MCQs in Pediatric asthma

Q1 :

A 12-year-old female is brought to the emergency department. She is breathing 30 times per minute,

is unable to speak in full sentences, and has a peak expiratory fl ow rate (PEFR) 50% predicted.

Th e preferred firs tline therapy for her asthma exacerbation is :

(A) Aminophylline IV.

(B)  B-agonist nebulization.

(C) Hydrocortisone IV.

(D) Magnesium sulfate IV

(E) B and D

(F) B and C

Answer

F


Patient is obviously in respiratory distress. Aggressive treatment with oxygen, systemic steroids, and shortacting bronchodilators is indicated. Ipratropium could also be added to the albuterol in the acute setting.

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Q 2:

A 12-year-old boy who has mild persistent asthma is brought to the emergency department
because of increased work of breathing of 1 day’s duration. He reports a low-grade fever and
nonproductive cough for the past 4 days, but this morning he developed difficulty breathing and
a cough that produced a small amount of yellowish sputum. His respiratory rate is 24
breaths/min, heart rate is 80 beats/min, and temperature is 99.0°F (37.3°C). He appears in no
respiratory distress, but his lung examination reveals bilateral rales and occasional wheezes. A
chest radiograph shows bilateral diffuse infiltrates with no effusions.
Of the following, the MOST likely etiologic agent causing his symptoms is

A. Haemophilus influenzae
B. Mycobacterium tuberculosis
C. Mycoplasma pneumoniae
D. Staphylococcus aureus
E. Streptococcus pneumoniae

Answer

C

Mycoplasma pneumoniae is a common cause of community-acquired pneumonia in school-age
children and adolescents, but it is rare in infants and toddlers. Other clinical manifestations of M
pneumoniae infection include upper respiratory tract infection, pharyngitis, bullous myringitis, and
bronchitis. Occasionally, systemic symptoms such as malaise and fever are present. Among
the rarer clinical presentations are hemolytic anemia, aseptic meningitis, and Stevens-Johnson
syndrome.

 M pneumoniae is responsible for many cases of pneumonia and acute chest syndrome in children who have sickle cell disease.

 Infection also may precipitate an exacerbation of asthma, as described for the boy in the vignette. A recent study showed that children of different ages may respond differently to infection with M pneumoniae.

Children younger than 5 years of age were more likely to experience coryza, vomiting and diarrhea, and
tachypnea than were older children.

Typical radiographic findings are bilateral, diffuse infiltrates, but occasionally a
lobar infiltrate or pleural effusion may be seen. The diagnosis frequently can be made clinically,
but serologic testing is available.

The complement fixation test and, more recently, immunofluorescence and enzyme immunoassay can be used to measure levels of immunoglobulin M (IgM) and IgG antibodies to M pneumoniae. High concentrations of IgM may be present for months after acute infection.

 Comparing acute and convalescent IgG titers allows for more accurate diagnosis, although this may be impractical. Serum cold hemagglutinin titers also may be positive in patients who have acute infection but have limited sensitivity and specificity.

 Direct polymerase chain reaction is both sensitive and specific for the diagnosis but is not widely available. Macrolide antibiotics generally are used for treatment, although many infections are self-limited, and a recent review found no conclusive evidence that antibiotics are effective in reducing symptoms

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Q 3:

You are evaluating a 12-year-old girl during her biannual asthma follow-up visit. She has a
history of mild persistent asthma that is well-controlled on a low-dose inhaled corticosteroid. You
review the asthma guidelines and recommend that she receive the influenza vaccine. Her
mother immediately replies, "Oh no, my daughter has a severe egg allergy and was told to never
get the influenza vaccine."
Of the following, the vaccine that is contraindicated in a patient who has a severe immunoglobulin E-mediated egg allergy is

A. human papillomavirus vaccine
B. measles-mumps-rubella
C. tetanus diphtheria
D. varicella
E. yellow fever

Pediatric Cases

Case 1:

A 4 year old girl presented with recurrent seizures since 1 year of age which are generalized tonic clonic convulsions with clenching occurring once in 2-3 months. Mother had appendicitis in first trimester of pregnancy. A milestone in form of speech is delayed. The child is a full term normal delivery with birth weight of 2.2 kg. On examination, the child has microphthalmia, microcornea, low set ears, microcephaly {head circumference = 44 cm}. There was an ejection systolic-murmur in pulmonary area. Other systems are normal.

Question :

What is the possible diagnosis?

This child has presented with recurrent seizures. In addition, the child has dysmorphic features such as microphthalmia, microcornea and microcephaly suggestive of either an intrauterine infection or genetic disorder. The commonest IU infection in this child would be congenital rubella and seizures could be due to brain calcifications, malformations. In this child, urine for rubella was positive suggestive of diagnosis of Congenital rubella. Echocardiography was normal. ECG showed sinus bradycardia. 

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Case 2


A 14 months old boy born of third degree consanguineous marriage presents with sudden inability to stand or sit since 15 days. There is no restriction of movement in upper limb. There is no convulsion, bladder or bowel complaints and no trauma. The child has fever since 3 days. His milestones and immunization history is adequate. There is no history of TB contact. On examination, the child has hypertonia in both lower limbs with decreased power and brisk reflexes in both knees. There is bilateral sustained ankle clonus and planter reflex was extensor. Cremasteric and Abdominal reflexes were absent. Upper limb reflexes were present. Pain and touch sensation appears intact. Other systemic examination is normal. 


Questions :

Mention 4 possible causes.


What is the most likely diagnosis.



Mention a useful informative test.


Answer

This child has presented with sudden onset paraplegia. One may consider possibilities such as poliomyelitis, trauma, GBS, transverse myelitis and spinal tumor which may have bled suddenly leading to intraspinal edema. Since the paralysis is symmetrical and the child has been immunized upto date, poliomyelitis seems unlikely. Trauma and vertebral facture is also unlikely as no history of trauma is available. Since the paralysis has remained static, GBS also seems unlikely as GBS is usually an ascending polyneuropathy. A sudden onset paraplegia can be a manifestation of transverse myelitis but it usually leads to a band of dermatomal involvement with band like pain at the level of the lesion and complete motor, sensory as well as bladder and bowel involvement below the upper level of involvement. In this child, bladder and bowel involvement cannot be commented upon as the child has still not achieved continence. However, pain and touch sensation are intact thus making a diagnosis of transverse myelitis less likely. Spinal tumors usually have an insidious onset of paraparesis. However, they may be totally asymptomatic and can present as sudden onset paraplegia if there is bleeding inside the tumor. Thus, one would consider a possibility of spinal tumor in this child. An MRI spine in this child was suggestive of right paravertebral soft tissue mass at the level of T1 to T5 spinal vertebrae extending into the spinal canal through T1 and T2 neural foramen with marked extradural compression of spinal cord suggestive of intraspinal tumor

MCQs In Pediatric GIT Disorders

Q1:

Which laxative should not be used to treat acute constipation because of its slow onset of action?

(A) glycerin

(B) bisacodyl suppository

(C) psyllium

(D) milk of magnesia

Answer

C


Glycerin and the bisacodyl suppository all produce stools in 30 mins to a few hours, whereas psyllium, a
bulk-forming laxative, produces stool in 24 to 72 hrs in the same manner as a normal bolus of food or fi ber.

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Q 2- 4 :



An 8-year-old girl presented with a 6-month history of fresh PR blood on the toilet paper and clearly mixed in with stools. She occasionally has pain on defecation, and intermittent, generalized sharp abdominal pain that resolves spontaneously. She denies any significant constipation. There is no vomiting, diarrhoea or weight loss. She has a normal diet and is growing well. On examination, her abdomen was soft and mildly
tender over the right side with stool palpable in her ascending colon. In the perianal region, there is a sentinel
skin tag but no evidence of a fissure. Urea, electrolytes and creatinine, liver function tests, Creactive
protein, erythrocyte sedimentation rate and full blood count were all within normal limits.




(Q2 ) What is the most likely diagnosis given this history?

