Download A newborn baby is apneic and blue. Her airway

Palestine Medical Council
Pediatrics Part I Exam
Previous MCQs
1. All true about bilirubin EXCEPT:A- Bilirubin is formed by breakdown of heme present in hemoglobin, myoglobin,
cytochromes, catalase, peroxidase and tryptophan pyrrolase.
B-Bilirubin is generated by degradation of heme mediated by two groups of enzymes:
Heme oxygenase and biliverdin reductase.
C-Water-insoluble unconjugated bilirubin is associated with all known toxic effects of
bilirubin.
D-Phototherapy converts bilirubin into lumirubin via structural isomerization that is not
reversible
E- Photo-oxidation reactions convert bilirubin to colorless, polar compounds that are
excreted primarily in the urine. This is a slow process but accounts for a major
proportion of bilirubin elimination.
2- Select the correct statement on hyaline membrane disease (RDS) in neonates:A. Surfactant deficiency is the primary cause of RDS because the resultant high surface
tension, especially at low volume, leads to alveolar collapse and diffuse atelectasis. The
relationship of the inflating pressure, surface tension, and radius of curvature is
illustrated by the model of a distal alveolus as a sphere connected to a distal airway and
described by LaPlace's law.
B. Hypoxemia results partially from mismatching of ventilation and perfusion due to
collapse of large portions of the lung. Extra pulmonary shunting is the major contributing
factor of hypoxemia in neonatal hyaline membrane disease.
C. Physiologic dead space is decreased in RDS.
D. Functional residual capacity is increased in RDS.
E. Static respiratory system compliance is increased in RDS.
3- All true on the management of a newborn with respiratory distress except:A. RDS is easily differentiated from bacterial pneumonia. As a result, we usually do not treat infants
with presumed RDS with antibiotics.
B. Severe RDS may be difficult to distinguish from some forms of congenital heart disease.
C. Administration of surfactant usually shortens the clinical course.
D. Arterial blood gas measurements typically show hypoxemia that responds to administration of
supplemental oxygen. PCO2 initially is normal or slightly elevated. It usually increases as the
disease worsens.
E. As the disease progresses, infants may develop hyponatremia. This results from water retention,
and usually improves with fluid restriction.
4- A newborn baby is apneic and blue. Her airway is cleared and she is stimulated. At
30 seconds positive –pressure ventilation is begun. At 60 seconds her heart rate is
40 beats per minute .Select the correct statement from the following:A. chest compressions should be started and positive pressure ventilation should be continued.
1
B. The preferred method of delivering chest compressions is the two finger technique.
C. If you anticipate that the baby will need medication by the umbilical route; it may be easier to
deliver chest compressions by two thumb technique.
D. The correct depth of chest compression is approximately one half of the anterior- posterior
diameter of the chest.
E. The Ratio of chest compression to ventilation should be 5 to 1
Match each antibiotic in the left column with its corresponding class on the right
column .Each class can be selected more than once or not at all.
A. A third generation cephalosporin
5- Imipenem
6- Cefixime
B. A carbapenem
C. A fourth generation cephalosporin
7- Cefipime
8- Ceftazidime
D. A second generation cephalosporin
9- All are true on the use of dexamethasone in bacterial meningitis except:A. In children with bacterial meningitis, dexamethasone appears to decrease the risk of
hearing loss (particularly in those with Haemophilus influenza type b meningitis).
However, it has no effect on other neurologic sequelae or mortality.
B. It is recommend not to use dexamethasone if more than one hour has elapsed since the
first dose of antimicrobial therapy
C. Dexamethasone is administered (0.15 mg/kg per dose) every six hours for two to four days
D. Dexamethasone also is indicated in the treatment of patients in whom aseptic,
nonbacterial, or gram-negative enteric meningitis is suspected
E. Adverse reactions to the use of dexamethsone may include: adrenal suppression, growth
suppression, gastrointestinal hemorrhage or perforation and cataract.
10- All are true regarding CHILDREN WITH ASPLENIA OR FUNCTIONAL
ASPLENIA EXCEPT:A. Streptococcus pneumonia is the most common pathogen that causes bacteremia in
children with asplenia.
B. The risk of bacteremia is higher in younger children than in older children, and risk may
be greater during the years immediately after splenectomy.
C. antimicrobial prophylaxis (in addition to immunization) should be considered for all
children with asplenia younger than 5 years of age and for at least 1 year after
splenectomy.
