Gaucher Disease Text A: Reading Test 12
Gaucher Disease Text A: Reading Test 12
Gaucher Disease Text A: Reading Test 12
PART A
Gaucher Disease
Text A
Gaucher disease is the most common of the lysosomal storage disorders (LSDs),
which are metabolic conditions caused by genetic defects in the lysosomal system.
The lysosome is an internal cell structure that contains numerous enzymes
responsible for degrading complex cellular components. LSDs result from the
absence or deficiency of a lysosomal enzyme and the subsequent accumulation of
the enzyme's particular substrate in the body. The incidence of LSDs is estimated
to range from one in 5,000 to one in 7,000 live births. Worldwide, Gaucher disease
has a prevalence estimated to range from one in 40,000 to one in 60,000 in the
general population and, though it is a panethnic disorder, in the Ashkenazi Jewish
population its frequency is markedly higher, ranging from one in 400 to one in
1,000 live births. Carrier frequency in those of Ashkenazi descent is estimated to
be as high as one in 18.
Text B
Skeletal Manifestations
The skeletal manifestations of Gaucher disease are often the most debilitating,
yet the pathogenesis of bone changes are not fully understood. Between 70% and
100% of patients with type 1 Gaucher disease have clinical or radiographic
evidence of bone disease. Irreversible complications may influence long-term
mobility and quality of life. The spine, pelvis, and femurs are usually affected;
several different mechanisms of bone injury have been identified. The
displacement of yellow marrow with red marrow because of Gaucher cell
infiltration produces both physical and biochemical changes in the bone marrow
microenvironment that can affect bone marrow vascularity and pressure,
potentially causing thrombosis, infarction, and impaired hematopoiesis.
Text C
Text D
1. A. condition that occurs due to an increase in the number of white cells in the
blood
Answer___________________.
7 .Weakening appearance
Answer___________________.
Questions 8-14
Answer each of the questions, 8-14, with a word or short phrase from one of the
texts. Each answer may include words, numbers or both.
Questions 15-20
Complete each of the sentences, 15-20, with a word or short phrase from one of the
texts. Each answer may include words, numbers or both.
15. The__________ have the capacity to exterminate various other cell structures.
16. With the appearance of red marrow, the___________ and__________ often get
affected more badly and may lead to infarction.
17 .The two of the common conditions that can occur due to Gaucher may
include _________ and _____________.
20. Studies reveal that there are various other __________ which can make the
conditions worse.
PART B
Questions 1-6
In this part of the test, there are six short extracts relating to the work of health
professionals. For questions 1-6, choose the answer (A, B or C) which you think
fits best according to the text.
1 ------ is an epidemic of infectious disease that has spread across a large region
Disease Transmission
Bioterrorism
Pandemics
C Low risk in the first two trimester compared to the third trimester.
Transmission
In general, 1 of every 150 to 200 babies in the USA is born with congenital CMV.
This makes CMV the most frequent congenital viral infection. Though this seems
like a large percentage of births, only 1 in 5 of these infants born with congenital
CMV will experience any adverse symptoms or long-term issues.
The virus has the potential to travel through the mother’s blood and pass through
the placenta, infecting the developing baby.
If the pregnant lady contracts the virus (primary infection) during pregnancy: It is
more likely to pass on CMV to the baby if it is primary infection during the
pregnancy. If the mother has a primary CMV infection during pregnancy, there is
approximately a 40% chance of passing the virus to the baby.
The risk of transmission from mother to baby is highest if she gets a primary CMV
infection in the third trimester (40-70%) and is lowest if the primary infection
begins in the first or second trimesters (30-40%).
3 The following table talks about
A Medication
B Preventive measure
C Effects of Disease
Allergic Rhinitis
Indicated as immunotherapy for short ragweed (Ambrosia artemisiifolia) pollen-
induced allergic rhinitis (with or without conjunctivitis) confirmed by positive skin
test or in vitro testing for ragweed-specific IgE antibodies
Initiate treatment 12 weeks before the expected onset of ragweed pollen season and
maintain it throughout the season 18-65 years: 1 tablet SL qDay; give the 1st dose
in physician’s office and observe for 30 min > 65 years: Not approved
4 What is wrong?
Effectiveness of PPE
Shortages of PPE
Multiple studies have found that the U.S. hospitals and healthcare agencies lack
sufficient PPE and even stockpiles have not provided adequate or correct supplies
to give healthcare personnel necessary PPE during past events. During the 2014
Ebola outbreak, PPE availability was severely limited, leading to potential
occupational exposures and healthcare personnel infection. When respirators are
limited, remaining supplies can be worn for extended periods of time or re-used
between patients. However, extending the use or re-using respirators puts nurses at
risk of exposure due to auto-inoculation when removing contaminated equipment
or from reduced compliance during long-term wear.
6 According to the table given, which is correct?
Univariate P-
Non-diabetics Diabetics
value
Categorical Variable
(%) Frequency
Frequency
(%)
Student 9 (1.7) --
Family history of
Yes 129 (24.57) 33 (41.77) 0.001
diabetes
In this part of the test, there are two texts about different aspects of
healthcare. For questions 7-22, choose the answer (A, B, C or D) which you
think fits best according to the text.
