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A. The least likely recurrent infection caused by primary immune
deficiency is:
a. Recurrent otitis media
b. Recurrent bacterial skin infection
c. Recurrent bacterial pneumonia
d. Recurrent osteomyelitis
e. Recurrent urinary tract infection
2. All of the following are potentially treatable causes of infantile
cholestasis except:a. Galactosemia.
b. Extra hepatic biliary atresia.
c. Choledochal cyst.
d. UTI and sepsis.
e. Paucity of intrahepatic bile ducts.
3.Disproportionate dwarfism is an important feature of one of the
following conditions:a. Achondroplasia.
b. Constitutional short stature.
c. Nutritional deprivation.
d. Familial short stature.
e. Psychological deprivation.
A. 4. Which one is the characteristic infection in patients with
terminal complement (C5-C9) deficiency?
a. MRSA
b. Pneumocystis carinii
c. Meningococcus
d. Catalase-positive organisms
e. Herpes viruses
5. A contraindicated vaccine in an isolated IgA deficiency patient is:
a. OPV
b. Varicella
c. Influenza
d. MMR
e. None of the above
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6. IVIG replacement is indicated in all of the following, except:
a. X-linked agammaglobulinemia (XLA)
b. X-linked hyper-IgM syndrome
c. Chronic granulomatous disease (CGD)
d. Wiskott-Aldrich syndrome (WAS)
e. Common variable immunodeficiency
7. What etiology of sudden onset of coughing in an active infant can be
effectively ruled out with a CXR?
a. Mycoplasma
b. Parainfluenza
c. Respiratory syncytial virus
d. Foreign body aspiration
e. None of the above
8. An experienced ward nurse asks you to correct an admission order
for pertussis. Which component is incorrect?
a. "Continuous cardio respiratory monitoring".
b. "Document episodes of cyanosis or post-tussive exhaustion".
c. "Daily weights".
d. "Deep suctioning q 3h".
e. "Instruct parent regarding maximal size of feedings.
9. Which one of the following is used as a screening test in HIV infection
diagnosis?
a. Enzyme immunoassay
b. Polymerase chain reaction
c. Western-blot
d. Immune fluorescence assay
e. Viral culture
10. Which one of the following is/are not shown to be a transmission
route for HIV infection?
a. Vertical transmission
b. Breast feeding
c. Vectors
d. Blood transfusion
e. Heterosexual sex
11. In Down syndrome the following are true except:a.
b.
c.
d.
e.
An increased incidence of diabetes mellitus.
In translocation Down syndrome the total no.of chromosomes is always 47.
Mosaiscism accounts for 1-2% of cases.
The commonest form is non-disjunction.
VSD is the commonest congenital heart disease in Down syndrome.
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12. Reasons for biopsy in a patient with nephrotic syndrome include:
a. Continued proteinuria after a week of prednisone therapy.
b. Age at onset of 10 months.
c. Relapse 1 year after initial course of therapy.
d. Cholesterol level greater than 400 mg/dl.
e. Female sex because lupus is more common in females.
13. A 5-year-old girl who has sickle cell disease develops a temperature
of 39.4°C. Findings on physical examination are normal. A
complete blood count reveals: hematocrit, 30%; white blood cell
count, 14,000/mm3; and platelet count, 140,000/mm3.
Among the following, the organism MOST likely to be isolated
from culture of this child's blood is
A.
B.
C.
D.
E.
14.
An 8-month-old infant had fever to 39.4°C for 3 days without any
other signs or symptoms. He is now afebrile but has developed a
maculopapular eruption on the trunk and arms with sparing of
the face and legs. Of the following, the MOST likely etiology of
this infant's findings is
A.
B.
C.
D.
E.
15.
Escherichia coli
Haemophilus influenzae type b
Neisseria meningitides
Salmonella species
Streptococcus pneumoniae
adenovirus
enterovirus
human herpesvirus 6
parvovirus B19
rubella
A tuberculin skin test is placed on a 15-month-old child.
Of the following, the factor that is MOST likely to affect the test
result is
A. administration of measles-mumps-rubella vaccine 8 weeks ago
B. an uncle living in the home who has human immunodeficiency virus
infection
C. concomitant administration of Haemophilus influenzae type b vaccine
D. history of receiving bacillus Calmette-Guerin vaccine at birth
E. oral corticosteroid therapy administered 6 months ago
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16.