D. When antimicrobial prophylaxis is used, the parents should be reassured that the risk for
fulminant septicemia will be completely eliminated
E. When surgical splenectomy is planned, immunization status for Hib, pneumococcus, and
meningococcus should be ascertained, and needed vaccines should be administered at least
2 weeks before surgery if possible.
11- All are true about Group A streptococcal infections except:
A. Children with streptococcal pharyngitis or skin infections should not return to school or
child care until at least 7days after beginning appropriate antimicrobial therapy
B. Children with nonbullous impetigo usually should be treated with an antimicrobial agent
active against both Group A Streptococcus and S aureus infections.
2
C. Antimicrobial therapy is not indicated for most GAS pharyngeal carriers
D. Tetracyclines;
sulfonamides,
including
trimethoprim-sulfamethoxazole;
fluoroquinolones should not be used for treating GAS pharyngitis
E. A clinical isolate of GAS resistant to penicillin never has been documented.
and
12-In bronchiolitis the major benefit of palivizumab prophylaxis is:
A.
B.
C.
D.
E.
Decreased hospitalization rate.
Improved treatment.
Increased cost-effectiveness.
Lower mortality rate.
Shorter duration of illness
13-Contraindications to immunization exist for all of the available live attenuated
vaccines (influenza, measles, mumps, rubella, and varicella). Which of the
following contraindications to immunization is shared by all of the live
attenuated vaccines?
A.
B.
C.
D.
E.
History of allergy to latex.
History of anaphylaxis to eggs.
History of anaphylaxis to neomycin or gelatin.
History of Guillain-Barre´ syndrome.
Possible or known immunodeficiency
14- At its origin at the base of the left ventricle, normally the ascending aorta
arises:
A.
B.
C.
D.
E.
Anteriorly & to the right of the pulmonary trunk.
Posteriorly & to the right of the pulmonary trunk.
Anteriorly & to the left of the pulmonary trunk.
Posteriorly & to the left of the pulmonary trunk.
In parallel with the pulmonary trunk.
15. The true statement regarding the normal mitral anatomy is:
A.
B.
C.
D.
E.
it has three papillary muscles.
it has four leaflets.
it is composed of two leaflets one anterior & the other posterior.
it is composed of two leaflets one median, the other lateral.
the mitral valve is normally inserted below the level of the tricuspid valve.
16. In an ECG trace done through the standard method, each big square
horizontally indicates a time of:
A.
B.
C.
D.
E.
0.04 seconds.
0.06 seconds.
0.08 seconds.
0.1 seconds.
0.2 seconds.
3
17. Which of the following is not true regarding fetal circulation?
A. The ductus venosus functionally close soon after birth, but it may remain anatomically open for
up to 20 days.
B. The fetal circulation is a parallel system.
C. The fetus has a limited reserve with which to increase cardiac output.
D. The blood with the highest oxygen content in the fetal circulation is found in the umbilical
circulation as it exits the placenta & courses toward the fetus.
E. 35% of fetal cardiac output returns to the left atrium via the pulmonary veins.
18. In Tetralogy of Fallot the basic determinant in the outcome of corrective
surgery is:
A.
B.
C.
D.
E.
the degree of hypoplasia of the pulmonary trunk.
the presence of one single VSD.
normal coronary anatomy.
the presence of an ASD.
the presence of tricuspid regurgitation.
19. Total body water in a newborn is what percentage of body mass?:
A.
B.
C.
D.
E.
40%.
55%.
60%.
70%
75%
20. The normal maintenance fluid in a child 20 kg NPO per 24 hours is:
A.
B.
C.
D.
E.
1000 ml.
1100 ml.
1200 ml.
1400 ml.
1500 ml.
21-An 8-month-old infant has a 2-day history of diarrhea and poor fluid intake. You
diagnose a 10 to 15% dehydration. Which of the following fluids is appropriate to
begin immediate resuscitation?
A.
B.
C.
D.
E.
D5 1⁄4 normal saline
D5 1⁄2 normal saline
Normal saline
Whole blood
D10W
22- A 6 weeks old male with 10 days history of projectile vomiting has been
diagnosed to have pyloric stenosis. Select the pattern of serum electrolytes
which Will be most consistent with his condition: A.
B.
C.
D.
E.
