NELSON'S MCQs Flashcards
1
Q
A mother of a 2-week-fullterm baby noted doll’s-eye movement of her baby eyes.
Of the following, The MOST appropriate next action is to
A. reassures her by informing her that, this is a normal reaction
B. refer the baby to an ophthalmologist
C. takes a detailed history of perinatal period
D. order brain ultrasound
E. order brain MRI
A
A- Normal finding
2
Q
he age at which the infant achieve early head control with bobbing motion when pulled to sit is A. 2 mo B. 3 mo C. 4 mo D. 5 mo E. 6 mo
A
(B). This is a developmental skill of 3 months of age which include also; lifts head and
chest with arms extended on prone; head above plane of body on ventral suspension;
reaches toward and misses objects; waves at toy; listens to music and says “aah, ngah”.
3
Q
The age at which the infant can reach an object, grasp it and bring it to mouth and seems exited when see the food is A. 4 mo B. 5 mo C. 6 mo D. 7 mo E. 8 mo
A
(A). By this age he also able to lifts head and chest, with head in approximately
vertical axis; achieve no head lag when pulled to sitting position, enjoys sitting with full
truncal support when held erect, pushes with feet when sees raisin, but makes no
move to reach for it, laughs out loud; and may show displeasure if social contact is
broken.
4
Q
By the age of 7 months the infant is able to do all the following EXCEPT A. transfer object from hand to hand B. bounces actively C. cruises D. grasp uses radial palm E. roll over
A
(C). Cruises or walks’ holding to furniture is by 10 months of age.
5
Q
You are specialist explaining the developmental implication of a 6-month-old baby
boy who “transfers object hand to hand” to college students.
Of the following, the BEST statement describing that is a/an
A. visuomotor coordination
B. voluntary release
C. comparison of objects
D. ability to explore small objects
E. increasing autonomy
A
(C). The developmental implication reflects cognitive development.
6
Q
A 10-month-old child can do all the following EXCEPT
A. follows one-step command without gesture
B. says “mama” or “dada”
C. points to objects
D. speaks first real word
E. inhibition to “no”
A
(D). This is a 12 months developmental skill.
7
Q
You are observing a 15-month-old toddler, he was able to do all the following EXCEPT A. walks alone B. makes tower of 3 cubes C. inserts raisin in a bottle D. responds to his/her name E. identifies 1 or more parts of body
A
(E). This is an 18 months developmental skill.
8
Q
The age by which the child can makes tower of 9 cubes and imitates circular stroke is A. 24 mo B. 30 mo C. 36 mo D. 42 mo E. 48 mo
A
(B). A 30 months old child also able to goes up stairs alternating feet, makes vertical
and horizontal strokes, refers to self by pronoun “I”; knows full name, helps put things
away and pretends in play.
9
Q
These facts are true regarding the developmental stage of preschool children
EXCEPT
A. handedness is achieved by 3 years of age
B. boys are usually later than girls in achieving bladder control
C. knowing gender by 4 years
D. egocentric thinking
E. musturbation
A
(C). Bowel and bladder control emerge during this period, with “readiness” for
toileting having large individual and cultural variation. Bed-wetting is normal up to age
4 yr in girls and age 5 yr in boys. Thinking in this period characterized by magical
thinking (the child might believe that people cause it to rain by carrying umbrellas, that
the sun goes down because it is tired), and egocentrism which refers to a child’s
inability to take another’s point of view. Gender identification usually achieved by 3
years of age. Curiosity about genitals and adult sexual organs is normal, as is
masturbation. But excessive masturbation interfering with normal activity, acting out
sexual intercourse, extreme modesty, or mimicry of adult seductive behavior all
suggests the possibility of sexual abuse or inappropriate exposure.
10
Q
A young couples of a 2-month-old baby girl with excessive crying seek your medical
advice. Mother said that the baby is crying about 3 hr in a day, 2-3 days per week. She
is intermittently spitting but she is gaining weight adequately. She is bottle fed baby.
Of the following, the LEAST important advice is to
A. master the situation in relaxed manner
B. adhere to precry cues
C. change milk formula
D. avoid sensory overstimulation
E. reassure about benign nature of illness
A
(C). Prolong crying episodes is present in about 20% of infants younger than 2 mo of
age.Crying (longer than 3 hr/day, longer than 3 days/wk lasting longer than 1 wk) and
more often crying/fussiness persisting longer than 3-5 mo might be associated with;
child abuse; behavioral problems when an older child (anxiety, aggression,
hyperactivity); decreased duration of breastfeeding and postnatal depression
(uncertain which is the cause or effect). Most infants with crying/fussiness do not have
gastroesophageal reflux, lactose intolerance, or cow-milk protein allergy.
11
Q
Between 2-6 months of life, the infant start to achieve a regular sleep–wake cycles.
All the following are true about infant sleep during this period EXCEPT
A. total sleep hours are about 14-16 hr/24 hr
B. sleeps about 9-10 hr concentrated at night
C. sleeps 2 naps/day
D. sleep electroencephalogram shows the mature pattern
E. the sleep cycle time is similar to that of adults
A
(E). The sleep cycle remains shorter than in adults (50-60 min vs approximately 90
min). As a result, infants who arouses frequently during the night, considered as
behavioral sleep problem.
12
Q
Of those babies who have prolonged crying episodes in the first 2 mo of life, the percentage that will remain having similar episodes is about 11 A. 1% B. 5% C. 10% D. 30% E. 50%
A
B
13
Q
The birthweight usually quadruples by the age of A. 1.5 yr B. 2 yr C. 2.5 yr D. 3 yr E. 3.5 yr
A
C
14
Q
The child who plays simple games (in “parallel” with other children); helps in
dressing and washes hands, his/her MOST appropriate developmental age is around
A. 15 mo
B. 18 mo
C. 24 mo
D. 30 mo
E. 48 mo
A
D
15
Q
The child who is able to imitates construction of “gate” of 5 cubes; draws a man with 2-4 parts besides head and identifies longer of 2 lines, his/her MOST appropriate developmental age is around A. 24 mo B. 30 mo C. 48 mo D. 54 mo E. 60 mo
A
C
16
Q
Regarding the physical growth of middle childhood (6-11 yr); all the following are true EXCEPT A. 3-3.5 kg wt increment/yr B. 6-7 cm height increment/yr C. brain myelinization stops by 8 yr D. first deciduous tooth falls by 6 yr E. risk for future obesity
A
(C). Brain myelinization continues into adolescence, with peak gray matter at 12-14
yr.
17
Q
The first permanent tooth to erupt is A. central incisor at 6 yr 13 B. molar at 6 yr C. premolar at 6-7 yr D. lower canine at 6-7 yr E. upper canine at 6-7 yr
A
(B). The first permanent molars usually erupt between ages 6 and 7 years. For that
reason, they often are called the “six-year molars.” They are among the “extra”
permanent teeth in that they don’t replace an existing primary tooth. These important
teeth sometimes are mistaken for primary teeth. The six-year molars also help
determine the shape of the lower face and affect the position and health of other
permanent teeth. The first deciduous teeth to fall are central incisors.
18
Q
The MOST common cause of sleeping difficulty in the first 2 months of life is
A. gastro-esophageal reflux
B. formula intolerance
C. colic
D. developmentally sleeping behavior
E. recurrent self resolving intussusceptions
A
(D). Most sleep issues that are perceived as problematic at this stage represent a
discrepancy between parental expectations and developmentally appropriate sleep
behaviors. Newborns that are noted by parents to be extremely fussy and persistently
difficult to console are more likely to have one of the mentioned medical issues.
19
Q
A mother of 4-month-old baby boy complaining that the baby never goes to sleep
unless he is rocked for quite long time. She needs to repeat the same issue whenever
he is awake at night. She was surprised as no one of her previous babies had the similar
behavior. She feels tired because of insufficient sleep.
Of the following, the MOST common cause of this baby problem is
A. an early signs of ADHD
B. behavioral insomnia of childhood, sleep-onset association
C. primary sleep disorder
D. restless legs syndrome
E. sleep terrors
A
(B). In this situation, the child learns to fall asleep only under certain conditions or
associations, which typically require parental presence, such as being rocked or fed,
and does not develop the ability to self-soothe. In babies, gradually weaning the child
from dependence, and in older children introduction of more appropriate sleep
associations that will be readily available to the child during the night (transitional
objects, such as a blanket or toy), in addition to positive reinforcement (i.e., stickers for
remaining in bed), is often beneficial
20
Q
All the following are compatible with the definition of obstructive sleep apnea
EXCEPT
A. repeated episodes of prolonged upper airway obstruction
B. apnea
C. ≥30% reduction in airflow
D. ≥ 30% O2 desaturation
E. disrupted sleep
A
(D). ≥ 3% O2 desaturation.
21
Q
All the following amino acids are indispensable in human diet EXCEPT 44 A. leucine B. methionine C. threonine D. valine E. alanine
A
(E). Alanine, aspartic acid, asparagine, glutamic acid, and serine are dispensable
amino acids.
22
Q
Essential fatty acids deficiency is associated with all the following features EXCEPT A. desquamating skin rashes B. alopecia C. thrombocytosis D. impaired immunity E. growth deficits
A
(C). Thrombocytopenia.
23
Q
All the following statements concerning iron requirement in children are true EXCEPT
A. breast milk provides optimal intake of iron for the first 4 months
B. iron present in animal protein is more bioavailable than that found in
vegetables
C. iron deficiency is the most common micronutrient deficiency
D. cow’s milk is a good source of bioavailable iron
E. iron supplements can interfere with zinc absorption
A
(D). Cow’s milk is a poor source of bioavailable iron.
24
Q
Zinc deficiency is associated with increased risk for all the following EXCEPT
A. stunting
B. impaired immune function
C. increased risk for respiratory diseases
D. increased risk for diarrheal diseases
E. increased risk for skin infections
A
(E). Zinc is Essential for proper growth and development, and an important catalyst
for about 100 specific enzymes.
25
```
Breast feeding has been suggested to have a possible protective effect against all
the following EXCEPT
A. otitis media
B. urinary tract infections
C. septicemia
D. childhood cancer
45
E. skin infections
```
(E). Diarrhea, otitis media, urinary tract infections, necrotizing enterocolitis,
septicemia, infant botulism, celiac disease, childhood cancer, allergy, hospitalizations,
and infant mortality all are conditions in which breastfeeding has been suggested to
have a possible protective effect.
26
```
An absolute contraindication to breastfeeding is
A. hepatitis c infection of the mother
B. alcohol intake
C. herpes simplex lesion of the lips
D. active pulmonary tuberculosis
E. maternal high grade fever
```
(D). Active pulmonary tuberculosis is a real contraindication to breastfeeding.
Herpes simplex infection of the breast is another contraindication.
27
Regarding breast engorgement; All the following are true EXCEPT
A. usually happens in the first stage of lactogenesis
B. poor breast feeding technique can cause engorgement
C. breastfeeding immediately at signs of infant hunger will eventually prevent
this
D. to reduce engorgement, breasts should be softened prior to infant feeding
with a combination of hot compresses and expression of milk
E. between feedings, cold compresses applied, and oral nonsteroidal antiinflammatory
medications administered
(A). Breast engorgement happens in the second stage of lactogenesis.
28
```
All the following organisms can cause mastitis EXCEPT
A. Staphylococcus aureus
B. Escherichia coli
C. Haemophilus influenza
D. Klebsiella pneumoniae
E. group B streptococcus
```
(E). Group A streptococcus, not group B.
29
```
The MOST commonly used index for nutritional status is
A. height-for-age
B. weight-for-height
C. body mass index
D. mid-upper arm circumference
E. weight-for-age
```
(E). Weight-for-age is the most commonly used index of nutritional status, although
a low value has limited clinical significance as it does not differentiate between wasting
and stunting. Weight-for-age has the advantage of being somewhat easier to measure
than indices that require height measurements.
30
```
For children <5 yr, the highest global prevalence of micronutrient and trace
elements deficiencies is that of
A. vitamin A deficiency
B. zinc deficiency
C. iron deficiency
D. iodine deficiency
E. copper deficiency
```
(A). For children <5 yr, the global prevalence is estimated to be 33% for vitamin A
deficiency, 29% for iodine deficiency, 17% for zinc deficiency, and 18% for irondeficiency
anemia. Prevalence of micronutrient deficiencies tends to be highest in
Africa.
31
```
In consideration of preventing infections in stabilization phase of malnutrition in
unimmunized 10-month-old boy.
Of the following, the vaccine that is recommended to be given is
A. oral polio vaccine
B. acellular pertussis vaccine
C. measles vaccine
D. pneumococcal vaccine
E. H. influenza vaccine
```
(C). Measles vaccine is given to unimmunized children age >6 mo.
32
```
The hallmark of refeeding syndrome is the development of severe
A. hypophosphatemia
B. hypokalemia
C. hypomagnesemia
D. hypernatremia
E. hyperglycemia
```
(A). The hallmark of refeeding syndrome is the development of severe
hypophosphatemia after the cellular uptake of phosphate duringthe 1st wk of starting
to refeed. Serum phosphate levels of ≤0.5 mmol/L can produce weakness,
rhabdomyolysis, neutrophil dysfunction, cardio-respiratory failure, arrhythmias,
seizures, altered level of consciousness, or sudden death.
33
```
The gastrointestinal hormone/response that stimulates appetite in children is
A. cholecystokinin
B. glucagon-like peptide-1
C. peptide yy
D. vagal neuronal feedback
E. ghrelin
```
(E). Gastrointestinal hormones, including cholecystokinin, glucagon-like peptide-1,
peptide YY, and vagal neuronal feedback promote satiety. Ghrelin stimulates appetite.
36.(E). Type 2 diabetes mellitus is a childhood obesity-associated comorbidity.
34
```
All the following are childhood obesity-associated comorbidities later in life EXCEPT
A. hypertension
B. gallbladder disease
C. tibia vara
D. asthma
E. type 1 diabetes mellitus
```
(E). Type 2 diabetes mellitus is a childhood obesity-associated comorbidity.
35
Brown-Vialetto-Van Laere syndrome (BVVLS), a neurologic disorder characterized
by progressive neurologic deterioration, sensorineural hearing loss, and pontobulbar
palsy usually responds to treatment with high doses of
A. niacin
B. biotin
C. riboflavin
D. pyridoxine
E. thiamin
C-Mutations in genes coding for riboflavin transporter proteins have been
identified in children with BVVLS.
36
The MOST convenient way to confirm a diagnosis of pellagra in children is
A. skin biopsy
B. urinary 2-pyridone
C. response to niacin treatment
D. urinary n1-methyl-nicotinamide
E. physical signs of glossitis and dermatitis
C-Rapid clinical response to niacin treatment is an important confirmatory test.
37
```
The MOST characteristic manifestation of pellagra is
A. dermatitis
B. dementia
C. diarrhea
D. anorexia
E. lassitude
```
(A). Dermatitis, the most characteristic manifestation of pellagra, can develop
suddenly or insidiously and may be initiated by irritants, including intense sunlight.
38
```
The dietary reference intake of pyridoxine for a healthy eight-month-old infant is
A. 0.1 mg/day
B. 0.3 mg/day
C. 0.5 mg/day
D. 1.0 mg/day
E. 3 mg/day
```
(B). 0-6 months: 0.1 mg/ day; 7-12 months: 0.3 mg/ day; 1-3 yr: 0.5 mg/day; 4-8 yr:
0.6 mg/day; 9-13 yr: 1.0 mg/day 14-18 yr: 1.2 - 1.3 mg/day.
39
```
Pyridoxine is not affected in patients receiving
A. isoniazid
B. valproate
C. phenytoin
D. penicillamine
E. corticosteroids
```
(B). Other distracters cause pyridoxine deficiency.
40
```
Folate is important during embryogenesis for the development of
A. lungs
B. genital organs
C. gastrointestinal system
D. central nervous system
E. cardiovascular system
```
D
41
All the following can cause folate deficiency EXCEPT
A. celiac disease
B. sickle cell anemia
C. 6-mercaptopurine therapy
D. inflammatory bowel disease
E. methylene tetrahydrofolate reductase deficiency
C Methotrexate not 6-mercaptopurine
42
25-hydroxy vitamin D level is reduced in
A. vitamin D deficiency
B. autosomal recessive hypophosphatemic rickets
C. autosomal dominant hypophosphatemic rickets
D. Fanconi syndrome
E. tumor-induced rickets
A
43
```
The best indicator of the body stores of vitamin C is
A. plasma ascorbate concentration
B. leukocyte ascorbate concentration
C. urinary ascorbate level
D. urinary amino acid level
E. plasma amino acid level
```
B
44
```
All the following are recognized causes of rickets EXCEPT
A. Mccune-Albright syndrome
B. epidermal nevus syndrome
C. tuberus sclerosis
D. neurofibromatosis
E. aluminum-containing antacids
```
(C). Aluminum-containing antacids cause phosphorus deficiency. McCune-Albright
syndrome, epidermal nevus syndrome, and Neurofibromatosis enhance renal losses.
45
```
The first factor to be affected by deficiency of vitamin K is
A. Factor I
B. Factor II
C. Factor VII
D. Factor IX
E. Factor X
```
(C). Factor VII has the shortest half life of the coagulation factors.
46
```
The MOST devastating consequence of untreated hypernatremia is
A. brain hemorrhage
B. seizures
C. central pontinemyelinolysis
D. extra pontinemyelinolysis
E. brain edema
```
(A). As the extracellular osmolality increases, water moves out of brain cells, leading
to a decrease in brain volume, this decrease can result in tearing of intra cerebral veins
and bridging blood vessels as the brain moves away from the skull and the meninges,
patients may have subarachnoid, subdural, and parenchymal hemorrhages. Seizures
and brain edema are more common during correction of hypernatremia.
47
```
Hypervolemic hyponatremia is caused by
A. cirrhosis
B. cerebral salt wasting
C. pseudohypoaldosteronism type I
D. obstructive uropathy
E. bowel obstruction
```
A
48
```
Asymptomatic hyponatremia is seen in
A. cirrhosis
B. tap water enema
C. child abuse
D. hyperglycemia
E. tubule -interstitial nephritis
```
(D). Because the manifestations of hyponatremia are a result of the low plasma
osmolality, patients with hyponatremia resulting from hyperosmolality do not have
symptoms of hyponatremia.
49
```
Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is characterized
by
A. extravascular volume expansion
B. high serum uric acid
C. high blood urea nitrogen
D. euvolemic hyponatremia
E. urine sodium <30 mEq/L
```
(D). Because SIADH is a state of intravascular volume expansion, low serum uric acid
and BUN levels are supportive of the diagnosis, the kidney increases sodium excretion
in an effort to decrease intravascular volume to normal; thus, the patient has a mild
decrease in body sodium(urine sodium >30 mEq/L).
50
```
The MOST common cause of hypokalemia in children is
A. alkalemia
B. distal rental tubular acidosis
C. gastroenteritis
D. diabetic ketoacidosis
E. loop diuretic
```
C
51
```
The mechanism of hypokalemia in emesis is mainly due to
A. gastric loss of potassium
B. gastric loss of hydrochloric acid
C. low aldosterone level
D. dehydration
E. hypomagnesaemia
```
(B). The gastric loss of hydrochloric acid (HCl), leading to a metabolic alkalosis and a
state of volume depletion. The kidney compensates for the metabolic alkalosis by
excreting bicarbonate in the urine, but there is obligate loss of potassium and sodium
with the bicarbonate. The volume depletion raises aldosterone levels, further
increasing urinary potassium losses and preventing correction of the metabolic
alkalosis and hypokalemia until the volume depletion is corrected.
52
```
The MOST common cause of hyperphosphatemia is
A. acute hemolysis
B. vitamin D intoxication
C. renal insufficiency
D. cow’s milk intake
E. hypoparathyroidism
```
C
53
```
Normal anion gap metabolic acidosis can occur in
A. renal failure
B. liver failure
C. severe anemia
69
D. malignancy
E. starvation ketoacidosis
```
(A). In renal failure, there is retention of unmeasured anions, including phosphate,
urate, and sulfate. The increase in unmeasured anions in renal failure is usually less
than the decrease in the bicarbonate concentration. Renal failure is thus a mix of an
increased gap and a normal gap metabolic acidosis. The normal gap metabolic acidosis
is especially prominent in children with renal failure as a result of tubular damage, as
occurs with renal dysplasia or obstructive uropathy, because these patients have a
concurrent RTA.Other distractors are causes of high anion gap metabolic acidosis.
54
Measuring serum concentrations of renin and aldosterone differentiates children
with metabolic alkalosis; both renin and aldosterone are elevated in
70
A. renovascular disease
B. glucocorticoid-remediable aldosteronism
C. Liddle syndrome
D. Cushing syndrome
E. 11β-hydroxylase deficiency
(A). Aldosterone is high and renin is low in patients with adrenal adenomas or
hyperplasia and glucocorticoid-remediable aldosteronism. Renin and aldosterone are
low in children with Cushing syndrome, Liddle syndrome, licorice ingestion, 17α-
74
hydroxylase deficiency, 11β-hydroxylase deficiency, and 11β-hydroxysteroid
dehydrogenase deficiency.
