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Intusseception
- Intermittent Pain
- Mass in RLQ
- Occult blood positive
-> Air contrast enema

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http://en.wikipedia.org/wiki/Addison%27s_disease

Addison's disease

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Goat's milk is not fortified with Vitamin D --> rickets

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 CONGENITAL CMV PRESENTATION
1) Majority are asymptomatic at birth
2) Periventricular calcifications
3) IUGR, developmental delay, microcephaly, sensorineural hearing loss,
retinitis, jaundice, hepatosplenomegaly, thrombocytopenia, hypotonia,
lethargy, poor suck
4) Preterm infants may appear septic – apnea, bradycardia, intestinal
distension)
5) Postnatal infections are generally asymptomatic

Presenting symptoms — The presenting symptoms reflect

the location of the primary tumor and the extent of metastatic
disease, if present. Patients with localized disease can be
asymptomatic, whereas children with advanced disease
appear ill at presentation, usually with systemic symptoms.

Signs and symptoms of neuroblastoma may include [5,6]:

●Abdominal mass (retroperitoneal or hepatic)

●Abdominal pain or constipation
●Proptosis
●Periorbital ecchymoses ("raccoon eyes", from
periorbital ecchymosis caused by orbital metastases)
●Horner syndrome (miosis, ptosis, anhidrosis)
●Localized back pain, weakness (from spinal cord
compression)
●Scoliosis, bladder dysfunction
●Palpable nontender subcutaneous nodules
●Opsoclonus myoclonus ataxia syndrome
●Otherwise unexplained secretory diarrhea (from
Hematogenous spread most often occur to bone, bone marrow, skin and liver. paraneoplastic production of vasoactive intestinal
polypeptide [VIP])
Bone—bone pain (esp. with ambulation) and in the younger child limp or irritability. Infiltration of ●Systemic symptoms (fever, weight loss)
periorbital bones may produce periorbital ecchymosis, ptosis and proptosis.
●Bone pain
Bone marrow—blood cunt abnormalities and fever
Liver—hepatomegaly ●Anemia
Skin—nontender papules and subcutaneous nodules ●Heterochromia iridis (different colors of the iris or
portions of the iris)
●Hypertension
●Unilateral nasal obstruction

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The presenting manifestations of SLE in children are as diverse as they are in adults. The most common
initial symptoms are the gradual onset of fever, malaise, and general deterioration over several months.
Children also may have small joint arthritis and renal disease. The classic malar rash is absent in two-
thirds of individuals.

In retrospective reviews from France and Canada, the onset of juvenile SLE is at a median age of 12 to 13
years, with the disease developing in the majority of patients after eight years of age. In these studies, the
most common presenting manifestations were as follows:

* Hematologic: anemia, cytopenia, and/or thrombocytopenia

* Mucocutaneous: malar rash and/or oral ulcers
* Musculoskeletal: arthritis or arthralgia
* Fever
* Renal abnormalities (ex, nephritis and nephrotic syndrome)

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B lymphocyte Function
Group B Strep
HIB
= Encapsulated

Bacterial infections with polysaccharide encapsulated bacteria, neutrophilia

Patients with B cell deficiencies are highly susceptible to encapsulated bacterial infections.

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 A varicocele (from the Latin word, varix for dilated vein; and the Greek
word, kele for tumor) is an enlargement of the veins in the scrotum and is
similar to varicose veins that occur in the legs. In younger men and boys,
an abnormal appearing or feeling scrotum is often the reason for coming to
a doctor. In adult men, varicoceles most often present during an evaluation
for infertility.

The anatomic defect in varicocele is an abnormal enlargement of the

pampiniform venous plexus that drains the testicle in the scrotum. The
pampiniform plexus is a collection of veins that helps regulate the
temperature of the testicle - as the testicle best makes sperm at
temperature two degrees lower than body temperature.

Varicoceles are graded on a scale from I-III, based on physical examination

findings and are often described as appearing "like a bag of worms" in the
scrotum

The first sign of tinea versicolor is often spots on the skin. The other signs and symptoms are:
- The spots are lighter (sometimes darker) than the surrounding skin. The color of the spots can be white, pink, salmon, red, tan, or
brown.
- The spots can appear anywhere on the body.
- Spots can be dry and scaly.
- Skin may itch where the spots appear.

