition
'development of secondary sexual characteristics before 8 years in females and 9 years in males'
more common in females
are rare, associated with rapid onset, neurological symptoms and signs and dissonance
e.g. McCune Albright syndrome
Question 58 of 80
A 4-year-old girl with sickle cell anaemia presents with abdominal pain. On examination she is noted to have splenomegaly and is clinically
anaemic. What is the most likely diagnosis?
Liver cirrhosis
Parvovirus infection
Sequestration crisis
Salmonella infection
Thrombotic crisis
Next question
Sickle-cell crises
Sickle cell anaemia is characterised by periods of good health with intervening crises
Four main types of crises are recognised:
Thrombotic crises
Sequestration crises
sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
acute chest syndrome: dyspnoea, chest pain, pulmonary infiltrates, low pO2 - the most common cause of death after childhood
Aplastic crises
Haemolytic crises
rare
fall in haemoglobin due an increased rate of haemolysis
Sickle cell trait (sickle cell carrier) occurs when a person inherits a gene for normal haemoglobin
(Hb A) from one parent and a gene for sickle cell haemoglobin from their other parent. Their
genotype is Hb AS. People who are sickle cell carriers rarely have clinical symptoms.
Sickle cell disease affects 1 in every 2400 live births in England and is now the most common
genetic condition at birth.
Sickle cell disease should be suspected if the person is in a high-risk ethnic group and:
Is a child aged 918 months with painful dactylitis (painful swelling of the bones of the hands
and feet). There may be chronic shortening of a digit due to epiphyseal damage.
Has a sudden severe infection.
Presents with features of an acute crisis, or presents with a history of features consistent with
an acute crisis.
Presents with features of a chronic complication of sickle cell disease.
Sickle cell disease is always diagnosed after both an initial and confirmatory test are positive.
A sickle cell crisis should be suspected if there is a sudden onset of pain, infection, or anaemia,
or other symptoms, such as a stroke or priapism. There is often a history of a previous crisis. The
person is often able to state whether their pain is typical of sickle cell disease.
The most common type of crisis is an acute painful crisis which usually starts with vague pain,
often in the back of limb bones, which gets gradually worse.
Acute chest syndrome is common in early childhood, when it may present like pneumonia.
Onset may be abrupt, and the person may deteriorate over a few hours. Typical features of
acute chest syndrome include: new onset of chest pain or pleuritic pain, cough, shortness of
breath and/or respiratory distress, tachypnea, fever, hypoxia, crepitations in the lung bases that
become generalized, and rib tenderness.
All people with clinical features of a sickle cell crisis should be admitted to hospital unless they
are:
A well adult with only mild or moderate pain who has a temperature of 38C or less.
A well child with mild or moderate pain who does not have an increased temperature.
All people with sickle cell disease will be followed up regularly in secondary care who will advise
on:
Immunizations.
Lifelong antibiotic prophylaxis.
When to suspect
Confirmation
Assessment of acute crisis
Management
Scenario: Screening : provides information on the national screening programme to detect sickle
cell disease and other disorders, such as sickle cell trait and thalassemia.
Scenario: Management - sickle cell crisis : covers the management of a sickle cell crisis that has
been identified in primary care.
Scenario: Management - chronic complications : covers the management of chronic
complications of sickle cell disease in primary care.
Scenario: Prevention of complications : covers the prevention of complications of sickle cell
disease, including the use of immunizations, antibiotics, and folic acid in primary care.
Scenario: Management - sickle cell trait : covers the management of sickle cell trait in primary
care.
Scenario: Contraception : covers recommendations for the use of contraception in people with
sickle cell disease.
Scenario: Screening
Scenario: Management - sickle cell crisis
Scenario: Management - chronic complications
Scenario: Prevention of complications
Scenario: Management - sickle cell trait
Scenario: Contraception
Evidence
Supporting evidence
Search strategy
References
Alhashimi, D., Fedorowicz, Z., Alhashimi, F. and Dastgiri, S. (2010) Blood transfusions for treating
acute chest syndrome in people with sickle cell disease (Cochrane Review). The Cochrane
Library. Issue 1. John Wiley & Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
BNF 59 (2010) British National Formulary. 59th edn. London: British Medical Association and
Royal Pharmaceutical Society of Great Britain.