55
```
Increased production of CO2 occurs in all the following EXCEPT
A. fever
B. emesis
C. excess caloric intake
D. high levels of physical activity
E. increased respiratory muscle work
```
B
56
```
Very-low birth weight infants can have insensible losses of
A. 10-20 mL/kg/24 hr.
B. 30-40 mL/kg/24 hr.
C. 50-60 mL/kg/24 hr.
D. 70-80 mL/kg/24 hr.
E. 100-200 mL/kg/24 hr.
```
E
57
```
For each 1°C increase in temperature above 38°C leads to increase in maintenance
water needs by
A. 5-10%
B. 10-15%
C. 15-20%
D. 20-25%
E. 25-30%
```
B
58
```
The best fluid bolus giving to a child with isolated vomiting and severe dehydration
is
A. normal saline
B. ringer lactate
C. half-normal saline
D. hypertonic (3%) saline
E. 5% dextrose + half-normal saline
```
A
59
The features of high quality CPR include all the following EXCEPT
A. rate at least 100/min
B. compression depth of 2/3 anterior-posterior diameter of the chest
C. allow complete chest recoil after each compression
D. minimize interruptions in chest compressions
E. avoid excessive ventilation
(B). The Depth of chest compression should be at least 1/3 anterior-posterior
diameter of chest, about 1 1/2 inches (4 cm) in infants and 2 inches (5 cm) in children.
60
After activation of emergency medical system, the next action to unresponsive child
is to
A. asses the air ways
B. check pulse
C. give rescue breathings
D. start chest compression
E. attach automated external defibrillator (AED) to find shakable rhythm
(B). After activation of EMS the next step is to check for pulse and find definite pulse
within 10 seconds.
61
Regarding Glasgow Coma scale in pediatrics, all the following are true EXCEPT
A. in modified type it uses 15 score points
B. it has 3 components
C. valid as a prognostic scoring system
D. score ≤8 require aggressive management
E. verbal response component has 5 possible points
(C). Although the GCS has not been validated as a prognostic scoring system for
infants and young children as it has been in adults, it is commonly used in the
assessment of pediatric patients with an altered level of consciousness. The GCS is the
most widely used method of evaluating a child’s neurologic function and has 3
components. Individual scores for eye opening, verbal response, and motor response
are added together, with a maximum of 15 points. Patients with a GCS score ≤8 require
aggressive management, including stabilization of the airway and breathing with
109
endotracheal intubation and mechanical ventilation, respectively, and, if indicated,
placement of an intracranial pressure monitoring device.
62
In pediatrics advanced life support (PALS) curriculum, regarding the format of
ABCDE, all the following are true EXCEPT
A. A refers to assessment of airways
B. B refers to assessment of breathing
C. C refers to assessment of circulation
D. D refers to assessment of dehydration
E. E refers to assessment of unidentified injuries
(D). The D means Disability which refers to assessment of the child’s neurologic
function in terms of the level of consciousness and cortical function. It can be assessed
quickly by checking the pupilary light respose and using Glasgow Coma scale, so the
response D is only partially true. The causes of decreased level of consciousness in
children are numerous and include conditions as diverse as respiratory failure with
hypoxia or hypercarbia, hypoglycemia, poisonings or drug overdose, trauma, seizures,
infection, and shock. All that system is represent the primary assessment of critically ill
child.
63
You received a call from your neighbor, he had a 12-month-old baby boy who
suddenly chock with a candy. When you arrive you find the baby unconscious.
Of the following, the NEXT proper action is to
A. open airway and give rescue breathing
B. give 5 back blows
C. give 5 chest thrusts
D. a series of 5 back blows and chest thrusts
E. perform endotracheal intubation
(A). In unconscious child, the child should be gently placed on the ground, supine,
and then the provider should open the airway with the head-tilt/chin-lift maneuver and
attempt mouth-to-mouth ventilation. If ventilation is unsuccessful, the airway is
repositioned, and ventilation attempted again. If there is still no chest rise, attempts to
remove a foreign body are indicated. In an infant <1 yr old, a combination of 5 back
blows and 5 chest thrusts is administered, After each cycle of back blows and chest
thrusts, the child’s mouth should be visually inspected for the presence of the foreign
body. If identified within finger’s reach, it should be removed with a gentle finger
sweep. If no foreign body is visual, ventilation is again attempted. If this is unsuccessful,
110
the head is repositioned, and ventilation attempted again. If there is no chest rise, the
series of back blows and chest thrusts is repeated. For a conscious child >1 yr old,
providers should give a series of 5 abdominal thrusts (Heimlich maneuver) with the
child standing or sitting; this should occur with the child lying down if unconscious.
64
All the following are an absolute indications for endotracheal intubation EXCEPT
A. inability to protect the airway against aspiration
B. failing to maintain adequate oxygenation
C. complete airway obstruction
D. failing to control blood carbon dioxide levels
E. paralysis is required for a procedure
(C). In settings of known complete airway obstruction, endotracheal intubation
should be avoided, and emergency cricothyroidotomy performed instead.
65
The MOST common pre-arrest rhythms in young children is
A. bradyarrhythmia
B. atrial flutter
C. ventricular fibrillation
D. supraventricular arrhythmia with WPW syndrome
E. atrioventricular block
A
66
You were engaged in CPR for 3-year-old child found unresponsive in the ward. You
were performing cycles of 15 chest compressions and 2 breathings alternatively with
102
another medical personal. After 2 min of resuscitation you evaluate the pulse which
shows PEA (pulseless electrical activity) status. The AED already is attached.
Of the following, the correct NEXT action is to
A. give a shock of 0.5 joule/kg
B. give a shock of 1 joule/kg
C. give a shock of 2 joules/kg
D. give adrenaline 0.01mg/kg iv
E. give adrenaline 0.1mg/kg iv
(D). Pulseless electrical activity (PEA) is a clinical condition characterized by
unresponsiveness and lack of palpable pulse in the presence of organized cardiac
electrical activity. In this situation (and in asystole) adrenaline is indicated in cycles of 3-
5 min, considering an advanced airways and reevaluation for the pulse status; if it
became shockable give shock of 2 joules/kg. You need to look also for correctable
causes (the 6H and 5T). If it became normal that goes for (ROSC) post-cardiac arrest
care.
67
An 11-year-old adolescent boy came to your clinic with a complaint of been
collapsed while bathing with hot water after he felt dizzy. Father confirms that the boy
was extremely pale when founded. He added that he toke few minutes to recover, also
he had past history of similar condition one month ago when he was urinating.
Of the following, the MOST likely explanation for this condition is
A. long QT syndrome
B. hypertrophic cardiomyopathy
C. neurocardiogenic syncope
D. drug abuse
104
E. seizure disorder
(C). Typically, the patient with syncope will have been standing for a period of time,
often on a hot day, or has gotten up suddenly from sleep or resting in a supine position.
Occurrence in the shower is common, presumably caused by standing and vasodilation
caused by hot water. For boys, the occurrence while urinating while standing is
sometimes reported. The occurrence of syncope in girls while sitting or standing and
having their hair brushed is common.
68
You are treating an 8-month-old baby in intensive care unit with cold shock. In the
first 15 min he received adequate fluid therapy and a colleague had started dopamine
for him. The baby condition is still unresponsive.
Of the following, the BEST option now is to start
A. epinephrine
B. hydrocortisone
C. norepinephrine
D. terlipressin
E. angiotensin
(A). After 15 min of treating shock, inotropes like dopamin need to be started, if the
shock still unresponsive epinephrine for cold and norepinephrine for warm shock
should be titrated. Hydrocortisone is considered after 60 min (Catecholamine resistant
shock). Other options are used in warm shock and usually after other options are
exhausted.
69
Septic shock usually shows a combination of hypovolemic and distributive shock in
addition to
A. obstructive shock
B. cardiogenic shock
C. acute respiratory distress syndrome
D. disseminated intravascular coagulation
E. decreased steroid synthesis
B
70
A 2-hour-old fullterm baby delivered to a mother with gestational diabetes by CS,
he developed an increasing respiratory distress and grunting with blood gases values
indicative of respiratory failure.
Of the following, the BEST mode to be used in mechanical ventilation is
A. intermittent mechanical ventilation (IMV)
B. synchronized Intermittent mechanical ventilation (SIMV)
C. synchronized Intermittent positive pressure ventilation (SIPPV)
D. pressure-regulated volume control (PRVC)
E. pressure support ventilation (PSV)
(D). IMV is not the choice because you need to paralyze the patient; options B and C
both are good choice as they provide synchrony with the patient and can achieve the
goal of ventilation by either controlling the pressure or volume values but in presence
the option D in your ventilator, making it the best because it will reduces both baro and
volutrauma.
71
```
In mechanical ventilation giving adequate PEEP can mainly reduces
A. volutrauma
B. barotrauma
C. oxytrauma
D. pneumonia
E. post-extubation airway obstruction
```
C
72
You are ventilating a fullterm baby with meconium aspiration syndrome, he was
fine on the machine, suddenly the monitor shows severe drop in O2 saturation. You
checked the ventilator; it was working well with its connections.
Of the following, the MOST likely cause is
A. O2 source supply
B. endotracheal tube blockade
C. self extubation
D. low inspiratory pressure value
E. low PEEP value
(B). All the given options are true causes for sudden drop of O2. The possible causes
of desaturating baby on ventilator are; Oxygen supply, machine and it is connections,
settings, tube block, self extubation and the patient himself. In the given scenario, the
most likely cause is B because of meconium aspiration. The best action to such scenario
is to disconnect the baby from the machine and use Ambu bagging gently to check the
air entry and rise of O2 saturation. If no rise you need to change the tube. In case of
unequal air entry, pneumothorax is another possibility.
73
A 32-week-preterm baby on mechanical ventilation, he is recovering from RDS, his
blood gas is satisfactory, and you reduced the ventilatory settings in process for
weaning.
Of the following, the MOST valuable indicator that the baby is ready for extubation is
A. frequency of respiration 35
B. fraction of inspired o2 0.55
C. peak expiratory end pressure 6
D. inspiratory pressure 8
E. inspiratory time 0.4 sec
(D). In absence of tachypnea, increased work of breathing, hypoxemia, hypercapnia,
acidosis, diaphoresis, tachycardia, and hypotension and when the ventilator rate is <5
breaths/min or (between 5-10 cm H2O in pressure controlled devices) indicate that the
contribution of the ventilation to minute ventilation is minimal and the baby is ready to
be extubated.
74
```
Prior to extubation and in order to reduce airway narrowing after extubation it is
advised to use
A. dexamethasone IV
B. betamethasone IV
C. inhaled budesonide
D. nebulized budesonide
E. hydrocortisone IV
```
(A). Administration of intravenous dexamethasone 0.5 mg/kg every 6 hr for 4 doses
prior to extubation has been shown to minimize the incidence of postextubation airway
obstruction. In patients in whom postextubation airway obstruction develops, the need
for re-intubation may be obviated by administration of nebulized racemic epinephrine
and heliox.
75
```
All the following mechanisms are contributing in pathophysiology of drowning
EXCEPT
A. laryngospasm
B. surfactant washout
C. anoxic injury
D. pulmonary aspiration
E. circulatory overload
```
E
76
```
A 4-year-old female child, a known case of ALL, she is developing an increasing
toxicity to methotrexate therapy.
Of the following, the MOST useful test is
115
A. homocysteine level
B. prothrombin test
C. factor V leiden
D. MTHFR DNA testing
E. folate level
```
(D). Knowledge of individual genotypes (pharmacogentic genetic testing) will guide
pharmacologic therapy, allowing customization of choice of drug and dosage to avoid
toxicity and provide a therapeutic response. An example of this is testing for
polymorphisms within the methylenetetrahydrofolate reductase (MTHFR) gene for
susceptibility of potentially increased toxicity to methotrexate antimetabolite therapy
for treatment of acute lymphoblastic leukemia.
77
The following are indications for genetic counseling EXCEPT
A. paternal age > 50 years
B. maternal age > 45 years
C. family history of intellectual disability
D. consanguinity
E. infertility
(B). Maternal age > or equal to 35 is an indication for genetic counseling. Genetic
counseling is a communication process in which the genetic contribution to health is
explained, along with specific risks of transmission of a trait and options to manage the
condition and its inheritance. The counselor is expected to present information in a
neutral, nondirective manner and to provide support to the individual and family to
cope with decisions that are made.
78
```
The organ that can be spared in mitochondrial disorders is
A. brain
B. eye
C. small intestine
D. liver
E. muscle
```
(C). The mitochondria are the cell’s suppliers of energy, so the organs that are most
affected by the presence ofabnormal mitochondria are those that have the greatest
energyrequirements, such as the brain, muscle, heart, and liver. Common
manifestations include developmentaldelay, seizures, cardiac dysfunction, decreased
muscle strengthand tone, and hearing and vision problems. Examples of
mitochondrialdisorders include MELAS (myopathy, encephalopathy, lactic acidosis,
andstrokelike episodes), MERRF (myoclonic epilepsy associated with ragged red fibers),
and Kearns-Sayre syndrome (ophthalmoplegia, pigmentary retinopathy, and
cardiomyopathy).
79
```
The MOST important reason for chromosomal analysis in persons suspected of
having Down syndrome is to
120
A. confirm clinical diagnosis
B. detect mosaicism
C. reassure the family
D. detect translocations
E. detect rare cases of ring chromosome
```
(D). Chromosome analysis is indicated in every person suspected of having Down
syndrome. If a translocation is identified, parental chromosome studies must be
performed to determine whether one of the parents is a translocation carrier, which
carries a high recurrence risk for having another affected child.
80
```
All the following are features of Edwards syndrome (Trisomy 18) EXCEPT
A. low birth weight
B. closed fists
C. cardiac malformations
D. short sternum
E. slopping forehead
```
(E). Slopping forehead is a feature of Trisomy 13.
81
```
All the following are features of Patau syndrome (Trisomy 13) EXCEPT
A. scalp defects
B. corneal abnormalities
C. holoprosencephaly
D. capillary hemangiomas
E. prominent occiput
```
(E). Prominent occiput is a feature of Edwards’s syndrome.
82
```
All the following are examples of microdeletion syndromes EXCEPT
A. Williams
B. Prader-Willi
C. Angelman
D. DiGeorge
E. Wolf-Hirschhorn
```
(E). Microdeletions involve loss of small chromosome regions so that the affected
individuals can have a distinctive phenotype depending on the number of genes
involved. All the mentioned syndromes are common microdeletions and the board
candidate should be familial with their phenotypic descriptions and clinical
manifestations. Wolf-Hirschhorn syndrome is chromosomal deletion syndrome.
83
```
All the following are recognized features of Turner syndrome EXCEPT
A. female phenotype
B. male phenotype
C. hypothyroidism
D. type I diabetes mellitus
E. gonadal dysgenesis
```
(D). Male phenotype can occur with mosaic type of Turner syndrome, and type II
insulin resistance is the true answer.
84
A 28-day-old girl presented with lethargy, poor feeding, and repeated vomiting for
last 5 days; IV fluid and empirical antibiotics were started; later she develops repeated
seizures not responding to IV calcium, glucose, B6, and anticonvulsant drugs. Septic
screen is negative; serum ammonia is elevated with normal anion gap and normal pH.
Family history reveals 2 siblings died with same scenario.
Of the following, the MOST likely diagnosis is
A. galactosemia
B. hyperglycinemia
C. organic acidemias
D. phenylketonurea
128
E. urea cycle defects
(E). Elevation of blood ammonia is usually caused by defects of urea cycle enzymes.
Infants with elevated blood ammonia levels from urea cycle defects commonly have
normal serum pH and bicarbonate values; without measurement of blood ammonia,
they may remain undiagnosed and succumb to their disease.
85
You suspect a metabolic problem in a 30-day-old girl presented with poor feeding,
vomiting, lethargy, and convulsion. Previous sibling died with the same condition.
Serum ammonia, pH, HCO3, and anion gap are normal.
Of the following, the MOST likely diagnosis is
A. porphyria
B. galactosemia
C. organic acidemia
D. urea cycle defect
E. aminoacidopathy
E
86
A 6-year-old boy with eczematoid rash, abnormal face, and prominent maxilla
complains from deterioration in school performance, hyperactivity, and seizures for the
last 2 years.
Of the following, the MOST likely enzymatic deficiency is
A. acid β-glucosidase
B. β-hexosaminidases
C. acid sphingomyelinase
D. phenylalanine hydroxylase
E. fumarylacetoacetate hydrolase
```
(D). Phenylketonurea is the most likely diagnosis; deficiency of the enzyme
phenylalanine hydroxylase (PAH) or of its cofactor tetrahydrobiopterin (BH4) causes
accumulation of phenylalanine in body fluids and in the brain.
```
87
A 10-month-old boy presented with failure to thrive, fever, jaundice, hepatomegaly,
and severe rickets. Investigations revealed hypoglycemia and normal anion gap
metabolic acidosis.
Of the following, the MOST likely enzyme deficiency is
A. acid β-glucosidase
B. β-hexosaminidases
C. acid sphingomyelinase
D. phenylalanine hydroxylase
E. fumarylacetoacetate hydrolase
(E). Tyrocinemia type 1 is a severe disease of the liver, kidney, and peripheral nerves
are caused by a deficiency of the enzyme fumarylacetoacetate hydrolase.
acid β-glucosidase ----------------- Gaucher disease
phenylalanine hydroxylase ------ Phenylketonurea
acid sphingomyelinase ---- Niemann-pick disease
fumarylacetoacetate hydrolase -------Tyrosinemia
β-hexosaminidases -----------=-- Tay-Sachs disease
88
```
The following are ocular manifestations of albinism EXCEPT
A. red reflex
B. strabismus
131
C. refractive errors
D. foveal hyperplasia
E. lack of binocular vision
```
(D). Ocular manifestations of albinism include hypopigmentation of iris and retina,
foveal hypoplasia, reduced visual acuity, refractive errors, nystagmus, alternating
strabismus, and a red reflex.
89
A 6-year-old girl that looks tall and thin with light skin. On examination she has a
peculiar malar flush, subluxation of the ocular lens, developmental delay, and severe
hypertension.
Of the following,the MOST likely cause of hypertension is
A. atherosclerosis
B. hyperthyroidism
C. thromboembolism
D. coarctation of aorta
E. renal artery stenosis
(C). In Homocystinuria, thromboembolic episodes involving both large and small
vessels, especially those of the brain, are common and may occur at any age. Optic
147
atrophy, paralysis, cor pulmonale, and severe hypertension (from renal infarcts) are
among the serious consequences of thromboembolism, which is caused by changes in
the vascular walls and increased platelet adhesiveness secondary to elevated
homocystine levels.
90
```
The MOST appropriate treatment of homocystinuria is
A. betaine
B. folic acid
C. vitamin C
D. vitamin B6
E. methionine restriction
```
(D). Treatment with high doses of vitamin B6 (200-1,000 mg/24 hr) causes dramatic
improvement in most patients who are responsive to this therapy.
91
A 2-year-old boy presented with failure to thrive, regression of developmental
milestone, and difficult to contact with surroundings. On examination, there are
hepatosplenomegaly and moderate lymphadenopathy.
Of the following, the MOST likely diagnosis is
A. Gaucher disease
B. Sandhoff disease
C. Tay-Sachs disease
133
D. Von Gierke disease
E. Niemann-pick disease
(E). Niemann-pick disease (NPD), inherited as autosomal recessive traits, the clinical
manifestations and course of type A NPD is uniform and is characterized by a normal
appearance at birth. Hepatosplenomegaly, moderate lymphadenopathy, and
psychomotor retardation are evident by 6 mo of age, followed by neurodevelopmental
regression and death by 3 yr. With advancing age, the loss of motor function and the
deterioration of intellectual capabilities are progressively debilitating; and in later
stages, spasticity and rigidity are evident. Affected infants lose contact with their
environment. Type B disease is a non-neuronopathic form observed in children and
adults NPD types A and B result from the deficient activity of acid sphingomyelinase.
Type C disease is a neuronopathic form that results from defective cholesterol
transport.
92
A 9-month-old boy presented with persistent head lag, rapid breathing, difficult
feeding, and hepatomegaly. Chest X- ray showed cardiomegaly.
Of the following, the MOST appropriate confirmatory test is
A. liver biopsy
B. muscle biopsy
C. electrocardiography
D. serum creatine phosphokinase
135
E. enzyme assay in dried blood spots
(E). The confirmatory step for a diagnosis of Pompe disease is enzyme assay
demonstrating deficient acid α-glucosidase. The enzyme assay is usually done in dried
blood spots, leukocytes, blood mononuclear cells, muscle, and cultured skin fibroblasts.
93
A 28-day-old boy presented with prolonged neonatal jaundice, lethargy, poor
feeding, and failure to regain birth weight. On examination there are hepatomegaly
and cataract.