A-  Constipation
B- Inflammatory bowel disease
C-  Campylobacter enterocolitis
D-  Meckel’s diverticulum

Abdominal X-ray revealed no faecal loading and a 99mTc pertechnetate scintigraphy (Meckel’s scan) showed no evidence of functional ectopic gastric tissue. An upper endoscopy and colonoscopy were done with biopsy of the intestinal mucosa. Histology showed no active inflammation, normal crypt architecture, no granulomas, no evidence of epithelial dysplasia nor evidence of malignancy. There was however raised mucosal eosinophil density with basophilic blush and positive Steiner staining.

(Q 3) This is diagnostic of

A-  Coeliac disease
B-  Helicobacter pylori infection
C-  Crohn’s disease
D-  Intestinal spirochaetosis
E-  C. enterocolitis

(Q 4) Treatment should be with

A-  Gluten exclusion diet
B-  Triple agent therapy: omeprazole, amoxicillin and metronidazole
C-  Elemental diet
D-  Metronidazole
E-  Symptomatic relief only

Answers

Q 2: A

Q 3: D

Q 4: D


PR bleeding has a number of different aetiologies. A comprehensive history and examination should be obtained to help direct the most appropriate investigations and in order to make an accurate diagnosis. Intestinal spirochaetes, IS, are difficult to diagnose because of non-specific symptoms. The causative organisms are Brachyspira aalborgi or Brachyspira pilosicoli.

Biopsy specimens obtained during endoscopy, can be suggestive of infestation when there is basophilic blush and positive Steiner staining with eosinophilic infiltration.

Definitive diagnosis is with electron microscopy that shows spirochaetes attached to epithelial cells However, if histology-staining is suggestive of IS, it may be pertinent to treat with a 7e10 day course of oral metronidazole, rather than proceeding to expensive confirmatory testing.

Inflammatory bowel disease, IBD, includes Crohn’s disease, CD, and ulcerative colitis, UC. They can present with abdominal pain, diarrhoea, bloody stools and weight loss. Weight loss is more common in CD, and blood loss in UC. CD can affect any part of the gastrointestinal tract whereas, by definition, UC will only affect the colon.

Diagnosing and differentiating IBD requires clinical assessment in correlation with biochemistry, radiology, endoscopy and histology investigations. UC is diagnosed by clinical history supported with colonoscopy findings. CD is diagnosed by history and the demonstration of focal lesions on colonoscopy with histology showing transmural inflammation and granulomas.

Constipation can be diagnosed on history and examination alone. Bleeding is often the result of an anal fissure. Abdominal X-ray can show faecal loading. Campylobacter enterocolitis can cause dysentery with
severe, crampy abdominal pain. It is transmitted mainly via infected food and its course is usually self-limiting over a few days.


Most children infected with Helicobacter pylori, are asymptomatic.


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Q 5:


A 12-year-old girl has had intermittent periumbilical abdominal pain for the past 4 years.
Sometimes the pain worsens when she drinks a glass of milk. A lactose breath hydrogen test
demonstrates a breath hydrogen of 40 ppm after 1 hour (normal, <20 ppm).
Of the following, the food that this girl is MOST likely to tolerate is

A. buttermilk
B. cheddar cheese
C. ice cream
D. skim milk
E. whole milk

Answer

B

Lactose intolerance (lactose maldigestion) is a common cause of pediatric recurrent abdominal
pain. Lactase is a digestive enzyme located on the intestinal villi that converts the disaccharide
lactose to the monosaccharides glucose and galactose. The monosaccharides can be
absorbed across the intestinal epithelial cells. In an individual who has low intestinal lactase
(lactose intolerance), the lactose cannot be broken down and passes into the lower intestine and
colon. The malabsorbed lactose can cause osmotic diarrhea or be fermented by bacteria,
resulting in pain, gas, and bloating. Lactose intolerance can either be primary (lactase activity
that gradually declines with aging) or secondary (an infection or enteropathy damages the villi,
resulting in lactose intolerance).

Lactose intolerance may be diagnosed clinically by elimination diet and rechallenge or
through lactose breath hydrogen testing. Breath testing offers the advantage of providing a more
definitive diagnosis, so milk is not withdrawn from the diet unnecessarily. In a breath hydrogen
test, an adult is given approximately 50 g of lactose (the equivalent of 4 cups of milk) as a single
dose. Patients who cannot digest and absorb this amount of lactose have the lactose fermented
to hydrogen by intestinal bacteria, which can be measured in the breath. Because intestinal
bacterial fermentation is essential for an accurate test, patients should not receive antibiotics for
at least 2 weeks prior to the test.

Many patients who have lactose intolerance diagnosed on breath test can tolerate small
amounts of lactose in their diet. Specifically, hard cheeses, such as cheddar, which often have
very little lactose (<0.5 g/serving), can be eaten by many patients who have lactose intolerance.
Other products, such as whole or skim milk, ice cream, or buttermilk, have more lactose
(approximately 10 g/serving) and are less likely to be tolerated

MCQs In Pediatric Emergencies

Q 1:

Parenteral calcium is used as an antidote for which of the following situations?

(A) Verapamil overdoses

(B) Hyperkalemia

(C) Cocaine intoxication

(D) Verapamil overdoses and hyperkalemia


Th e correct answer is :

 D

Parenteral calcium is used to reverse the cardiac effects of calcium-channel blocker overuse and hyperkalemia.

Q 2:



A 4-year-old girl is brought to the emergency department after sticking a hair pin in a household
electrical outlet. The mother reports that she heard the child scream, and when she investigated,
smoke was coming from the outlet and the child was crying, holding her right hand. There was a
black imprint on her fingers in the shape of the hair pin. She washed the fingers with soap and
water and drove the child to the emergency department for further evaluation. On physical
examination, the child is tearful but awake and alert. Her right index finger and thumb have
erythematous burn imprints with small blisters surrounded by soot. She has no other burns or
other findings of note on the remainder of her examination.
Of the following, it is MOST important to evaluate this patient for

A. arrhythmias
B. compartment syndrome
C. immunization status
D. myoglobinuria
E. skin grafting

Answer

C


The girl in the vignette has a surface thermal burn related to contact with the heated hair pin,
and the presence of soot on her finger is evidence of flash contact. She has no clinical features
or risk factors for rhabdomyolysis, myoglobinuria, compartment syndrome, or arrhythmias. The
wound is small and should not require skin grafting. Therefore, other than ascertaining her
tetanus immunization status and providing wound care, no further evaluation is indicated


Pediatric electrical injuries typically are related to contact with household, low-voltage sources such as
electric cords or wall outlets. The primary determinant of tissue damage following electrical
exposure is current strength, which is directly proportional to voltage and inversely related to
tissue resistance. Because voltage is the only variable commonly known, electrical exposures
are classified as low- (<1,000 V) and high- (>1,000 V) voltage. Standard household electricity in
the United States is 110 V; utility power lines carry in excess of 14,000 V.

The most common injury following contact with electricity is burns. Excluding lightening
injuries, electrical burns may occur from any of the following mechanisms: electrothermal
heating from direct contact with the electrical source, arc exposure in which the body becomes
part of the electrical circuit, flash contact in which the current arc strikes the skin but does not
enter the body, and flame burns that result when clothing or other objects combust in the
presence of electrical current. Oral burns due to chewing on an electrical cord
usually are electrothermal. Arc exposure can be associated with deep tissue burns and internal
organ involvement as the current flows through body; the extent of injury may be
underestimated by the appearance of the skin wounds. Flash contact is characterized by
surface burns accompanied by soot, as described for the girl in the vignette.

Although both high- and low-voltage exposures can lead to injuries in a variety of organ
systems, high-voltage exposures are responsible for most significant injuries. The most
commonly affected organ is the skin, with burns resulting as described previously. Arrhythmias,
including asystole and ventricular fibrillation, may occur, usually at the time of contact. Deep
thermal injuries to bone and muscle may occur after arc exposure and lead to compartment
syndrome or rhabdomyolysis with subsequent renal damage. Risk factors for these more
serious injuries include extensive full-thickness burns, cardiac arrest, or high-voltage exposure.
It is rare for children exposed to household current to sustain injuries other than burns.