BNF for Children (2010) British National Formulary for Children. London: British Medical
Association and the Royal Pharmaceutical Society of Great Britain.
British Committee for Standards in Haematology (1996) Guidelines for the prevention and
treatment of infection in patients with an absent or dysfunctional spleen. Working Party of the
British Committee for Standards in Haematology Clinical Haematology Task Force. BMJ
312(7028), 430-434. [Abstract] [Free Full-text]
British Committee for Standards in Haematology (2003) Guidelines for the management of acute
painful crisis in sickle cell disease. British Journal of Haematology 120(5), 744-752. [Free Fulltext]
Chinegwundoh, F.I. and Anie, K.A. (2004) Treatments for priapism in boys and men with sickle
cell disease (Cochrane Review). The Cochrane Library. Issue 4. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Claster, S. and Vichinsky, E.P. (2003) Managing sickle cell disease. British Medical Journal
327(7424), 1151-1155. [Free Full-text]
Davies, J.M., Barnes, R. and Milligan, D. (2002) Update of guidelines for the prevention and
treatment of infection in patients with an absent or dysfunctional spleen. Clinical Medicine 2(5),
440-443. [Abstract] [Free Full-text]
Davis, C.J., Mostofi, F.K. and Sesterhenn, I.A. (1995) Renal medullary carcinoma. The seventh
sickle cell nephropathy. American Journal of Surgical Pathology 19(1), 1-11. [Abstract]
de Montalembert, M. (2008) Management of sickle cell disease. British Medical Journal 337(Sep
8), a1397.
Dimashkieh, H., Choe, J. and Mutema, G. (2003) Renal medullary carcinoma: a report of 2 cases
and review of the literature. Archives of Pathology & Laboratory Medicine 127(3), e135-e138.
[Abstract] [Free Full-text]
Dunlop, R. and Bennett, K.C.L.B. (2006) Pain management for sickle cell disease in children and
adults (Cochrane Review). The Cochrane Library. Issue 2. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Falletta, J.M., Woods, G.M., Verter, J.I. et al. (1995) Discontinuing penicillin prophylaxis in
children with sickle cell anemia. Prophylactic Penicillin Study II. Journal of Pediatrics 127(5), 685690. [Abstract]
FSRH (2009) UK medical eligibility criteria for contraceptive use. Faculty of Sexual and
Reproductive Healthcare. www.fsrh.org [Free Full-text]
Gaston, M.H., Verter, J.I., Woods, G. et al. (1986) Prophylaxis with oral penicillin in children with
sickle cell anemia. A randomized trial. The New England Journal of Medicine 314(25), 15931599. [Abstract]
Gill, F.M., Brown, A., Gallagher, D. et al. (1989) Newborn experience in the Cooperative Study of
Sickle Cell Disease. Pediatrics 83(5), 827-829.
Hirst, C. and Owusu-Ofori, S. (2002) Prophylactic antibiotics for preventing pneumococcal
infection in children with sickle cell disease (Cochrane Review). The Cochrane Library. Issue 3.
John Wiley & Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
Hirst, C. and Wang, W.C. (2002) Blood transfusion for preventing stroke in people with sickle cell
disease (Cochrane Review). The Cochrane Library. Issue 1. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Joint Royal Colleges Ambulance Liaison Committee (2009) UK Ambulance Service clinical
practice guidelines: sickle cell crisis - update. University of Warwick. www2.warwick.ac.uk [Free
Full-text]
Jones, A.P., Davies, S.C. and Olujohungbe, A. (2001) Hydroxyurea for sickle cell disease
(Cochrane Review). The Cochrane Library. Issue 2. John Wiley & Sons, Ltd.