Of the following, the MOST likely injured organs in this disease are
A. eyes, liver, and brain
B. kidney, liver, and eyes
C. kidney, liver, and brain
D. kidney, heart , and brain
E. pancreas, liver, and brain
(C). In galactosemia, without the transferase enzyme, the infant is unable to
metabolize galactose-1-phosphate, the accumulation of which results in injury to
kidney, liver, and brain.
94
A 20-day-old girl presented with prolonged neonatal jaundice, lethargy, and poor
feeding treated as sepsis by empirical antibiotics and nothing by mouth. The patient
got partial response, when oral feeding resumed, the condition deteriorate again.
In this condition, the blood culture MOST likely reveals growth of
A. Escherichia coli
B. Staphylococcal aureus
C. group B streptococcus
D. Listeria monocytogens
E. Pseudomonas aeruginosa
(A). Patients with galactosemia are at increased risk for Escherichia coli neonatal
sepsis; the onset of sepsis often precedes the diagnosis of galactosemia.
95
```
All the following drugs are unsafe in acute intermittent porphyria EXCEPT
A. nifedipine
B. diclofenac
C. cimetidine
D. valproic acid
E. carbamazepine
```
C
96
```
All the following are clinical features of acute intermittent porphyria EXCEPT
A. neuropathy
B. tachycardia
C. abdominal pain
D. bladder dysfunction
E. cutaneous photosensitivity
```
E Porphyria cutanea tarda
97
```
The MOST common presentation of primary carnitine deficiency is
A. liver disease
B. cardiomyopathy
C. skeletal myopathy
D. endocardial fibroelastosis
E. hypoketotic hypoglycemia
```
(B). The most common presentation of primary carnitine deficiency is progressive
cardiomyopathy with or without skeletal muscle weakness beginning at 1-4 yr of age.
98
```
Leukokoria (white pupillary reflex) in newborn infant suggests the following
disorders EXCEPT
A. cataracts
B. tumor
C. congenital glaucoma
D. chorioretinitis
E. retinopathy of prematurity
```
(C). A cornea >1cm in diameter in a term infant (with photophobia and tearing)
suggests congenital glaucoma and requires prompt ophthalmologic consultation.
99
```
A single umbilical artery in a newborn infant increases the risk for
A. meningomyelocele
B. occult renal anomaly
C. omphalocele
D. gastroschisis
E. omphalitis
```
(B). A single umbilical artery increases the risk for an occult renal anomaly.
100
```
The following drugs can be given with caution to breast-feeding mother
A. psychotropic drugs
B. amphetamines
C. bromocriptine
D. chloramphenicol
E. methimazole
```
(A). Amphetamines, bromocriptine, chloramphenicol, and methimazole are
contraindicated in breast-feeding mother.
101
```
One of the following drugs may cause pyloric stenosis if administered to a
premature infant
A. intravenous vitamin E
B. indomethacin
C. enteric gentamicin
D. prostaglandins
E. dexamethasone
```
(D). Prostaglandins and erythromycin may cause pyloric stenosis if administered to a
premature infant.
102
Seizures beginning in the delivery room or shortly thereafter may be due to
A. hypoxic-ischemic encephalopathy
B. unintentional injection of maternal local anesthetic into the fetus
C. intracranial hemorrhage
D. cerebral anomaly
E. hypoglycemia
(B). Seizures beginning in the delivery room or shortly thereafter may be due to the
unintentional injection of maternal local anesthetic into the fetus. Convulsions may
also result from hyponatremia and water intoxication in the infant after the
administration of large amounts of hypotonic fluid to the mother shortly before and
during delivery.
103
```
Vomiting in the neonatal period is usually due to
A. pyloric stenosis
B. milk allergy
C. overfeeding
D. stress ulcer
E. an inborn error of metabolism
```
(C). Vomiting during the 1st day of life suggests obstruction in the upper digestive
tract or increased intracranial pressure. Roentgenographic studies are indicated when
obstruction is suspected. Vomiting may also be a nonspecific symptom of an illness
such as septicemia. It is a common manifestation of overfeeding, inexperienced feeding
technique, or normal reflux and is rarely due to pyloric stenosis, milk allergy, duodenal
ulcer, stress ulcer, an inborn error of metabolism (hyperammonemia, metabolic
acidosis), or adrenal insufficiency.
104
Early postnatal exposure to dexamethasone, within the 1st wk of life, in VLBW
infants, is associated with the following EXCEPT
A. metabolic derangements
B. poor growth
C. increased risk for sepsis
D. increased risk of spontaneous bowel perforation
E. cerebral palsy
(E). Infants exposed to postnatal steroids after the 1st wk of life have an increased
risk of cerebral palsy and developmental delay.
105
The following are true regarding apnea EXCEPT
A. apnea is a common problem in preterm infants
B. in term infants, apnea is always worrisome
C. obstructive apnea is characterized by absence of airflow but persistent chest
wall motion
D. serious apnea is defined as cessation of breathing for longer than 20 sec
E. bradycardia follows the apnea by 1-2 sec in more than 95% of cases and is most
often nodal
(E). Bradycardia follows the apnea by 1-2 sec in more than 95% of cases and is most
often sinus, but on occasion it can be nodal. Vagal responses and, rarely, heart block
are causes of bradycardia without apnea.
106
Regarding CPAP, the following are true EXCEPT
A. its prevents collapse of surfactant-deficient alveoli
B. its indicated, if oxygen saturation cannot be kept > 95%
C. its improves functional residual capacity FRC
D. its improves ventilation-perfusion matching
E. its reduces ventilatory needs
(B). Warm humidified oxygen should be provided at a concentration initially
sufficient to keep arterial oxygen pressure between 50 and 70 mm Hg (91-95%
saturation) in order to maintain normal tissue oxygenation while minimizing the risk of
oxygen toxicity. If oxygen saturation cannot be kept >90% at inspired oxygen
concentrations of 40-70% or greater, applying CPAP at a pressure of 5-10 cm H2O via
nasal prongs is indicated and usually produces a rapid improvement in oxygenation.
CPAP reduces collapse of surfactant-deficient alveoli and improves both FRC and
ventilation–perfusion matching.
107
```
One of the following is FALSE in the chest radiograph of transient tachypnea of the
newborn (TTN)
A. prominent pulmonary vascular markings
B. fluid in the intralobar fissures
C. overaeration
D. flat diaphragms
E. diffuse reticulogranular pattern
```
(E). The distinctive features of transient tachypnea are rapid recovery of the infant
and the absence of radiographic findings for RDS (hypoaeration, diffuse
reticulogranular pattern, air bronchograms) and other lung disorders.
108
```
The following are predisposing factors for persistent pulmonary hypertension of the
newborn (PPHN) EXCEPT
A. anemia
B. meconium aspiration syndrome
C. early-onset sepsis
D. hypoglycemia
E. birth asphyxia
```
(A). Persistent pulmonary hypertension of the newborn (PPHN) occurs in term and
post-term infants. Predisposing factors include birth asphyxia, MAS, early-onset sepsis,
RDS, hypoglycemia, polycythemia, maternal use of nonsteroidal anti-inflammatory
drugs with in utero constriction of the ductus arteriosus, maternal late trimester use of
selective serotonin reuptake inhibitors, and pulmonary hypoplasia due to
diaphragmatic hernia, amniotic fluid leak, oligohydramnios, or pleural effusions. PPHN
is often idiopathic.
109
Congenital diaphragmatic hernia (CDH) can be diagnosed on prenatal
ultrasonography (between 16 and 24 wk of gestation) in > 50% of cases.
Findings on ultrasonography may include the following EXCEPT
A. chest mass
B. mediastinal shift
C. gastric bubble
D. olighydramnios
E. a liver in the thoracic cavity
(D). Polyhydramnios
110
Absolute indication for surgery in neonatal necrotizing enterocolitis (NEC) include
A. positive result of abdominal paracentesis
B. failure of medical management
C. a single fixed bowel loop on radiographs
D. abdominal wall erythema
E. a palpable mass
(A). A surgeon should be consulted early in the course of treatment. Indications for
surgery include evidence of perforation on abdominal roentgenograms
(pneumoperitoneum) or positive result of abdominal paracentesis (stool or organism
on Gram stain preparation from peritoneal fluid). Failure of medical management, a
single fixed bowel loop on radiographs, abdominal wall erythema, and a palpable mass
are relative indications for exploratory laparotomy.
111
```
The following factors are associated with decreased risk of significant jaundice
EXCEPT
A. gestational age ≥41 wk
B. male gender
C. exclusive bottle-feeding
D. black race
E. discharge from hospital after 72 hr
```
(B). Male gender is a minor risk factor.
112
Jaundice, consisting of either indirect or direct bilirubin that is present at birth or
appears within the 1st 24 hr of life may be due to all the following EXCEPT
A. erythroblastosis fetalis
B. Crigler-Najjar syndrome
C. concealed hemorrhage
D. sepsis
E. congenital infections
(B). Jaundice that first appears on the 2nd or 3rd day is usually physiologic but may
represent a more severe form. Familial nonhemolytic icterus (Crigler-Najjar syndrome)
and early-onset breast-feeding jaundice are seen initially on the 2nd or 3rd day.
113
```
Early ultrasonographic signs of hydrops include
A. double–bowel wall sign (bowel edema)
B. polyhydramnios
162
C. ascites
D. pleural effusions
E. scalp edema
```
(A). Early ultrasonographic signs of hydrops include organomegaly (liver, spleen,
heart), the double–bowel wall sign (bowel edema), and placental thickening.
Progression to polyhydramnios, ascites, pleural or pericardial effusions, and skin or
scalp edema may then follow.
114
The blood smear of infant with hemolytic disease of the newborn (erythroblastosis
fetalis) typically shows the following EXCEPT
A. polychromasia
B. increasedreticulocyte count
C. a marked increase in nucleated RBCs
D. thrombocytopenia
E. low white blood cell count
(E). The white blood cell count is usually normal but may be elevated.
115
One of the following is FALSE in hemolytic disease of the newborn caused by Rh
incompatibility
A. infant is Rh Positive (D, sometimes C)
B. never occur in first-born infant
C. maternal antibody titers can help predict the severity of fetal disease
D. severe anemia is frequent
E. stillbirth/hydrops is frequent
B). 5% of first-born infants may be affected.
116
In Infants of diabetic mothers, the following are true EXCEPT
A. hypoglycemia develops in about 25-50% of infants of diabetic mothers
B. increased weight of the placenta and all infant organs
C. heart failure occurs in 5-10% of infants of diabetic mothers
D. the incidence of congenital anomalies is increased threefold in infants of
diabetic mothers
E. lumbosacral agenesis is one of most common congenital anomalies
(B). The probable pathogenic sequence is that maternal hyperglycemia causes fetal
hyperglycemia, and the fetal pancreatic response leads to fetal hyperinsulinemia; fetal
174
hyperinsulinemia and hyperglycemia then cause increased hepatic glucose uptake and
glycogen synthesis, accelerated lipogenesis, and augmented protein synthesis. Related
pathologic findings are hypertrophy and hyperplasia of the pancreatic islet β cells,
increased weight of the placenta and infant organs except for the brain, myocardial
hypertrophy, increased amount of cytoplasm in liver cells, and extramedullary
hematopoiesis. The incidence of congenital anomaliesis increased 3-fold in infants of
diabetic mothers; cardiac malformations (ventricular or atrial septal defect,
transposition of the great vessels, truncus arteriosus, doubleoutlet right ventricle,
tricuspid atresia,
117
```
Agents that commonly cause nosocomialneonatal infection are
A. group B streptococci GBS
B. coagulase-negative staphylococci
C. proteus
D. gonococci
E. chlamydiae
```
(B). Agents that commonly cause nosocomial infection are coagulase-negative
staphylococci, gram-negative bacilli (E. coli, Klebsiella pneumoniae, Salmonella,
Enterobacter, Citrobacter, Pseudomonas aeruginosa, Serratia), enterococci, S. aureus,
and Candida. Viruses contributing to nosocomial neonatal infection include
enteroviruses, CMV, hepatitis A, adenoviruses, influenza, respiratory syncytial virus
(RSV), rhinovirus, parainfluenza, HSV, and rotavirus. Community-acquired pathogens
such as Streptococcus pneumoniae may also cause infection in newborn infants after
discharge from the hospital.
118
```
Limb hypoplasia may be a feature of the following transplacental infections
A. cytomegalovirus
B. herpes simplex virus
166
C. varicella-zoster virus
D. HIV
E. rubella
```
C
119
```
Intracranial calcification may be a feature of one of the following transplacental
infections
A. cytomegalovirus
B. herpes simplex virus
C. varicella-zoster virus
D. syphilis
E. rubella
```
(A). Intracranial calcification may be a feature of CMV, HIV, toxoplasmosis, and T.
cruzi.
120
```
Pneumatosis intestinalis is pathognomonic for
A. Hirschsprung's disease
B. necrotizing enterocolitis (NEC)
C. pseudomembranous enterocolitis
D. neonatal ulcerative colitis
E. meconium ileus
```
(E). Pneumatosis intestinalis can be seen in various other conditions, including
Hirschsprung's disease, pseudomembranous enterocolitis, neonatal ulcerative colitis,
and ischemic bowel disease. However, it is a characteristic finding in 85% of patients
with NEC. Dark, concentric rings within the bowel wall represent hydrogen as a
byproduct of bacterial metabolism
121
```
The following are manifestations of hypocalcemia in the neonate EXCEPT
A. jitteriness
B. seizures
C. carpopedal spasm
D. high-pitched cry
E. laryngospasm
```
(C). Chvostek's sign (facial muscle twitching on tapping), and Trousseau's sign
(carpopedal spasm) may be present, but more commonly these are absent during the
neonatal period.
122
```
Penile growth occur during SMR
A. 1
B. 2
C. 3
D. 4
E. 5
```
C
123
```
Smoking during pregnancy is associated with an average decrease in fetal weight by
A. 100 g
B. 200 g
C. 300 g
D. 400 g
E. 500 g
```
(B). Smoking during pregnancy is associated with an average decrease in fetal
weight of 200 g; this decrease, added to the already smaller size of infants born to
teenagers, increases perinatal morbidity and mortality.
124
```
Evaluation for pubertal delay in female should be done if she lacks any pubertal
signs by the age of
A. 12 yr
B. 13 yr
C. 14 yr
D. 15 yr
E. 16 yr
```
B
125
```
The age at which an evaluation for primary amenorrhea should be undertaken is
A. 12 yr
B. 13 yr
C. 14 yr
D. 15 yr
E. 16 yr
```
D
126
A 3-year-old boy presented with recurrent attacks of pneumonia and otitis media
since the 1st birthday. Your diagnosis is X-linked agammaglobulinemia.
Of the following, the MOST likely offending organism is
A. CMV
B. Mycoplasma
C. Pneumocystis jiroveci
D. Staphylococcus aureus
E. Streptococcus pneumonia
(E). They acquire infections with extracellular pyogenic organisms, such as
Streptococcus pneumoniae and Haemophilus influenzae, unless they are given
prophylactic antibiotics or immunoglobulin therapy.
127
```
The following conditions can cause eosinophilia EXCEPT
A. malaria
B. filariasis
C. giardiasis
D. ascariasis
E. amebiasis
```
(C). Eosinophilia are caused by tissue-invasive as helminth infections; ascariasis,
filariasis, amebiasis, malaria, and scabies whereas entrobius vermicularis and Giardia
lamblia are solely intraluminal in gastrointestinal tract.
128
```
The highest white blood cells count is seen at
A. birth
B. 12 hour of life
C. 1 week of life
D. 1 year of life
E. adolescence
```
(A). For newborns, the mean WBC count at birth is high, followed by a rapid fall
beginning at 12 hr through the 1st wk of life. Thereafter, values are stable until 1 yr of
age, after which a slow steady decline in the WBC count continues throughout
childhood until adult values are reached during adolescence.
129
```
Severe neutropenia is defined as absolute neutrophil count (ANC) less than
A. 500/μL
B. 750/μL
C. 1000/μL
D. 1250/μL
E. 1500/μL
```
(A). Mild neutropenia, is an absolute neutrophil count (ANC) of 1,000-1,500/μL.
Moderate neutropenia, is an absolute neutrophil count (ANC) of 500- 1,000/μL. Severe
neutropenia, is an absolute neutrophil count (ANC) <500/μL.
130
The MOST vital initial treatment in the management of severe asthma
exacerbations is
A. supplemental oxygen
B. inhaled β -agonist
C. intramuscular injection of epinephrine
D. inhaled ipratropium
E. intramuscular injection of β-agonist
A
131
```
The best ‘‘rescue’’ medication in the treatment of acute asthma symptoms is
A. oral SABA
B. inhaled SABA
C. oral corticosteroid
D. inhaled ipratropium
E. inhaled corticosteroid
```
(B). Inhaled SABA, up to 3 treatments in 1 hr. If the child has an incomplete
response to initial treatment with rescue medication (persistent symptoms and/or a
PEF value <80% of personal best), a short course of oral corticosteroid therapy
(prednisone 1-2 mg/kg/day [not to exceed 60 mg/day] for 4 days), in addition to
inhaled β-agonist therapy, should be instituted.
132
```
A common finding of chest radiograph in a child with asthma is
A. peribronchial thickening
B. atelectasis
C. pneumothorax
D. bronchiectasis
E. pneumomediastinum
```
(A). Peribronchial thickening and flattening of diaphragm.
133
```
The hallmark of atopic dermatitis is
A. intense pruritus
B. cutaneous reactivity
C. lichenification
D. fibrotic papules
E. dry skin
```
(E). The hallmark of atopic dermatitis is dry skin, is results from compromise of the
epidermal barrier, which leads to excess transepidermal water loss, allergen
penetration, and microbial colonization.
134
```
The cardinal feature of atopic dermatitis is
A. skin rash
B. lichenification
C. fibrotic papules
D. intense pruritus
E. dry skin
```
(D). Intense pruritus, especially at night, and cutaneous reactivity are the cardinal
features of atopic dermatitis.
135
```
The best choice of antibiotic in treating localized impetiginous lesions in patients
with atopic dermatitis is
A. oral erythromycin
B. oral cephalexin
C. topical mupirocin
D. topical ampicillin
E. topical gentamycin
```
(C). Erythromycin and azithromycin are usually beneficial for patients who are not
colonized with a resistant S. aureus strain; a first-generation cephalosporin (cephalexin)
is recommended for macrolide-resistant S. aureus. Topical mupirocin is useful in the
treatment of localized impetiginous lesions, with systemic antibiotics for widespread
infections.
136
One of the following statements is TRUE regarding skin tests in identifying food
allergies in patients with atopic dermatitis
A. negative skin and blood test results for allergen-specific ige have a low
predictive value for excluding suspected allergens
B. positive results of skin or blood tests using foods often correlate with clinical
symptoms and no need to be confirmed with controlled food challenges
C. extensive elimination diets are commonly required
D. even with multiple majority of patients react to more than 3 foods
E. potential allergens can be identified by a careful history and performing
selective skin prick tests or in vitro blood testing for allergen- specific IgE
(E). For the other stems; A- high predictive value, B- Positive skin test often do not
correlate with clinical symptoms, C- Rarely required, and D- The majority react to less
than 3 foods.
137
In atopic dermatitis, the presence of punched out erosions, vesicles, and infected
skin lesions that fail to respond to oral antibiotics suggests infection with
A. Herpes zoster
B. Herpes simplex
C. cutaneous warts
D. Trichophyton rubrum
E. Molluscum contagiosum
(B). Herpes simplex virus (HSV) can provoke recurrent dermatitis and may be
misdiagnosed as S. aureusinfection, which can be diagnosed by a Giemsa-stained
Tzanck smear of cells scraped from the vesicle base or by viral polymerase chain
reaction or culture.
138
Exfoliative dermatitis may develop in patients with atopic dermatitis and extensive
skin involvement, usually caused by inappropriate therapy or superinfection with
A. E. coli
B. Herpes zoster
C. Herpes simplex
D. Streptococcal pyogen
E. Trichophyton rubrum
(C). Exfoliative dermatitis associated with generalized redness, scaling, weeping,
crusting, systemic toxicity, lymphadenopathy, and fever, and is usually caused by
superinfection (e.g., with toxin-producing S. aureus or HSV infection) or inappropriate
therapy. In some cases, the withdrawal of systemic glucocorticoids used to control
severe AD precipitates exfoliative erythroderma.
139
```
The MOST common hypersensitivity response of the eye is
A. allergic conjunctivitis
B. vernal keratoconjunctivitis
C. atopic keratoconjunctivitis
D. giant papillary conjunctivitis
E. contact allergy
```
(A). Allergic conjunctivitis is the most common hypersensitivity response of the eye,
affecting approximately 25% of the general population and 30% of children with atopy.