MCQs In Pediatric Endocrinology Part II

Q 1:

You are evaluating a 13-year-old girl who complains of malaise, fatigue, and occasional

abdominal discomfort. You diagnosed hypothyroidism due to chronic lymphocytic thyroiditis

(Hashimoto thyroiditis) 6 years ago. She has normal serum immunoglobulin A concentrations. A

tissue transglutaminase antibody study was negative 1 month before this visit, and free

thyroxine and thyroid-stimulating hormone (TSH) values were normal at that time. She reports that she has been eatinAg poorly and has lost 5 lb since you saw her at the beginning of the summer.

Of the following, the MOST important laboratory studies to obtain at this time are

A. complete blood count and erythrocyte sedimentation rate

B. duodenal biopsy for cryptic celiac disease

C. measurement of cortisol and adrenocorticotropic hormone

D. measurement of free thyroxine and TSH

E. mononucleosis spot test and liver function study

Answer

C

The malaise, fatigue, weight loss, abdominal discomfort, and tanned skin reported
for the girl in the vignette are signs of Addison disease. Adrenal insufficiency causes an
elevation in serum potassium concentrations, decrease in serum sodium concentrations, and
shifts in muscle electrolyte concentrations that result in weakness, myalgias, and
gastrointestinal symptoms. Skin pigmentation is increased by high concentrations of
adrenocorticotropic hormone. The presence of both autoimmune hypothyroidism and suspected
adrenal insufficiency in this girl suggests the diagnosis of autoimmune polyglandular syndrome
type 2. The genetic defect in this disorder is not yet known, but the girl is at risk for other
endocrine autoimmunities, including ovarian failure and diabetes.

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Q 2-3


A 12-year-old girl with a past medical history of brittle asthma presents with a history of loss of weight, dizziness and palpitations over the past few weeks. Her maternal grandmother had ‘thyroid problems’. On examination she looks flushed with warm peripheries, pulse rate 110 bpm. She has a smooth goitre 5 6 cm with an audible bruit.

2. What ONE investigation would be MOST useful in establishing a diagnosis? 

a- Thyroid stimulating antibodies
b- Ultrasound scan of the neck
c- Thyroid function tests (TSH & Free T4)
d- Fine needle biopsy of the goitre
e- Antithyroid peroxisomal antibodies

3- As part of your management plan which ONE of the following would you recommend: 

a- Radioactive iodine treatment
b- Surgical removal of the thyroid gland
c- Pharmacotherapy with carbimazole
d- Beta blockade with propanolol
e- Lugol’s iodine

Answers


2. c. Thyroid function tests
3. c. Pharmacotherapy with carbimazole

Graves’ disease: Graves’ disease describes a form of thyrotoxicosis caused by the presence of thyroid stimulating antibodies resulting in excess thyroid hormone secretion.

Graves’ disease is much more common in females (between 5:1 and 10:1 F:M ratio) and is associated with a strong family history of thyroid disease.

Typically Graves’ disease presents with weight loss, heat intolerance, sweatiness, palpitations, diarrhoea, anxiety and psychosis e all signs of a hyper-metabolic state. The initial investigation is a thyroid profile to include free thyroxine (FT4) and thyroid stimulating hormone (TSH) levels.

The FT4 will typically be elevated with suppressed TSH levels confirming thyrotoxicosis.

Other routine investigations include an USS of the neck (to rule out any “hot” nodules), and TSI (thyroid stimulating immunoglobulins).

Radio-iodine scans or fine needle biopsies should only be requested in the light of unusual clinical findings such as a nodule.

Free triiodothyronine (FT3) may be useful in diagnosing rare ‘T3 toxicosis’. Antithyroid antibodies
may be strongly positive (often seen in Down syndrome), and lead to eventual spontaneous resolution of
the disease to the hypothyroid state e “Hashitoxicosis”.

First line treatment is pharmacotherapy with a thionamide drug e.g. carbimazole/methimazole or propylthiouracil (PTU). 

PTU is recommended as a second-line treatment if side effects prevent the use of carbimazole.

The two treatment options are :

 (1) ‘block and replace’ to reduce the hormone production with large doses of carbimazole until the patient is rendered ‘hypothyroid’ followed by added thyroxine tablets versus

(2) the ‘titration’ method which effectively titrates the amount of thionamide given to maintain euthyroidism.

There is currently a national trial to establish the relative benefit of these two regimens. In any case close attention to the full blood count during treatment is required, to detect agranulocytosis, and all families are
given instructions to attend urgently if unexplained fever and sore throat occurs on treatment.

Beta-blockade in the first 6 weeks reduces the symptoms of thyrotoxicosis but is contraindicated in the presence of asthma.

 Lugol’s iodine can produce rapid reduction in thyroxine levels in thyrotoxiccrisis. It is important to discuss all later definitive treatment options (radio-iodine/surgery) with the patient.

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Q : 4-6


A 5-year-old Asian boy is referred to the paediatric endocrinology. He has previously been well however 10 months earlier he was noticed to have developed pubic hair, which has been increasing steadily. He has an adult type body odour, and he has developed acne on his face. On examination you note pubic hair and penis Tanner stage 3, testicular volumes of 5 ml bilaterally. Abdominal and neurological examinations are normal.

Q6. How would you proceed with this patient? Select ONE answer only please.

a Perform a gonadotrophin-releasing hormone (GnRH) test and measure testosterone levels
b Reassure parents that this is likely to be a variant of normal as some Asian children can be quite hirsute
     and appear more virilized


c Arrange to see the family back in outpatients in 6 weeks to review the child and calculate his growth
     velocity before requesting any tests
d Perform an HCG test with androgen levels before and after stimulation
e Perform a short Synacthen test with measurement of adrenal androgens and 17 alpha hydroxyprogesterone

5. Which TWO of the radiological tests below would you perform? Select TWO answers only please.

a X-ray head
b Testicular ultrasound scan
c MRI hypothalamus and pituitary
d CT chest
e X-ray of the hand and wrist for bone age
f Ultrasound scan of the adrenal glands

6. All the following are possible causes of precocious puberty EXCEPT: Select ONE answer only please.

a McCuneeAlbright syndrome
b Anorexia nervosa
c Hypothyroidism
d Hyperthyroidism
e Neurofibromatosis type 1

Answers:


4 a. Perform a GnRH test and measure testosterone levels
5. c. MRI Head, f. Ultrasound scan (USS) of the adrenal
glands
6. b. Anorexia nervosa

Precocious puberty in males: precocious puberty refers to the onset of puberty outside of the lower limit of the normal age range. For boys this is considered as puberty at an age less than 9 years.

The first sign of progression into puberty is the enlargement of the testicles, and a volume of 4 ml heralds the onset of puberty.

It is important to consider premature adrenarche/pubarche which is considered a variation of normal but where testicular enlargement is not seen.

 Clinicians should also be aware of virilizing conditions which result in gonadotrophin-independent
precocity such as non-salt-losing congenital adrenal hyperplasia, androgen producing tumours where testicles may be smaller than normal, familial testotoxicosis and McCuneeAlbright syndrome with polyostotic fibrous
dysplasia of bone and caf e au lait patches.


Precocious puberty is classified into gonadotrophindependent precocious puberty (GDPP) sometimes called
‘true’ or central precocious puberty, and gonadotrophinindependent precocious puberty (GIPP) pseudo-precocious puberty, In GDPP puberty progresses in a concordant manner and is driven by the activation of the hypothalamic-pituitary axis, while GIPP tends to progress in a discordant manner.

GDPP in boys is rarely idiopathic and as such all boys should be investigated appropriately by a qualified clinician to rule out an underlying cause. Investigations should
include:

Blood: testosterone, FSH/LH in response to GnRH stimulation
Radiology: Bone age, MRI Head.

It is important to determine the underlying cause and treat this as appropriate. Depending on the age of presentation it may be important to stop the progression of puberty to reduce psychological distress and to allow longer to grow before epiphyseal fusion.