www.thecochranelibrary.com [Free Full-text]
Lees, C., Davies, S.C. and Dezateux, C. (2000) Neonatal screening for sickle cell disease (Cochrane
Review). The Cochrane Library. Issue 1. John Wiley & Sons, Ltd. www.thecochranelibrary.com
[Free Full-text]
Legardy, J.K. and Curtis, K.M. (2006) Progestogen-only contraceptive use among women with
sickle cell anemia: a systematic review. Contraception 73(2), 195-204. [Abstract]
Lucas, S.B., Mason, D.G., Mason, M. and Weyman, D. (2008) A sickle crisis? A report of the
national confidential enquiry into patient outcome and death (2008). National Confidential
Enquiry into Patient Outcome and Death. www.ncepod.org.uk [Free Full-text]
Mart-Carvajal, A.J., Conterno, L.O. and Knight-Madden, J.M. (2007) Antibiotics for treating acute
chest syndrome in people with sickle cell disease (Cochrane Review). The Cochrane Library.
Issue 2. John Wiley & Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
Mehta, S.R., Afenyi-Annan, A., Byrns, P.J. and Lottenberg, R. (2006) Opportunities to improve
outcomes in sickle cell disease. American Family Physician 74(2), 303-310. [Abstract] [Free Fulltext]
NHS Antenatal and Newborn Screening Programmes (2006) Sickle cell disease in childhood:
standards and guidelines for clinical care. . .London: NHS Sickle Cell and Thalassaemia Screening
Programme in partnership with the Sickle Cell Society. http://sct.screening.nhs.uk [Free Fulltext]
NHS Sickle Cell and Thalassaemia Screening Programme (2006) Laboratory data report:
development towards a quality report. NHS Sickle Cell and Thalassaemia Screening Programme.
http://sct.screening.nhs.uk
O'Brien, P. (2001) New WHO medical eligibility criteria for contraceptives: adaptation for use in
a local service in UK. Journal of Family Planning and Reproductive Health Care 27(3), 149-152.
Okomo, U. and Meremikwu, M.M. (2007) Fluid replacement therapy for acute episodes of pain
in people with sickle cell disease (Cochrane Review). The Cochrane Library. Issue 2. John Wiley &
Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
Oringanje, C., Nemecek, E. and Oniyangi, O. (2009) Hematopoietic stem cell transplantation for
children with sickle cell disease (Cochrane Review). The Cochrane Library. Issue 1. John Wiley &
Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
Platt, O.S., Brambilla, D.J., Rosse, W.F. et al. (1994) Mortality in sickle cell disease - life
expectancy and risk factors for early death. New England Journal of Medicine 330(23), 16391644. [Abstract] [Free Full-text]
RCOG (2004) Venous thromboembolism and hormonal contraception. . Guideline 40. Royal
College of Obstetricians and Gynaecologists. www.rcog.org.uk [Free Full-text]
Ryan, K., Bain, B.J., Worthington, D. et al. (2010) Significant haemoglobinopathies: guidelines for
screening and diagnosis. British Journal of Haematology 149(1), 35-49. [Free Full-text]
Sears, D.A. (1978) The morbidity of sickle cell trait: a review of the literature. American Journal
of Medicine 64(6), 1021-1036.
Sickle Cell Society (2005) Sickle cell: a guide for GPs, nurses and other health professionals. .
.London: Sickle Cell Society. www.sicklecellsociety.org
Sickle Cell Society (2008) Standards for the clinical care of adults with sickle cell disease in the
UK. . .London: Sickle Cell Society. http://sct.screening.nhs.uk [Free Full-text]
Swartz, M.A., Karth, J., Schneider DT et al. (2002) Renal medullary carcinoma: clinical,
pathologic, immunohistochemical, and genetic analysis with pathogenetic implications. Urology
60(6), 1083-1089. [Abstract]
Teach, S.J., Lillis, K.A. and Grossi, M. (1998) Compliance with penicillin prophylaxis in patients
with sickle cell disease. Archives of Pediatrics Adolescent Medicine 152(3), 274-278. [Abstract]
[Free Full-text]
Vichinsky, E. (2001) Transfusion therapy in sickle cell disease. Information Center for Sickle Cell
and Thalassemic Disorders. http://sickle.bwh.harvard.edu [Free Full-text]
Watanabe, I.C., Billis, A., Guimaraes, M.S. et al. (2007) Renal medullary carcinoma: report of
seven cases from Brazil. Modern Pathology 20(9), 914-920. [Abstract] [Free Full-text]
Weatherall, D.J. (2010) Disorders of the synthesis or function of haemoglobin. In: Warrell, D.A.,
Cox, T.M., Firth, J.D. and Benz, E.J.Jr (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford
University Press. 4420-4444.