140
```
The MOST sight-threatening type of ocular allergy is
A. contact allergy
B. vernal keratoconjunctivitis
C. giant papillary conjunctivitis
D. seasonal allergic conjunctivitis
E. perennial allergic conjunctivitis
```
(B). Vernal keratoconjunctivitis and atopic keratoconjunctivitis are sight-threatening
entities and causes visual morbidity and suspected cases need to be referred to an
ophthalmologist.
141
```
The drug of choice for cold-induced urticaria is
A. loratadine
B. epinephrine IM
C. cyproheptadine
D. diphenhydramine
E. oral corticosteroid
```
C
142
```
Systemic lupus erythematosus can be caused by
A. isoniazid
B. sulfonamide
C. hydrochlorothiazide
D. allopurinol
E. nonsteroidal anti-inflammatory drug
```
(A). Along with Hydralazine and procainamide.
143
```
Vasculitis can be cause by
A. isoniazid
B. sulfonamide
C. penicillamine
D. calcium channel blockers
E. tetracycline
```
(C). Along with Hydralazine and propylthiouracil.
144
Nonsteroidal anti-inflammatory drugs (NSAIDs) are prescribed to decrease acute and
chronic inflammation associated with various rheumatic diseases; however, many
adverse effects have been encountered with long term use.
Of the following, the NSAID that has the highest toxicity is
A. celecoxib
B. meloxicam
C. indomethacin
D. naproxen
E. ibuprofen
(C). Certain agents (indomethacin) have a higher risk of toxicity than others
(ibuprofen); naproxen has an intermediate risk. Selective COX-2 inhibitors (such as
celecoxib and meloxicam) inhibit receptors responsible for promoting inflammation
with potential for fewer gastrointestinal adverse effects.
145
A 7-year-old boy developed small hypopigmented depressed scars after fingernail
scratches; he has been treated with nonsteroidal antiinflammatory drugs (NSAIDs) for a
rheumatic disease 6 weeks ago.
Of the following, the NSAID that is MOST likely to cause such a unique skin reaction is
A. celecoxib
B. meloxicam
C. indomethacin
D. naproxen
E. ibuprofen
(D). Naproxen is more likely than other NSAIDs to cause pseudoporphyria.
Pseudoporphyria is more likely to occur in fair-skinned individuals and on sun-exposed
areas. If pseudoporphyria develops; the inciting NSAID should be discontinued because
scars can persist for years or be permanent.
146
Sulfasalazine is an effective drug in many rheumatic diseases; however, it is
associated with severe systemic hypersensitivity reaction.
It is approved in all the following rheumatic disease EXCEPT
A. polyarticular juvenile idiopathic arthritis (JIA)
B. oligoarticular JIA
225
C. systemic JIA
D. peripheral arthritis associated with juvenile ankylosing spondylitis
E. enthesitis associated with juvenile ankylosing spondylitis
(C). Sulfasalazine is generally considered contraindicated in children with active
systemic JIA because of increased hypersensitivity reactions.
147
You are meeting parents of a 14-year-old girl who has been treated with a monthly
intravenous cyclophosphamide for SLE-associated renal failure for the last 6 months;
the mother is asking about the long-term complications of this drug.
All the following are long-term complications EXCEPT
A. bone marrow suppression
B. bladder cancer
C. leukemia
D. lymphoma
E. infertility
(A). Nausea, vomiting, anorexia, alopecia, mucositis, hemorrhagic cystitis, and bone
marrow suppression are potential short-term adverse effects.
148
Juvenile idiopathic arthritis has many subtypes. Arthritis must be present to make a
diagnosis of any subtype; involved joints oftenly have the following signs EXCEPT
A. swelling
B. warm
C. erythema
D. limitation of movement
E. pain on movement
(C). Involved joints are often swollen, warm to touch, and painful on movement or
palpation with reduced range of motion, but usually are not erythematous.
149
Oligoarthritis, the most common subtype of juvenile idiopathic arthritis, is defined as
involving ≤4 joints within the 1st 6 mo of disease onset.
Of the following, the MOST commonly affected joint is
A. hip
B. knee
C. elbow
D. wrist
E. metatarsal
(B). In oligoarthritis, it predominantly affects the large joints of the lower extremities,
such as the knees and ankles. Isolated involvement of upper extremity large joints is
less common.
150
```
Oligoarthritis predominantly affects the joints of the lower extremities rather than
upper extremity joints. Which of the following joints is never a presenting sign of
oligoarthritis?
A. hip
B. knee
C. ankle
D. metatarsal
E. interphalangeal
```
(A). Isolated involvement of the hip is almost never a presenting sign and suggests
extended oligoarticular juvenile idiopathic arthritis.
151
A 4-year-old girl recently diagnosed with persistent oligoarticular juvenile idiopathic
rheumatoid arthritis (JIA); she has 3 involved joints including the right knee, right ankle,
and left elbow; antinuclear antigen (ANA) is significantly positive.
Of the following, the MOST important step in the management of this girl is
A. regular examination of locomotor system
B. periodic slit-lamp examination
C. periodic ANA monitoring
D. periodic erythrocyte sedimentation rate (ESR) monitoring
E. frequent C-reactive protein (CRP) monitoring
(B). The presence of a positive ANA confers increased risk for asymptomatic anterior
uveitis, requiring periodic slit-lamp examination.
152
```
Reactive arthritis is defined as joint inflammation caused by a sterile inflammatory
reaction following a recent infection.
Of the following, the LEAST likely micro-organism that may cause reactive arthritis is
A. Clostridium difficile
B. Salmonella paratyphi
C. Shigella flexneri
D. Yersinia enterocolitica
E. Campylobacter jejuni
```
(A). Reactive arthritis typically follows enteric infection with Salmonella sp., Shigella
flexneri, Yersinia enterocolitica, Campylobacter jejuni, orgenitourinary tract infection
with Chlamydia trachomatis. Escherichiacoli and Clostridium difficile are also causative
enteric agents, although less common.
153
```
Postinfectious arthritis describes arthritis that occurs after infectious illnesses,
mainly viruses.
Of the following, the LEAST likely causative viral infection is
A. rubella
B. varicella-zoster
C. cytomegalovirus
D. Epstein-Barr virus
E. herpes-simplex virus
```
D
154
A 4-year-old boy has an acute severe left groin pain that refers to thigh.
Examination reveals limitation of movement of the left hip joint. Lab investigations
reveal normal blood count and indices, and normal ESR; radiologic imaging shows
widening of the hip joint space with effusion. You suspect transient synovitis.
Of the following, the BEST therapeutic approach for this boy is
A. systemic steroids
230
B. methotrexate
C. aspiration of joint fluid
D. intravenous antibiotics
E. observation
(C). In transient synovitis (toxic synovitis), aspiration of joint fluid is often necessary
to exclude septic arthritis and typically results in dramatic clinical improvement.
155
Reactive arthritis has the potential for evolving to chronic arthritis, especially after
bacterial enteric infection or genitourinary tract infection (UTI).
Of the following, the microorganism that is MOST likely causing chronic arthritis after
UTI is
A. Escherichia coli
B. Chlamydia trachomatis
C. Proteus mirabilis
D. Pseudomonas aeruginosa
E. Staphylococcus saprophyticus
B
156
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized
by multisystem inflammation and the presence of circulating autoantibodies directed
against self-antigens. It occurs in both children and adults.
All the following are features of childhood SLE EXCEPT
A. it is has a more severe course
B. there is more widespread organ involvement
C. it usually presents before 5 year of age
D. fever, fatigue, hematologic abnormalities, and arthritis are common clinical
manifestations
E. although renal disease is asymptomatic, it is oftenly present as nephrotic
syndrome in adolescent age group
(C). Childhood SLE is rare before 5 yr of age.
157
Drug-induced lupus refers to the presence of SLE manifestations triggered by
exposure to specific medications, including antibiotics.
Of the following, the drug that is MOST likely associated with drug-induced lupus is
A. isoniazid
B. rifampin
C. nitrofurantoin
231
D. penicillin
E. tetracycline
A
158
Systemic lupus erythematosus (SLE) is often characterized by periods of flare and
disease quiescence or may follow a more smoldering disease course.
All the following lab tests correlate with active disease EXCEPT
A. positive anti-nuclear antibody titer
B. positive anti–double-stranded DNA level
C. low serum complement level
D. high erythrocyte sedimentation rate
E. elevated C-reactive protein (CRP) value
(A). ANA titers are not reflective of disease activity; therefore, repeating ANA titers
is not helpful in disease management.
159
A 12-year-old female adolescent has a recent diagnosis of SLE; she has a faint malar
rash and mild arthritis including both elbow and knee joints as well as the joints of
small fingers.
Of the following, the MOST appropriate initial therapy for this patient is
A. systemic steroids
B. methotrexate
C. leflunomide
D. cyclophosphamide
E. hydroxychloroquine
(E). Hydroxychloroquine is recommended for all individuals with SLE if tolerated. In
addition to treating mild SLE manifestations such as rash and mild arthritis,
hydroxychloroquine prevents SLE flares, improves lipid profiles, and may have a
beneficial impact on mortality and renal outcomes. Corticosteroids are a mainstay for
treatment of significant manifestations of SLE. Methotrexate, leflunomide, and
azathioprine are often used to treat persistent moderate disease, including arthritis,
significant cutaneous or hematologic involvement, and pleural disease.
Cyclophosphamide is reserved for the most severe, potentially life threatening SLE
manifestations, such as renal, neurologic, and cardiopulmonary disease.
160
A 3-week-old male baby develops malar rash involving the face and periorbital area
after exposure to sun light; you suspect neonatal lupus.
Of the following, the NEXT step of management is
A. measurement of anti-Ro and anti-La antibodies of the mother
B. measurement of anti-Ro and anti-La antibodies of the baby
C. cardiac evaluation of the mother
D. cardiac evaluation of the baby
E. hematological evaluation of the baby
(D). The most serious complication due to neonatal lupus is congenital heart block.
161
Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in
children characterized by skin rash and proximal muscle weakness.
All the following are common cutaneous manifestations of JDM EXCEPT
A. heliotrope rash of the eyelids
B. photosensitivity to ultraviolet light
C. facial erythema sparing the nasolabial folds
D. Gottron papules
E. periungual telangiectasias
(C). In JDM, facial erythema is crossing the nasolabial folds, in contrast to the malar
rash without nasolabial involvement typical of systemic lupus erythematosus.
162
Amyloidosis is the most serious complication of familial Mediterranean fever (FMF),
and in its absence FMF patients may live a normal life span.
Of the following, the organ that is not affected by secondary amyloidosis of FMF is
A. kidney
B. lung
C. nerve
D. heart
E. teste
(C). Amyloidosis may develop most commonly in the kidneys, gastrointestinal tract,
spleen, lungs, testes, thyroid, and adrenals. Rarely, cardiac amyloidosis may develop;
macroglossia and amyloid neuropathy is generally not seen with the amyloidosis of
FMF.
163
Kawasaki disease (KD) has unique clinical manifestations; however, less consistent
clinical presentation may occur.
All the following are common classic clinical features of KD EXCEPT
A. bilateral non exudative bulbar conjunctivitis
B. cracked lips
C. edema and erythema of the hands and feet
D. vesicular rash
E. unilateral non suppurative cervical lymphadenopathy
(D). Typical rash is maculopapular, erythema multiforme, or scarlatiniform. Bullous,
pustular, or vesicular rashes are uncommon skin manifestations.
164
ou are evaluating a 1-year-old boy with an established diagnosis of Kawasaki
disease (KD) since 4 days; the caring nurse asks you about cardiac involvement during
this stage.
All the following are possible cardiac complications at this stage EXCEPT
A. myocarditis
B. pericarditis
C. coronary artery aneurysm
D. mitral regurgitation
E. cardiogenic shock
(C). Coronary artery aneurysm usually develops in the subacute phase in the 2nd or
3rd week of the illness.
165
A follow up two-dimensional echocardiography is performed to a 1.5-year-old boy
with Kawasaki disease (KD) 3 weeks after the diagnosis; it shows a small solitary
aneurysm of the left anterior descending coronary artery without thrombosis.
Of the following, the MOST appropriate therapy for this child is
A. aspirin for 6 months
B. life-long aspirin
C. IVIG and aspirin for 14 days
D. aspirin and clopidogrel antiplatelet for 8 weeks
E. aspirin and warfarin for 6 months
(B). The patient with KD who has had a small solitary aneurysm should continue
aspirin indefinitely. Patients with larger or numerous aneurysms may require the
addition of other antiplatelet agents or anticoagulation.
166
```
Rotavirus vaccine should not be initiated for infants older than
A. 11 wk
B. 13 wk
C. 15 wk
D. 17 wk
E. 19 wk
```
C
167
```
Serious reactions to IVIG include all the following EXCEPT
A. anaphylactoid events
B. thromboembolic disorders
C. aseptic meningitis
D. carditis
E. renal insufficiency
```
D
168
```
The minimum interval between the 2 doses of MMR is
A. 2 wk
B. 4 wk
C. 2 mo
D. 6 mo
E. 1 yr
```
B
169
```
The minimum age for the last dose of hepatitis B vaccine is
A. 16 weeks
B. 20 weeks
C. 24 weeks
D. 28 weeks
244
E. 32 weeks
```
(C). The minimum interval between 2nd and 3rd dose is 8 weeks andat least 16 weeks
after 1st dose. Minimum age for the last dose is 24 weeks.
170
Preterm infant, weight 1600 gm, should not receive the following vaccine at birth
A. BCG
B. hepatitis B, if born to a HBs Ag negative mother
C. polio
D. DPT
E. MMR
(B). Preterm infants generally can be vaccinated at the same chronologic age as fullterm
infants according to the recommended childhood immunization schedule. An
exception is the birth dose of hepatitis B vaccine. Infants weighing ≥2 kg and who are
stable may receive a birth dose. However, hepatitis B vaccination should be deferred in
infants weighing <2 kg at birth until 30 days of age, if born to a HBs Ag negative
mother. All preterm, low birth weight infants born to HBs Ag positive mothers should
receive hepatitis B immunoglobulin and hepatitis B vaccine within 12 hr of birth.
However, such infants should receive an additional 3 doses of vaccine starting at 30
days of age.
171
```
Drugs that are known to cause fever include
A. chlorpheniramine
B. allopurinol
C. diphenhydramine
D. acetazolamide
E. adenosine
```
(B). Drugs that are known to cause fever include vancomycin, amphotericin B, and
allopurinol.
172
```
Bulbar conjunctivitis in a child with FUO suggests
A. leptospirosis
B. coxsackievirus infection
C. tuberculosis
D. infectious mononucleosis
E. lymphogranuloma venereum
```
(A). Palpebral conjunctivitis in a febrile patient may be a clue to measles,
coxsackievirus infection, tuberculosis, infectious mononucleosis,
lymphogranulomavenereum, or cat-scratch disease. In contrast, bulbar conjunctivitis in
a child with FUO suggests Kawasaki disease or leptospirosis.
173
```
Which of the following represent a major criterion for the diagnosis of
staphylococcal toxic shock syndrome?
A. rash
B. conjunctival hyperemia
C. myalgia
D. thrombocytopenia
E. vomiting
```
(A).
MAJOR CRITERIA (ALL REQUIRED)
1. Acute fever; temperature >38.8°C (101.8°F)
2. Hypotension
3. Rash (erythroderma with convalescent desquamation)
MINOR CRITERIA (ANY 3 OR MORE)
1. Mucous membrane inflammation (vaginal, oropharyngeal or conjunctival
hyperemia, strawberry tongue)
2. Vomiting, diarrhea
3. Liver abnormalities (bilirubin or transaminase greater than twice upper limit of
normal)
4. Renal abnormalities (urea nitrogen or creatinine greater than twice upper limit
of normal, or greater than 5 white blood cells per high-power field)
5. Muscle abnormalities (myalgia or creatinine phosphokinase greater than twice
upper limit of normal)
6. Central nervous system abnormalities (alteration in consciousness without
focal neurologic signs)
7. Thrombocytopenia (100,000/mm3 or less)
174
```
Kawasaki disease closely resembles toxic shock syndrome clinically. However, many
of the clinical features of toxic shock syndrome are usually absent or rare in Kawasaki
disease like
A. fever unresponsive to antibiotics
B. hyperemia of mucous membranes
C. erythematous rash
D. desquamation
E. diffuse myalgia
```
(E). Many of the clinical features of TSS are usually absent or rare in Kawasaki
disease, including diffuse myalgia, vomiting, abdominal pain, diarrhea, azotemia,
hypotension, acute respiratory distress syndrome, and shock.
175
S. pneumoniae is the most frequent cause of bacteremia, bacterial pneumonia,
otitis media, and bacterial meningitis in children. Children at increased risk of
pneumococcal infections include those with the following conditions EXCEPT
A. megaloplastic anemia
B. deficiencies in humoral immunity
C. HIV infection
D. cerebrospinal fluid leak
E. cochlear implants
(A). Children at increased risk of pneumococcal infections include those with sickle
cell disease, asplenia, deficiencies in humoral (B cell) and complement-mediated
immunity, HIV infection, certain malignancies (e.g., leukemia, lymphoma), chronic
heart, lung, or renal disease (particularly nephrotic syndrome), cerebrospinal fluid leak,
and cochlear implants.
176
Impetigo (or pyoderma) has traditionally been classified into 2 clinical forms:
bullous and nonbullous.
Of the following, which statement is TRUE?
A. bullous impetigo is more common
B. nonbullous lesions are most common on the trunk and perineum
C. regional lymphadenitis is commonly associated with nonbullous lesions
D. nonbullous impetigo is generally accompanied by fever
E. bullous impetigo usually involve the face and extremeties
(C). Nonbullous impetigo is the more common form. The lesions may occur
anywhere but are most common on the face and extremities. Regional lymphadenitis is
common. Nonbullous impetigo is generally not accompanied by fever or other systemic
signs or symptoms. Bullous impetigo is less common and occurs most often in neonates
and young infants. The usual distribution involves the face, buttocks, trunk, and
perineum.
177
In which of the following circumstances the diagnosis of acute rheumatic fever can
be made without strict adherence to Jones criteria?
A. when chorea occurs as the only major manifestation of acute rheumatic fever
B. when indolent carditis is the only manifestation months after the apparent
onset of acute rheumatic fever
C. in a limited number of patients with recurrences of acute rheumatic fever in
particularly high-risk populations
D. all of the above
E. none of the above
D
178
```
Group B streptococcus (GBS), or Streptococcus agalactiae, is a major cause of
neonatal bacterial sepsis.
Of the following, the MOST common syndrome associated with childhood GBS disease
beyond early infancy is
A. bacteremia without a focus
B. meningitis
C. ventriculitis
D. septic arthritis
E. pneumonia
```
(A). Early-onset neonatal GBS disease presents as sepsis; pneumonia and meningitis.
Late-onset neonatal GBS disease most commonly manifests as bacteremia (45-65%)
and meningitis (25-35%). Invasive GBS disease in children beyond early infancy is
uncommon. Bacteremia without a focus is the most common syndrome associated
with childhood GBS disease beyond early infancy.
179
```
Recommended duration of therapy for manifestations of group B streptococcus are
as follow
A. bacteremia without a focus: 7 days
B. meningitis: 3-6 weeks
C. ventriculitis: at least 8 weeks
D. septic arthritis: 3-4 weeks
E. osteomyelitis: 6-8 weeks
```
```
(D).
o bacteremia without a focus 10 days
o meningitis 2-3 wk
o ventriculitis at least 4 wk
o septic arthritis 3-4 wkase
o osteomyelitis 3-4 wkas
```
180
Diphtheria is an acute toxic infection; toxic cardiomyopathy occurs in 10-25% of
patients with respiratory diphtheria and is responsible for 50-60% of deaths.
Of the following, the mainstay of therapy is
A. antitoxin
B. penicillins
C. erythromycin
D. clindamycin
E. rifampin
(A). Specific antitoxin is the mainstay of therapy and should be administered on the
basis of clinical diagnosis. The role of antimicrobial therapy is to halt toxin production,
treat localized infection, and prevent transmission of the organism to contacts.
181
A painless, slow-growing, hard mass producing cutaneous fistulas, a condition
commonly known as lumpy jaw is usually caused by
A. Staphylococcus
B. Actinomyces
C. Nocardia
D. Yersinia
E. Leptospira
(B). Three major forms of actinomycosis—cervicofacial, abdominal and pelvic, and
pulmonary. In the patient with cervicofacial actinomycosis, there is often a history of
oral trauma, oral surgery, dental procedures, or caries, facilitating entry of organisms
into cervicofacial tissues. Cervicofacial actinomycosis usually manifests as a painless,
slow-growing, hard mass and can produce cutaneous fistulas, a condition commonly
known as lumpy jaw. Less frequently, cervicofacial actinomycosis manifests clinically as
an acute pyogenic infection with a tender, fluctuant mass with trismus, firm swelling,
and fistulas with drainage containing the characteristic sulfur granules.