Tinea versicolor is caused by the dimorphic, lipophilic organisms in the genus Malassezia, formerly known as Pityrosporum. Eleven
species are recognized within this classification of yeasts, of which Malassezia globosa and Malassezia furfur are the predominant
species isolated in tinea versicolor

Tx) Terbanifine, Ketoconazole

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Appropriate blood glucose targets can be established based on the pattern of blood glucose responses and the ability of
the individual athlete to make appropriate adjustments to keep glucose in the target range. At the beginning of sports
training, it is often necessary to adjust insulin therapy by decreasing the total insulin dose by 20–50%. Reductions may
be needed in both short- or rapid-acting insulin and intermediate- or long-acting insulin. Athletes on pump therapy may
need to decrease bolus doses by 20–50% and may choose to discontinue basal insulin during exercise and decrease
the basal insulin by 25% after exercise is completed.

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 Short Stature
Delayed menses
Androgen -> hair growth -> HPA activated
Weak Femoral Pulses (Coarctation)

THINK TURNER’S

Treponema pallidum is a spirochete that is responsible for syphilis.

First stage: Presents as a painless ulcer (Chancre) at the site of inoculation 3 weeks after infection, and the
lesion resolves.
- The chancre of primary syphilis usually begins as a single, painless papule that rapidly becomes eroded and
indurated, with a surrounding red areola. The edge and base of the ulcer have a cartilaginous (buttonlike)
consistency on palpation.

Second stage: The patient may present with fever malaise, headache, adenopathy, generalized body rash,
and mucosal lesions (condylomata lata). The rash of secondary syphilis can be confused with pitiriasis rosea.
The symptoms of secondary syphilis resolves spontaneously and the infection enters a latent stage.

Third stage: Can present years later and may manifest as neurologic, cardiovascular, and skeletal disease.
HPV: Condylomata are discrete, papillary, cauliflowerlike lesions that involve multiple sites on moist surfaces.
Keratotic warts are often seen on dry surfaces like the labia majora. Warts vary in size and can form large,
exophytic, cauliflowerlike masses (see the images below). Discrete papules 1-3 mm in size can present on the
shaft of the penis. The growth can extend into the vagina, urethra, cervix, perirectal epithelium, anus, and
rectum

HSV: Tender Bilateral lymphadenopathy. Lesions itch. Vesicular Lesions on an erythematous base

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An infant can experience symptoms either very quickly after feeding (rapid onset) or up to several days or even weeks after first
consuming the cow's milk protein (slower onset). The most common symptoms are gastrointestinal (50-60%), skin (50-60%) and
respiratory (20-30%). Many infants may experience two or more of these symptoms.3

IgE-associated cases of CMPA tend to show an early reaction even to a small quantity of CMP, with symptoms including irritability,
urticaria, dermatitis, swelling, or vomiting occurring within minutes. Although anaphylaxis is more common in other food allergies,
in rare cases, anaphylaxis can occur in conjunction with CMPA. In these cases, CMPA may be the first expression or predictor of
future atopy and other food allergies in the infant, and there is frequently a family history of atopy.1

Laryngotracheobronchitis
Croup manifests as hoarseness, a seal-like barking cough, inspiratory stridor, and a variable degree of respiratory distress. However,
morbidity is secondary to narrowing of the larynx and trachea below the level of the glottis (subglottic region), causing the
characteristic audible inspiratory stridor (see the image below).

Inspiratory stridor suggests a laryngeal obstruction, whereas expiratory stridor suggests tracheobronchial obstruction. A reactive
inflammatory response causes subglottic edema. Narrowing of the airway can be life threatening in infants and young children
because of their small airway

“Pencil Sign” on AP X-ray

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 cholelithiasis -- patients with spherocytosis/hemolysis have high risk of gallstones from bilirubin

Hereditary Spherocytosis (HS) is the most common hemolytic anemia due to a red blood cell (RBC) membrane disorder. HS is due to
a mutation in spectrin, ankyrin, band 3 protein, or protein 4.2. These proteins normally maintain the biconcave disc shape of
RBCs.With a defect in these proteins, the RBC typically takes on a spherical shape.
- These RBCs are not as flexible as the regular RBC, and can undergo hemolysis when trying to pass through the small capillaries of
the reticuloendothelial system (RES).
- This then causes the spleen to become engorged, which can be palpable on physical exam.

Cholelithiasis — due to severe hemolysis, patients can develop cholelithiasis and acute cholecystitis due to the formation of pigmented
gallstones.

Administer surfactant and IV ABX?

Congenital cardiac defects — these can occur in 40 to 60% of patients with Down’s syndrome, and include complete AV canal
defects, VSD and tetralogy of Fallot. Because of this risk, ALL newborns with Down syndrome should have an echocardiogram
and ECG to screen for heart defects. If found, the patient should be referred to a pediatric cardiologist.