A 9-month-old girl has had rhinorrhea for 2 weeks; for the past
week she has had an increasingly severe paroxysmal cough with
posttussive emesis. Findings on physical examination are normal
except for tachypnea. The white blood cell count is 20,000/mm3,
with 70% lymphocytes and 30% polymorphonuclear leukocytes.
Among the following, the BEST reason to treat this child with
erythromycin is to decrease the
A.
B.
C.
D.
E.
17.
A 15-month-old boy has pneumococcal pneumonia. He had
pneumococcal bacteremia at 3 months of age and has had
multiple episodes of acute otitis media and sinusitis over the past
year. Of the following, the MOST useful initial screening test to
evaluate this child's immune system would be
A.
B.
C.
D.
E.
18.
duration of the coughing spells
risk of bacterial super infection
risk of pneumothorax
severity of the coughing spells
spread of disease to others
a pneumococcal antibody test
a total hemolytic complement level
intradermal skin testing for delayed type hypersensitivity
serum immunoglobulin levels
T and B cell counts
A woman who has tested positive for hepatitis B surface antigen
delivers at term. After careful bathing, the BEST management for
this woman's infant would be to administer
A. hepatitis B immune globulin (HBIG) and hepatitis B (HB) vaccine
immediately and HB vaccine at 1 month and 6 months of age
B. HB vaccine at 2 months, 4 months, and 6 to 18 months of age
C. HBIG immediately and HB vaccine at 2 months, 4 months, and 6 to 18
months of age
D. HBIG immediately and at 1 month and 6 months of age
E. HBIG immediately and at 1 month of age and HB vaccine at 2
months, 4 months, and 6 to 18 months of age
19. A 6-month-old girl has a urinary tract infection caused by
Pseudomonas aeruginosa. Among the following antibiotics, which is
MOST likely to treat the infection effectively with minimal toxicity?
A.
B.
C.
D.
E.
Amikacin
Ceftazidime
Clindamycin
Gentamicin
Vancomycin
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20. A 5-year-old boy develops a temperature of 38.8°C, right
periorbital erythema and edema, and mild proptosis of the right
eye several days following an upper respiratory tract infection.
Findings on neurological examination are normal except for mild
impairment of lateral and upward gaze. Visual acuity is normal.
Among the following, the organisms that MOST often cause the
findings reported for this patient are
A.
B.
C.
D.
E.
Bacteroides fragilis and Peptostreptococcus
Candida albicans and Aspergillus fumigatus
Escherichia coli and Streptococcus pneumoniae
Haemophilus influenzae and Streptococcus pneumoniae
Pseudomonas aeruginosa and Moraxella catarrhalis
21. An 8-year-old girl is in the same school with a boy who developed
meningococcal meningitis. The two children often sit at the same lunch
table, but they have no classes together. No other cases of meningococcal
infection have been reported in the school. The BEST management of
this girl is that
A. she and all her schoolmates should be given a prophylactic dose of
rifampin
B. she and her family should be given a prophylactic dose of rifampin
C. she should be cultured for meningococcus and treated with rifampin if
she is a carrier
D. she should be evaluated promptly if she becomes ill during the next
few weeks
E. she should be immunized with meningococcal polysaccharide vaccine
22.
A 2-year-old boy is diagnosed with otitis media. Thirty-six hours after starting
oral amoxicillin, he has a seizure. Examination of the cerebrospinal fluid (CSF) reveals:
white blood cell count, 510/mm3 with 60% polymorphonuclear cells; protein, 100 mg/dL;
glucose, 42 mg/dL; and no organisms seen on Gram stain. Simultaneous serum glucose
concentration is 90 mg/dL.
In this patient, the MOST helpful and specific test to diagnose bacterial meningitis would
be
A.
countercurrent immunoelectrophoresis for Escherichia coli
B.
determination of CSF/serum glucose ratio
C.
latex agglutination test for Haemophilus influenzae
D.
measurement of CSF tumor necrosis factor
E.
rapid antibody test for Listeria monocytogenes
23.