182
Prophylaxis for people who have had acute rheumatic fever with carditis but
without residual heart disease persist for
A. 5 yr or until 21 yr of age, whichever is longer
B. 10 yr or until 21 yr of age, whichever is longer
C. 5 yr or until 40 yr of age, whichever is longer
252
D. 10 yr or until 40 yr of age, whichever is longer
E. Lifelong
B).
o Rheumatic fever without carditis : 5 yr or until 21 yr of age, whichever is longer
275
o Rheumatic fever with carditis but without residual heart disease : 10 yr or until
21 yr of age,whichever is longer
o Rheumatic fever with carditis and residual heart disease: 10 yr or until 40 yr of
age, whichever is longer; sometimes lifelong prophylaxis
183
```
The MOST common secondary site involved in cases of pulmonary nocardiosis is
A. brain
B. skin
C. kidney
253
D. liver
E. bone
```
(A). The brain is the most common secondary site and is involved in 15-40% of cases
of pulmonary nocardiosis. Brain abscess is the most common presentation, and
meningitis is the second most common presentation.
184
```
Which of the following is a gram negative bacterium?
A. Staphylococcus aureus
B. Streptococcus pneumoniae
C. Actinomyces
D. Haemophilus influenza
E. Nocardia
```
(D). Actinomyces, staphylococcus, streptococcus, diphtheria, enterococcus, listeria
and nocardia are gram positive.
185
```
The MOST common clinical manifestation of meningococcal infection is
A. asymptomatic carriage
B. meningococcal meningitis
C. bacteremia without sepsis
D. meningococcal septicemia
E. pneumonia
```
(A). The most common clinical manifestation of meningococcal infection is
asymptomatic carriage of the organism in the nasopharynx.
186
Gonorrhea is manifested by a spectrum of clinical presentations from asymptomatic
carriage, to the characteristic localized urogenital infections, to disseminated systemic
infection.
Regarding disseminated gonococcal infection, the following statement is TRUE
A. hematogenous dissemination occurs in 10-30% of all gonococcal infections
B. men account for the majority of cases
C. meningitis and osteomyelitis are the most common manifestations
D. skin lesions found in75% of patients
E. acute endocarditis is an uncommon but often fatal manifestation
(E). Hematogenous dissemination occurs in 1-3% of all gonococcal infections, more
frequently after asymptomatic primary infections than symptomatic infections. Women
account for the majority of cases, with symptoms beginning 7-30 days after infection
and within 7 days of menstruation. The most common manifestations are asymmetric
arthralgia, petechial or pustular acral skin lesions, tenosynovitis, suppurative arthritis,
and, rarely, carditis, meningitis, and osteomyelitis. Only 25% of patients complain of
skin lesions.
187
All patients who are presumed or proven to have gonorrhea should be evaluated
for concurrent presence of all the following infections EXCEPT
A. syphilis
B. hepatitis B
C. HIV
D. C. trachomatis
E. HSV2
(E). All patients who are presumed or proven to have gonorrhea should be
evaluated for concurrent syphilis, hepatitis B, HIV, and C. trachomatis infection.
188
```
The preferable antibiotic in neonate with pertussis is
A. azithromycin
B. erythromycin
C. clarithromycin
D. trimethoprim-sulfamethoxazole
E. amoxycilline
```
(A).
o Azithromycin: Recommended agent 10 mg/kg/day in a single dose for 5 days
o Erythromycin: Not preferred
o Clarithromycin: Not recommended
o trimethoprim-sulfamethoxazole: Contraindicated for infants <2 mo of age
189
Regarding diagnosis of typhoid fever, one of the following is TRUE
A. blood cultures are positive in 65-80% of the patients
B. urine culture results become positive within the 1st wk
C. thrombocytosis may be a marker of severe illness
D. diagnosis by Widal test alone is prone to error
E. leukocytosis is rare in young children
(D). Results of blood cultures are positive in 40-60% of the patients seen early in the
course of the disease, and stool and urine culture results become positive after the 1st
wk. Thrombocytopenia may be a marker of severe illness and may accompany
disseminated intravascular coagulopathy. Blood leukocyte counts are frequently low in
relation to the fever and toxicity, there is a wide range in counts; in younger children
leukocytosis is common and may reach 20,000-25,000 cells/μL.
190
All the following are clinical features of shigellosis EXCEPT
A. an incubation period of 12 hr to several days
B. most children never progress to the stage of bloody diarrhea
C. untreated diarrhea can last more than 4 weeks
D. neurologic findings are the most common extraintestinal manifestations
E. neonatal shigellosis is rare
(C). Untreated diarrhea can last 7-10 days; only approximately 10% of patients have
diarrhea persisting for longer than 10 days. Persistent diarrhea occurs in malnourished
infants, children with AIDS, and occasionally previously normal children.
191
Cholera is a dehydrating diarrheal disease caused by Vibrio cholerae, of more than
200 serogroups; the serogroups that have been associated with epidemics are
A. O131
B. O139
C. O151
D. O159
E. O165
(B). Of the more than 200 serogroups, only serogroups O1 and O139 have been
associated with epidemics, although some non-O1, non-O139 V.choleraestrains (e.g.,
O75 and O141) are pathogenic and can cause small outbreaks.
192
```
The MOST common long-term sequela associated with congenital CMV infection is
A. renal failure
B. hearing loss
C. heart failure
D. vision loss
E. hepatic failure
```
(B). Hearing loss is the most common long-term sequela associated with
congenital CMV infection, the failure of an infant to pass a newborn hearing screening
exam should raise the possibility of congenital CMV infection. Hearing loss in the older
infant and young child should also alert the clinician to the possibility of congenital
CMV infection, as approximately 50% of infants with hearing loss associated with
congenital CMV infection will pass an initial hearing screening exam but develop
hearing loss in later infancy and early childhood.
193
Human herpesvirus 6 (HHV-6A and HHV-6B) and human herpesvirus 7 (HHV-7)
cause infection in infancy and early childhood. HHV-6B is responsible for the majority of
cases of roseola infantum (exanthema subitum or sixth disease).
All the following are true EXCEPT
A. 95% of children being infected with HHV-6 by 2 yr of age
B. peak age of primary HHV-6B infection is 6-9 mo of life
C. congenital infection with HHV-6 occurs in 1% of newborns
D. congenital infection with HHV-7 is well recognized
E. breast milk does not play a role in transmission of either HHV-6 or HHV-7
(D). Congenital infection with HHV-7 has not been demonstrated.
194
Subacute sclerosing panencephalitis (SSPE) is a chronic complication of measles
with a delayed onset and an outcome that is nearly always fatal.
SSPE is characterized by
A. females are affected twice as often as males
B. clinical manifestations begin insidiously 3-6 yr after primary measles infection
C. the hallmark of the 1st stage is massive myoclonus
D. the 3rd stage is characterized by loss of critical centers
E. clinical trials using isoprinosine suggest significant benefit
(C). Acute otitis media is the most common complication of measles and
pneumonia is the most common cause of death in measles.
195
```
The MOST common finding among infants with congenital rubella syndrome is
A. psychomotor retardation
B. cataracts
C. deafness
D. patent ductus arteriosus
E. neonatal purpura
```
(E). Males are affected twice as often as females, and there appear to be more
cases reported from rural than urban populations. Clinical manifestations of SSPE begin
insidiously 7-13 yr after primary measles infection. The hallmark of the 2nd stage is
massive myoclonus, which coincides with extension of the inflammatory process site to
deeper structures in the brain, including the basal ganglia. The 4th stage is
characterized by loss of critical centers that support breathing, heart rate, and blood
pressure. Death soon ensues.
196
```
The MOST common complication of mumps is
A. meningitis
263
B. conjunctivitis
C. optic neuritis
D. pneumonia
E. thrombocytopenia
```
(A). The most common complications of mumps are meningitis, with or without
encephalitis, and gonadal involvement. Uncommon complications include
conjunctivitis, optic neuritis, pneumonia, nephritis, pancreatitis, and
thrombocytopenia.
197
Measles is a serious infection characterized by high fever, an enanthem, cough,
coryza, conjunctivitis, and a prominent exanthem.
All the following are true EXCEPT
A. incubation period is 8-12 days
B. Koplik spots have been reported in 50-70% of measles cases
C. the rash begins on the forehead, behind the ears, and on the upper neck
D. the rash fades over about 3 days in the same progression as it evolved
E. in more severe cases, generalized lymphadenopathy may be present
(D). The rash fades over about 7 days in the same progression as it evolved, often
leaving a fine desquamation of skin in its wake.
198
```
Giardiasis should be considered in children who have the following presentations
EXCEPT
A. acute dysenteric diarrhea
B. persistent diarrhea
C. failure to thrive
D. malabsorption
E. chronic crampy abdominal pain
```
(A). Giardiasis should be considered in children who have acute nondysenteric
diarrhea, persistent diarrhea, intermittent diarrhea and constipation, malabsorption,
chronic crampy abdominal pain and bloating, failure to thrive, or weight loss.
199
```
Hand-foot-mouth disease is one of the more distinctive rash syndromes; it is
MOST frequently caused by
A. coxsackievirus A6
B. coxsackievirus A16
C. enterovirus 71
D. coxsackievirus B2
E. coxsackievirus B12
```
(B). Hand-foot-and-mouth disease, one of the more distinctive rash syndromes, is
most frequently caused by coxsackievirus A16, sometimes in large outbreaks, and can
also be caused by enterovirus 71; coxsackie A viruses 5, 6, 7, 9, and 10; coxsackie B
viruses 2 and 5; and some echoviruses.
200
```
Typically, the first sign of infection in infants with respiratory syncytial virus (RSV)
is
A. rhinorrhea
B. cough
C. low-grade fever
D. increased respiratory rate
E. subcostal retractions
```
A
201
```
Secretory diarrhea can be caused by
A. neuroblastoma
B. laxative abuse
C. lactase deficiency
D. irritable bowel syndrome
E. thyrotoxicosis
```
(A). Laxative abuse and lactase deficiency are causes of osmotic diarrhea while
irritable bowel syndrome and thyrotoxicosis are caused by increased bowel motility.
202
```
Acute diarrhea in infancy is commonly caused by
A. primary disaccharidase deficiency
B. overfeeding
C. Hirschsprung toxic colitis
D. adrenogenital syndrome
E. neonatal opiate withdrawal
```
```
(B). Rare causes of acute diarrhea in infancy include
Primary disaccharidase deficiency
Hirschsprung toxic colitis
Adrenogenital syndrome
Neonatal opiate withdrawal
```
203
```
The following are features of achalasia EXCEPT
A. dysphagia for solids and liquids
B. may be accompanied by undernutrition
C. may be accompanied by chronic cough
D. may be misdiagnosed as asthma
E. usually diagnosed before school age
```
E
204
```
Gastroesophageal reflux disease (GERD) is the most common esophageal disorder
in children of all ages.
Of the following, the MOST common symptom in infants is
A. excessive crying
B. regurgitation
C. failure to thrive
D. abnormal posturing
E. sleeping disturbances
```
(B). Infantile refluxmanifests more often with regurgitation(especially
postprandially), signs of esophagitis (irritability, arching, choking, gagging, feeding
aversion), and resulting failure to thrive; symptoms resolve spontaneously in the
majority of infants by 12-24 mo.
205
The following procedures satisfactorily improve gastroesophageal reflux disease
(GERD) symptoms in infants EXCEPT
A. modified feeding volumes
B. hydrolyzed infant formulas
C. prone position, when the infant is awake and observed
D. avoidance of smoke exposure
E. seated position
(E). Seated position worsens infant reflux and should be avoided in infants with
GERD.
206
```
Direct person-to-person contact outbreaks of gastroenteritis are usually caused by
A. Shigella
B. Salmonella
C. Rotavirus
D. Giardia
E. Clostridium difficile
```
(A). Direct person-to-person contact outbreaks of gastroenteritis are usually caused
by norovirus and Shigella species. Unknown agents are seen in 30-40%; other
pathogens include Salmonella, rotavirus, Giardia, Cryptosporidium, Clostridium difficile,
and C. jejuni.
207
```
The first-line treatment for children with Vibrio cholera is
A. azithromycin
B. ciprofloxacin
C. doxycycline
D. ampicillin
E. TMP-SMX
```
(A). Treatment of Vibrio cholerae is supportive care with aggressive oral and
intravenous rehydration.Doxycycline is recommended as first-line treatment for adults,
whereas azithromycin is recommended as first-line treatment for children and
pregnant women. Ciprofloxacin and doxycycline recommended as second-line drugs for
children.
208
Plain abdominal radiographs may be helpful in selected cases of abdominal
pain/suspected appendicitis. Plain abdominal x-rays can demonstrate the following
findings in acute appendicitis EXCEPT
A. sentinel loops of bowel and localized ileus
B. scoliosis from psoas muscle spasm
C. a colonic air–fluid level above the right iliac fossa
D. a RLQ soft-tissue mass
E. a calcified appendicolith (50% of cases)
(E). A calcified appendicolith (5-10% of cases),
209
```
The test of choice for acute pancreatitis is
A. serum amylase
B. serum lipase
C. hyperglycemia
D. hypocalcemia
E. elevated γ-glutamyl transpeptidase
```
(B). Serum lipase is now considered the test of choice for acute pancreatitis as it is
more specific than amylase for acute inflammatory pancreatic disease and should be
determinedwhen pancreatitis is suspected.
210
```
Grunting is produced by expiration against a partially closed glottis and is an attempt
to maintain positive airway pressure during expiration for as long as possible.
It is MOST commonly prominent in
A. epiglottitis
B. hyaline membrane disease
C. asthma
D. croup
E. choanal atresia
```
(B). Grunting is most beneficial in alveolar diseases that produce widespread loss of
FRC, such as in pulmonary edema, hyaline membrane disease, and pneumonia.
Grunting is also effective in small airway obstruction (bronchiolitis) to maintain a higher
positive pressure in the airway during expiration, decreasing the airway collapse.
211
```
Rigid bronchoscopy is preferentially indicated for
A. persistent pneumonia
B. extracting foreign bodies
C. atelectasis
D. persistent wheeze
E. interstitial disease
```
(B). Rigid bronchoscopy is preferentially indicated for extracting foreign bodies, for
removing tissue masses, and in patients with massive hemoptysis. In other cases, the
flexible scope offers the advantages that it can be passed through endotracheal or
tracheostomy tubes can be introduced into bronchi that come off the airway at acute
angles, and can be safely and effectively inserted with topical anesthesia and conscious
sedation.
212
```
Foreign bodies are often placed in the nose by small children and developmentally
delayed children.
Of the following, the MOST common presenting clinical symptom is
A. mucopurulent nasal discharge
B. foul nasal odor
C. epistaxis
D. nasal obstruction
E. mouth breathing
```
(A). Presenting clinical symptoms include history of insertion of foreign bodies (86%),
mucopurulent nasal discharge (24%), foul nasal odor (9%), epistaxis (6%), nasal
obstruction (3%), and mouth breathing (2%).
213
Common causes of childhood epistaxis include the following EXCEPT
A. epistaxis digitorum
B. foreign bodies
C. significant gastroesophageal reflux into the nose
D. dry air
E. allergic rhinitis
(C). Common causes of nosebleeds from the anterior septum include digital trauma,
foreign bodies, dry air, and inflammation, including upper respiratory tract infections,
sinusitis, and allergic rhinitis. Young infants with significant gastroesophageal reflux
into the nose rarely present with epistaxis secondary to mucosal inflammation.
214
```
A “silent chest” with infiltrates should arouse suspicion of all the following EXCEPT
A. alveolar proteinosis
B. bronchopulmonary dysplasia
C. Pneumocystis jiroveci infection
D. interstitial pneumonitis
E. tumors
```
(B). A “silent chest” with infiltrates should arouse suspicion of alveolar proteinosis,
Pneumocystis jiroveci infection, genetic disorders of surfactant synthesis and secretion
causing interstitial pneumonitis, or tumors.
215
```
The barking cough typical of croup is rare in
A. laryngotracheobronchitis
B. acute epiglottitis
C. acute infectious laryngitis
D. spasmodic croup
E. measles croup
```
(B). This dramatic, potentially lethal condition is characterized by an acute rapidly
progressive and potentially fulminating course of high fever, sore throat, dyspnea, and
rapidly progressing respiratory obstruction. The barking cough typical of croup is rare.
216
```
The MOST common congenital laryngeal anomaly that produces stridor is
A. vocal cord paralysis
B. laryngomalacia
C. congenital subglottic stenosis
D. congenital laryngeal web
E. congenital subglottic hemangioma
```
(B). Laryngomalacia is the most common congenital laryngeal anomaly and the most
common cause of stridor in infants and children. Sixty percent of congenital laryngeal
anomalies in children with stridor are due to laryngomalacia.
217
```
Cardiovascular causes of wheezing include
A. right atrial enlargement
B. left atrial enlargement
C. right ventricular enlargement
D. left ventricular enlargement
E. coarctation of aorta
```
(B). Cardiovascular causes of wheezing include dilated chambers of the heart
including massive cardiomegaly, left atrial enlargement, and dilated pulmonary
arteries.
218
Bronchiolitis obliterans (BO) is a rare chronic obstructive lung disease of the
bronchioles and smaller airways. An insult to the lower respiratory tract occurs,
resulting in fibrosis of the small airways.
Of the following, the BEST mean of establishing its diagnosis is
A. chest radiographs
B. pulmonary function tests
C. ventilation-perfusion scans
D. chest CT
E. open lung biopsy
(E). Closure of all supracristal ventricular VSDs at the time of diagnosis is commonly
recommended to prevent the development of aortic regurgitation, even in an
asymptomatic child.
219
Pulmonary edema is traditionally separated into two categories according to cause
(cardiogenic and non-cardiogenic). Non-cardiogenic pulmonary edema, in its most
severe state, is also known as acute respiratory distress syndrome (ARDS).
The following radiographic features is usually present in non-cardiogenic pulmonary
edema
A. septal lines
B. peribronchial cuffing
C. pleural effusions
D. air bronchograms
E. even or central distribution of edema
(C). The radial and femoral pulses should always be palpated simultaneously for the
presence of a radial-femoral delay. Normally, the femoral pulse occurs slightly before
the radial pulse. A radial-femoral delay occurs when blood flow to the descending aorta
is dependent on collaterals, in which case the femoral pulse is felt after the radial pulse.
220
The recurrent aspiration of small quantities of gastric, nasal, or oral contents can
lead to several clinical presentations, including recurrent bronchitis or bronchiolitis;
recurrent pneumonia; atelectasis; wheezing; cough; apnea; and/or laryngospasm.
The MOST common underlying problem associated with recurrent pneumonias in
hospitalized children is
A. oropharyngeal incoordination
B. esophageal foreign body
C. nasoenteric tube
D. poor oral hygiene
E. bronchopulmonary dysplasia
(C). A variety of lesions may give rise to chronic pulmonary venous hypertension,
which when extreme may result in pulmonary arterial hypertension and right-sided
heart failure. These lesions include congenital mitral stenosis, mitral insufficiency, total
anomalous pulmonary venous return with obstruction, left atrial myxomas, cor
triatriatum (stenosis of a common pulmonary vein), individual pulmonary vein stenosis,
and supravalvular mitral rings.
221
The MOST common etiology of pulmonary infiltrates with eosinophilia (PIE) is
A. simple pulmonary eosinophilia
B. acute eosinophilic pneumonia
C. chronic eosinophilic pneumonia
D. allergic bronchopulmonary aspergillosis
E. parasitic Infections
(E). This lesion is not progressive in childhood, and specific therapy is not indicated.
Antibiotic prophylaxis is no longer recommended during surgery and dental
procedures.
222
```
The MOST frequent pathogens of pneumonia in children 5 yr and older is
A. Streptococcus pneumoniae
B. Mycoplasma pneumoniae
313
C. group A streptococci
D. H. influenzae (type b, nontypable)
E. adenovirus
```
(B). Sinus rate <90 beats/min in neonates and <60 beats/min in older children is
considered to be sinus bradycardia.
223
```
The MOST frequent pathogen of pneumonia in children 4 mo-4 yr is
A. Streptococcus pneumoniae
B. Mycoplasma pneumoniae
C. group A streptococci
D. H. influenzae (type b, nontypable)
E. Respiratory syncytial virus
```
(E). Ocular pressure must never be performed, and carotid sinus massage is very
rarely effective.
224
The MOST common physical finding of the pulmonary exam in bronchopulmonary
dysplasia (BPD) is
A. tachypnea
B. mouth breathing
C. increased anteroposterior diameter of the chest
D. intercostal retractions
E. clear breath sounds
(A). Physical findings of the pulmonary exam vary with the severity of disease.
Tachypnea is a common finding.
225
Bronchopulmonary dysplasia (BPD) is a syndrome characterized by signs and
symptoms of chronic lung disease that originates in the neonatal period. An accepted
definition includes a postnatal oxygen requirement for
A. 7 days
B. 14 days
C. 21 days
D. 28 days
E. 35 days
(D). An accepted definition includes an oxygen requirement for 28 days postnatally,
and the disorder is graded as mild, moderate or severe on the basis of supplemental
oxygen requirement and gestational age.