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 HEMOLYTIC DISEASE OF THE NEWBORN
The diagnosis is made when the infant is A, B or AB and has a positive direct Coombs test and a
positive indirect Coombs result for anti-A or anti-B. The mother will lack the A or B antigen which
is positive in the indirect Coombs test.

TX) Exchange Transfusion

Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) fall into the broader
category of thrombotic microangiopathies (TMA). Thrombotic microangiopathies are characterized by the
involvement of widespread occlusive microvascular thromboses resulting in thrombocytopenia,
microangiopathic hemolytic anemia, and variable signs and symptoms of end-organ ischemia.

Specifically, E coli serotype O157:H7 has been associated with more than 80% of infections leading to
hemolytic uremic syndrome.

TRIAD of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.

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 Cremaster reflex would still be present in epididymitis? Epididymitis is the most frequent cause
of acute onset scrotal pain in adults. In
contrast with men who have testicular
torsion, the cremasteric reflex
(elevation of the testicle in response to
stroking the upper inner thigh) is not
altered.

Status Epilepticus: Benzos to abort -> progress to Phenytoin -> Phenobarb if doesn’t work

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 slit lamp exam -- I didn't think it was septic arthritis given that she feels
better with usage. ANA+ made me think of autoimmune causes of joint
pain, which made me think about associated eye findings. Case Files
says that uveitis is an important thing to catch in JIA and recommends
doing a slit lamp exam on everyone suspected to have JIA.

Nephroblastoma: Usually asymptomatic kid, with mass found by parents

- Does NOT cross midline

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Leukocoria or a white pupillary reflex is an ominous finding in infants. It can be caused by opacification of any
of the structures that lie in the visual axis including the cornea, the lens, the vitreous, and the retina. The
majority of these conditions are significant for visual impairment that may not be appreciated until the child is
older. However, retinal conditions such as retinoblastoma (RB) can be life-threatening. Therefore screening for
and evaluation of leukocoria should be performed at every well-baby and well-child visit

The most common presenting feature in the US is leukocoria (an absence of the red reflex of the eye).
Strabismus (misalignment of the eyes) is the second most common presenting feature.

Retinoblastoma confined within the eye has excellent prognosis with reported survival rates of above 90%.
When this disease goes undetected, as is often the case in developing countries, tumor often spills from the
sclera into the orbit or beyond the optic nerve leading to seeding in the vitreous cavity or extension into the
subretinal space, thereby decreasing survival rates to around 50%.

Hemolytic disease of the newborn -- it is not physiologic before 24-36hr of life.

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 Hyperinflation: Obstructive lung disease => Asthma

Endoscopy should be performed within the first 12-48 hours following ingestion as endoscopy
occurring more than 48 hours after ingestion increases the risk of perforation of the weakened tissue.

An exception to this is children who ingested household bleach often do not require endoscopy due to
the very low likelihood of tissue damage. The presence and extent of injury is documented using
flexible endoscopy. Endoscopic ultrasonography has been demonstrated to more accurately show the
depth of lesions and may be used more widely in the future to aid in diagnosis and treatment.

Ingestions of alkaline substances cause tissue damage by liquefactive necrosis. This involves
saponification of fats, denaturation of proteins, and disruption of cellular membranes, allowing the
alkaline substance to diffuse into deeper layers of the mucosa. Small vessel thrombosis and heat
production also occur.

Agents with a pH greater than 11 cause the most damage.

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Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalized vasculitis
involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central
nervous system (CNS).

Subsequently, symptoms develop, of which the following are the most common:

- Rash (95-100% of cases), especially involving the legs; this is the hallmark of the disease
- Abdominal pain and vomiting (35-85%)
- Joint pain (60-84%), especially involving the knees and ankles
- Subcutaneous edema (20-50%)
- Scrotal edema (2-35%)
- Bloody stools

Reactive arthritis is a painful form of inflammatory arthritis (joint disease due to inflammation). It occurs in reaction to an infection
by certain bacteria. Most often, these bacteria are in the genitals (Chlamydia trachomatis) or the bowel (Campylobacter,
Salmonella, Shigella and Yersinia)

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 Associated with low C3 (hypocomplementemia): "PMS" = poststreptococcal glomerulonephritis,
membranoproliferative, and SLE (lupus nephritis)
Acute post-streptococcal glomerulonephritis (PSGN) can occur after an episode of strep throat or, in rare cases, impetigo (a skin
infection). The Streptococcus bacteria do not attack the kidney directly, but an infection may stimulate the immune system to
overproduce antibodies, which are circulated in the blood and finally deposited in the glomeruli, causing damage. PSGN can bring on
sudden symptoms of swelling (edema), reduced urine output (oliguria), and blood in the urine (hematuria). Tests will show large
amounts of protein in the urine and elevated levels of creatinine and urea nitrogen in the blood, thus indicating reduced kidney function.
High blood pressure frequently accompanies reduced kidney function in this disease.