A 1-year-old boy with hydrocephalus underwent surgery to revise his
ventriculoperitoneal shunt 6 weeks ago. Among the following findings, which would be
MOST supportive of shunt infection in this patient?
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A.
B.
C.
D.
E.
Fever, malaise, and feeding problems
Focal or generalized seizure
Full fontanelle and "setting sun" sign
Poorly compressible shunt reservoir
Swelling and erythema of the scalp at the shunt site
24.
Twelve children at a child care center have been diagnosed with hepatitis A.
Among the following, the BEST method for controlling further spread of this disease is
to
A.
administer oral acyclovir to affected children
B.
administer immune globulin to all staff and children
C.
close the center for 6 months
D.
isolate the infected children for 1 month
E.
screen all staff for hepatitis A
25.
An adolescent with familial nephritis (Alport syndrome) develops fever, shaking
chills, and flank pain. Administration of which of the following antibiotics is MOST
likely to cause significant renal toxicity?
A.
B.
C.
D.
E.
Ampicillin
Cefaclor
Cefotaxime
Gentamicin
Trimethoprim-sulfamethoxazole
26.
A 3-year-old boy has low-grade fever, mild irritability, and swelling and
tenderness over the parotid glands bilaterally. He has not been immunized against
mumps, and you assume he has the disease.
Which of the following is MOST commonly associated with mumps in a child this age?
A.
B.
C.
D.
E.
Cerebrospinal fluid lymphocytosis
Chronic Parotitis
Facial nerve paralysis
Orchitis
Unilateral hearing loss
27.
A 3.5-year-old boy has had recurrent pneumonia, sinusitis, and otitis media.
Immunoglobulin G (IgG) level is 65 mg/dL (normal, 929 +/- 228 mg/dL). No rise in
specific antibody titer was detected following immunization with either Haemophilus
influenzae vaccine or pneumococcal vaccine. Tympanography reveals normal mobility
in both ears. The BEST chronic management for this patient is
A.
B.
C.
D.
administration of antibiotics alone for acute infectious episodes
administration of immune globulin intramuscularly each month
administration of immune globulin intravenously each month
referral to an otolaryngologist
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E.
reimmunization with both Haemophilus influenzae and pneumococcal
vaccines
28.
During the 22nd week of pregnancy, a woman had an illness characterized by
malar flush and a symmetric macular eruption on the arms, trunk, and buttocks that lasted
for 2 weeks. Shortly after recovering, she delivered a stillborn infant with nonimmune
hydrops fetalis.
The MOST likely cause of the infant's death is infection due to
A.
B.
C.
D.
E.
congenital herpesvirus
human immunodeficiency virus
parvovirus B19
rubella virus
Toxoplasma gondii
29.
You are evaluating a 10-year-old child with chronic abdominal pain.
Of the following, which is LEAST helpful in confirming a diagnosis of gastritis due to
Helicobacter pylori infection?
A.
B.
C.
D.
E.
Culture of biopsy specimen from the antral curve
Gross appearance of gastric mucosa on endoscopy
Histological appearance of biopsy specimen from the stomach
Isotope-labeled urea breath test
Level of urease production by biopsy specimen
30.
A 13-year-old girl has systemic lupus erythematosus that has been controlled with
nonsteroidal anti-inflammatory agents since diagnosis. Serial laboratory studies reveal
that anti-DNA titers are increasing and that C3 and C4 complement levels are decreasing.
Of the following, the MOST appropriate diagnostic study to obtain next is
A.
B.
C.
D.
E.
a fluorescent antinuclear antibody test
a urinalysis
an erythrocyte sedimentation rate
an antinuclear cytoplasmic antibody titer
another anti-DNA titer
31. An 8-year-old girl is limping. Three weeks ago she had varicella. Physical
examination reveals an afebrile child with tenderness and swelling of the right knee. The
MOST likely diagnosis is
A.
B.
C.
D.
E.
acute rheumatic fever
post infectious arthritis
rheumatoid arthritis
septic arthritis
varicella arthritis
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32.
A 6-month-old boy with a history of upper respiratory tract infection is brought to
the emergency room because of respiratory distress. He is noted to be semi-alert. Physical
examination reveals tachycardia, a gallop rhythm, hepatomegaly, cool skin, poor
peripheral perfusion, and multiple scattered purpuric lesions.