NA 135 Meq/L CL 115Meq/L K 6Meq/L HCO3 12Meq/L
Na 130 Meq/L Cl 87Meq/L
K 3Meq/L HCO3 30Meq/L
Na 150 Meq/L Cl 113Meq/L K 2.9Meq/L HCO3 12Meq/L
Na 128 Meq/L Cl 90 Meq/L K 7Meq/L HCO3 31Meq/L
Na 145 Meq/L Cl 105Meq/L K 4Meq/L HCO3 23Meq/L
4
23-The renin-angiotensin-aldosterone system is important in the regulation of water
& electrolyte balance as well as the blood pressure. All of the following increases
rennin release except:
A.
B.
C.
D.
E.
Decreased blood volume.
Decreased macula densa concentration of chloride.
Decreased beta adrenergic activity.
Increased prostaglandin secretions.
Decreased perfusion pressure.
24-Which one of the following organisms grown in throat culture requires treatment
with antibiotics:A.
B.
C.
D.
E.
Group A beta hemolytic streptococcus in a 7 years old boy
Group B beta hemolytic streptococcus in a 5 years old girl
Streptococcus pneumonia in a 4 year old boy with sickle cell disease
Pseudomonas aeurogenosa in 10 year old male with recurrent otitis media
Methicillin resistant staphylococcus aureus in 2 year old boy with temp of 39.5 rectally.
25-Select the most common cause of bacterial pneumonia in a 12 year old boy:A.
B.
C.
D.
E.
Staphylococcus aureus
Mycoplasma pneumonia
Hemophilus influenza type b
Moraxella catarrhalis
Streptococcus pneumonia
26-Skeletal maturation, all are true except:
A.
B.
C.
D.
E.
In familial short stature, bone age is normal (comparable to chronological age).
In endocrinologic short stature, the bone age is delayed.
Skeletal maturation is linked more closely to sexual maturity rating than to chronological age.
It is more rapid and less variable in girls than in boys.
Bone age does not correlate well with stage of pubertal development.
27-The best formula to approximate average weight (kg) for a 4-year old is:
A.
B.
C.
D.
E.
Age (years) x 7-5/2
Age (years) x 2 + 8
Age (months) + 9/2
Age (years) x 5 + 17
Age (years) x 7 + 5
28-Growth during the years between 6 and 12y is characterized by annual weight and
height increments of:
A.
B.
C.
D.
E.
3.5kg, 6cm
6kg, 3.5cm
5kg, 10cm
10kg, 5cm
1.5kg, 5cm
Match the following serum HBV serologic markers with their correct interpretations.
Each lettered choice may be used once, more than once, or not at all.
29. Positive HBe Antibody
5
30. Positive HBe Antigen
31. Positive HBs Antibody with negative HBc Antibody
A.
B.
C.
D.
E.
Acute infection.
High viral replication rate.
Immunity after immunization.
Immunity after recovery from infection.
Low viral replication rate.
32-A 5-day-old child is brought to the emergency department because he has been
difficult to arouse over the last 6 hours. His parents report that he has not been
interested in feeding today and that he has been breathing rapidly and with a
grunting noise. On physical examination, the infant’s heart rate is 185 beats/min,
respiratory rate is 80 breaths/min, and blood pressure is 55/40 mm Hg. A pulse is
palpable in the right brachial region, but not in the feet. All of his extremities are
cool and mottled, with a capillary refill of more than 2 seconds.
Of the following, the MOST appropriate next step is to
A.
B.
C.
D.
E.
arrange for echocardiography at the first appointment in the morning
initiate a furosemide infusion
initiate a prostaglandin infusion
obtain a computed tomography scan of the head
obtain a lumbar puncture
33-A 4 years old child was recently diagnosed with type 1 diabetes mellitus.
He was started on insulin therapy. In one month period, the dose of insulin was
tapered down and eventually discontinued as the child developed recurrent
severe hypoglycemic reactions. The most likely explanation of this phenomenon
is:A.
B.
C.
D.
E.
Regeneration of some of β cells in the pancreas.
Increased of the peripheral sensitivity of the insulin receptors.
Absolutely wrong diagnosis of type 1 diabetes.
Decreased metabolism of peripheral insulin.
None of the above is correct.
34- In the coming few weeks, the child in question # 33 will have which one of the
following scenarios?
A.
B.
C.
D.
E.
Type 2 diabetes is inevitable.
The child will require oral hypoglycemic agents to control his blood sugar.
The child will require insulin indefinitely.