226
```
CHARGE association is characterized by the following cardiac feature
A. endocardial cushion defect
B. supravalvular aortic stenosis
C. tetralogy of Fallot
D. patent ductus arteriosus
E. anomalous pulmonary venous return
```
(C). CHARGE association (coloboma, heart, atresia choanae, retardation, genital and
ear anomalies) is associated with TOF, aortic arch and conotruncal anomalies.
227
The chest x-ray may help to differentiate the types of congenital heart defects.
Increased pulmonary markings (increased pulmonary blood flow) is seen in
A. pulmonary atresia
B. truncus arteriosus
C. tetralogy of Fallot
D. tricuspid atresia
E. Ebstein anomaly
(B).
Decreased pulmonary markings (diminished pulmonary blood flow)
Pulmonary atresia or severe stenosis
Tetralogy of Fallot
Tricuspid atresia
Ebstein anomaly
Increased pulmonary markings (increased pulmonary blood flow)
341
Transposition of great arteries
Total anomalous pulmonary venous return
Truncus arteriosus
228
Of the following, the MOST common clinical sign of coarctation of the aorta in older
children is
A. cardiac enlargement
B. notching of the inferior border of the ribs
C. a systolic ejection click or thrill in the suprasternal notch
D. differential blood pressure: arms > legs
E. diminished or absent femoral or lower-extremity pulses
(D). The most common clinical sign of coarctation of the aorta in older children is
differential blood pressure: arms > legs (100%).
229
Sinus bradycardia is due to slow discharge of impulses from the sinus node, the
heart's natural pacemaker. Sinus bradycardia in neonates is considered if sinus rate is
A. <100 beats/min
B. <90 beats/min
C. <80 beats/min
D. <70 beats/min
E. <60 beats/min
(B). Sinus rate <90 beats/min in neonates and <60 beats/min in older children is
considered to be sinus bradycardia.
230
```
The leading causative agents for endocarditis in pediatric patients are
A. group D enterococci
B. viridans-type streptococci
C. Pseudomonas aeruginosa
D. fungal organisms
E. Serratia marcescens
```
(B). Viridans-type streptococci (α-hemolytic streptococci) and Staphylococcus
aureus remain the leading causative agents for endocarditis in pediatric patients.
231
The following is a major Duke criterion for the diagnosis of endocarditis
A. new valve regurgitant flow by echocardiography
B. Osler nodes
C. single positive blood culture
D. serologic evidence of infection
E. high erythrocyte sedimentation rate
(A). The Duke criteria help in the diagnosis of endocarditis. Major criteria include
(1) positive blood cultures (2 separate cultures for a usual pathogen, 2 or more for less
typical pathogens), and (2) evidence of endocarditis on echocardiography (intracardiac
mass on a valve or other site, regurgitant flow near a prosthesis, abscess, partial
dehiscence of prosthetic valves, or new valve regurgitant flow).
232
The typical radiologic configuration in tetralogy of Fallot as seen in the
anteroposterior view consists of all the following EXCEPT
A. normal heart size
B. some elevation of the cardiac apex
C. convexity in the region of the main pulmonary artery
D. right-sided aortic arch
E. diminished pulmonary vascularity
(C). Concavity in the region of the main pulmonary artery.
233
Obstructed total anomalous pulmonary venous return (TAPVR) is a pediatric cardiac
surgical emergency because
A. prostaglandin therapy is usually not effective
B. severe pulmonary congestion develop
C. left atrium may be small
D. left ventricle may be small
E. pulmonary hypertension develop
(A). Obstructed TAPVR is a pediatric cardiac surgical emergency because
prostaglandin therapy is usually not effective.
234
In neonates with total anomalous pulmonary venous return and marked pulmonary
venous obstruction, the chest x-ray demonstrates
A. small heart
B. enlarged heart
C. prominent pulmonary artery
D. prominent right ventricle
E. subaortic stenosis
(A). In neonates with marked pulmonary venous obstruction, the chest x-ray
demonstrates a very dramatic perihilar pattern of pulmonary edema and a small heart.
235
In truncus arteriosus, a single arterial trunk arises from the heart and supplies the
systemic, pulmonary, and coronary circulations. All the following are true EXCEPT
A. VSD is always present
B. the truncus overriding the defect
C. both ventricles are at systemic pressure
D. heart failure ensues immediately after birth
E. clinical cyanosis is usually mild
(D). When pulmonary vascular resistance is relatively highimmediately after birth,
pulmonary blood flow may be normal; as pulmonary resistance drops in the 1st mo of
life, blood flow to the lungs is greatly increased and heart failure ensues.
236
Pentalogy of Cantrell consists of the following EXCEPT
A. ectopia cordis
B. midline supra umbilical abdominal defect
C. deficiency of the anterior diaphragm
D. defect of the upper sternum
E. intracardiac defect
D). Defect of the lower sternum.
237
The MOST common cause of pulmonary hypertension in pediatric patients is
A. idiopathic pulmonary arterial hypertension
B. pulmonary venoocclusive disease
C. left-sided valvular heart disease
D. chronic obstructive pulmonary disease
E. interstitial lung disease
(A). Idiopathic or familial disease is the most common in pediatric patients (~55%),
followed by pulmonary hypertension secondary to congenital heart disease (~35%) and
chronic respiratory disorders (~15%).
238
All patients with clinical evidence of a PDA are at increased risk for endocarditis. As
result, PDA should be considered for closure EXCEPT
A. PDA with severe irreversible pulmonary hypertension
B. small, hemodynamically insignificant PDA
C. small, hemodynamically significant PDA
D. moderate size PDA
E. large PDA
(A). All PDAs except for small silent PDAs and those patients with severe irreversible
pulmonary hypertension should be considered for closure.
239
```
Phenytoin is used for treatment of
A. supraventricular tachycardia
B. atrial fibrillation
C. atrial flutter
D. ventricular tachycardia
E. digitalis intoxication
```
(E). Other options are treated by procainamide.
240
The definition of hypertension in children is
A. average systolic blood pressure (SBP) and/or diastolic BP that is ≥95th
percentile for age, sex, on ≥3 occasions
B. average systolic blood pressure (SBP) and/or diastolic BP that is ≥95th
percentile for age, sex, and height on ≥2 occasions
C. average systolic blood pressure (SBP) and/or diastolic BP that is ≥95th
percentile for age, sex, and height on ≥3 occasions
D. average systolic blood pressure (SBP) and/or diastolic BP that is ≥90th
percentile for age, sex, and height on ≥3 occasions
E. average systolic blood pressure (SBP) and/or diastolic BP that is ≥99th
percentile for age, sex, and height on ≥3 occasions
(C). The National High Blood Pressure Education Program Working Group on High
Blood Pressure in Children and Adolescents published the Fourth Report on the
Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and
Adolescents (Fourth Report) in 2004.
The Fourth Report defined hypertension as average systolic blood pressure (SBP)
and/or diastolic BP that is ≥95th percentile for age, sex, and height on ≥3 occasions.
Prehypertension was defined as average SBP or diastolic BP that are ≥90th percentile
but <95th percentile.
241
```
Renal childhood diseases responsible for chronic hypertension include
A. hemolytic-uremic syndrome
B. acute tubular necrosis
C. congenital dysplastic kidney
D. pyelonephritis
E. renal trauma
```
(C). Other options are responsible for acute/ intermittent hypertension.
242
```
The valuable diagnostic feature that differentiate anemia of chronic disorder from
iron deficiency anemia is
A. low serum iron
B. low or normal serum transferrin
C. normal bone marrow cellularity
D. normochromic normocytic RBC
E. leukocytosis
```
(B). The serum iron level in anemia of chronic disorder is low, but without the
increase in total iron-binding capacity (serum transferrin) that occurs in iron deficiency.
This pattern of low serum iron and low to normal iron-binding protein (serum
transferrin) is a regular and valuable diagnostic feature.
243
All the following are true regarding physiologic anemia of infancy EXCEPT
A. it is exaggerated in premature infants
B. the hemoglobin concentration range is between 9-11 g/dL
C. the hemoglobin concentration rarely falls below 10 g/dL in healthy infants
D. vitamin E deficiency does not play a role
E. iron deficiency is a contributing factor
(E). The iron previously stored from the degraded fetal RBCs can be used for
hemoglobin synthesis. The supply of stored iron is sufficient for hemoglobin synthesis,
even in the absence of dietary iron intake, until approximately 20 wk of age. Unless
there has been significant blood loss, iron stores should be sufficient to maintain
erythropoiesis early on.
244
The first laboratory marker in progressive iron deficiency anemia is
A. depletion of bone marrow hemosiderin
B. falling of serum ferritin
C. decrease of serum iron and increase of the iron-binding capacity
D. decrease hemoglobin synthesis
E. hypochromic microcytic anemia
(A). In progressive iron deficiency, a sequence of biochemical and hematologic
events occurs. First, the tissue iron stores represented by bone marrow hemosiderin
disappear.
245
```
An extremely high RBC distribution width (RDW) is MOST likely consistent with
348
A. iron deficiency anemia
B. megaloblastic anemia
C. sideroblastic anemia
D. thalassemia
E. sickle cell anemia
```
(C). RDW is usually high in IDA, megaloblastic anemia, and sideroblastic anemia, but
it is extremely high in the last.
246
```
In hereditary spherocytosis, all the following will resolve postsplenectomy EXCEPT
A. osmotic fragility
B. anemia
C. reticulocytosis
D. hyperbilirubinemia
E. hypoplastic/aplastic crises
```
(A). After splenectomy, osmotic fragility often improves because of diminished
splenic conditioning and less RBC membrane loss, but not resolved.
247
Splenectomy is recommended in all the following conditions EXCEPT
A. a 6-year-old child with hereditary spherocytosis and significant hemolysis
B. a 7-year-old child with hereditary elliptocytosis and a hemoglobin level of 7
g/dL and corrected reticulocytes count of more than 15%
C. an 8-year-old child with hereditary stomatocytosis with hemolysis
D. a 6-year-old child with thalassemia major with splenomegaly and frequent
blood transfusion requirement
E. a 12-year-old child with chronic immune thrombocytopenia not responding to
all modalities of medical treatment
(C). Hemolytic anemia may be associated with hereditary stomatocytosis, but
splenectomy is not a recommended treatment. Persistent symptomatic thrombocytosis
may follow splenectomy if the hemolysis is not eliminated or markedly decreased.
Patients have developed a life-threatening tendency toward in situ thrombosis after
splenectomy in association with the abnormal adherence of the stomatocytic RBC to
vascular endothelium in conjunction with the thrombocytosis.
248
```
The best assessment of iron overload for patients with thalassemia major is
achieved by
A. liver MRI
B. bone marrow biopsy
C. serum iron
D. serum ferritin
E. total iron binding capacity
```
(A). Quantitative liver iron by approved MRI technology is the best indicator of totalbody
iron stores and should be obtained in chronically transfused patients after chronic
transfusion therapy has initiated.
249
You are evaluating a 4-year-old child with ß-thalassemia major; he is on chronic
transfusion therapy since the age of 1 year; you suspect transfusion-induced
hemosiderosis.
Of the following, the organ that is LEAST likely to be affected by iron deposition at this
time is
A. liver
B. pancreas
C. heart
D. pituitary gland
E. thyroid gland
(C). Iron is initially deposited in the liver. Liver hemosiderosis develops after 1 yr of
chronic transfusion therapy and is followed by iron deposition in the endocrine system.
This leads to a high rateof hypothyroidism, hypogonadotropic gonadism, growth
hormone deficiency, hypoparathyroidism, and diabetes mellitus. After 10 yr of
transfusion, cardiac dysfunction secondary to hemosiderosis begins.
250
A healthy 5-mo-old boy appears pale. Examination is unremarkable. Lab findings
include: Hb 8.1 g/dl; WBC 4,800/mm3; platelets 144,000/mm3; MCV, 111 fl; blood film
showed hypersegmented neutrophils; serum B12, 65 pg/mL (low). The infant is
vigorously breast fed.
Of the following, the NEXT step of the management is
A. performing Schilling test
B. check serum B12 of the mother
C. parenteral administration of vitamin B12
D. administration of both folic acid and vitamin B12
E. transfuse packed RBCs
(B). Older children and adults have sufficient vitamin B12 stores to last 3-5 yr.
However, in young infants born to mothers with low vitamin B12 stores (breast fed
364
infants of vegans mothers or having pernicious anemia), clinical signs of cobalamin
deficiency can become apparent in the first 4-5 mo of life.
251
A 9-month-old child with a hemoglobin concentration of 10 gm/dL and marked
microcytosis; serum iron and total iron binding capacity are within normal limits; serum
ferritin and hemoglobin electrophoresis are also normal.
Of the following, the MOST likely diagnosis is
A. iron deficiency anemia
B. sideroblastic anemia
C. ß-thalassemia minor
D. α-thalassemia trait
E. anemia due to chronic disease
(D). The α-thalassemia traits present as a microcytic anemia that can be mistaken
for iron-deficiency anemia. The hemoglobin electrophoresis is normal. The diagnosis is
confirmed by DNA testing.
252
```
All the following may cause autoimmune hemolytic anemia EXCEPT
A. systemic lupus erythematosus (SLE)
B. immunodeficiency
C. mycoplasma pneumoniae infection
D. lymphoproliferative disorder
E. penicillin drug administration
```
(E). Penicillin may cause hemolysis via the "hapten" mechanism (immune but not
autoimmune) by binding tightly to the RBC membrane. Antibodies to the drug, either
newly or previously formed, bind to the drug molecules on RBCs, mediating their
destruction in the spleen.
253
```
Fresh frozen plasma (FFP) transfusion is the best treatment for which of the
following conditions
A. hemophilia a
B. hemophilia b
C. factor vii deficiency
D. immunoglobulin replacement therapy
E. bleeding due to warfarin therapy
```
(E). Transfusion of FFP is no longer recommended for treatment of patients with
severe hemophilia A or B or for factor VII deficiency because safer factor VIII, IX, and VII
concentrates are available; also it is not indicated for correction of hypovolemia or as
immunoglobulin replacement therapy, because safer alternatives exist; An important
use of FFP, albeit rare in children, is for rapid reversal of warfarin effects in patients
who are actively bleeding or who require emergency surgery (i.e., in whom functional
deficiencies of factors II, VII, IX, and X cannot be rapidly reversed by vitamin K).
254
A 9-year-old male child is about to do elective tonsillectomy; preoperative
investigations are all normal except for a significant prolonged partial thromboplastin
time PTT; there was no previous bleeding after circumcision and emergency
appendectomy.
Of the following, the MOST likely diagnosis is
A. hemophilia A
B. hemophilia B
C. hemophilia C
D. prekallikrein deficiency
E. factor XIII deficiency
(D). Deficiencies of the contact factors prolong the PTT but are not causes of clinical
bleeding. These factors include factor XII, prekallikrein, and high molecular weight
kininogen.
255
A 16-year-old female is found to have multiple bruising and hypochromic microcytic
anemia; during her first pregnancy she noticed disappearance of the bruises; she has a
past history of uncomplicated emergency appendectomy at the age of 10 year.
Of the following, the drug MOST likely beneficial for alleviation of her symptoms is
A. ferrous sulphate
B. desmopressin acetate (DDAVP)
C. recombinant factor VIII
D. recombinant factor IX
E. aminocaproic acid
(B). Von Willebrand disease (VWD) is the most likely cause for this case;
menstruating female may present with iron deficiency due to menorrhagia. Because
VWF is an acute-phase protein, stress will increase its level; thus, patients may not
bleed during appendectomy and childbirth. Bruising symptoms may diminish during
pregnancy, because the VWF levels may double or triple during pregnancy.
256
```
Lupus anticoagulants usually affect the following laboratory test
A. bleeding time (BT)
B. partial thromboplastin time (PTT)
C. prothrombin time (PT)
D. thrombin time (TT)
E. clot solubility test
```
(B). The lupus anticoagulant causes a prolongation of the PTT that fails to correct on
1:1 mixing with normal plasma. The antibody is directed against the phospholipid used
as a reagent in the PTT.
257
A healthy 5-day-old male neonate develops bruising and melena. The pregnancy,
and delivery course were unremarkable. The mother received heparin therapy for
recurrent abortions secondary to lupus anticoagulants. The infant is vigorously
breastfeeding. Examination reveals only multiple bruises. Lab tests reveals:
hemoglobin, 8.4 g/dL; WBC, 8600/mm3; platelets count, 200000/mm3; PTT, 74
seconds; PT, 28 seconds; serum fibrinogen is normal.
Of the following, the MOST likely cause of this bleeding is
A. disseminated intravascular coagulopathy (DIC)
B. hemophilia
C. vitamin K deficiency
D. heparin therapy
E. lupus anticoagulants
(C). Heparin does not cross the placenta, and so it does not affect the baby. DIC
causes prolonged PTT, PT, thrombocytopenia and low serum fibrinogen. Hemophilia
causes prolonged PTT only. Lupus anticoagulants may cause thrombosis rather than
bleeding and usually associated with prolonged PTT because of the presence of
antiphospholipids that affect the phospholipid reagent.
258
previously healthy 10-month-old boy develops pallor over 2 weeks. Examination
is unremarkable except for pallor. Lab tests includes: hemoglobin, 4.9 g/dL; WBC,
6700/mm3; platelets count, 180000/mm3; MCV, 79 fL; reticulocyte count 0.8%;
hemoglobin electrophoresis, normal.
Of the following, the MOST likely cause for these findings is
A. pure red cell aplasia
B. transient erythroblastopenia of childhood
C. thalassemia minor
D. aplastic anemia
E. parvovirus infection
(B). Normal hemoglobin electrophoresis usually excludes thalassemia and Diamond-
Blackfan syndrome. Anemia due to parvovirus infection usually occurs in children with
hemolytic anemia or immunodeficiency. In aplastic anemia, the anemia is usually
macrocytic rather than normocytic.
259
You are evaluating a 5-year-old male child with multiple ecchymoses distributed all
over the extremities and trunk; examination reveal a palpable spleen 3 cm below the
left costal margin. Lab findings include: hemoglobin, 11.3 g/dL; white blood cell count,
8700/mm3; platelets count, 21000/mm3.
Of the following, the most proper NEXT step is
A. bleeding time
B. coagulation profile
C. platelets aggregation test
D. abdominal ultrasonography
E. bone marrow study
(E). Although spleen may be palpable in 10% of children with ITP, bone marrow
examination is mandatory when there is an unusual presentation.
260
```
Which of the following is least likely to be associated with prolonged
thrombocytopenia in children?
A. immune thrombocytopenia
B. systemic lupus erythematosus (SLE)
C. HIV infection
D. type 2B von Willebrand disease
E. Wiskott-Aldrich syndrome (WAS)
```
(A). In 70-80% of children who present with acute ITP, spontaneous resolution of
their ITP will occur within 6 mo.
261
```
Which of the following drugs is least likely to cause thrombocytopenia in children?
A. valproic acid
B. phenytoin
C. sulfonamides
D. trimethoprim-sulfamethoxazole
E. heparin
```
(E). Common drugs used in pediatrics that cause thrombocytopenia include valproic
acid, phenytoin, sulfonamides, and trimethoprim-sulfamethoxazole. Heparin-induced
thrombocytopenia is seldom seen in pediatrics.
262
Which of the following is not a feature of Wiskott–Aldrich Syndrome (WAS)
A. thrombocytopenia
B. eczema
C. recurrent otitis media and pneumonia
D. propensity to develop autoimmune disorders
E. giant platelets by blood smear
E
263
Each of the following may cause thrombocytopenia EXCEPT
A. heparin therapy
B. aspirin ingestion
C. disseminated intravascular coagulation (DIC)
D. systemic lupus erythematosus (SLE)
360
E. hemolytic-uremic syndrome (HUS)
(B). Aspirin may affect platelets function rather than the count.
264
Alport Syndrome AS, is a genetically heterogeneous disease caused by mutations in
the genes coding for type IV collagen, it is characterized by
A. asymptomatic microscopic hematuria
B. proteinuria<1 g/24 hr.
C. congenital sensor neural hearing loss
D. ocular abnormalities in 80% of X-linked type
E. leiomyomatosis of the esophagus is common
(A). All patients with AS have asymptomatic microscopic hematuria, which may be
intermittent in girls and younger boys. Progressive proteinuria, often exceeding 1 g/24
hr., is common by the 2nd decade of life and can be severe enough to cause nephrotic
syndrome. Bilateral sensor neural hearing loss, which is never congenital, Ocular
abnormalities, which occur in 30-40% of patients with X-linked AS, include anterior
lentic onus (extrusion of the central portion of the lens into the anterior chamber),
macular flecks, and corneal erosions. Leiomyomatosis of the esophagus,
tracheobronchial tree, and female genitals in association with platelet abnormalities
has been reported, but is rare.