Wethers, D.L. (2000) Sickle cell disease in childhood: part II. Diagnosis and treatment of major
complications and recent advances in treatment. American Family Physician 62(6), 1309-1314.
[Abstract] [Free Full-text]
Wierenga, K.J., Hambleton, I.R. and Lewis, N.A. (2001) Survival estimates for patients with
homozygous sickle cell disease in Jamaica: a clinic based population study. Lancet 357(9257),
680-683. [Abstract]
Question 59 of 80
Each one of the following statements regarding glue ear is correct, except:
Incidence peaks at 2 years of age
Next question
The majority of children will have at least one episode during childhood
Glue ear
Glue ear describes otitis media with an effusion (other terms include serous otitis media). It is common with the majority of children having
at least one episode during childhood
Risk factors
male sex
siblings with glue ear
higher incidence in Winter and Spring
bottle feeding
day care attendance
parental smoking
Features
grommet insertion - to allow air to pass through into the middle ear and hence do the job normally done by the Eustachian tube.
The majority stop functioning after about 10 months
adenoidectomy
Question 60 of 80
Which one of the following is most strongly associated with coeliac disease?
HLA-DQ3
HLA-B4
HLA-DR4
HLA-DR3
HLA-DQ2
Next question
failure to thrive
diarrhoea
abdominal distension
older children may present with anaemia
Diagnosis
Question 61 of 80
Which one of the following statements regarding developmental dysplasia of the hip is true?
Affects around 10% of newborns
Next question
DDH is slightly more common in the left hip. Around 20% of cases are bilateral.
Clinical examination is made using the Barlow and Ortolani tests:
Management
Question 62 of 80
24
28
32
36
Next question
Dentition
Basics
The age at which children develop their first teeth varies from birth (rare) to around 1 year of age, but it tends to occur at around 6 months.
The first teeth to appear are usually the lower incisors
Children usually develop all their deciduous teeth by two-and-a-half years of age.
Question 63 of 80
A mother brings her child as she is concerned he is clumsy compared to other similar aged children. At what age would the average child
acquire a good pincer grip?
5-6 months
7-8 months
12 months
18 month
2 years
Next question
Milestone
3 months
9 months
12 months
Bricks
Age
Milestone
15 monthsTower of 2
18 monthsTower of 3
2 years
Tower of 6
3 years
Tower of 9
Drawing
Age
Milestone
18 monthsCircular scribble
2 years
3 years
Copies circle
4 years
Copies cross
5 years
Book
Age
Milestone
Notes
hand preference before 12 months is abnormal and may indicate cerebral palsy
Question 64 of 80
Seizures
Next question
Reye's syndrome
Reye's syndrome
Reye's syndrome is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys
and pancreas. The aetiology of Reye's syndrome is not fully understood although there is a known association with aspirin use and a viral
cause has been postulated
The peak incidence is 2 years of age, features include:
Management is supportive
Although the prognosis has improved over recent years there is still a mortality rate of 15-25%.
Question 65 of 80
What is the risk of a newborn infant at 27 weeks gestation having surfactant deficient lung disease?