The child will live with no insulin therapy at all.
None of the above.
35-In Persistent hyperinsulinemic hypoglycemia of infancy( nesidioblastosis) all of
the following is true except:.
A. The presence of ketotic hypoglycemia
B. Inappropriately high plasma concentrations of insulin and C-peptide at the time of
hypoglycemia
6
C. Increased glucose requirements (>15 mg/kg per minute to maintain normoglycemia (>40
mg/dL )
D. Glycemic response (increase of ≥ 30 mg/dL) to glucagon despite hypoglycemia
36-The above picture is a gram stain smear which was obtained from
cerebrospinal fluid sample of a 9 months old infant. From the following select
the FALSE statement:A. The disease could have been prevented by administering Pneumovax vaccine to this
infant.
B. The initial treatment should include Cefotaxime in addition to Vancomycin
C. Vancomycin should not be continued if the organism is susceptible to other appropriate
non–beta-lactam antimicrobial agents
D. Asplenia puts this infant at increased risk for recurrent infections with this organism
E. Antimicrobial chemoprophylaxis is not recommended for contacts of this infant,
regardless of their immunization status.
37-What is the average yearly linear growth rate for a boy during the second year of
life?
A.
B.
C.
D.
12 cm
8 cm
6 cm
4 cm
38-An 8 year-old child had grown normally in the 25th percentile between age 2-5
and has now deteriorated to the 2nd percentile in height and 15th percentile in
weight. He drinks 4 liters of water a day. This is compatible with:
A. Congenital hypothyroidism
B. Malnutrition
7
C. Intestinal absorption defect
D. Maternal deprivation
E. A large tumor in the sella turcica
39- At three years old child can do all of the following EXCEPT:
A.
B.
C.
D.
E.
Draw a circle
Walk down stairs one foot per step
Speak four word sentences
Know some colors
Kick a ball
40-The presence of atrioventricular canal (endocardial cushion defect) is most
likely to be associated with:
A.
B.
C.
D.
E.
Turner syndrome
Noonan syndrome
Down syndrome
Marfan syndrome
Hunter syndrome
41- In infancy Social smile usually appears at the age of?
A.
B.
C.
D.
E.
6 months
8 weeks
2 weeks
4 months
9 months
42-One of the following congenital heart diseases is ductus arteriosus dependent:A.
B.
C.
D.
E.
Pulmonary Stenosis (mild)
Critical (severe) aortic stenosis
Atrio-ventricular (AV) canal
Tetralogy of Fallout
Truncus arteriosus
43-To be effective for preventing myelomeningocele, administration of folic acid
needs to begin:
A.
B.
C.
D.
E.
By 3 months of gestation
At the first missed period
Before conception
By 30 days of gestation
At 3 months prior to delivery
44-A 4-yr-old boy is evaluated for his first generalized tonic-clonic seizure, which
lasted 10 min. There is no history of illness or fever, and findings on
examination an hour after the seizure are completely normal. The most
appropriate management is:
A. Begin therapy with carbamazepine
B. Order an EEG
8
C. Order a CT scan of the brain
D. Order an MRI study of the brain
E. Order G6PD
45-A 15-mo-old girl is evaluated for a 10-min-long generalized seizure associated
with a temperature of 40oC. Which of the following factors in the history is
most likely to increase the risk of future seizures?
A.
B.
C.
D.
E.
APGAR score of 7 at 5 min
Family history of epilepsy
Clinical evidence of roseola
Female gender
Presence of 1 café-au-lait spots
46- A 5-yr-old child is referred with a 6-wk history of morning headaches, often
associated with vomiting. His parents have noted that during this period he has
become irritable and moody. Which of the following would be most likely to be
identified during the physical examination?
A.
B.
C.
D.
E.
Marked reduction of blood pressure
Tenderness on percussion of frontal sinuses
Papilledema
Orbital pain
Refractive error
47-A 10-yr-old girl is being evaluated for new onset of school problems, obsessivecompulsive behavior, and occasional uncontrolled movements of the hands. She
has been healthy and has not taken any medications. Further evaluation is most
likely to reveal:
A.
B.
C.
D.
E.
Posterior fossa tumor
Partial complex epilepsy
Evidence of streptococcal infection
Hydrocephalus
Gluten sensitivity
48-A 11-mo-old girl is presented to the emergency department with gradual onset of
fever, lethargy, and irritability. Her immunizations are up to date.