265
Which of the following is pathognomonic in diagnosis of X-linked type of hereditary
nephritis?
A. gross hematuria
B. discontinuous epidermal basement membrane staining
C. platelet abnormalities
D. hearing loss
E. macular flecks
(B). Absence of epidermal basement membrane staining for the α5 chain of type IV
collagen in male hemi zygotes and discontinuous epidermal basement membrane
staining in female heterozygotes on skin biopsy is pathognomonic for X-linked AS and
can preclude diagnostic renal biopsy.
266
```
The best single test to document cutaneous streptococcal infection is
A. low serum C3 level
B. elevated antistreptolysin O titer
C. positive streptozyme screen
D. antideoxyribonuclease B level
E. depressed serum CH50
```
(D). Four differences in APSGN follow throat or skin infections:
Post streptococcal GN commonly follows streptococcal pharyngitis during cold-weather
months and streptococcal skin infections or pyoderma during warm-weather months.
Epidemics of nephritis have been described in association with throat (serotypes M1,
M4, M25, and some strains of M12) and skin (serotype M49) infections.
267
Renal biopsy in acute post streptococcal glomerulonephritis should be considered
in all the following EXCEPT
A. acute renal failure
B. nephrotic syndrome
C. absence of evidence of streptococcal infection
D. low C3 level in the first 2 months
E. initially normal complement levels
(D). Renal biopsy is considered when hematuria and proteinuria, diminished renal
function, and/or a low C3 level persist more than 2 mo. after onset.
268
Acute post- infectious glomerulonephritis is one of the most common glomerular
causes of gross hematuria in children.
Of the following, the TRUE statement is
A. can follow fungal infection
B. early systemic antibiotic, eliminate the risk of glomerulonephritis
C. all family members of patient need antibiotic treatment
D. antibiotic affect the natural history of the disease
E. recurrences are common
(A). Acute GN can occur after certain fungal, rickettsial, protozoan, parasitic, or viral
diseases (influenza and parvovirus) thatis why the terms APSGN and acute post
infectious GN are used synonymously. Early systemic antibiotic therapy for
streptococcal throat and skin infections does not eliminate the risk of GN. Family
members of patients with acute GN, especially young children, should be considered at
risk and be cultured for group A β-hemolytic streptococci and treated if positive. Family
pets, particularly dogs, have also been reported as carriers. A 10 day course of systemic
antibiotic therapy with penicillin is recommended to limit the spread of the
nephritogenic organisms, antibiotic therapy does not affect the natural history of
APSGN, and recurrences are extremely rare.
269
```
The MOST common etiology of secondary membranous nephropathy (MN) in
children is
A. neuroblastoma
B. measles
C. systemic lupus erythematosis
D. chronic hepatitis C
395
E. penicillin
```
(C). MN also associated with chronic hepatitis B infection and congenital syphilis,
other chronic infections, including malaria, which is likely the most common cause of
nephrotic syndrome worldwide. Certainmedications, such as penicillamine and gold, or
chronic factor replacement in patients with hemophilia. Rare causes such as
neuroblastoma, or other idiopathic systemic diseases.
270
Type I membranoproliferative glomerulonephritis is
A. less common than type II membranoproliferative glomerulonephritis
B. not to be mediated by immune complexes
C. associated with partial lipodystrophy
D. called dense deposit disease
E. characterized by low C3 complement level
(E). Type II MPGN (dense deposit disease) is less common than type I
membranoproliferative glomerulonephritis, not to be mediated by immune complexes,
demonstrate an associated systemic disease called partial lipodystrophy, serum C3
complement levels are low in the majority of cases(while in membranous nephropathy
is normal), Patients present in equal proportions with nephrotic syndrome, acute
nephritic syndrome, or persistent asymptomatic microscopic hematuria and
proteinuria (while in membranous nephropathy usually manifest as nephrotic
syndrome).
271
```
WHO classification of lupus nephritis is based on a combination of features
including light microscopy, immunofluorescence, and electron microscopy.
Of the following, the WORST outcome is associated with
A. class I nephritis
B. class II nephritis
C. class III nephritis
D. class IV nephritis
E. class V nephritis
```
(D). Renal biopsy should be performed in all patients with SLE because
histopathologic findings are used to determine the selection of specific
immunosuppressive therapies.
272
```
Henoch-Schönlein purpura is the most common small vessel vasculitis in childhood,
aggressive therapy may be reasonable in those with
396
A. isolated microscopic hematuria
B. insignificant proteinuria
C. severe systemic manifestations
D. >50% crescents on renal biopsy
E. IgA deposits intensity
```
(D). Spontaneous and complete resolution of the nephritis typically occurs in the
majority of patients with mild initial manifestations (isolated hematuria with
insignificant proteinuria), and the severity of the systemic manifestations does not
correlate with the severity of the nephritis.
273
"Rapidly progressive” RPGN describes the clinical course of several forms of
glomerulonephritis whose unifying feature is the histopathologic finding of crescents in
the majority of glomeruli. Plasmapheresis has been reported to be of benefit in
patients with
A. post infectious glomerulonephritis
B. immunoglobulin A nephropathy
C. Henoch-Schönleinpurpura
D. Good pasture disease
E. endocarditis
(D). Plasmapheresis may also benefit patients with ANCA-associated crescentic GN,
in particular those with the most severe renal dysfunction at presentation. The possible
benefits of plasmapheresis in other forms of RPGN are unclear.
274
Hemolytic-uremic syndrome is a common cause of community acquired acute
kidney injury in young children; atypical type may be triggered by
A. toxin-producing E-coli
B. raw milk
C. Streptococcus pneumoniae
D. shiga toxin
E. cyclosporine
E
275
In hemolytic-uremic syndrome, thrombotic microangiopathies are associated with
aII the following EXCEPT
A. genetically determined factor H deficiency
B. neuraminidase-producing Streptococcus pneumonia infection
C. systemic lupus erythematosis
D. malignancy
E. hypotension
(E). Malignant hypertension.
276
In hemolytic-uremic syndrome (HUS). Of the following, the MOSTcorrect statement
is
A. majority of patients of diarrhea-associated enteropathictype develops HUS
397
B. stool culture is often positive in patients who have diarrhea-associated HUS
C. kidney biopsy is rarely indicated to diagnose HUS
D. partial thromboplastin and prothrombin times are usually low
E. Coombs test is usually negative in in pneumococci-induced HUS
(C). Kidney biopsies are only rarely performed in HUS because the diagnosis is
usually established by clinical criteria and the risks of biopsy are significant during the
active phase of the disease. The organisms that cause HUS may be rapidly cleared,
therefore, the stool culture is often negative in patients who have diarrhea-associated
HUS, Partial thromboplastin and prothrombin times are usually normal, the Coombs
test is negative, with the exception of pneumococci-induced HUS, where the Coombs
test is usually positive.
277
All the following matching are true EXCEPT
A. nephrotic syndrome---Penicillin
B. nephrogenic diabetes inspidus ---cisplatin
C. nephrolithiasis ---furosemide
D. renal tubular acidosis---lithium
E. interstitial nephritis---cimetidine
(A). Nephrotic syndrome--- penicillamine while interstitial nephritis--- penicillin
278
```
Hypercalciuria can be seen in
A. hypoparathyroidism
B. corticosteroid therapy
C. vitamin D deficiency
D. oral thiazide diuretics therapy
398
E. Addison`s disease
```
(B). Hypercalciuria can accompany conditions resulting in hypercalcemia, such as
hyperparathyroidism, vitamin D intoxication, immobilization, and sarcoidosis.
Hypercalciuria may be associated with Cushing syndrome, corticosteroid therapy,
tubular dysfunction secondary to Fanconi syndrome (Wilson disease,
oculocerebrorenal syndrome), Williams syndrome, distal renal tubular acidosis, or
413
Bartter syndrome. Oral thiazide diuretics can normalize urinary calcium excretion by
stimulating calcium reabsorption in the proximal and distal tubules.
279
```
Hemorrhagic cystitis can occur in response to all the following EXCEPT
A. cyclophosphamide
B. adenovirus infection
C. cyclosporine
D. polyoma BK virus infection
E. amyloidosis
```
(C). The hemorrhagic cystitis can occur in response to chemical toxins
(cyclophosphamide, penicillin’s, busulfan, thiotepa, dyes, insecticides), viruses
(adenovirus types 11 and 21 and influenza A), radiation, and amyloidosis. The polyoma
BK virus, present latently in immunocompetent hosts, is associated with the
development of drug-induced cystitis in immunosuppressed patients.
280
Autosomal dominant polycystic kidney disease is the most common hereditary
human kidney disease, all the following are true EXCEPT
A. gross or microscopic hematuria
B. may be seen in neonates
C. abnormal renal sonography in the absence of symptoms
D. is a multiorgan disorder
E. right sided valvular heart disease
(E). Mitral valve prolapse is seen in approximately 12% of children; aortic and
coronary artery aneurysms and aortic valve insufficiency are noted in affected adults. It
is a systemic disorder with possible cyst formation in multiple organs (liver, pancreas,
spleen, brain) and the development of saccular cerebral aneurysms.
281
```
Steroid resistance nephrotic syndrome is defined as failure to achieve remission
after
A. 4wk of corticosteroid therapy
B. 6 wk. of corticosteroid therapy
C. 8 wk. of corticosteroid therapy
D. 10 wk. of corticosteroid therapy
E. 12 wk. of corticosteroid therapy
```
(C). Steroid-resistant nephrotic syndrome is usually caused by focal segmental
glomerulosclerosis (80%), minimal change nephrotic syndrome, or
membranoproliferativeglomerulonephritis.
282
To reduce the risk of serious infections in children with nephrotic syndrome, all the
following immunizations can be used EXCEPT
A. pneumococcal 13-valent conjugant vaccine
B. influenza vaccine annually to the child
C. pneumococcal 23-valent polysaccharide vaccine
D. varicella-zoster vaccine
E. influenza vaccination annually to their household contacts
(D). Following close contact with varicella infection, give immune compromised
children on immunosuppressive agents varicella-zoster immune globulin if available.
283
Congenital nephrotic syndrome is defined as nephrotic syndrome manifesting at
birth or within the 1st 3 mo of life,
The etiologies include all the following EXCEPT
A. syphilis
B. herpes infection
C. mercury exposure
D. mutations in the NPHS1
E. infantile systemic lupus erythematosis
(B). Congenital nephrotic syndrome may be classified as primary or as secondary to
a number of etiologies such as in utero infections (cytomegalovirus, toxoplasmosis,
syphilis, hepatitis B and C, HIV).
284
```
All the following matching are true EXCEPT
A. proximal RTA----trimethoprim
B. distal RTA----amphotericin B
C. hyperkalemic RTA----cyclosporine
D. distal RTA----lithium
E. proximal RTA----gentamicin
```
(A). Hyperkalemia RTA---- Trimethoprim/sulfamethoxazole.
285
```
Pathogenesis of hyperkalemic (Type IV) renal tubular acidosis is due to
A. hyperaldosteronism
B. inhibiting ammonia genesis
C. pseudohyperaldosteronism
D. massive bicarbonate wasting
E. all of the above
```
(B). Type IV RTA occurs as the result of impaired aldosterone production
(hypoaldosteronism) or impaired renal responsiveness to aldosterone
(pseudohypoaldosteronism), proximal RTA pathogenesis is massive bicarbonate
wasting. Acidosis results because aldosterone has a direct effect on the H+/ATPase
responsible for hydrogen secretion. In addition, aldosterone is a potent stimulant for
potassium secretion in the collecting tubule; consequently, lack of aldosterone results
415
in hyperkalemia. This further affects acid–base status by inhibiting ammoniagenesis
and, thus, H+ excretion.
286
Acute kidney injury AKI is a clinical syndrome in which a sudden deterioration in
renal function results in the inability of the kidneys to maintain fluid and electrolyte
homeostasis.
Of the following, the MOST correct matching is
A. low urine sodium (UNa< 20 mEq/L) : intrinsic AKI
B. fractional excretion of sodium >2% : prerenal AKI
C. elevated urine osmolality (UOsm>500 mOsm/kg) : post renal AKI
D. specific gravity of <1.010 : intrinsic AKI
E. red blood cell casts :prerenal AKI
(D). Patients whose urine shows an elevated specific gravity (>1.020), elevated urine
osmolality (UOsm>500 mOsm/kg), low urine sodium (UNa< 20 mEq/L), and fractional
excretion of sodium <1% (<2.5% in neonates) most likely have prerenal AKI. Those with
a specific gravity of <1.010, low urine osmolality (UOsm< 350 mOsm/kg), high urine
sodium (UNa> 40 mEq/L), and fractional excretion of sodium >2% (>10% in neonates),
red blood cell casts most likely have intrinsic AKI and low urine osmolality (UOsm< 350
mOsm/kg) in Post renal AKI.
287
Medical management of acute kidney injury include all the following EXCEPT
A. bladder catheter should be placed immediately
B. intravenous administration of boluses isotonic saline
C. furosemide may be administered as a single IV dose
D. fluid restriction is essential if there is no response to a diuretic challenge
E. mannitol is not effective in prevention of pigment induced renal failure
(E). Mannitol may be effective in prevention of pigment (myoglobin, hemoglobin)
induced renal failure.
288
Hyperkalemia can lead to cardiac arrhythmia, cardiac arrest, and death. Serum
potassium can be lowered by all the following EXCEPT
A. regular insulin with glucose
B. calcium gluconate
C. sodium bicarbonate
D. Kayexalate
E. Dialysis
(B). Calcium gluconate counteracts the potassium-induced increase in myocardial
irritability but does not lower the serum potassium level.
289
```
Indications for dialysis in acute kidney injury include all the following EXCEPT
A. anuria/oliguria
B. persistent hypercalcemia
C. severe metabolic acidosis
D. volume overload
E. blood urea nitrogen >100-150 mg/dl
```
(B). Persistent hyperkalemia.
290
Chronic kidney disease in children <5-yr-old is MOST commonly a result of
A. lupus nephritis
B. familial juvenile nephronophthisis
C. Alport syndrome
D. focal segmental glomerulosclerosis
E. autosomal dominant polycystic kidney disease
(D). After 5 yr of age, acquired diseases (various forms of glomerulonephritis
including lupus nephritis) and inherited disorders (familial juvenile nephronophthisis,
Alport syndrome) predominate.
291
```
Pathogenesis of chronic kidney disease (CKD) include all the following EXCEPT
A. hyperfiltration injury
B. hyperkalemia
C. proteinuria
D. hypertension
E. hyperphosphatemia
```
(B). Hyperkalemia is the result of CKD not a cause.
292
```
Infections need to be identified and treated before renal transplantation. Infectious
disease screening includes all the following EXCEPT
A. tuberculosis skin test
B. cytomegalovirus IgG
C. measles antibody
407
D. hepatitis A serology
E. varicella titer
```
(D). Infections need to be identified and treated before renal transplantation are;
Hepatitis B and Hepatitis C
293
```
Glomerular filtration rate in full term neonate is
A. 15 mL/min/1.73 m2
B. 25 mL/min/1.73 m2
C. 35mL/min/1.73 m2
D. 45 mL/min/1.73 m2
E. 55 mL/min/1.73 m2
```
B
294
```
Potter syndrome (bilateral renal agenesis) is characterized by all the following
EXCEPT
A. widely separated eyes
B. polyhydramnios
C. low set ears
D. broad nose
E. limb anomalies
```
B. Oligohydramnios
295
```
Death in neonates with bilateral renal agenesis is due to
A. renal failure
B. hypertension
C. pulmonary insufficiency
D. congenital heart disease
E. sepsis
```
C). Death is due to pulmonary insufficiency from pulmonary hypoplasia rather than
renal failure.
296
```
The MOST common cause of an abdominal mass in the newborn is
A. polycystic kidney disease
B. neuroblastoma
C. renal vein thrombosis
D. hydronephrosis
E. multicystic dysplastic kidney
```
E
297
```
One of the complications of the horseshoe kidney is
A. renal abscess
B. neuroblastoma
C. bladder outlet obstruction
D. familial renal dysplasia
418
E. multicystic dysplastic kidney
```
(E). Wilms tumors are 4 times more common in children with horseshoe kidneys than
in the general population, stone disease and hydronephrosis secondary to
ureteropelvic junction obstruction are other potential late complications.
298
```
Symptoms of cystitis include
A. fever
B. polyuria
C. nausea
D. suprapubic pain
E. urinary retention
```
(D). Symptoms include dysuria, urgency, frequency, suprapubic pain, incontinence,
and malodorous urine. Cystitis does not cause fever and does not result in renal injury;
malodorous urine is not specific for a UTI.
299
```
All the following are indications for admission to hospital in patients with urosepsis
EXCEPT
A. dehydration
B. unable to drink fluids
C. neonatal period
D. vomiting
E. microscopic hematuria
```
(E). Microscopic hematuria is common in acute cystitis, but micro hematuria alone
does not suggest UTI.
300
Regarding vesicoureteral reflux VUR (the retrograde flow of urine from the bladder
to the ureter and kidney).
All the following are true EXCEPT
A. it is usually congenital
B. it may cause hypertension in children
C. the mean age at VUR resolution is 10yr
D. severity is graded according to voiding cystourethrogram
E. reimplantation of the ureters corrects the condition
(C). The mean age at VUR resolution is 6yr.
301
```
The MOST common cause of daytime incontinence is
A. an overactive bladder
B. infrequent voiding
C. detrusor–sphincter dyssynergia
D. bladder outlet obstruction
E. behavioral
```
A
302
```
Staccato urinary stream is seen in
A. hypospadias
B. meatal stenosis
C. ureteral ectopia
D. posterior urethral valves
E. non neurogenic bladder
```
(E). In posterior urethral valves the urinary stream is weak. Hypospadias is typically
ventral deflection or severe splaying. Meatal stenosis; if the meatus is pinpoint, boys
void with a forceful, fine stream that goes a great distance. Ureteral ectopia
characterized by constant urinary dripping all day.
303
```
The MOST effective way of treatment in older children with nocturnal enuresis is
A. motivational therapy
B. conditioning therapy
423
C. desmopressin acetate
D. oxybutynin chloride
E. imipramine
```
(B). This form of therapy has a reported success of 30-60%, although the relapse
rate is significant, while motivational therapy; there is no evidence that this approach is
428
beneficial, Pharmacologic therapy is intended to treat
304
```
The gold standard diagnostic investigation for precocious puberty is
A. Tanner maturating rate
B. left wrist x-ray
C. serum luteinizing hormone levels
D. gonadotropins measurement
E. brain MRI
```
(D). Measurement of gonadotropins after gonadotropin-releasing hormone or
gonadotropin-releasing hormone–agonist stimulation.
305
```
Of the following, the MOST likely cause of bloody nipple discharge in infants is
A. chronic nipple irritation
B. ducts of Montgomery
C. hypothalamic tumors
D. Intraductal cysts
E. mammary duct ectasia
```
E
306
```
The MOST common cause of breast pain in adolescents is
A. benign fibroadenoma
B. exercise
C. mastitis
D. lipoma
E. Intraductal papilloma
```
(B). Physiologic swelling and tenderness occur on a cyclic basis, most commonly
during the premenstrual phase, and are secondary to hormonal stimulation and
resulting proliferative changes. Hormonal imbalance can cause exaggerated responses
in the breast tissue, especially in the upper and outer quadrants. Nodularity, poorly
localized tenderness, and a soreness radiating to the axilla and arm are usual
accompanying findings.
307
```
Of the following, the MOST common solid mass seen in adolescent girls is
A. lipoma
B. fibroadenoma
C. lymphangioma
D. hematoma
E. hamartoma
```
B
308
```
All the following may cause hirsutism EXCEPT
A. hydrochlorothiazide
B. acetazolamide
C. cyclophosphamide
D. anabolic steroids
E. penicillamine
```
C - alopecia
309
```
Typical insulin dosage requirement in prepubertal children after the "honeymoon"
period is
A. 0.4 U/kg/day
B. 0.5 U/kg/day
C. 0.6 U/kg/day
437
D. 0.7 U/kg/day
E. 1 U/kg/day
```
(B). Prepubertal children generally require about 0.5 U/kg/day. During the middle of
puberty, dosages often exceed 1 U/kg/day, whereas postpubertal individuals require
0.75-1.0 U/kg/day. Athletes or those with a low caloric intake may require less insulin.
For most children with type 1 diabetes, there are a variety of strategies and
combinations of insulin that are currently used. There is a trend toward three or more
doses of insulin each day. In addition, more children are on insulin pump therapy.
310
```
Neonates and young infants with central diabetes insipidus are often best treated
with
A. fluid therapy
B. vasopressin analogs
C. thiazide diuretics
D. indomethacin
E. amiloride
```
(A). Neonates and young infants are often best treated solely with fluid therapy,
given their requirement for large volumes (3 L/m2/24 hr) of nutritive fluid.