Of the following, the MOST likely diagnosis is
A.
B.
C.
D.
E.
bronchiolitis
cardiogenic shock
pneumothorax
status epilepticus
supraventricular tachycardia
33.
An infant is born at 34 weeks' gestation. Abdominal distention, lethargy, and
gastrointestinal bleeding develop 72 hours after the initiation of feeding with human
milk. A radiograph of the abdomen reveals pneumatosis intestinalis; there is no evidence
of free air.
The MOST common late complication of this patient's disorder is
A.
B.
C.
D.
E.
abscess
cholestasis
fistula
malabsorption
stricture
34.
An 11-year-old girl has had abdominal pain for the past year. It is now worse and
causes her to miss school frequently. The pain is located in the mid-abdomen and is
described only as "bad." The mother is concerned and requests a full evaluation.
Which of the following is MOST suggestive of organic pathology for the pain?
A.
Frequent nausea, headaches, and dizziness
B.
Improvement in pain within 20 minutes following administration of
acetaminophen
C.
Long duration of symptoms
D.
Occasional diarrhea containing small amounts of bright red blood
E.
Occurrence in the early evening unrelated to time of the evening meal
35. Chest pain occurs in children of all ages. Which of the following is considered the
least frequent cause of this pain:a. Costochondritis.
b. Asthma.
c. Cardiac disorders.
d. Gastrointestinal disorders.
e. Psychogenic factors
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36. Positive T wave in the leads V1-V4 is considered a normal finding in which of the
following age group:a. The first week of life.
b. The first 3 weeks of life.
c. The first month of life.
d. Till the age of 3 months.
e. None of the above.
37. A left to right shunt lesions, such as VSD and aortopulmonary window, are
considered operable if the ratio of the pulmonary cardiac output over the systemic cardiac
output (QP/QS) is :a. 1/1
b. 1.3/1
c. 1.8/1
d. > 2/1
e. None of the above.
38. Which one of the following is not considered in the treatment of Eisenmenger
Complex secondary to left to right shunt :a.
Calcium
b. Sildenafil (Viagra)
c. Postacyclin analog (Prostanoids).
d. Endothelin antagonists.
e. Aspirin.
channel
blockers.
39. The incidence of coronary artery complications in children affected with Kawasaki
disease who were not treated with intravenous immunoglobulin (IV IG) is
a. 5 %
b. 10 %
c. 20 %
d. 30 %
e. 36 %
40. Which of the following cardiac tumors is considered a typical finding of tuberous
sclerosis?
a. Myxomas
b. Fibromas
c. Sarcomas
d. Rhabdomyomas
e. Hemangiomas.
41. In a one year old child with supraventricular tachycardia, all of the following are
standard treatments except:-
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a. IV Verapamil.
b. Valsalva maneuver.
c. IV Adenosine.
d. Ocular compression.
e. IV Propranolol.
42. Hypertrophic cardiomyopathies have multiple etiologies. Which of the following is
not considered to cause hypertrophic cardiomyopathy: a. Certain metabolic diseases (Inborn errors of metabolism).
b. Adriamycin treatment.
c. Malformation syndromes such as Noonan syndrome.
d. Neuromuscular diseases such as Fredreich’s Ataxia.
e. Carnitine deficiency.
43. Transposition of great arteries is a rare congenital heart disease. It is considered
curable provided the baby undergoes switch surgery.The perfect timing for this surgery is
a. First week of life.
b. Between 7 days to 14 days of age.
c. Between 1 month to 2 months of age.
d. After the age of 3 months.
e. None of the above.
44. Common clinical findings in children with phenylketonuria include all of the
following except: a. Mental retardation.
b. Musty odor.
c. Seizures.
d. Macular cherry-red spots.
e. Recurrent vomiting.
45. A 4 year old male child was referred for evaluation of developmental delay,
sublaxation of the ocular lens and skeletal abnormalities resembling Marfan syndrome.
The most appropriate next step to establish the diagnosis is to request: a. Serum amino acid chromatography.
b. Urine for organic acids.
c. Plasma level of very long chain fatty acids.
d. Brain MRI.
e. Muscle biopsy.