Regaining lost weight after a diet often leads to another weight loss attempt. With
repetitive loss and gain of 10-50 pounds, a pattern of weight cycling emerges.
Weight cycling is a high-risk behavior for the development of Type 2 diabetes
(T2D), as regained weight is more metabolically unhealthy because it
preferentially deposits as visceral fat. Visceral adipose tissue, as opposed to
subcutaneous adipose tissue, produces more inflammatory factors, as well as
resistin and visfatin, hormones that are linked to insulin resistance. In summary,
while all weight gain can lead to negative metabolic changes, regained weight is
especially likely to promote insulin resistance and inflammation.
Emotional eating often results in consumption of excess food or poor food choices
and often leads to weight gain. Beginning a stressful new job or going through a
divorce are just two examples of life events that can spur emotional eating. Asking
patients if they have noticed a change in their eating habits, and if that coincided
with any other changes in their lives, should be part of the history if weight gain
has occurred. If the patient acknowledges overriding fullness cues and eating more
than usual, the underlying stress, emotional pain, depression, and/or anxiety could
be helped in a variety of ways.
Sleep hygiene can help to get sleep on time as well as sleeping more
deeply. Patients should be apprised of the need to avoid caffeine for 7 hours, and
alcohol for 2 hours before bedtime. Also turning off of “blue light” an hour before
bedtime is a good way to help increase the natural sleep hormone melatonin. For
patients who still find it difficult to fall asleep or stay asleep, recommending
melatonin supplements can help. If restorative sleep still evades the patient, it is
important to assess for obstructive sleep apnea (OSA), a major impediment of
deep, restful sleep and is associated with obesity.
A reduced activity could be a result of fatigue, chronic pain, old injuries, or newly
developing arthritis. For those suffering from pain, referral to an appropriate
specialist may be indicated. Physical therapy can also help to maximize their
mobility and to find an exercise that is also enjoyable, sustainable, and suitable for
their limitations. Research shows that threats to health, such as lack of food, sleep,
or long periods of exertion, are perceived by homeostatic sensors as threats to
human survival. Dieting, which often requires ignoring hunger may be perceived
as a threat, whereas intuitive eating, which honors internal cues is perceived as
reassuring to the body. This alternative approach to dieting was started by lay
health writers in the 1980’s and has dieticians more likely to use intuitive eating
than restrictive practices.
By having patients make sure they have healthy and delicious food available for
when ideal hunger sets in (neither starving nor hardly hungry) so they can eat until
they are satisfied, which is a key for visceral eating. Whether an advance practice
nurse or a nutritionist helps guide the patient, this non-dieting approach to eating
helps patients replace an antagonistic relationship with the body for a nurturing
one.
Text 1: Questions 7-14
7 The first paragraph talks about;
A. Fat Triggers
B .How to lose weight?
C .Healthy lifestyle
D .Nature of Leptin
X-ray diffraction imaging (XDI) scanner has been developed for security screening
applications by Morpho Detection GmbH in Hamburg, Germany. The main
rationale for developing this XDI scanner is the existence of explosive materials
whose densities overlap those of common materials, such as water, leading to
unacceptably high false alarm rates for scanners employing merely transmission x-
ray data. As the x-ray diffraction probes molecular structure, XDI yields more
features for material identification than transmission x-rays, leading to higher
detection rates and lower false alarm rates.
The XDI scanner employs the Multiple Inverse Fan-Beam (MIFB) topology
(Harding et al, 2012). The MIFB topology is a multiple-focus, multiple-beam,
multiple-detector extension of that originally described by Harding. These
extensions increase the photon throughput by over five orders of magnitude
relative to that of the original system. The MIFB topology features an x-ray
multisource, comprising a linear array of 16 focal spots that are sequentially
irradiated by a magnetically-deflected electron beam. The accelerating voltage is
140 kV; whereas the tube DC power is 6 kW and the beam dwell time for each
focus is 200 µs.
The inherent contrast of molecular coherent scatter from body tissues is much
greater than that originating in the linear attenuation coefficient accessed by
transmission radiation, when the momentum dimension is included in tissue
discrimination. As each voxel is irradiated from several directions, a modest degree
of transmission tomosynthesis can be reconstructed from the transmission data;
The fusion of data from scatter and transmission sensors allows a significant
improvement in image quality relative to that obtained when each is separately
depicted; the conveyor belt speed is sufficient to allow an anatomical region, such
as head, thorax or abdomen, to be scanned in only a few seconds; the measured
dose imparted in an XDI scan is negligible compared with that of the natural
radiation background, taken as ~ 3 mGy / year.
The x-ray imparted by the XDI scanner was repeatedly measured with a PTW
Diados E dosimeter inserted into the center of a D100 QRM thorax phantom. The
thorax phantom, visible to the right of the picture, was inserted in a luggage bin
that was moved by conveyor belt through the scanner. The dosimeter signal was
read out through the cable. As the dosimeter was inserted into the center of the
phantom, it was shielded from radiation emitted by the x-ray multisource owing to
an overlying material; hence the skin dose will be significantly higher.
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