Examination reveals a febrile infant who does not interact with the examiner and
cries inconsolably. A lumbar puncture is performed, and the cerebrospinal fluid
contains 1,500 white blood cells/mm3, 84% of which are granulocytes; a glucose
concentration of 12 mg/dL; and a protein concentration of 30 mg/dL. Gram stain is
negative. The most likely etiologic agent for this infection is:
A.
B.
C.
D.
E.
An enterovirus
Herpes simplex virus
Neisseria meningitides
Group B streptococci
Epstein bar encéphalites
9
49-Causes of large head include all of the following except:
A.
B.
C.
D.
E.
Hydrocephalus
Chronic subdural effusions
Canavan disease
Congenital CMV infection
Sotos syndrome
50-The Gowers sign demonstrates:
A.
B.
C.
D.
E.
Poor reflexes
Spinal dysraphism
Tethered cord
Proximal motor weakness
Mental retardation
51- Characteristics of simple motor partial seizures include all of the following
except:
A.
B.
C.
D.
Loss of consciousness
Duration of 10-20 sec
No postictal state
Abnormal EEG findings
52-Physical features of Sydenham chorea include all of the following except:
A.
B.
C.
D.
E.
Hypertonia and spasticity
Choreic hand
Darting tongue
Emotional lability
Writing difficulty
53-The infant pictured above develops infantile spasms. The disorder most likely to
be affecting this infant is:A. Neurofibromatosis
10
B. Tuberous sclerosis
C. Incontinentia pigmenti
D. Pityriasis rosea
E. Psoriasis
54-All of the following are true about Neurofibromatosis type 1 (NF1) Except:A. It is usually inherited as an autosomal dominant condition.
B. About 50% of new cases are new mutations.
C. The diagnosis demands one or more café au lait patches more than 5mm before the onset
of puberty.
D. It is associated with optic nerve gliomas
E. In children may be associated with mental retardation.
55. In Breath holding attacks all the following are true Except:
A.
B.
C.
D.
E.
Are commoner in infants and younger children
Can be confused with a generalized convulsion.
May be precipitated by a minor injury.
Should be treated with sedatives.
Are never fatal.
56-A Four years old boy presents with frequent short staring spells, each
lasting for few seconds. The child is usually not responsive during the
attack. The most likely diagnostic procedure is:A.
B.
C.
D.
E.
Chest X-ray.
EEG
Brain CT scan
Muscle biopsy
Calcium level
57-In Duchene muscular dystrophy all of the following are true Except:
A.
B.
C.
D.
E.
Commonest muscular dystrophy affecting male children.
A history of recurrent falls
It is an X-linked recessive condition.
Urinary incontinence
The serum creatine kinase is usually very elevated before muscular weakness becomes
clinically evident
11
58An eight month old with failure to thrive and chronic
diarrhea with these lesions below and similar lesions in the
diaper area
-
From among the following laboratory investigations performed on this infant
select the most likely abnormal test result :A.
B.
C.
D.
Low serum ferritin level
Low serum copper level
Low serum folate level
Low serum zinc level
12
59-
The above represents the external genitalia of a 5 days old female newborn. This
baby may have all of the following abnormal laboratory investigations except:A.
B.
C.
D.
E.
Na =128 mEq/L ,K=2.9 mEq/L Cl=98 mEq/L
Elevated serum 17-OH progesterone
Elevated serum testosterone
Hypoglycemia
Low serum cortisol
60-
13
All of the following represent risk factors for the abnormality seen in the above pelvic
x-ray of a 7 months old infant except:A.
B.
C.
D.
Male gender
Breech presentation
A positive family history for the condition
Meningomyelocele
61- The above peripheral blood film was done on a 7 year old male with anemia and
splenomegaly. All the following statements regarding his condition are true
except:A.
B.
C.
D.
He is at risk of a plastic crisis if infected with parvovirus 19 infection.
He is at risk of developing gallbladder stones
The risk of transmitting the disease to his off springs is 25%
Splenectomy is curative
14
62-Select the proper endotracheal tube size and placement for a 2 kg premature
neonate:A.
B.
C.
D.
E.