25%
50%
75%
90%
95%
Next question
male sex
diabetic mothers
Caesarean section
second born of premature twins
Clinical features are those common to respiratory distress in the newborn, i.e. tachypnoea, intercostal recession, expiratory grunting and
cyanosis
Chest x-ray characteristically shows 'ground-glass' appearance with an indistinct heart border
Management
Question 66 of 80
You review a 4-year-old boy in clinic. He has been diagnosed with asthma after having multiple wheezy episodes over the past 3 years.
Around 4 months ago he was admitted with shortness-of-breath and wheeze and was diagnosed as having a viral exacerbation of asthma
by the paediatric team. Prior to his discharge he was given a Clenil (beclometasone dipropionate) inhaler 100mcg bd in addition to
salbutamol 100mcg prn via a spacer.
His mother reports that he has a persistent night-time cough and is regularly having to use his salbutamol inhaler. Clinical examination of
his chest today is normal.
What is the most appropriate next step in management?
Add a long-acting beta agonist
Next question
Step 3 of asthma management in children 2-5 years: add a leukotriene receptor antagonist
Children aged 2-5 years: trial of a leukotriene receptor antagonist. If already taking leukotriene receptor antagonist reconsider inhaled
corticosteroids
Children aged under 2 years: refer to respiratory paediatrician
3
2. Assess control of asthma:
StepTherapy
Question 67 of 80
A 2 month-old boy presents to the department with a desquamating rash affecting the palms of the hand and soles of the feet. On
examination he has a low grade fever of 37.8C and there is generalised lymphadenopathy, hepatosplenomegaly and a muco-purulent
discharge from the nose.
What is the most likely diagnosis?
Cytomegalovirus
Enterovirus
Parvovirus B19
Rubella virus
Congenital syphilis
Next question
Syphilis
Syphilis is a sexually transmitted infection caused by the spirochaete Treponema pallidum. Infection is characterised by primary, secondary
and tertiary stages. The incubation period is between 9-90 days
Primary features
Tertiary features
gummas
aortic aneurysms
general paralysis of the insane
tabes dorsalis
Question 68 of 80
A 17-year-old male with a history of cystic fibrosis presents to clinic for annual review. What is the most appropriate advice regarding his
diet?
High calorie and low fat with pancreatic enzyme supplementation for every meal
High calorie and low fat with pancreatic enzyme supplementation for evening meal
Normal calorie and low fat with pancreatic enzyme supplementation for every meal
High calorie and high fat with pancreatic enzyme supplementation for evening meal
High calorie and high fat with pancreatic enzyme supplementation for every meal
Next question
regular (at least twice daily) chest physiotherapy and postural drainage. Parents are usually taught to do this. Deep breathing
exercises are also useful
high calorie diet, including high fat intake*
vitamin supplementation
pancreatic enzyme supplements taken with meals
heart and lung transplant
*this is now the standard recommendation - previously high calorie, low-fat diets have been recommended to reduce the amount of
steatorrhoea
Question 69 of 80
A 4-year-old girl is brought to surgery due to dysuria. On examination her temperature is 37.2C, abdominal examination is unremarkable
and urine dipstick is positive for leukocytes and nitrites. She has no past medical history of note. What is the most appropriate
management, other than urine microscopy?
Oral antibiotics for 10 days + follow-up if not settled + static radioisotope scan after 4 months
Oral antibiotics for 10 days + follow-up if not settled + ultrasound within 6 weeks
Next question
The 2007 NICE guidelines are controversial - many paediatricians are still taking the approach of imaging more children than strictly
suggested by the guidelines.
Management
children aged more than 3 months old with a lower UTI should be treated with oral antibiotics for 3 days according to local
guidelines, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin. Parents should be asked to bring the children back
if they remain unwell after 24-48 hours
antibiotic prophylaxis is not given after the first UTI but should be considered with recurrent UTIs
Arranging a review (e.g. after 48 hours) to ensure response to treatment and to reassess
the antibiotic choice, if applicable.
Offering appropriate advice to parents or carers (such as to return for assessment if the
child is still unwell after 2448 hours of treatment or if they suspect a repeat UTI).