Treatment may be given with long-acting GnRH analogues (in combination with cyproterone acetate initially) until the family and child are psychologically prepared for puberty and a reasonable final
height likely to be achieved.

Anorexia nervosa causes delayed puberty as the low body mass index (BMI) does not allow for a normal progression of puberty.

 Patients with anorexia nervosa have multiple hormone disturbances including low levels of FSH and LH.


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Q 7, 8:


You review a 6-year-old girl in your clinic with delayed motor milestones and mild learning disability. She was born at term, weighing 3.3 kg, to a 29-year-old mother following an uncomplicated pregnancy. She reports that the child had problems with feeding for the first few years of life, and in particular
needed nasogastric tube feeds for the first 18 months of life.

This has now settled and she is feeding very well. On examination the child is overweight with a weight on the 91st centile and a height on the 9th centile. She has short stubby fingers, one caf e-au-lait birthmark on her abdomen and almond shaped eyes. You suspect the child has Pradere Willi Syndrome.

7. What is the likely underlying genetic cause of this condition? 

a Triploidy on chromosome 21
b Maternal uniparental isodisomy of chromosome 7
c Deletion of paternal copies of genes on chromosome 15
d Monosomy X
e Duplication of the long arm of the X chromosome

8. Which ONE of the following would you NOT recommend as part of the therapy for this child?

a Dietician review
b Physiotherapy
c Speech and language therapy
d Growth hormone therapy
e Cardiology review


Answers:


7. c. Deletion of paternal copies of genes on chromosome 15

8. e. Cardiology review

PradereWilli Syndrome (PWS): PWS is a rare genetic disorder involving chromosome 15. The underlying genetic cause is loss of the paternal PWS region on chromosome 15 (15q11e13) either by deletions, maternal uniparental disomy (UPD) or translocations. The incidence of PWS is
approximately 1 in 20,000.

During pregnancy mothers may report decreased foetal movement and a breech presentation. Most babies have a good birth weight, but are noted to have the following features: hypotonia, difficulty establishing respiration, poor feeding, hypogonadism. Babies with PWS are classically described as having short stubby fingers, and almond shaped eyes. Problems relating to poor feeding persist through infancy, often manifesting as poor weight gain. In early childhood delayed milestones become evident. By the age of about 3 years children with PWS usually begin to display signs of hyperphagia and exaggerated food seeking behaviour.
If unchecked this results in an excessive weight gain.

A significant number of patients go on to develop complications including obstructive sleep apnoea, poor sleep patterns, scoliosis, short stature, poor coordination, and delayed milestone with possible mild to moderate learning difficulties.

Management of PWS patients and their families involves a multi-disciplinary team involving: geneticists, endocrinologists, physiotherapists, occupational therapists, dieticians, community physicians, speech and language therapists. Endocrine management of patients with PWS focuses on preventing excessive weight gain, and optimizing body composition in childhood; and addressing
hypogonadism and infertility issues in adolescence and as
a young adult.

Growth hormone (GH) therapy is used primarily for the improvement of body composition. The increase in muscle mass improves body tone, and helps overcome the delay in developmental milestones, and this helps with weight control. Height velocity is also sometimes noted to improve, particularly in the first year of therapy. Some unexpected deaths have been reported in patients with PWS on GH. As part of the pre-treatment work-up it is advised to perform an antero-posterior (AP) spinal X-ray to assess for scoliosis
and a polysomnograph, however abnormal results would not be an absolute contraindication to treatment, though more stringent follow up may be prudent. The other genetic disorders listed above include Down
Syndrome (Trisomy 21) which can present like PWS in the neonatal period, with a floppy infant with poor feeding.

These children usually have other features characteristic of Down Syndrome, including a single palmer crease and upslanting palpebral fissures. They have a tendency to gain weight as they grow older, but not to the same degree as children with PWS.

Maternal uniparental disomy on chromosome 7 results in RusselleSilver Syndrome, which presents with a small baby with intra-uterine growth retardation. These babies usually have small triangular faces, and clinodactyly (incurving) of the 5th finger. They tend to remain small throughout their lives.

Monosomy X results in Turner Syndrome (karyotype X0), which presents phenotypically as a girl with short
stature, webbing of the neck and a wide carrying angle. Duplication of the long arm of the X chromosome will result in a phenotype quite similar to Turner Syndrome, with short stature, webbing of the neck, but in addition there are other associated features like development delay, microcephaly, and seizures.

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Q 9:

You are asked to see an 8-year-boy in whom medulloblastoma was diagnosed at age 3 years. Treatment at that time consisted of chemotherapy and craniospinal irradiation. During the past year, he grew 2 cm, although he is eating normally, and his weight is appropriate for height. Despite spinal irradiation, the upper-to-lower segment ratio is normal for his age.

Of the following, the MOST likely diagnosis is

A. acquired growth hormone deficiency
B. chemotherapy-induced renal failure
C. Cushing syndrome
D. irradiation-induced epiphyseal fusion
E. tumor recurrence

Answer:

A

Cranial irradiation has gradual deleterious effects on pituitary hormone secretion due to damage
to hypothalamic releasing factors. Growth hormone, thyroid-stimulating hormone (TSH), and the
gonadotropins tend to be affected most, with adrenocorticotropic hormone (ACTH) relatively
protected, although all neuroendocrine function can be disturbed by cranial irradiation. The boy
described in the vignette has a normal upper-to-lower segment ratio, indicating that his spine still
is growing despite radiation. This suggests that his growth attenuation is due to an acquired
endocrine deficiency of either growth hormone or thyroid hormone. Early in the deficiency
process, if this child were tested with standard stimuli to growth hormone release, he might be
able to release growth hormone, but if continuous nocturnal growth hormone sampling were
performed, diminished and disorderly spontaneous growth hormone release might be
documented. With time, secretory function to stimuli also would be lost.

Renal failure from chemotherapy would be expected to have manifested earlier and been
recognized by this time. Cushing syndrome is a possible but rare cause of growth attenuation
caused by adrenal or pituitary oversecretion of cortisol or ACTH, respectively, and is not
associated with cranial irradiation. Irradiation-induced epiphyseal fusion at the level of the spine
is common following spinal irradiation. The epiphyseal fusion of the spine results in an increased
lower segment (measured from the pubic symphysis to the heel) compared with the upper
segment because the legs continue to grow while the spine does not. This results in an
increased lower segment-to-upper segment ratio. Tumor recurrence has more obvious
manifestations than statural growth attenuation

MCQs In Hepatobiliary Diseases

Q 1:

A 13-year-old girl presents with a 4-month history of right upper quadrant abdominal pain. The

pain occurs at different times, but seems to strike primarily after meals, more frequently after

she eats fatty foods. In your office, she complains of intermittent pain to deep palpation of the

right upper quadrant. Complete blood count, alanine aminotransferase, alkaline phosphatase,

serum bilirubin, amylase, and lipase findings are normal. Abdominal ultrasonography shows no

evidence of stones or gallbladder thickening. Upper endoscopy and biopsy results are normal,

with no evidence of ulcers or gastritis.

Of the following, the MOST appropriate next step is

A. abdominal computed tomography scan

B. endoscopic retrograde cholangiopancreatography (ERCP)

C. nuclear medicine gallbladder emptying scan with fatty meal

D. psychiatric consultation to rule out depression or anxiety

E. referral to an acupuncturist for chronic pain management

Answer

C


The colicky abdominal pain in the right upper quadrant after the ingestion of fatty foods
described for the patient in the vignette is strongly suggestive of gallbladder disease. However,
results of laboratory studies and abdominal ultrasonography are within normal limits. Because of
the symptom profile, the possibility of chronic acalculous cholecystitis with gallbladder dysmotility
should be considered, and the test of choice to evaluate for this condition is a radionuclide
gallbladder emptying scan. If the patient has markedly delayed gallbladder motility, consideration
should be given to performing a cholecystectomy.