2.5 mm ,7 cm from the upper lip
3 mm ,8 cm from the upper lip
3 mm, 7 cm from the upper lip
2 mm ,7 cm from the upper lip
4 mm ,8 cm from the upper lip
63. The following statements are true regarding asthma EXCEPT:
A. Leukotriene inhibitors have a useful role in some patients
B. The diagnosis of asthma involves blood tests
C. Treatment can be divided into those that prevent and those that relieve
symptoms
D. Inhaled glucocorticoids improve lung function in asthmatics
E. Peak flow monitoring can be used in most children aged 5 years and above
64-A 10-yr-old child has intermittent symptoms of mild asthma.
The most appropriate treatment option is:
A.
B.
C.
D.
E.
Environmental control and patient education only no medication is indicated
Oral theophylline
Inhaled 2-agonist as needed for symptoms
Daily inhaled corticosteroid
Oral Cromolyn
65-A 3-yr-old boy has been coughing daily for 2 mo. The cough is nonproductive and
occurs during sleep in the early morning hours as well as during the day,
particularly when the child is active. On physical examination both height and
weight are in the 50-75th percentile, and chest examination is unremarkable.
There is no evidence of digital clubbing. A chest roentgenogram is interpreted as
normal. The diagnostic procedure most likely to ascertain the cause is:
A.
B.
C.
D.
E.
Sputum cytology and culture
Sweat chloride testing
Bronchoscopy
Complete blood count
Trial of bronchodilator therapy pulmonary
66. A 9-year-old girl has just been started on captopril therapy for management of
hypertension resulting from renal scars. Which of the following is a serious
potential adverse effect of captopril (and other angiotensin converting enzyme
inhibitors)?
A. Hypercalcemia.
15
B.
C.
D.
E.
Hyperkalemia.
Hypermagnesemia.
Hypernatremia.
Hyperuricemia
67-A 1-year-old year boy who has chronic kidney disease from posterior urethral
valves presents to your office because his breathing has been noisy for the past 2
hours. His usual medications include calcium carbonate and vitamin D. On
physical examination, you note inspiratory stridor.
He has not had upper respiratory tract symptoms or fever, and there is no history
of choking.
The mother reports that the boy was seen by the nephrologist 1 week ago, and
because of worsening renal function, he was begun on sodium bicarbonate.
Of the following, the electrolyte abnormality that BEST explains his current
symptoms is
A.
B.
C.
D.
E.
hyperkalemia
hypermagnesemia
hypocalcemia
hyponatremia
hypophosphatemia
68-A 12-month-old girl is found to have a hemoglobin of 9.7 g/dL (97 g/L) during a
health supervision visit.
Other findings on the complete blood count are mean cell volume of 63 fL, red cell
distribution width (RDW) of 15.1%, and red blood cell count of 5.3_103/mcL
5.3_109/L. She is given a trial of oral iron sulfate, and after 3 weeks, the
hemoglobin measures 9.6 g/dL (96 g/L). The most likely explanation for the failure
of the hemoglobin to increase is:
A.
B.
C.
D.
E.
Administration of the oral iron with meals.
Failure to take oral iron.
Incorrect diagnosis.
Malabsorption of iron.
Ongoing blood loss.
69-Wide fixed split of S2 heart sound is pathognomonic of
A.
B.
C.
D.
E.
Ventricular septal defect (VSD).
Coarcitation of aorta.
Total anomalous venous return.
Atrial septal defect (ASD).
Patent ductus arteriosus (PDA).
70-Rib notching on chest X ray is seen in
A. VSD.
B. ASD.
C. Tetra logy of Fallout.
16
D. PDA.
E. Coarcitation of aorta.
71-For a patient who has autosomal recessive congenital nephrogenic diabetes
insipidus, the administration of vasopressin during a water deprivation test
will result in:
A.
B.
C.
D.
E.
Significantly higher serum osmolality.
Significantly higher urine osmolality.
Significantly lower serum osmolality.
Significantly lower urine osmolality.
Unchanged urine osmolality
72-A 4 months old, has chronic diarrhea, failure to thrive, fever, persistent
bronchiolitis for the last 4 weeks. You suspect severe combined immunodeficiency.
The finding on the CBC that will support your suspicion is which one of the
following?
A.
B.
C.
D.
E.
Anemia.
Thrombocytopenia.
Neutropenia.
Lymphopenia.
Low MCV
73- Conditions associated with Cholelithiasis in children include all EXCEPT:
A.
B.
C.
D.
E.