Infants/children aged over 3 months with suspected UTI are at:
High risk of serious illness if they are systemically unwell, dehydrated, or vomiting and
cannot tolerate oral fluids and medication or have a history or clinical features
suggesting urinary tract obstruction.
Low risk of serious illness if temperature is less than 38C with no history of fever and
there is no loin pain/tenderness.
Intermediate risk of serious illness if they do not satisfy the criteria for being at high or
low risk.
Have I got the right topic?
Age from 0 months to 16 years
This CKS topic is based on the National Institute for Health and Care Excellence guideline
Urinary tract infection in children: diagnosis, treatment and long-term management
[National Collaborating Centre for Women's and Children's Health, 2007].
This CKS topic covers the management in primary care of urinary tract infection (UTI) in
infants and children.
This CKS topic does not cover the management of recurrent UTI in sexually active girls,
or UTI in infants or children with:
Indwelling urinary catheters.
Neurogenic bladders.
Using intermittent catheterization.
Significant uropathy.
Underlying renal disease.
Immunosuppression.
This CKS topic does not cover in any detail the investigations used to detect renal tract
abnormalities in children with UTI as these will usually be arranged and performed in
secondary care. However referral to secondary care for consideration of further
investigation is discussed.
There are separate CKS topics on Urinary tract infection (lower) - women, and Urinary
tract infection (lower) - men.
The target audience for this CKS topic is healthcare professionals working within the
NHS in the UK, and providing first contact or primary health care.
How up-to-date is this topic?
Changes
Update
Goals and outcome measures
Goals
QIPP - options for local implementation
Background information
Definition
Causal organisms
Prevalence
Risk factors
Prognosis
Complications
Diagnosis
Diagnosis (< 3 months of age)
Diagnosis (3 months to 3 years of age)
Diagnosis (> 3 years of age)
Management
Scenario: UTI less than 3 months of age : covers the management of infants less than
three months of age, with a urinary tract infection.
Scenario: UTI 3 months to 3 years of age : covers the management of children between
three months and three years of age, with a urinary tract infection.
Scenario: UTI over 3 years of age : covers the management of children over three years
of age, with a urinary tract infection.
Scenario: UTI less than 3 months of age
Scenario: UTI 3 months to 3 years of age
Scenario: UTI over 3 years of age
Prescribing information
Important aspects of prescribing information relevant to primary healthcare are covered
in this section specifically for the drugs recommended in this CKS topic. For further
information on contraindications, cautions, drug interactions, and adverse effects, see
the electronic Medicines Compendium (eMC) (http://medicines.org.uk/emc), or the
British National Formulary (BNF) (www.bnf.org).
Trimethoprim
Nitrofurantoin
Cefalexin
Cefixime
Amoxicillin and co-amoxiclav
Paracetamol
Evidence
Supporting evidence
Search strategy
References
ABPI Medicines Compendium (2010) Summary of product characteristics for Augmentin
125/31 SF Suspension. Electronic Medicines CompendiumDatapharm Communications
Ltd. www.medicines.org.uk [Free Full-text]
ABPI Medicines Compendium (2012a) Summary of product characteristics for Cefalexin
250mg Capsules. Electronic Medicines CompendiumDatapharm Communications Ltd.
www.medicines.org.uk [Free Full-text]
MHRA (2011) Press release: more exact paracetamol dosing for children to be
introduced. Medicines and Healthcare products Regulatory Agency. www.mhra.gov.uk
[Free Full-text]
MHRA (2014) Nitrofurantoin now contraindicated in most patients with an estimated
glomerular filtration rate (eGFR) of less than 45 ml/min. Drug Safety Update 8(2), A3.
[Free Full-text]
Miron, D., Daas, A., Sakran, W. et al. (2007) Is omitting post urinary-tract-infection renal
ultrasound safe after normal antenatal ultrasound? An observational study. Archives of
Disease in Childhood 92(6), 502-504. [Abstract] [Free Full-text]
National Collaborating Centre for Women's and Children's Health (2007) Urinary tract
infection in children: diagnosis, treatment and long-term management (full NICE
guideline). . Clinical guideline 54. National Institute for Health and Care Excellence.
www.nice.org.uk [Free Full-text]
NICE (2009) When to suspect child maltreatment (NICE guideline). . Clinical guideline 89.