311
The clinical presentation of adrenal insufficiency depends on the age of the patient,
the usual presentation of adrenal insufficiency in infancy is
A. ketosis
B. hyperpigmentation
C. orthostatic hypotension
D. hypoglycemia
E. hypernatremia
(D). Hyperkalemia, hyponatremia, and hypoglycemia are prominent presenting signs
of adrenal insufficiency in infants.
312
The MOST definitive test for adrenal insufficiency is measurement of
A. blood sugar
B. cortisol before and after administration of ACTH
C. serum sodium
D. arterial blood gases
E. urinary excretion of sodium and chloride
(B). The most definitive test for adrenal insufficiency is measurement of serum
levels of cortisol before and after administration of ACTH; resting levels are low and do
not increase normally after administration of ACTH.
313
```
The MOST common cardiac defect in Noonan syndrome is
A. aortic coarctation
B. pulmonary valvular stenosis
C. aortic stenosis
D. mitral valve prolapse
E. anomalous pulmonary venous drainage
```
(B). The phenotype differs from Turner syndrome in several respects. Cognitive
impairment is often present, the cardiac defect is most often pulmonary valvular
stenosis or an atrial septal defect rather than an aortic defect, normal sexual
maturation usually occurs but is delayed by 2 yr on average, and premature ovarian
failure has been reported.
314
```
An elevated polymorphonuclear (PMN) cells count in cerebrospinal fluid (CSF)
suggests
A. tuberculous meningitis
B. early phase of aseptic meningitis
C. fungal meningitis
D. demyelinating diseases
E. brain or spinal cord tumor
```
(B). Normal CSF contains up to 5/mm3 white blood cells (WBCs), and a newborn can
have as many as 15/mm3. Polymorphonuclear (PMN) cells are always abnormal in a
child, but 1-2/mm3 may be present in a normal neonate. An elevated PMN count
suggests bacterial meningitis or the early phase of aseptic meningitis. CSF
lymphocytosis can be seen in aseptic, tuberculous, or fungal meningitis; demyelinating
diseases; brain or spinal cord tumor; immunologic disorders, including collagen vascular
diseases; and chemical irritation (following myelogram, intrathecal methotrexate).
315
Regarding myelomeningocele, all the following are true EXCEPT
A. risk of recurrence after one affected child is 3-4%
B. maternal periconceptional use of folic acid supplementation reduces the
incidence of neural tube defects (NTDs) in pregnancies at risk by at least 50%
C. anticonvulsant valproic acid causes NTDs in approximately 1-2% of pregnancies
when administered during pregnancy
D. hydrocephalus in association with a type II Chiari malformation develops in at
least 80% of patients with myelomeningocele
E. the lower the deformity is in the neuraxis (sacrum), the more likely is the risk of
hydrocephalus
(E). Generally, the lower the deformity is in the neuraxis (sacrum), the less likely is the
risk of hydrocephalus.
316
Mobius syndrome is characterized by the following EXCEPT
A. bilateral facial weakness
B. paralysis of the abducens nerve
C. hypoplasia or agenesis of brainstem nuclei
D. feeding difficulties
E. mental retardation
(E). The immobile, dull facies might give the incorrect impression of mental
retardation; the prognosis for normal development is excellent in most cases.
317
```
Causes of communicating hydrocephalus include
A. achondroplasia
B. aqueductal stenosis
C. Chiari malformation
D. Dandy-Walker malformation
E. Klippel-Feil syndrome
```
(A). Aqueductal stenosis, Chiari malformation, Dandy-Walker malformation, and
Klippel-Feil syndrome are causes of non-communicating hydrocephalus.
318
```
PHACE syndrome denotes to the following EXCEPT
A. posterior fossa malformations
B. hemangiomas
C. anal anomalies
D. coarctation of the aorta
E. eye abnormalities
```
(C). Arterial anomalies.
319
```
The MOST common cause of neonatal seizures is
A. vascular events
B. intracranial infections
C. brain malformations
D. hypoxic-Ischemic encephalopathy
E. metabolic disturbances
```
(D). Hypoxic-ischemic encephalopathy is the most common cause of neonatal
seizures, accounting for 50-60% of patients. Seizures secondary to this encephalopathy
occur within 12 hr of birth.
320
```
Many inborn errors of metabolism cause generalized convulsions in the newborn
period. Prominent hiccups, persistent generalized seizures, and lethargy rapidly leading
to coma are features of
A. propionic academia
B. maple syrup urine disease
C. nonketotic hyperglycinemia
D. Leigh disease
E. neonatal adrenoleukodystrophy
```
(C). Nonketotic hyperglycinemia, an intractable condition characterized by markedly
elevated plasma and CSF glycine levels, prominent hiccups, persistent generalized
seizures, and lethargy rapidly leading to coma.
321
```
The initial drug used to control acute neonatal seizures is usually
A. diazepam
B. midazolam
C. phenobarbital
D. lorazepam
E. phenytoin
```
(D). The initial drug used to control acute seizures is usually lorazepam. Lorazepam
is distributed to the brain very quickly and exerts its anticonvulsant effect in <5 min. It
is not very lipophilic and does not clear out from the brain very rapidly. Its action can
last 6-24 hr. Usually, it does not cause hypotension or respiratory depression. The dose
is 0.05 mg/kg (range: 0.02-0.10 mg/kg) every 4-8 hr.
322
Intravenous phenytoin is not widely used to control acute neonatal seizures
because of all the following EXCEPT
A. reduced solubility
B. severe local cutaneous reactions
C. interaction with other drugs
D. possible cardiac toxicity
E. not possible to mix with saline solutions
(E). It is not possible to mix phenytoin or fosphenytoin with dextrose solutions.
323
Status epilepticus is a medical emergency that should be anticipated in any patient
who presents with an acute seizure. It is defined as continuous seizure activity or
recurrent seizure activity without regaining of consciousness lasting for more than
A. 5 min
B. 15 min
C. 30 min
D. 45 min
E. 60 min
A
324
```
The MOST severe form of CP is
A. spastic diplegia
B. spastic quadriplegia
C. spastic hemiplegia
D. athetoid CP
E. dyskinetic CP
```
(B). Spastic quadriplegia is the most severe form of CP because of marked motor
impairment of all extremities and the high association with intellectual disability and
seizures.
325
```
All the following can be used for treatment of cerebral palsy (CP) EXCEPT
A. benzodiazepines
B. baclofen
C. dantrolene
D. botulinum toxin
E. hyperbaric oxygen
```
(E). Hyperbaric oxygen has not been shown to improve the condition of children
with CP.
326
```
The MOST common cause of childhood subarachnoid and intraparenchymal
hemorrhagic stroke (HS) is
A. moyamoya disease/syndrome
B. arteriovenous malformations
C. cerebral sinovenous thrombosis
D. hemolytic uremic syndrome
E. idiopathic thrombocytopenic purpura
```
(B). Arteriovenous malformations are the most common cause of childhood
subarachnoid and intraparenchymal HS and may occur anywhere.
327
The diagnosis of diffuse CNS infections depends on examination of cerebrospinal
fluid (CSF) obtained by lumbar puncture (LP). The cerebrospinal fluid (CSF) protein may
reach 3,000 (mg/dL) in
A. acute bacterial meningitis
B. tuberculous meningitis
C. fungal meningitis
D. amebic (naegleria) meningoencephalitis
481
E. subdural empyema
(B). In tuberculous meningitis, CSF protein is 100-3,000; may be higher in presence
of block, while in other options, it may reach 500.
328
```
The CSF leukocyte count in normal healthy neonates may reach
A. 10 leukocytes/mm3
B. 15 leukocytes/mm3
C. 20 leukocytes/mm3
D. 25 leukocytes/mm3
E. 30 leukocytes/mm3
```
(E). Normal healthy neonates may have as many as 30 leukocytes/mm3 (usually
<10), but older children without viral or bacterial meningitis have <5 leukocytes/mm3
in the CSF; in both age groups there is a predominance of lymphocytes or monocytes.
329
Seizures are common during the course of bacterial meningitis. Immediate therapy
for seizures includes intravenous diazepam (0.1- 0.2 mg/kg/dose) or lorazepam (0.05-
0.10 mg/kg/dose).
After immediate management of seizures, patients should receive the following
anticonvulsant to reduce the likelihood of recurrence
A. valproic acid
B. phenytoin
C. phenobarbital
D. lorazepam
E. carbamazepine
(B). After immediate management of seizures, patients should receive phenytoin
(15-20 mg/ kg loading dose, 5 mg/kg/24 hr maintenance) to reduce the likelihood of
recurrence. Phenytoin is preferred to phenobarbital because it produces less CNS
depression and permits assessment of a patient’s level of consciousness. Serum
phenytoin levels should be monitored to maintain them in the therapeutic range (10-20
μg/mL).
330
```
The MOST common neurologic sequelae of bacterial meningitis is
A. recurrent seizures
B. delay in acquisition of language
C. hearing loss
D. cognitive impairment
E. visual impairment
```
(C). Sensorineural hearing loss is the most common sequela of bacterial meningitis
and, usually, is already present at the time of initial presentation. It is a result of
cochlear infection and occurs in as many as 30% of patients with pneumococcal
meningitis, 10% with meningococcal, and 5-20% of those with H. influenzae type b
meningitis. Hearing loss may also be caused by direct inflammation of the auditory
nerve. All patients with bacterial meningitis should undergo careful audiologic
assessment before or soon after discharge from the hospital. Frequent reassessment
on an outpatient basis is indicated for patients who have a hearing deficit.
331
```
The MOST common presenting complaint of intramedullary spinal cord tumors is
A. gait disturbance
B. sensory deficits
C. scoliosis
D. back pain
484
E. urinary urgency
```
(D). With the exception of the uncommon malignant glial tumors of the spinal
cord, which tend to present precipitously, intramedullary spinal cord tumors present in
a very insidious manner. Back pain related to the level of the tumor is a common
presenting complaint. It is likely that this pain will awaken the child from sleep and
improve as the day progresses.
332
```
The MOST common involved segments in transverse myelitis (TM) are in the
A. cervical region
B. thoracic region
C. lumbar region
D. lumbo-sacral region
E. sacral region
```
B
333
Duchenne muscular dystrophy (DMD) is the most common hereditary
neuromuscular disease affecting all races and ethnic groups.
All the following are features of DMD EXCEPT
A. scoliosis
B. contracture
C. fasciculation
D. cardiomyopathy
E. intellectual impairment
(C). Fasciculations of muscle, which are often best seen in the tongue, are a sign of
denervation is seen in spinal muscular atrophy.
334
The characteristic features of Duchenne muscular dystrophy (DMD) are progressive
weakness, intellectual impairment, hypertrophy of the calves, and proliferation of
connective tissue in muscle.
Of the following, the BEST initial test for diagnosis is
A. PCR
B. muscle biopsy
C. electromyography
D. serum creatine kinase
E. nerve conduction study
(D). The diagnosis should be confirmed by blood PCR or muscle biopsy in every
case.
335
In Guillain-Barré syndrome, the paralysis usually follows a nonspecific
gastrointestinal or respiratory infection by approximately 10 days.
Of the following, the MOST likely respiratory infection that triggers the disease is
A. Chlamydia trachomitis
B. Staphylococcal aureus
C. Haemophilus influenzae
D. Mycoplasma pneumoniae
E. Streptococcal pneumonia
(D). The original infection might have caused only gastrointestinal (especially
Campylobacter jejuni, but also Helicobacter pylori) or respiratory tract (especially
Mycoplasma pneumoniae) symptoms.
336
```
All the following vaccines may induce Guillain-Barré syndrome (GBS) EXCEPT
A. OPV
B. Rota
C. Rabies
D. Influenza
E. Conjugated meningococcal vaccine
```
B
337
In Guillain-Barré syndrome, the onset is gradual and progresses over days or weeks.
The maximal severity of weakness is usually reached by
A. 2 wk
B. 4 wk
C. 6 wk
D. 8 wk
E. 10 wk
(B). Weakness usually begins in the lower extremities and progressively involves the
trunk, the upper limbs, and, finally, the bulbar muscles, a pattern known as Landry
ascending paralysis. The onset is gradual and progresses over days or weeks; the
process plateaus in 1-28 days. Weakness can progress to inability or refusal to walk and
later to flaccid tetraplegia. Maximal severity of weakness is usually reached by 4 wk
after onset.
338
Guillain Barré syndrome is an autoimmune disorder often considered a
postinfectious polyneuropathy involving mainly motor but also sensory and sometimes
autonomic nerves.
All the following are required in diagnosis EXCEPT
A. CSF study
B. muscle biopsy
C. electromyography
D. sural nerve biopsy
E. motor nerve conduction study
(B). Muscle biopsy is not usually required for diagnosis; specimens appear normal in
early stages and show evidence of denervation atrophy in chronic stages.
339
```
The best diagnostic test in Guillain-Barré syndrome (GBS) is
A. CSF study
B. electromyography
C. sural nerve biopsy
D. serum creatine kinase
E. motor nerve conduction study
```
(A). The dissociation between high CSF protein and a lack of cellular response in a
patient with an acute or subacute polyneuropathy is diagnostic of Guillain-Barré
syndrome.
340
Bell palsy is an acute unilateral peripheral facial nerve palsy that is not associated
with other cranial neuropathies or brainstem dysfunction.
Of the following, the MOST traditional treatment is
A. acyclovir
B. prednisone
C. laser therapy
D. physiotherapy
E. surgical decompression
(B). Oral prednisone (1 mg/kg/day for 1 wk, followed by a 1 wk taper) started within
the 1st 3-5 days results in improved outcome and is a traditional treatment, its efficacy
confirmed in a recent long-term prospective study in the United Kingdom.
341
Bell palsy usually develops abruptly about 2 wk after a systemic viral infection.
Of the following, the MOST common viral cause is
A. Mumps virus
B. Cytomegalovirus
C. Epstein-Barr virus
D. Herpes simplex virus
E. Human herpes virus 6
(D). Active or reactivation of herpes simplex or varicella-zoster virus may be the
most common cause of Bell palsy.
342
```
Charcot-Marie-Tooth disease, the most common genetically determined
neuropathy.
Of the following, the MOST severely affected nerve is
A. radial
B. facial
C. femoral
D. peroneal
E. mandibular
```
(D). The peroneal and tibial nerves are the earliest and most severely affected.
343
The MOST common cause of dizziness in young children is
A. cholesteatoma in the mastoid or middle ear
B. eustachian tube middle ear disease
C. labyrinthitis
D. vestibular neuronitis
E. benign paroxysmal vertigo
B
344
```
The MOST common infectious cause of congenital sensorineural hearing loss is
A. Measles virus
B. Cytomegalovirus
C. Toxoplasma gondii
D. Rubella virus
E. Treponema pallidum
```
B
345
The MOST common cause of bacterial meningitis that results in sensorineural
hearing loss after the neonatal period is
A. H. influenza
B. E. Coli
C. Streptococcus pneumoniae
D. Listeria monocytogenes
E. Staphylococcus aureus
C
346
A 3-day-old neonate presented with numerous firm, yellow-white, 1-2 mm papules
and pustules with a surrounding erythematous flare in several sites of the body surface
but the palms and soles were spared. Intralesional contents demonstrated eosinophils
in Wright-stained smears and culture was sterile.
Of the following, the MOST likely diagnosis is
A. pyoderma
B. erythema toxicum
C. candidiasis
D. herpes simplex
E. transient neonatal pustular melanosis
(
347
A 3-day-old neonate presented with unvarying red purple hue restricted to left
lower limb with atrophic underlying subcutaneous tissue. The lesions become more
pronounced during changes in environmental temperature, physical activity, or crying.
Of the following, the MOST likely diagnosis is
A. reticulate capillary malformation
B. cutis marmorata telangiectatica congenital
C. harlequin color change
D. cutis marmorata
E. cutis verticis gyrate
B
348
A 3-month-old infant presented with an intensely erythematous, confluent plaque
with a scalloped border, a sharply demarcated edge, and satellite pustules in the
perianal skin, perineum, and inguinal folds.
Of the following, the MOST likely diagnosis is
A. seborrheic dermatitis
B. atopic dermatitis
C. primary irritant contact dermatitis
D. candidal diaper dermatitis
E. bacterial dermatitis
D
349
A 4-year-old child presented with a 2 pearly, skin-colored, smooth, dome-shaped, 4
mm papules with a central umbilication on the face.
Of the following, the MOST likely diagnosis is
A. molluscum contagiosum
B. ectopic sebaceous glands
C. keratoacanthoma
D. warty dyskeratoma
E. cryptococcosis
(A). They typically have a central umbilication from which a plug of cheesy material
can be expressed. The papules may occur anywhere on the body, but the face, eyelids,
neck, axillae, and thighs are sites of predilection.
350
```
The MOST common risk factor for scurvy in children is
A. prolonged antibiotics use
B. psychiatric disease
C. exclusive breast feeding
D. chronic diarrhea
E. cow milk protein allergy
```
(B). In children, the most common risk factors are behavioralor psychiatric disease
that results in poor nutrition.
351
An adolescent girl presented with pain beneath the patella, walking up and down
stairs aggravates the pain; squatting, running, and other vigorous physical activities also
exacerbate the pain. There is no history of antecedent trauma and there is no swelling.
Pain is often relieved through knee extension.
Of the following, the MOST likely diagnosis is
A. osteochondroma
B. osteochondritis dissecans
C. popliteal cyst
D. patellofemoral pain syndrome
E. juvenile idiopathic arthritis
(D). Patellofemoral pain syndrome (PFPS) is one of the most common causes of knee
pain, particularly in adolescent girls. The precise etiology of the knee pain remains
unknown and is likely multifactorial.
352
Toddler fractures occur in young ambulatory children. The age range for this
fracture is typically around 1-4 yr.
All the following statement are true EXCEPT
A. children in this age group are usually unable to describe the area of injury well
B. radiographs may show no fracture
C. classic symptom is refusal to bear weight
D. Inflammatory markers may be ordered to rule out infectious processes
E. fracture is treated with bed rest and analgesia for approximately 2 wk
(E). The fracture is treated with an above-knee cast for approximately 3 wk.
353
```
The two MOST common causative organisms of osteomyelitis in children with sickle
cell anemia are
A. S. aureus and streptococci
B. Salmonella spp. and S. aureus
C. S. aureus and Pseudomonas aeruginosa
D. Enterobacteriaceae and S. aureus
E. S. aureus and Bartonella henselae
```
(B). Streptococcus pneumoniae most commonly causes osteomyelitis in children
younger than 24 mo of age and in children with sickle cell anemia, but its frequency has
declined because of pneumococcal conjugate vaccines. Cases of Pseudomonas
infection are related almost exclusively to puncture wounds of the foot, with direct
inoculation of P. aeruginosa from the foam padding of the shoe into bone or cartilage,
which develops as osteochondritis. Bartonella henselae can cause osteomyelitis of any
bone, but especially in pelvic and vertebral bones.
354
A 3 year old male presented to your clinic with chronic
constipation since early infancy. Patient's weight and height are
normal for age. However, patient was noted to only have 1-2
bowel movement per week. On birth history, passage of
meconium was noted on the third day of life. DRE revealed good
sphincteric tone, with no stool in the rectal vault. The
pathophysiology of this disease entity is usually located at the
A. Small intestines
B. Pylorus of the stomach
C. Recto-sigmoid area
D. Esophagus
C
355
```
The most common etiologic agent for upper respiratory tract
infection in children is
A. S. pneumoniae
B. RSV
C. Rhinovirus
D. Rotavirus
```
C
356
```
The most common pediatric inflammatory myopathy is
characterized by the following except?
A. Gottron Papules
B. Heliotrope Rash
C. Janeway lesions
D. None of the Above
```
C
357
```
The functional closure of the ductus arteriosus in the neonate
occurs within
A. 10-15 hours after birth
B. 2-3 weeks
C. 2-3 months
D. 2-3 years
• Functional closure of
```
A
358
A 1 year-old male came for consult for rashes. Four days prior,
patient started having runny nose with clear nasal discharge
with mild conjunctival redness. Three days ago, patient had a
fever of 39°C which resolved on the 3rd day but a few hours
after there was appearance of a rash starting on the trunk which
eventually spread to the face and extremities. PE revealed small
pinkish maculopapular lesions over the trunk, face and
extremities, HR: 120, RR: 33, T> 37.2°C. What is the most likely
diagnosis?
A. Rubeola
B. Rubella
C. Roseola
D. Kawasaki
C
359
A 5 year-old male presents with fever and rashes. 6 days prior,
he was noted to have a fever of 37.9°C, dry cough, runny nose
and redness of the eye with tearing. Then 3 days prior, the
mother noted flat red spots starting on the hairline, behind the
ears and upper neck spreading to the entire face, neck, upper
arms and chest. Today, the rash spread over the back, abdomen,
and thighs which prompted them to seek consult. PE: HR 110,
RR 32 T> 39.5°C, generalized maculopapular rash with areas of
confluence. What is the most likely diagnosis?
A. Rubeola
B. Rubella
C. Roseola
D. Kawasaki
A