Classically, cholecystitis occurs when the gallbladder is inflamed and irritated by gallstones.
Such gallstones typically are classified as cholesterol stones and pigment (bilirubin) stones. Risk
factors for cholesterol stones include older age, female sex, pregnancy, and overweight. Risk
factors for pigment stones include parenteral nutrition and hemolysis (as seen in children who
have sickle cell disease). For patients who have stones, cholecystitis commonly presents with
pain in the right upper quadrant, epigastrium, and back. Fever, jaundice, and abnormal liver
enzyme values also may be present, especially if a gallstone is in the biliary tree
(choledocholithiasis).

MCQs In Pediatric Hypertension

Q1:

You are evaluating a 16-year-old boy whom you have known since early childhood. He is

complaining of headaches over the past 2 weeks. He has a history of asthma, which has been

well controlled, and he is an otherwise healthy member of the varsity football team at school. He

has had a significant weight gain of 30 lb (13.5 kg) since his visit to you 1 year ago. He denies

using illicit or prescription drugs. On physical examination, he appears very muscular and has a

blood pressure of 180/120 mm Hg. You repeat the measurement using a leg cuff to ensure

adequate cuff size and obtain the same result.

Of the following, the BEST management plan is

A. angiotensin-converting enzyme inhibition as an outpatient

B. beta blocker therapy as an outpatient

C. diuretic therapy as an inpatient

D. repeat blood pressure measurement in 1 to 2 weeks

E. vasodilator therapy as an inpatient

A nswer

E



The patient described in the vignette has a significantly elevated blood pressure that
involves marked and reproducible systolic and diastolic hypertension. The best management
plan is to monitor his blood pressure while the cause is ascertained and treatment begun, which
involves admission to the hospital and initial treatment with an intravenous antihypertensive
agent. The goal of such therapy is to reduce the blood pressure by 25% or less over the first 8
hours and gradually normalize it over the next 48 hours to avoid complications (eg,
cerebrovascular accident).

The choice of chronic antihypertensive therapy depends, in part, on the cause of the
hypertension, but for immediate short-term management, vasodilators (eg, calcium channel
blockers, hydralazine, nitroprusside) are useful. These agents reduce the afterload against
which the left ventricle pumps, thereby reducing its work and oxygen consumption. Alternatively,
short-acting beta blockers could be used in the acute setting. When using beta blockers,
however, the clinician must bear in mind their potential complications, including exacerbation of
underlying asthma. Of importance, pharmacologic management of severe hypertension and
hypertensive crisis should use medications that can be titrated to effect readily and have a fast
onset of action. Diuretics, particularly the thiazide class, often are used as first-line
antihypertensive agents for those who have mild or moderate hypertension that can be
controlled on an outpatient basis. These may be used in combination with other agents, including
but not limited to angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, if
adequate control is not obtained with a single agent. The significant hypertension reported for the
boy in the vignette requires immediate action; repeating the blood pressure measurement in 1 to
2 weeks is not appropriate.


Q2 :


Before antihypertensive therapy begins, secondary causes of hypertension should be ruled out.
Laboratory findings that suggest an underlying cause of hypertension include:

A. a decreased serum potassium level.
B. an increased urinary catecholamine level.
C. an increased blood cortisol level.
D. All of the above.

Answer

D

All these investigations are needed.


Low serum potassium levels in a hypertensive patient suggest primary aldosteronism. Elevated urinary catecholamines suggest a pheochromocytoma; other signs and symptoms of this tumor include weight loss, episodic flushing, and sweating. Elevated serum cortisol levels suggest Cushing syndrome; the patient is also
likely to have a round (moon) face and truncal obesity.

Secondary hypertension requires treatment of the underlying cause; supplementary antihypertensive drug
therapy may also be needed.

-------------------

Q2- 3:


A 12-year-old boy presented with a 2-year history of intermittent tachycardia. More recently he had developed headaches, hot flushes and weight loss. He denies any drug misuse. He was hypertensive with a blood pressure of 218/ 134 mmHg. Investigations showed a normal cardiac ECHO
and ECG showed sinus tachycardia of 130/minutes and ventricular ectopics.

Full blood count, Thyroid function and LFTs were normal. Urinary normetadrenaline levels were raised.
Abdominal ultrasound scan showed an abdominal mass. CT scan identified this as a 5 cm 4 cm vascular mass arising from Organ of Zuckerkandl. Meta-iodobenzylguanidine (MiBG) scan showed increased uptake in an isolated avid lesion at the bifurcation of the aorta.

(Q 2) What is the most likely diagnosis?

A- Grave’s disease
B- Phaeochromocytoma
C-  Cushing’s disease
D-Neuroblastoma

(Q 3) What is the prognosis?

A- Good outcome but requires lifelong hormone replacement
B- 5-year survival rates of <10%
C-  Potentially curable with surgical resection
D- Invariably fatal, no known cure

Answer :

2 : B
3: C


Phaeochromocytoma is a tumour of the sympathetic nervous system that presents with episodes of tachycardia/ palpitations, headaches, sweating and pallor. These are precipitated because of catecholamine-releasing properties of this tumour. Symptomatic relief can be achieved with the use of alphaand
betablockers, such as bisoprolol and phenoxybenzamine respectively. Doses should be titrated up to effect
.
Tumours are most commonly located in the adrenal glands, but as in this case, have a number of extra-renal
sites where they may be found. The Organ of Zuckerkandl is not normally visualized on scanning, and if seen, is suggestive of pathology such as neuroblastoma or phaeochromocytoma.

In this age group, phaeochromocytoma is a much more likely diagnosis. Undiagnosed, the tumour can be fatal due to the cardiovascular risk. Early diagnosis lends itself to curative treatment by surgical resection.

Approximately 4% of paediatric tumours are malignant, they can be locally invasive or metastazise to lung, bone, liver and brain. It is more common in boys than girls with a ratio of 2:1.

The differential diagnosis should include primary hypertension, thyrotoxicosis, co-arctation of the aorta and
neuroblastoma. Investigations should be performed in order to make a comprehensive diagnosis, to include blood pressure monitoring, cardiac assessment, radiological imaging with ultrasound or CT and urinary catecholamines.

MCQs In Breastfeeding

Q 1:

An 18-year-old girl presents to your school-based clinic with her 3-day-old newborn for an early

discharge follow-up visit. The young mother looks very tired and is tearful. She states that she is

trying to breastfeed but has to supplement the feeding with a bottle because she “doesn’t have

any milk.”

Of the following, the MOST appropriate course of action is to

A. call the infant’s grandmother immediately to come to the office

B. recommend that she stop breastfeeding

C. schedule a same-day visit with a lactation consultant in the office or hospital

D. schedule another follow-up visit in 1 month

E. suggest that she attend prenatal classes for her next pregnancy


Answer

C


For the young mother described in the vignette, community resources such as referral to a
lactation consultant at the hospital where she delivered or in the community, low-cost infant care
classes, videotapes of infant care topics, and child care resources for resumption of school or
work are important. Cessation of breastfeeding almost never is indicated. Calling the
grandmother to help may be appropriate if there are signs of postpartum depression, as might
rehospitalization or referral to a mental health professional, but such action is unlikely to
empower the young mother. Recommending a prenatal visit for subsequent pregnancies does
not help the mother with her current difficulties in caring for her infant. Delaying a follow-up visit
for 1 month is inappropriate for a breastfed infant who has risk factors for poor weight gain and
for this mother, who is at risk for postpartum depression.

MCQs In Pediatric Otolaryngology Part II

Q 1:

An 18-month-old girl is brought to the emergency department by her mother, who reports that

the girl has had a high fever and difficulty breathing for 1 day. She has had a barking cough for

the last 3 days but had been afebrile and breathing comfortably until today. Her immunizations

are up to date. Her temperature is 104ºF (40°C), her respiratory rate is 50 breaths/min, she

exhibits inspiratory stridor and a brassy cough, and she appears ill. Lung examination reveals

decreased breath sounds bilaterally with transmitted upper airway sounds. An anterior neck

radiograph shows subglottic narrowing and a ragged tracheal air column.