National Institute for Health and Care Excellence. www.nice.org.uk [Free Full-text]
NICE (2013) Key therapeutic topics - medicines management options for local
implementation. National Institute for Health and Care Excellence. www.nice.org.uk
[Free Full-text]
NPC (2011) Key therapeutic topics 2010/11 - Medicines management options for local
implementation. National Prescribing Centre. www.npc.nhs.uk [Free Full-text]
NPC (2012) Key therapeutic topics - medicines management options for local
implementation. National Prescribing Centre. www.npc.nhs.uk [Free Full-text]
Shaikh, N., Ewing, A.L., Bhatnagar, S. and Hoberman, A. (2010) Risk of renal scarring in
children with a first urinary tract infection: a systematic review. Pediatrics 126(6), 10841091. [Abstract] [Free Full-text]
Stamm, W.E. (1998) Urinary tract infections and pyelonephritis. In: Fauci, A.S.,
Braunwald, E. and Isselbacher, K.J. (Eds.) Harrison's principles of internal medicine. New
York: McGraw-Hill.
Tomson, C. and Armitage, A. (2010) Urinary tract infection. In: Warrell, D.A., Cox, T.M.
and Firth, J.D. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University
Press. 4103-4122.
Tullus, K. (2007) Personal communication. Consultant Paediatric Nephrologist, Great
Ormond Street Hospital: London.
Williams, G. and Craig, J.C. (2011) Long-term antibiotics for preventing recurrent urinary
tract infection in children (Cochrane Review). The Cochrane Library. Issue 3. John Wiley
& Sons, Ltd. www.thecochranelibrary.com [Free Full-text]
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Question 70 of 80
Abdominal x-ray
DMSA
Micturating cystourethrogram
Ultrasound
Next question
Vesicoureteric reflux
Vesicoureteric reflux (VUR) is the abnormal backflow of urine from the bladder into the ureter and kidney. It is relatively common
abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI), being found in around 30% of children who
present with a UTI. As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI
Pathophysiology of VUR
ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle
therefore shortened intramural course of ureter
vesicoureteric junction cannot therefore function adequately
Grade
I
II
III
IV
Dilation of the renal pelvis and calyces with moderate ureteral tortuosity
Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity
Investigation
Question 71 of 80
Which one of the following is not a risk factor for child abuse?
Disability
Chronic illness
Prematurity
Next question
prematurity
low birth weight
disability
chronic illness
psychiatric disorder
isolation
large family size
Question 72 of 80
Marfan syndrome
Kallman's syndrome
Klinefelter's syndrome
Homocystinuria
Next question
Tall stature
Causes of tall stature
constitutional
excessive growth hormone (pituitary gigantism)
hyperthyroidism
Marfan syndrome
homocystinuria
Klinefelter's syndrome
Congenital adrenal hyperplasia may be associated with a premature growth spurt but early fusion of the epiphyses results in short stature
Question 73 of 80
Which one of the following statements regarding iron deficiency anaemia in children is incorrect?
The prevalence is around 2%
Management can be done in primary care and involves dietary advice and iron supplementation
Next question
Prevention
*whilst breast milk is relatively low in iron it is present in a form that is easily absorbed
Question 74 of 80
A 13-month-old baby girl has her first MMR vaccination. If side-effects do occur, which on of the following best describes the likely
symptoms?