For Questions 46-50, Match each of the following metabolic disorders (in column A)
with each characteristic association or clinical finding (in column B). Each item from
column B may be used once, more than once, or not at all: Column A
Column B
10
46. Biotinidase deficiency.
a. Exaggerated startle reaction.
47. None ketotic hyperglycinemia
b. Hypoketotic hypoglycemia.
48. Fatty acid oxidation defects.
c. Profound hypotonia.
49. Gaucher disease.
d. Alopecia.
50. Tay-Sachs disease.
e. Pathological fracture.
51. In evaluating children with short stature, all the following are true except:a- Height more than 2 SD below the mean always merits further consideration.
b- Target height can be estimated from parent's heights.
c- Achondroplasia affects directly growth plate.
d- Children who have endocrine disorders usually have low weight for height ratio.
e- Height velocity is usually normal in genetic short stature.
52. A 7 months old infant can make a variety of sounds that include vowels. This ability
is best described as:a- Babbling.
b- Cooing.
c- Jargoning.
d- Talking.
e- None of the above.
53. The average Na concentration in diarrheal stool is:a- 15 meq /L
b- 25 meq /L
c- 55 meq/L
d- 90 meq /L
e- 120 meq /L
54. All of the following are recognized causes of increased water need except:a-Hypothyroidism.
b-Fever.
c-Tracheostomy.
d-Third spacing.
e-Radiant warmer.
55. Which neoplasm is least likely in a 3 year old child?
A-Wilm's tumor.
B-Neuroblastoma.
C-Leukemia.
D-Posterior fossa brain tumor.
E-Osteogenic sarcoma.
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56. Deficiency of which vitamin can cause petechial bleeding:A-Vitamin B1.
B-Vitamin B12.
C-Folate.
D-Vitamin C.
E-Vitamin K.
57. Which one of the following statements about glucose-6-phosphate dehydrogenase
(G6PD) deficiency is correct?
a- Hemolysis worsens with continued exposure to the offending oxidative drug.
b- It is associated with neonatal jaundice
c- Hemolysis typically lasts four to six weeks after ingestion of the offending drug.
d- Breastfeeding mothers of a baby with G6PD deficiency may safely take oxidative
medications.
e- Bacterial infections can cause hemolysis, whereas viral infections do not.
58. Which of the following sites is least likely obstructed by ingested foreign bodies in
children?
a- Cricopharyngeal area.
b- Upper one third of the esophagus.
c- Pylorus.
d- Ileocecal valve.
59. Which one of the following agents should be considered for treatment of a moderate
to severe acute exacerbation of asthma in a child in conjunction with a short-acting
beta2 agonist?
a- A. Inhaled ipratropium bromide (Atrovent or Aerovent).
b- An intravenous beta2 agonist.
c- Oral theophylline.
d- An oral beta2 agonist
60. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal
infections are thought to cause which one of the following?
a- Depression.
b- Attention-deficit disorder.
c- Obsessive-compulsive or tic disorders.
d- Panic attacks and anxiety disorders.
61-
Finger clubbing can be seen in all of the following conditions except:a- Asthma.
b- Cystic fibrosis.
c- Chronic liver disease
d- Cyanotic heart disease
e- Bronchiectasis.
62-The most common cause of diarrhea in children:
a- Shigella
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b- Salmonella
c- Adenovirus
d- Rota virus
e- Giardia
63- All of the following conditions may be associated with stridor except:a- Laryngomalacia
b- Vocal cord paralysis
c- Vascular ring
d- Sub-glottic hemangioma
e- Foreign body in Rt lung
64- Which one of the following is the most common malignancy in children?
a- Lymphoma
b- Leukemia
c-Brain tumors.
d- Lung tumors
e- Wilm’s tumor.
65- Which malignancy you expect to find in a child with aniridia and congenital
genitourinary abnormalities?
a- Lymphoma
b- Sarcoma
c- Neuroblastoma
d- Wilms
E- Adrenal carcinoma
66- What is the treatment for an asymptomatic newborn of a mother who developed
Varicella (Chickenpox) one day after delivery?
a- Oral Acyclovir.
b- Intravenous Acyclovir.
c- Varicella Zoster Immunoglobulin (VZIG).
d- Gancyclovir
e- Vaccination to varicella.