Of the following, the MOST likely diagnosis is

A. acute viral laryngotracheitis

B. angioneurotic edema

C. bacterial tracheitis

D. epiglottitis

E. retropharyngeal abscess

Answer

C


Bacterial tracheitis is a serious infection causing significant upper airway obstruction. It typically
occurs as a secondary staphylococcal infection following viral laryngotracheobronchitis, but it
also may develop as a primary infection without preceding symptoms of croup. Clinical features
include a toxic appearance, with high fever, tachypnea, and brassy cough, as described for the
girl in the vignette. Airway obstruction can be pronounced due to purulent airway secretions, and
lower airway disease such as wheezes or rales also may be present. Neck radiographs often
reveal a ragged air column or subglottic narrowing, and peripheral white blood cell counts
frequently are elevated. Treatment consists of intravenous antibiotics and airway control.
Endotracheal intubation frequently is required until the infection is treated and the severity of
secretions improves.

Acute viral laryngotracheitis typically causes a barking cough with occasional inspiratory
stridor, but affected children usually do not have high fevers or appear ill.

Angioneurotic edema is a response to histamine release with an allergic reaction and often is accompanied by
urticaria. Upper respiratory tract mucosae become edematous, so inspiratory stridor may be
present, but fever is unlikely.

Children who have epiglottitis appear toxic and have significant airway compromise and inspiratory stridor due to the swollen epiglottis. Cough is rare. Lateral neck radiograph reveals an enlarged epiglottis (“thumb sign”) rather than a ragged air column.

 Further, epiglottitis is unlikely in a child whose immunizations are up to date. A retropharyngeal abscess can develop with pharyngitis, and inspiratory stridor and respiratory distress with high fever is typical. The lateral neck radiograph reveals widening of the prevertebral tissues, and computed tomography scan of the neck can confirm the presence of the abscess.

----

Q 2:


A 2-year-old boy has had a purulent drainage from the right nostril for a week. He is afebrile and has had no associated symptoms, such as cough. Which of the following is the most likely diagnosis?

(A) sinusitis
(B) nasal polyps
(C) an upper respiratory infection
(D) a foreign body in the right nostril
(E) allergic rhinitis

Answer :

(D)

 Children frequently insert foreign bodies into the nose. Initial symptoms are local obstruction, sneezing, and pain. Subsequently, there is swelling and infection leading to a purulent, malodorous, and often bloody discharge.

The infection clears after removal of the foreign body. Nasal polyps cause obstruction of the nasal passages, hyponasal speech, and mouth breathing; gray, grape-like masses can be visualized on nasal examination. An
upper respiratory infection is usually suggested by a careful history. Initial symptoms include a scratchy throat, followed by development

of thin nasal discharge and sneezing. Myalgia, low-grade fever, headache,malaise, and decreased appetite may be present. By the 2nd or 3rd day, the discharge becomes thicker and more purulent. Cough is common. Symptoms usually resolve by 7–10 days. Adolescents with sinusitis may have classic
symptoms of headache and sinus tenderness.

In children, cough and nasal discharge arecommon; the cough is worse when supine. If upper respiratory infection symptoms persist without improvement for >10 days, sinusitis should be considered. A more acute form may occur, with a shorter duration and more severe symptoms such as fever >39°C, purulent nasal
discharge, headache, and eye swelling. Children with allergic rhinitis present with sneezing,
clear watery, rhinorrhea, and itching of the nose, palate, pharynx, and eyes. Itching, redness, and
tearing of the eyes may be present. This occurs in response to exposure to an allergen such as pollen, mold spores, and animal or mite antigens.

MCQs In Pediatric GIT Emergencies

Q 1:

A 12-year-old girl presents with an episode of “feeling faint” and melena. On physical

examination, you note a gallop rhythm and mild, nonspecific abdominal tenderness. Stool is

guaiac-positive. Laboratory analysis demonstrates anemia, with a hematocrit of 18% (0.18). You

administer fluid resuscitation and packed red blood cells, and the patient’s hemodynamic status

stabilizes.

Of the following, the next MOST appropriate diagnostic test is

A. angiography

B. barium contrast upper gastrointestinal tract radiography

C. Doppler ultrasonography of portal and esophageal veins

D. upper gastrointestinal endoscopy

E. video capsule study

Answer

D


Upper gastrointestinal endoscopy remains the initial diagnostic test of choice for most upper
gastrointestinal hemorrhages because it is not only highly sensitive for mucosal lesions such as
peptic ulcers and varices, but also allows the endoscopist to treat any bleeding lesions.


Upper gastrointestinal bleeding (bleeding proximal to the ligament of Treitz) may be either acute
(presenting with melena and hemodynamic instability) or chronic (presenting with anemia).
Common causes include gastric or duodenal ulcers, chronic gastritis, esophageal or gastric
varices, and reflux esophagitis. Vascular lesions such as arteriovenous malformations or
telangiectasias (as seen in hereditary hemorrhagic telangiectasia [Osler-Weber-Rendu
disease]) also can present with chronic gastrointestinal blood loss. Dieulafoy lesion is another
cause of upper gastrointestinal hemorrhage that presents with massive and recurrent bleeding
and is caused by an abnormally enlarged arteriole in the gastric cardia or duodenum that
periodically bleeds into the gastric lumen.

MCQs In Neonatal Infections

Q 1:

During your morning nursery rounds, you find you have a new patient who was born to a mother

infected with human immunodeficiency virus (HIV). You introduce yourself to the mother, and

she asks you about any precautions she needs to take in the care of her newborn due to her

HIV infection.

Of the following, you are MOST likely to tell the mother that she should

A. add a teaspoon of liquid bleach to the infant’s bath water

B. avoid breastfeeding

C. avoid sharing eating utensils

D. take no specific action

E. wear gloves while changing diapers

Answer

B

The risk of transmission of human immunodeficiency virus (HIV) to an infant from an infected
mother without interventions is approximately 15% to 25%. Breastfeeding by an infected mother
increases the risk by 5% to 20% to a total of approximately 20% to 45%. Therefore, in countries
where safe alternatives to breastfeeding are readily acceptable, feasible, available, affordable,
and sustainable, avoidance of all breastfeeding by HIV infected mothers is recommended. In
countries where there are no safe alternatives to breastfeeding, the World Health Organization
supports exclusive breastfeeding for the first 6 postnatal months due to the higher death rates
for children who receive formula.

--------------

Q 2:


A woman who has chorioamnionitis and had a positive group B streptococcal (GBS) screening
culture at 36 weeks’ gestation delivers an infant at term. The infant becomes ill in the first 4
hours after birth, demonstrating tachypnea, inability to maintain temperature, and poor perfusion.
He is admitted to the intensive care nursery. On physical examination, there are retractions of
the chest wall, coarse and shallow breath sounds, and delayed capillary refill. There is no heart
murmur or cyanosis, but arterial oxygen saturation determined by pulse oximetry is only 80% on
room air.
Of the following, the clinical manifestation that is observed MORE often in early-onset than lateonset
GBS infection is

A. cellulitis
B. meningitis
C. osteomyelitis
D. pneumonia
E. septic arthritis

Answer

D

Early-onset GBS typically presents in the first 24 hours after birth as a systemic sepsis
syndrome that involves apnea, respiratory distress from pneumonia or pulmonary hypertension,
shock, and occasionally meningitis (<10% of cases).

 Late-onset GBS infection presents after the first 7 days of postnatal life, most commonly in the third or fourth week. Meningitis is far more common in late-onset GBS infection, but other presentations include septic shock and focal infections such as cellulitis, arthritis, and osteomyelitis.