Diarrhoea: occurs after 5-10 days and lasts around 2-3 days
Diarrhoea: occurs after 2-3 days and lasts around 1-2 days
Malaise, fever and rash: occurs after 5-10 days and lasts around 2-3 days
Arthralgia: occurs after 5-10 days and lasts around 2-3 days
Malaise, fever and rash: occurs after 2-3 days and lasts around 1-2 days
Next question
MMR vaccine
Children in the UK receive two doses of the Measles, Mumps and Rubella (MMR) vaccine before entry to primary school. This currently
occurs at 12-15 months and 3-4 years as part of the routine immunisation schedule
Contraindications to MMR
severe immunosuppression
allergy to neomycin
children who have received another live vaccine by injection within 4 weeks
pregnancy should be avoided for at least 1 month following vaccination
immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are
present)
Adverse effects
malaise, fever and rash may occur after the first dose of MMR. This typically occurs after 5-10 days and lasts around 2-3 days
Question 75 of 80
What is the most common cause of childhood hypothyroidism in the United Kingdom?
Iodine deficiency
Autoimmune thyroiditis
Grave's disease
Next question
Hypothyroidism in children
The most common cause of hypothyroidism in children (juvenile hypothyroidism) is autoimmune thyroiditis.
Other causes include
post total-body irradiation (e.g. in a child previous treated for acute lymphoblastic leukaemia)
iodine deficiency (the most common cause in the developing world)
Question 76 of 80
Next question
Breast feeding
Advantages
Disadvantages
Mother
bonding
involution of uterus
protection against breast and ovarian cancer
cheap, no need to sterilise bottle
Transmission of drugs
Immunological
IgA (protects mucosal surfaces), lysozyme (bacteriolytic enzyme) and lactoferrin Vitamin K deficiency
(ensures rapid absorption of iron so not available to bacteria)
Breast milk jaundice
reduced incidence of ear, chest and gastro-intestinal infections
reduced incidence of eczema and asthma
reduced incidence of type 1 diabetes mellitus
At what age would the average child start to play alongside, but not interacting with, other children?
3 months
6 months
12 months
2 years
4 years
Next question
Milestone
Laughs
Enjoys friendly handling
6 monthsNot shy
9 months
Shy
Takes everything to mouth
Feeding
Age
Milestone
6 months
Drinks from cup + uses spoon, develops over 3 month period 12 -15 months
Competent with spoon, doesn't spill with cup
2 years
3 years
5 years
Dressing
Age
Milestone
12-15 months
18 months
2 years
Can dress and undress independently except for laces and buttons 4 years
Play
Age
Milestone
Plays 'peek-a-boo'
9 months
Waves 'bye-bye'
Plays 'pat-a-cake'
12 months
Age
Milestone
18 months
4 years
Question 78 of 80
A newborn male baby is found to have an undescended right testicle during the routine newborn exam. It is neither palpable in the scrotum
or inguinal canal. What is the most appropriate management?
Outpatient referral to urology to be seen within 6 weeks
Next question
If the testicle has not descended by around 6 months then referral should be considered for orchidopexy
Undescended testis
Undescended testis occurs in around 2-4% of term male infants., but is much more common if the baby is preterm. Around 25% of cases
are bilateral
Complications of undescended testis
infertility
torsion
testicular cancer
psychological
Management
orchidopexy: referral should be considered from around 6 months of age. Surgical practices vary although the majority of
procedures are performed at around 1 year of age
Question 79 of 80
24
28
32
36
40
Next question
Dentition
Basics
The age at which children develop their first teeth varies from birth (rare) to around 1 year of age, but it tends to occur at around 6 months.
The first teeth to appear are usually the lower incisors
Children usually develop all their deciduous teeth by two-and-a-half years of age.
Question 80 of 80
Which one of the following conditions is most strongly associated with supravalvular aortic stenosis?
Pierre-Robin syndrome
Edward's syndrome
William's syndrome
Patau syndrome
Noonan syndrome
Next question
Childhood syndromes
Below is a list of common features of selected childhood syndromes
Syndrome
Key features
Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers
Fragile X
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
Noonan syndrome
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
Pierre-Robin syndrome*
Micrognathia
Posterior displacement of the tongue (may result in upper airway obstruction)
Cleft palate
Prader-Willi syndrome
Hypotonia
Hypogonadism
Obesity
William's syndrome
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
*this condition has many similarities with Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome is
autosomal dominant so there is usually a family history of similar problems