67- Which one of the following electrolyte disturbance you expect to find in a child with
Supravalvular Aortic stenosis?
a- Hypercalcemia
b- Hypocalcemia
c- Hypernatremia
d- Hyponatremia
e- Hypophosphatemia
68- All of the following conditions are usually associated with coarse facial features
except:a- Hunter syndrome.
b- Hurler syndrome.
c- Mucolipidosis.
d- I-cell disease
13
e- Glycogen Storage Disease (GSD) type Ib.
69. All of the following conditions may cause erythema nodosum except:a. Acute Rheumatic Fever.
b. Sarcoidosis.
c. Tuberculosis
d. Ulcerative colitis.
e. Systemic Lupus Erythematosus.
70. All of the following conditions may cause elevation of chloride concentration in the
sweat (false positive sweat chloride test) except:a. Ectodermal dysplasia.
b. Hypothyroidism.
c. Hypoparathyroidism.
d. G6PD deficiency.
e. Hypoproteinic edema
71. The metabolic disturbance that usually occurs in cystic fibrosis is:a. Hypochloremic metabolic acidosis.
b. Hypermetabolic metabolic acidosis.
c. Hyperchloremic metabolic alkalosis.
d. Hypochloremic metabolic alkalosis.
e. No metabolic disturbance.
72. A six week old male infant presents with frequent projectile vomiting. Physical
findings and imaging studies are consistent with hypertrophic pyloric stenosis. Which
of the following electrolyte disturbances you expect to find when you perform serum
electrolytes analysis:a. Hyperkalemia, hyponatremia, normal chloride.
b. Hypokalemia, normal sodium, hyperchloremia.
c. Hypokalemia, normal sodium, hypochloremia.
d. Hyperkalemia, hypernatremia, hypochloremia.
e. Hyperkalemia, normal sodium, hyperchloremia.
73. All of the following can cause bronchiolitis obliterans except:a. After bone marrow transplantation.
b. After lung transplantation.
c. After respiratory infection with Adenovirus.
d. Scleroderma.
e. Home dust.
74. Growth hormone may be used in the treatment of short/small stature associated with
all of the following conditions except:a. Turner syndrome.
b. Prader Willi syndrome.
c. End stage renal failure (before kidney transplantation).
d. Intra uterine growth retardation.
e. Achondroplasia.
14
75.
75. All of the following statements are true for celiac disease Except
a) It is a permanent (lifelong) sensitivity to gluten in wheat and related
proteins found in barley and rye. It occurs in genetically susceptible
persons
b) Usual presentation between 6-24 month with steatorrhea and abdominal
distention and poor weight gain.
c) Isolated short stature as many as 10% of children referred for evaluation of
growth failure can be the only manifestation in patient with celiac disease
d) Treatment consists of excluding Gluten from diet (that contains proteins
derived from wheat, rye, and barley) for the first 5 years of life.
e) Endomysial (EMA) and tissue transglutaminase (TTG) are good screening
tests for celiac disease with good sensitivity and specificity.
76.
Which of the following conditions should be considered specifically for children
with head growth deceleration, abnormal respiration, seizures, and hand wringing in
whom Autism Spectrum Disorder is suspected?
a.
b.
c.
d.
e.
Lead poisoning
Celiac
Fragile X syndrome
Rett Syndrome
Krabbe disease
disease
77.
All of the following statements about breastfeeding and hyperbilirubinemia are
true except:
a. It is likely that dehydration itself is the most important cause of
hyperbilirubinemia in breastfed infants.
b. To help prevent hyperbilirubinemia, nursing mothers should breastfeed their
infants at least eight to 12 times a day for the first several days.
c. Breastfed infants tend to develop hyperbilirubinemia because of the effect of
caloric deprivation on enterohepatic circulation of bilirubin.
d. The poor caloric intake associated with inadequate breastfeeding appears to
play an important role in the development of severe hyperbilirubinemia.
e. Kernictrus may occur with severe hyperbilirubinemia due to breastfeeding.
78. Which of the following cutaneous abnormalities is most likely found in an infant
with an absent radius?