PSGN is most common in children between the ages of 3 and 7, although it can strike at any age, and it most often affects boys. It lasts
only a brief time and usually allows the kidneys to recover.

BUN:Cr > 20

Prerenal acute renal failure / AKI / azotemia

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Biliary atresia is a disorder unique to the neonatal period. The fetal/perinatal form is evident within the first 2 weeks of life; the
postnatal type presents in infants aged 2-8 weeks.

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Asplenic patients need prophylactic Penicillin

Kind of like sickle cell patients

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Infantile hemangiomas (IH) are benign vascular neoplasms with a course marked by early proliferation and spontaneous
involution

- IH are the most common tumors of infancy

- IH can be cutaneous or extracutaneous. Common extracutaneous sites include: Liver, Gastrointestinal tract, Larynx, CNS

Kassabach-Merritt Syndrome- Thrombocytopenia and/or coagulopathy resulting from platelet trapping within a vascular tumor.
Infantile hemangiomas are rarely responsible for this syndrome! More commonly tufted angioma or
kaposiformhemangioendothelioma

Hirchsprung’s disease: Hirchsprung’s disease, or congenital aganglionic megacolon, is a

motor disorder of the colon that causes a functional intestinal obstruction. It occurs in 1/5000
infants with a male to female predominance of 4:1. The pathogenesis of the disease is
failure of migration of the neural crest cells that form the colonic ganglion cells. Without
parasympathetic innervation, the colon cannot relax or undergo peristalsis, resulting in a
functional obstruction.

The aganglionic segment is limited to the rectosigmoid in the majority of patients. 10% have
full colonic involvement and in 10% more, there is lack of ganglion cells into the small bowel.

Diagnosis of Hirchsprung’s disease can be made with barium enema, revealing a transition
zone between the constricted aganglionic segment and the proximal, normally dilated
segment.

Confirmation of the diagnosis is made with rectal suction biopsy, which will demonstrate
absent ganglion cells, hypertrophied nerve fibers, and elevated acetylcholinesterase activity.

The treatment of Hirchsprung’s disease is through surgical resection of the aganglionic

bowel and approximation of normal, ganglionic tissue near the anus.
The major complications of the disease, even after surgical resection, are bowel obstruction
and enterocolitis, though most patients go on to normal or near-normal bowel function.

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Puberty in Females
- Onset between 7-13
- Thelarche -> Adrenarche -> Menarche (Age 9-15)

Latent TB infection
- Positive PPD
- >5mm for those in close contact
- >10mm younger than 4, endemic region, chronic medical condition
- >15mm older than 4 with no other risk factors
- Negative physical and chest x-ray
- Children rarely progress to to TB disease

Tx) Isoniazid for 9 months. Older adolescents are given B6 to prevent neuro complications

TB Disease
Tx) INH, Rifampin, Pyrazinamide for 2 months, followed by INH and Rifampin for 4 months

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 Kawasaki Treatment: IVIG and Aspirin to prevent Coronary Aneurysm

In addition to aspirin?

Likely a viral URI --> supportive treatment, self-limiting

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 HHT
- Nose Epistaxis from Telangiectasias
- AVM in brain and large organs
Neurofibromatosis Type 1
- Cafe au Lait Tuberous Sclerosis
- Neurofibromas - Hamartomas
- Bone Defects - Ash Leaf Macules
- Scoliosis - Benign tumors to grow in the brain and on other vital organs
- Lisch Nodules such as the kidneys, heart, eyes, lungs, and skin. A
- Optic Nerve Gliomas combination of symptoms may include seizures, intellectual
disability, developmental delay, behavioral problems, skin
abnormalities, lung and kidney disease.
Sturge Weber
- Port Wine Stain Von Hippel Disease
- Calcifications of Brain Clinical hallmarks include the development of retinal and central nervous
- Weakness of body opposite of stain system (CNS) hemangioblastomas (blood vessel tumors),
pheochromocytomas, multiple cysts in the pancreas and kidneys, and an
increased risk for malignant transformation of renal cysts into renal cell
carcinoma.

About a week to 11 days after the MMR injection, some children get a very mild
form of measles. This includes a rash, high temperature, loss of appetite and a
general feeling of being unwell for about two or three days.

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