Spherocytosis
Sickle Cell Disease
Cystic Fibrosis
Acute Pancreatitis
Chronic Pancreatitis
74-A 7-year-old boy has crampy abdominal pain and a rash on the back of his legs
and buttocks as well as on the extensor surfaces of his forearms. Laboratory
analysis revealed proteinuria and microhematuria. He is most likely to be
affected by
A.
B.
C.
D.
E.
Systemic lupus erythrematosis.
Anaphylactoid purpura.
Poststreptococcal glomurolonephritis.
Polyarteritis nodosa.
Dermatomyositis.
75-Iron deficiency anemia in 1 year old male, (choose the correct statement)
A.
B.
C.
D.
E.
It is macrocytic anemia.
It is very rare in Palestine.
Red cell distribution width is normal
It is usually caused by incorrect nutritional practices.
Cancer colon should be ruled out.
17
76-You diagnosed a 6 months old male patient with simple uncomplicated inguinal
hernia. In discussing repair of the hernia with the parents, you should inform
them that:
A. Herniorraphy can be postponed until the age of 2 years because many hernias close
spontaneously.
B. Herniorraphy can be postponed until the age of 12 years because oligospermia only
occurs if repair is carried out before this age.
C. Herniorraphy should be scheduled at the earliest convenient time.
D. Herniorraphy should be scheduled as an emergency.
E. There is no need to repair a hernia in childhood unless incarceration occurs.
77-All of the following tests would be indicated in evaluation of a 2 years old child
with recurrent chest infections except?
A.
B.
C.
D.
E.
Quantitative immunoglobulin levels.
Complete blood count.
Sweat chloride test.
Spirometry.
Isohemagluttinins
78-A 6-week-old infant is admitted to the hospital because of obstructive jaundice.
Each of the following disorders could be responsible EXCEPT:
A.
B.
C.
D.
Cyctic fibrosis.
Choledochal cyst.
Alpha-antitrypsin deficiency.
Gilbert’s disease.
E. Biliary atresia
79-“Normal” or “physiologic” saline has each of the following characteristics
except:
A.
B.
C.
D.
E.
Na+ 154meq/L and Cl- 154 meq/L.
9 g of NaCl in 1 L of water.
Approximate isotonicity with blood.
Physiologic ratio of Na to Cl.
0.9% NaCl.
80-The amount of calories in the breast milk is close to
A.
B.
C.
D.
E.
24 kcal/oz .
28 kcal/oz .
20 kcal/oz .
15 kcal/oz .
30 kcal/oz .
18
81- A 6 month old patient with cow milk protein allergy. Which of the following
formulas is the treatment of choice?
A.
B.
C.
D.
E.
Protein hydrolysate formula.
Soy protein based formula.
Galactose free formula.
MCT oil formula.
Phenyl alanine free formula.
82-A 4 months old infant is brought to the emergency room with sudden onset of
lethargy, poor feeding, constipation, generalized hypotonia and weakness, and
ophthalmoplegia. The infant is a febrile. The mother sweetens the milk with
honey. The most likely diagnosis is
A.
B.
C.
D.
E.
Gray syndrome.
Hypothyroidism
Werding- Hoffmann’s disease.
Infant botulism.
Hypomagnesaemia
83-ALL the following statements about Wilson’s disease are true EXCEPT that:
A.
B.
C.
D.
E.
It is inherited as a sex-linked trait.
In children it may present with hepatomegaly and liver failure.
Ceruloplasmin levels are typically decreased.
Total serum copper concentration is usually low.
It is often associated with renal disease (Fanconi syndrome
84-The most important maneuver in preserving renal function in septic shock is:
A.
B.
C.
D.
Intravenous infusion of furosemide
Infusion of dopamine at a rate of 1-3 g/kg/min
Rapid restoration of the circulating volume
Proper antibiosis against the offending organism
19
85-
20
The above is the x-ray of lateral neck on a 7 year old child who presented with
significant stirdor, fever and drooling. All statements regarding his disease are true
except:A- Defer examination of the pharynx in this child with signs of moderate/severe respiratory
distress
B- Administer empiric antimicrobial therapy:
Cefotaxime OR ceftriaxone
PLUS Clindamycin OR vancomycin
C- The incidence of the disease shown in the x-ray did not decline following the introduction
Hib vaccine to the routine infant immunization schedule
D- Blood cultures are positive in the majority of children affected with this condition
E- Secure airway before diagnostic evaluation since respiratory distress is severe
For question86-88, read the following clinical scenario and answer the questions
below:21
"A 7 days old male newborn presents to the emergency room with vomiting, rapid
breathing and poor sucking of 1 day duration. His physical exam was positive for
lethargy, poor Moro and sucking reflexes and mild hepatomegaly. Septic work up was
performed and he was started on antibiotics. His routine lab tests were unremarkable
and blood gases showed no acidosis. Next day, serum lactate and ammonia were
obtained. Lactate was normal but he had high serum ammonia of 320
micromole/liter."