A- Petichiae
B- Café-au-lait spots
C- Hemangioma
D- Hypoplastic nails
E- Pigmented nevi
15
79. A 46 XY infant has a functional gonad, internally normal male genitalia and
externally ambiguous genitalia. Which of the following is the most likely cause of this
disorder?
ABCDE-
Inability to produce H & Y antigen
Inability to produce Mullerian inhibitory factor
Inability to produce testosterone
5-alpha reductase deficiency
E. Androgen insensitivity
80. Which of the following clinical presentation is consistent with Erb-Duchenne
paralysis?
A. An internally rotated shoulder, extended and pronated elbow, flexion of
the wrist and fingers, with hand movement
B. An internally rotated shoulder, limp at the elbow, wrist and fingers, with
no movement below the elbow
C. Weakness and absence of movement from the shoulder down
D. WNL shoulder posture, flexion of elbow, weakness of wrist & fingers with
absent grasp
E. Normal upper extremities posture, but asymmetric Moro reflex weak grasp
and Horner syndrome on the affected side
81. Which of the following statements regarding gastroschisis is true?
A. The defect is usually found inferior to the base of the umbilicus
B. The small intestine is usually foreshortened
C. Associated cardiac anomalies are common
D. Intestinal atresia exists in more than 50% of the cases
E. Chromosomal Anomalies are common
82. A 5 Year old child has chorioathetoid movement, upward gaze palsy, and tooth
enamel dysplasia. Brain stem evoked potential demonstrated high frequency
sensorineural hearing loss. Which of the following is the most likely cause of these
findings?
A- Hypoxic ischemic encephalopathy.
B- Mercury ingestion.
C- Genetic disorder.
D- Hypoglycemic brain damage
16
E- Bilirubin encephalopathy
83. A 2wk old, FT infant with strabismus has a family history of leukokoria in
infancy. CT of the head reveals extensive calcification of the right eye.
Which of the following is the most likely diagnosis?
A. Neuroblastoma
B. Retinoblastoma
C. Congenital Cataract
D. Galactosemia
E. Congenital Rubella
84. Anemia of prematurity is best explained by which of the following?
A. Inadequate iron stores in the preterm infant
B. Production by the liver of a complex erythropoietin that is inactive
C. Rapid destruction of fetal RBCs
D. Circulating antagonist to erythropoietin
E. Inadequate erythropoietin production by the kidney
85. A 1.5 Kg infant is to receive dopamine at 10 mcg/Kg/min. How much dopamine
(mg) should be added to 100 ml of D10W at infusion rate of 2ml/hour?
A. 3
mg.
B. 4.5 mg.
C. 15 mg.
D. 30 mg.
E. 45 mg.
86. A 6hour-old full term infant has cloudy corneas. Re-exam at 72h of age shows no
change in ocular findings. Which of the following is the most appropriate management?
A. Evaluate the infants for increased ocular pressure
B. Treat the infants with propranolol
C. Reassure the parents that these findings are benign
D. Evaluate the infant for retinoblastoma
E. Evaluate the infant for renal abnormalities
87. Cystic hygroma and lymphatic channel malformations are associated with which of
the following karyotypes?
A- 47,XX,+18
B- 69,XXX
17
C- 46,XX
D- 45,X
E- 47,XX,+13
88. A fetus whose mother has SLE with anti-Ro (SS-A) antibodies in her blood is
especially at risk for which of the following conditions?
A-
SVT
B-
Sick sinus syndrome
C-
Complete heart block
D-
Ventricular ectopia (unifocal)
E-
Atrial flutter
89.
A 1 Day Old, FT infant born to a mother who was treated with phenytoin and
phenobarbital for seizure disorder during pregnancy has a bloody stool. Examination
shows a large cephalohematoma, UA: shows hematuria, other Labs: Platelets 230K; PT
> 50 sec; a-PTT > 100 sec & fibrinogen = 200. After treatment with FFP, administration
of which of these is most likely to correct the abnormality?
A-
Activated protein C
B-
Cryo-precipitate
C-
Vitamin K
D-
Factor IX concentrate
E-
Heparin
90. Which of the following disorders in an oliguric infant is most likely associated with
a urinary Na concentration of < 20?
A-
ATN (Acute Tubular Necrosis).