The infant described in the vignette has early-onset GBS infection, with tachypnea and
temperature instability prompting an evaluation for sepsis. His mother, who is a GBS carrier, had
chorioamnionitis at the time of delivery, which increases his risk for acquiring GBS. The
respiratory distress may be due to pneumonia that is indistinguishable from hyaline membrane
disease on chest radiography. In cases of GBS bacteremia, a GBS toxin also may mediate
pulmonary vascular constriction and cause pulmonary hypertension, even when pneumonia is
not present. With GBS bacteremia or septicemia, approximately 10% of infants have
corresponding meningitis

-----------------

Q3:


A mother brings in her child because she found a tick on the girl’s shoulder yesterday and is
worried about Lyme disease. The mother found a site on the Internet that suggests her daughter
needs an antibiotic called ceftriaxone. You assure the mother that only very few children who
suffer tick bites actually develop a tick-associated disease.
Of the following, the manifestation of Lyme disease for which ceftriaxone administration is MOST
appropriate is

A. acute arthritis
B. carditis
C. disseminated erythema migrans
D. isolated facial palsy
E. peripheral neuropathy

---------------

Q  3-6 :



A 10-day-old boy presents to the emergency department with a 1-day history of lesions on his lips. He was born at 38 weeks by Caesarean section because his mother had previously had a Caesarean section. The pregnancy was uneventful; in particular there was no history of Group B Streptococcus or herpes simplex virus infection. The baby was discharged home on day 2, feeding well. He developed sticky eyes on day 4, which his mother treated with overthe- counter medication. Otherwise, there had been no concerns and he continued to feed well.

On the morning of presentation, his mother had noticed yellow crusty lesions on his upper lip and at the angle of his mouth. Although feeding well, he seemed more unsettled
than normal. At triage, his observations were as follows:

Axillary temperature 36.4 1C
Heart rate 140 beats per minute
Central capillary refill time less than 2 s
Respiratory rate 35 breaths per minute.

Clinical assessment revealed the baby to be unsettled, and he disliked being examined. Cardiovascular, respiratory and abdominal examination was unremarkable. He had a yellow crusty lesion at the angle of his mouth and a small one on his central upper lip. There was yellow discharge from both eyes, but his conjunctivae were normal. His skin was diffusely erythematous and seemed to be tender. He was well hydrated.

3. What is the most likely diagnosis?

(a) Herpes simplex infection
(b) Neonatal bullous impetigo
(c) Neonatal erythema toxicum
(d) Staphylococcal scalded skin syndrome
(e) Toxic epidermal necrolysis

4. What is the most likely pathogen?

(a) Group B Streptococcus
(b) Human herpes virus 6
(c) Staphylococcus aureus enterotoxin
(d) Staphylococcus aureus epidermolytic toxins A and B
(e) Streptococcus pyogenes exotoxin




5. What would be the most appropriate treatment?

(a) High-dose oral flucloxacillin
(b) Intravenous benzylpenicillin and flucloxacillin
(c) Intravenous aciclovir
(d) A swab lip lesion and await sensitivities before
commencing the appropriate antibiotic
(e) Topical fucidin

6. What is the usual natural progression of this condition if treated appropriately and promptly?

(a) Drying of the erosions followed by desquamation and healing within 14 days
(b) The formation of pustules that burst, leaving hyperpigmented macules
(c) Septicaemia leading to circulatory collapse, with a high rate of morbidity and mortality
(d) Stripping of the epidermis followed by severe scarring with loss of the nails and hair
(e) Uncomplicated healing but a recurrence of the lesions precipitated by minor illness

MCQs In Pediatric Emergency- Part II

Q 1:

You are evaluating a 3-year-old boy in the urgent care clinic for fever. His mother tells you that

he had been well until yesterday, when he had a temperature that she recorded by oral

thermometer at 103°F (39.5°C). He has had a clear nasal discharge, cough, and one episode of

emesis. At the time of your evaluation, the patient is eating ice chips from a cup that he is holding

while he is sitting on the bed. He has a temperature of 102.7°F (39.3°C), a heart rate of 140

beats/min, a respiratory rate of 30 breaths/min, and a blood pressure of 110/66 mm Hg. He has

coarse breath sounds with good air movement bilaterally. His pulses are strong throughout. His

capillary refill time is between 3 and 4 seconds in his hands and 2 seconds in his feet.

Of the following, the BEST plan of management is

A. blood pressure measurement and pulse oximetry in the four extremities

B. echocardiography for coarctation of the aorta

C. empiric intravenous antibiotics for suspected bacteremia

D. inotropic therapy with dopamine for shock

E. repetition of the perfusion examination with the patient supine and hands warmed

Answer

E


The febrile child in the vignette has delayed capillary refill in his hands, but not in his feet,
which can be explained by the ice chips that he is holding. His perfusion, and specifically his
capillary refill, should be re-evaluated when his hands have been warmed. A febrile patient may
have diminished perfusion, but a differential capillary refill is unexpected. All other aspects of his
physical examination and behavior suggest fever without diminished perfusion, and neither
empiric antibiotics nor inotropic therapy is warranted. Coarctation leads to differential blood
pressure and pulse findings when extremities that receive their blood flow proximal to the area of
coarctation are compared with those that are distal. Blood pressure measurement and pulse
oximetry in all four extremities are redundant tests in a 3-year-old child and would not yield
relevant clinical information.

MCQs in Dysmorphology

Q1:

A mother who is new to your area brings her 6-year-old daughter to your office for evaluation

due to poor school performance and behavioral concerns. The child is in the first grade and is

struggling with math and reading. The mother says that the girl is “hyper” and does not play well

with other children. Despite almost being hit by a car recently, she repeatedly runs into the road

in front of their house. On physical examination, you note that the child’s height, weight, and

head circumference are less than the 10th percentile. She has short palpebral fissures, a

smooth philtrum, and a thin upper lip .

Of the following, the MOST likely diagnosis is

A. Down syndrome

B. fetal alcohol syndrome

C. hypochondroplasia

D. Russell-Silver syndrome

E. 47,XXX

Answer

B


The behavioral and physical features described for the girl in the vignette are most consistent
with fetal alcohol syndrome (FAS). FAS is at one end of a spectrum of abnormalities associated
with prenatal exposure to alcohol termed “fetal alcohol spectrum disorders” (FASD)


Children who have FAS typically have height, weight, and occipitofrontal circumference
(OFC) below the 10th percentile from birth and continue to grow slowly. Characteristic facial
features include midface hypoplasia (including flat nasal bridge and epicanthal folds), short
palpebral fissures, relatively smooth philtrum, and a narrow upper lip that has a poorly defined
“cupid’s bow” (Item C40A). Psychomotor development is delayed, and there is a typical pattern
of both cognitive and behavioral abnormalities, including poor impulse control and poor judgment.
An FASD diagnosis should be ascertained as early as possible to ensure the best possible
outcome for affected children. Management issues include parent education, careful physical
examination (with attention to vision, hearing, the heart, the kidneys, and the musculoskeletal
system), appropriate referrals, neuropsychological testing with proper school placement, and
anticipatory guidance

------------

Q 2


A neighbor approaches you regarding her concerns about her newborn grandson. Her 23-year old
daughter, the mother of the baby, had an uneventful pregnancy, labor, and delivery, and “all
of her prenatal tests were normal.” The baby has been in the normal newborn nursery for 3
days, and he is feeding poorly. He “just can’t seem to get the hang of sucking.” She thinks he is
probably just “lazy,” and she describes him as feeling like a “rag doll” when she holds him. She
has noticed that he has “a lot of skin” at the back of his neck, and his fifth fingers are “crooked”
. She thinks his penis is small. You suggest that she alert the baby’s physician to
her concerns.


Of the following, the MOST likely diagnosis is
A. 22q11 deletion
B. achondroplasia
C. cri-du-chat syndrome
D. Down syndrome
E. Prader-Willi syndrome
Copyright

Answer

D



The hypotonia, poor feeding, excess nuchal skin, incurving (clinodactyly) of the fifth fingers, and
questionably small phallus described for the infant in the vignette are consistent with Down
syndrome (DS). Hypotonia and excess nuchal skin are present in 80% of newborns who have
DS, fifth finger clinodactyly is present in approximately 50% (Item C136A), and a relatively small
penis with reduced testicular volume occurs frequently.