86. The most probable diagnosis is:A.
B.
C.
D.
E.
Isovaleric acidemia.
Methylmalonic acidemia.
Maple syrup urine disease.
Ornithine transcarnamylase (OTC) deficiency.
Nonketotic hyperglycinemia (NKH).
87. The mode of inheritance of this condition is:A.
B.
C.
D.
E.
Autosomal recessive.
Autosomal dominant.
X-linked recessive.
X-linked dominant.
None of the above.
88. What are the 2 important medications used to treat this condition?
A.
B.
C.
D.
E.
Sodium benzoate and sodium phenylacetate.
Sodium valproate and sodium bicarbonate.
Sodium citrate and sodium chloride.
Sodium acetate and sodium diclofenate.
Sodium bicarbonate and sodium phosphate.
Questions 89-91
Read the following clinical scenario and answer the questions below.
“Sami is a 4 year old boy who presents to the doctor’s office with coarse facial
features, stiffness of joints, short stature and mental retardation. His parents and a 10
yr old sister and an 8 year old brother all appear unaffected. Sami’s mother is
pregnant. She also had a brother who died at 15 years of age with similar findings
that seemed to worsen with age. She also has a nephew (her sister’s son) who has
similar features. You suspect a diagnosis of Hunter Syndrome.
89. The risk that the fetus is affected is:A.
B.
C.
D.
E.
100 %
67 %
50 %
25%
Virtually 0.
90. Amniocentesis is performed to determine the sex of the fetus. What is the risk
that the fetus will be affected if it is female?
22
A.
B.
C.
D.
E.
100 %
b 67 %
50%
25 %
Virtually 0.
91. What is the risk that the fetus will be affected if it is male?
A.
B.
C.
D.
E.
100 %
67 %
50%
25 %
Virtually 0.
Questions 92-96
Match the following,; each may be used once, more than once or not at all.
A.
B.
C.
D.
E.
Austosomal dominant
Austosomal recessive
X-linked recessive
Chromosomal
Multifactorial
92. Male to male transmission makes this mode unlikely.
93. Elevated maternal age is characteristic.
94. Parents with three affected children have a higher recurrence risk than parents
with two affected children.
95. Consanguinity is characteristic.
96. Mutation in one gene is enough to cause disease in both sexes.
97. Down syndrome is characterized by all of the following Except:
A.
B.
C.
D.
E.
It is the single most common genetic cause of moderate mental retardations.
The incidence in live births is approximately 1/750.
The life expectancy for children with Down syndrome is approximately 50 years.
The occurrence of trisomy 21 increase with advanced maternal age.
The parental origin of the supernumerary chromosome 21 is maternal in 60% of the cases
as a result of errors in meiosis.
For Questions 98-99
23
Read the following and answer the questions below:
A baby is born with spina bifida, a defect in the lower spinal cord and meninges
that may cause bladder and lower limb dysfunction. The family history reveals
that the father had a small spina bifida that was repaired by surgery,
98. Regarding this particular condition, choose the correct statement:A. Folic acid supplementation has been found to reduce the risk of neural tube defects and is
usually started shortly after the lady gets pregnancy.
B. Spina bifida patients usually have urinary incontinence and nocturnal enuresis because of
the paralysis of the muscles of the bladder neck.
C. In patients with meningomyelocoele hydrocephalus is rare and it usually develops because
of cord tethering.
D. Children with meningomyelocele have a high risk of developing latex allergy; therefore,
they should not be exposed to latex from birth.
E. High meningomyeloceles does not result in lower extremity paralysis.
99. The most likely diagnostic category of this condition is :A.
B.
C.
D.
E.
Multiple congenital anomalies.
Mendelian disorder.
Isolated major anomaly.
Isolated minor anomaly.
Multiple anomaly syndrome.
100.The recurrence risk for the couple's next child to have spina bifida is:A.
B.
C.
D.
E.
>1%
<1%
>6%
<6%
25%
24
25