B-
Renal dysplasia
C-
Post Urethral Valve
D-
Low circulating blood volume
E-
Chronic hypoxia
91. An AGA (Appropriate for gestational age) full term infant has marked pallor but
minimal respiratory distress. Heart Rate =180 beats/min. with no evidence of
hepatosplenomegaly. At 2 hours of age the infant has hemoglobin of 8 gm/dl, Bilirubin
of 2 mg/dl & Retic count of 6%. The study that most likely to establish the diagnosis is?
A-
Kliehauer Betki test
B-
G-6-PD assay
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C-
Osmotic fragility
D-
Direct Coombs Baby’s blood
E-
Indirect Coombs Mom’s blood
92.
Which of the following screening tests is most appropriate for determining
chronic granulomatous disease?
A-
Measurement of quantitative serum immuno-globulin concentration
B-
Total lymphocyte count
C-
Total neutophil count
D-
Total hemolytic complement
E-
Nitroblue tetrazolium dye test
93. Concerning cerebral palsy (CP), one of the following statements is correct: a.
Motor
handicap
is
a
constant
criterion
for
diagnosis.
b. Either lower or upper limbs are affected in paraplegia.
c. The incidence of CP has decreased with improvement of perinatal and
postnatal
care.
d. With the advances in neuroimaging studies, the causes of CP are identified in
most
cases.
e. It is a progressive disorder of posture and movement due to a defect or lesion
in the developing brain
94.
One of
correct:
A.
B.
C.
D.
E.
the following concerning Attention Deficit Hyperactivity Disorder (ADHD) is
Is always associated with neurological abnormalities.
Etiology is already understood.
Children with ADHD have significantly low IQ scores
Poor ability to attend to a task, motor hyperactivity, and impulsivity.
Distinguishing between ADHD, conduct disorder, and learning disabilities is not
difficult.
95.
All of the following are manifestations of Infantile Autism except:
A. Undeveloped or poorly developed verbal and nonverbal communication skills.
B. Stereotypic body movements.
C. Tantrum-like rages may accompany disruptions of routine.
D. The autistic child is withdrawn and often spends hours in solitary play.
E.
Persistent and prolonged eye contact.
96.
All the following statements are true except:A. Hypsarrhythmia in EEG suggests West Syndrome.
B. A 3 per second spike and slow waves discharge is suggestive of Absence attack
C. Attacks of convulsion during acute meningitis, of previously healthy child, are not
epileptic
D. Absence attacks are associated with a decrease of muscular tone
E.
By definition, 1st attack of unprovoked seizure is not epileptic
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97.
MELAS is a recognized mitochondrial disorder. All the following features are characteristic
of this disorder except:
A. Muscle biopsy typically show ragged red fibers (RRF)
B. Presentation by stroke-like episodes with acute hemiparesis.
C. Presence of lactic acidosis with increased serum lactate levels during acute episodes.
D. Lowering the serum lactate concentration in those with severe lactic acidosis may
result in marked clinical improvement.
E.
Patients with this disorder are abnormal since birth.
98.
One of the following is true concerning Guillain Barré Syndrome (GBS):
A. Demyelination is mainly motor and the sensory nerves are not affected.
B. Urinary incontinence and retention are against the diagnosis of GBS.
C. Bulbar involvement is a rare occurrence.
D. Miller-Fisher syndrome consists of acute external ophthalmoplegia, ataxia and
areflexia.
E.
CSF studies are essential for diagnosis. Normal CSF findings in the 1st days rules out
GBS.
In Pseudotumor Cerebri, all of the following are correct except:
99.
A.
B.
C.
D.
E.
100.
Increased intracranial pressure.
Vomiting is the most frequent symptom.
Normal ventricular size, anatomy and position.
Normal CSF.
It may follow prolonged treatment with corticosteroids.
The contrast media used in brain CT imaging in used to obtain variation in density, so some
normal structures are best seen and certain anomalies are best identified. All of the following
are correct except:
A. Intact hemato-cephalic barrier (capillary endothelium of the CNS) blocks the fast
diffusion of contrast media.
B. In normal brain, the contrast media remains in the vascular path.
C. In normal CNS tissue, the contrast media accentuates differentiation between white
and Gray matter.
D. On skull bones, the contrast media makes no changes.
E.
CSF will be more visualized with contrast media.
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