MCQs in Pediatric RHeumatology

Q 1:

A 12-year-old girl who has juvenile idiopathic arthritis (juvenile rheumatoid arthritis) presents with

a history of chronic anemia. Her current medications include ibuprofen, methotrexate, and folic

acid. Physical examination demonstrates tenderness to palpation of the upper abdomen and a

left knee effusion. The stool is positive for occult blood. Results of laboratory studies include a

hematocrit of 28% (0.28), mean cell volume of 72 fL, reticulocyte count of 1%, white blood cell

count of 4.2x103/mcL (4.2x109/L), serum iron concentration of 14 mcg/dL (2.5 mcmol/L), and

total iron binding capacity of 400 mcg/dL (71.6 mcmol/L). Transaminases, bilirubin, amylase, and

lipase values are normal. You initiate iron therapy.

Of the following, the MOST appropriate diagnostic test at this time is

A. abdominal ultrasonography

B. abdominal spiral computed tomography scan

C. mesenteric angiography

D. radionuclide tagged red cell study

E. upper endoscopy with biopsy

Answer

E


The patient described in the vignette presents with iron deficiency anemia and guaiac-positive
stool, which suggest the presence of inflammation and bleeding of the gastrointestinal tract. It is
unlikely that she has an acute gastrointestinal hemorrhage because she is hemodynamically
stable. The differential diagnosis of chronic gastrointestinal bleeding and anemia in this patient is
broad and includes Helicobacter pylori infection, celiac disease, and Crohn disease. However,
her chronic use of ibuprofen points toward gastritis and ulcer disease due to chronic use of
nonsteroidal anti-inflammatory drugs (NSAIDs) (ibuprofen). Upper endoscopy is indicated to
establish a diagnosis of NSAID gastropathy and to exclude other conditions.

MCQs In Pediatric Dermatology

Q 1:

A 12-year-old girl presents for evaluation of areas of skin thickening, tightness, and discoloration

that developed 2 months ago. Physical examination reveals shiny, hypopigmented patches with

brown borders on the leg and ankle . The affected skin is immobile, firm, and has a

“bound-down” feeling.

Of the following, the MOST likely diagnosis is

A. lichen sclerosus et atrophicus

B. linear scleroderma

C. pityriasis alba

D. progressive systemic sclerosis

E. vitiligo

Answer:

B


Scleroderma is a rare connective tissue disease that is believed to have an autoimmune cause.
It may be categorized as localized and systemic, with the localized form predominating.
Localized scleroderma begins as areas of indurated skin that have violaceous borders. Over
time, the violaceous color is lost, and the skin takes on a waxy, ivory appearance. As the
disease remits, affected areas become atrophic and hypo- or hyperpigmented. Three clinical
patterns of localized scleroderma exist: linear scleroderma, morphea, and generalized morphea.
In linear scleroderma, as exhibited by the patient described in the vignette, lesions appear in a
bandlike distribution, typically are unilateral, and usually involve the extremities.

The abnormal tissue may span joints, resulting in diminished range of motion or deformity, and
may extend to soft tissue, muscle, or bone. In morphea, one or two discrete areas are affected,
often on the trunk. Generalized morphea is characterized by the presence of widespread or
coalescent lesions.

The treatment of localized scleroderma is difficult and may include the application of potent
topical corticosteroids or topical calcipotriene.

-----------------------------------------------------

Q 2:


A 13-year-old boy requests treatment for his acne. He is using no medications and has no known drug allergies. Physical examination of the face reveals a few small inflammatory papules and numerous blackheads and whiteheads; there is no scarring . No acne lesions are present on the chest and back. Of the following, the MOST appropriate treatment is

A. benzoyl peroxide topically
B. benzoyl peroxide topically and tetracycline orally
C. benzoyl peroxide topically and tretinoin topically
D. clindamycin topically
E. tretinoin topically

Answer

C


The patient described in the vignette has facial acne characterized by a few small inflammatory lesions and numerous obstructive lesions (ie, blackheads and whiteheads) . As a result, he requires treatment with two agents, one to control inflammatory lesions and one to control obstructive lesions. Because the inflammatory component of his acne is mild and limited to the face, it may be treated topically with benzoyl peroxide applied each morning.

Alternatives might include a topical combination product (eg, benzoyl peroxide combined with either clindamycin or erythromycin) or possibly a topical antibiotic alone (although bacterial resistance to these agents when they are used without benzoyl peroxide is common).

MCQs In Pediatric Orthopedics

Q1:

A 12-year-old basketball player presents to your office with an acute shoulder injury. He reports

that an opponent ran into his left, posteriorly outstretched arm as he was guarding him. He

developed pain in his left shoulder and has been unable to move his arm since the injury.

Physical examination reveals asymmetry of his shoulders, with increased shoulder slope on the

left. The left arm is externally rotated and slightly abducted. Fullness is palpable inferior to the left

mid-clavicle, and there is no pinprick sensation over the lateral deltoid. You place the patient’s

left arm in a sling, administer acetaminophen and codeine, and send him for radiographs.

Of the following, the MOST helpful radiographic view for diagnosing his injury is

A. anteroposterior view of the clavicle

B. anteroposterior view of the humerus

C. anteroposterior view of the left scapula

D. axillary view of the shoulder

E. lateral view of the humerus

Answer

D


Shoulder dislocations are relatively uncommon injuries in children and adolescents because of
the strength of the shoulder capsule and the glenohumeral ligaments.

Most often seen in sportsrelated trauma, these injuries should be reduced urgently in an appropriate setting.

 Most shoulder dislocations involve anterior displacement of the humeral head, resulting from posteriorly directed force on an abducted, extended arm.

The patient complains of significant shoulder pain and holds the arm slightly abducted in external rotation, as described for the boy in the vignette.

 The humeral head may be palpated inferiorly to the mid-clavicle on the affected side. The axillary nerve is the most commonly injured nerve following anterior shoulder dislocation, and its function should be assessed and documented. Axillary nerve injury results in numbness over the deltoid and the inability to abduct or extend the shoulder>




Although the diagnosis typically is based on clinical findings, radiographs are indicated for confirmation and to evaluate for any associated bony abnormalities, such as fractures of the humeral head or glenoid fossa .

 The initial radiologic evaluation should include anteroposterior, lateral, and axillary views of the shoulder. 

The axillary view is the most sensitive for seeing the relationship between the humeral head and the glenohumeral fossa. 

Humeral, scapular, or clavicular radiographs alone are not adequate to assess the location of the humeral
head.

MCQs In Growth

Q1:

During the annual health supervision visit of a 9-year-old boy, you note that he has grown very

little in the past year . He has been otherwise well. On physical examination, he has

slightly increased abdominal fat and decreased muscle mass.

Of the following, the MOST likely diagnosis is

A. celiac disease

B. constitutional delay of maturation

C. craniopharyngioma

D. hypochondroplasia

E. renal insufficiencyAnswer

C


A hallmark of short stature due to endocrine disease is central adiposity and somewhat
decreased muscle mass, as described for the boy in the vignette, which is found in growth
hormone deficiency, hypothyroidism, and Cushing syndrome. Craniopharyngioma may present
with endocrine deficiency disorders such as growth hormone and thyroid-stimulating hormone
deficiency, leading to hypothyroidism. Celiac disease and renal insufficiency usually
lead to weight loss when associated with slowing growth, but additional symptoms would be
expected. Hypochondroplasia is associated with moderate short-limbed dwarfism that begins in
early childhood.

MCQs In Pediatric Parasitic Infections

Q 1:

You are taking your family on a safari in Africa. Everyone has received hepatitis A and typhoid

immunization. Your husband is worried about “catching malaria” and asks how you are planning

to prevent this.

Of the following, the BEST chemoprophylaxis to use for malaria is

A. atovaquone

B. chloroquine

C. dapsone

D. mefloquine

E. no prophylactic medications

Answer

D


Chloroquine-resistant malaria (due to Plasmodium falciparum) is common throughout Africa,
requiring the use of other medications, such as mefloquine, doxycycline, or atovaquoneproguanil,
for chemoprophylaxis. Mefloquine is taken once weekly starting 1 week prior to travel,
once weekly while traveling, and for 4 weeks after the travel has concluded.