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1. Two weeks after a viral syndrome, a 9-year-old boy presents to your clinic
with a complaint of several days of weakness of his mouth. In addition to the
drooping of the left side of his mouth, you note that he is unable to completely
shut his left eye. His smile is asymmetric, but his examination is otherwise
normal. Which of the following is the most likely diagnosis?
e. Bell palsy
2. An infant can regard his parent's face, follow to the midline, lift his head from
the examining table, smile spontaneously, and respond to a bell. He does not yet
regard his own hand, follow past the midline, nor lift his head to a 45° angle off
the examining table. Which of the following is the most likely age of the infant?
a. 1 month
3. A child is brought to your clinic for a routine examination. She can put on
a T-shirt but requires a bit of help dressing otherwise. She can copy a circle well
but has difficulty in copying a square. Her speech is understandable and she
knows four colors. She balances proudly on each foot for 2 seconds but is
unable to hold the stance for 5 seconds. Which of the following is the most
likely age of this child?
d. 4 years
4. A 4-year-old girl is noticed by her grandmother to have a limp and a somewhat
swollen left knee. The parents report that the patient occasionally complains
of pain in that knee. An ophthalmologic examination reveals findings
as depicted in the photograph. Which of the following conditions is most likely
to be associated with these findings?
a. Juvenile rheumatoid arthritis
5. A previously healthy 4-year-old child pictured below presents to the emergency
room (ER) with a 2-day history of a brightly erythematous rash and
temperature of 40°C (104°F). The exquisitely tender, generalized rash is
worse in the flexural and perioral areas. The child is admitted and over the
next day develops crusting and fissuring around the eyes, mouth, and nose.
The desquamation of skin shown in the photograph occurs with gentle traction.
Which of the following is the most likely diagnosis?
b. Staphylococcal scalded skin syndrome
6. A mother brings to your office an article from the Internet suggesting
that infants in day care have a statistically higher incidence of upper respiratory
infections (p < 0.05) as compared to children not in day care. You explain to
her that this means which of the following?
d. The odds are less than 1 in 20 that the differences in upper respiratory infection
rates observed were only a chance variation.
7. A patient comes to your office for a hospital follow-up. You had sent him
to the hospital 3 weeks earlier for persistent fevers but no other symptoms;
he was diagnosed with endocarditis and is currently being treated appropriately.
Advice to this family should now include which of the following?
c. Provide the child with antibiotic prophylaxis for dental procedures.
8. A mother calls you on the telephone and says that your 4-year-old son
bit the hand of her 2-year-old son 48 hours previously. The area around the
injury has become red, indurated, and swollen, and he has a temperature of
39.4°C (103°F). Which of the following is the most appropriate response?
b. Admit the child to the hospital immediately for surgical debridement and antibiotic
9. The adolescent shown presents with a 14-day history of multiple oval
lesions over her back. The rash began with a single lesion over the lower
abdomen (A); the other lesions developed over the next days (B). These lesions
are slightly pruritic. Which of the following is the most likely diagnosis?
b. Pityriasis rosea
10. A chubby 6-month-old baby boy is brought to the clinic by his father.
His father is concerned that his penis is too small (see photograph). The child
is at the 95% for weight and the 50% for length; he has been developing
normally and has had no medical problems. Which of the following is the
most appropriate first step in management of this child?
b. Evaluation of penile length after retracting the skin and fat lateral to the penile
11. A previously healthy 5-year-old boy has a 1-day history of low-grade
fever, colicky abdominal pain, and a rash. He is well-appearing and alert. His
vital signs, other than a temperature of 38°C (100.5°F) are completely normal.
A diffuse, erythematous, maculopapular, and petechial rash is present on his
buttocks and lower extremities, as shown in the photograph. He has no localized
abdominal tenderness or rebound; bowel sounds are active. Laboratory data
Urinalysis: 30 red blood cells (RBCs) per high-powered field,
Stool: Guaiac positive
Platelet count: 135,000/μL
These findings are most consistent with which of the following?
a. Anaphylactoid purpura
12. A 4-month-old baby boy arrives to the ER cold and stiff. The parents
report that he had been healthy and that they put him to bed as usual for the
night at the regular time. When they next saw him, in the morning, he was
dead. Physical examination is uninformative. A film from a routine skeletal
survey is shown below. Which of the following is the most likely diagnosis?
13. A 6-year-old boy is often teased at school because he has stooled in his
underwear almost daily for the last 3 months. He was toilet trained at 2 years
of age without difficulty, but over the last 2 years he had developed ongoing
constipation. His family is frustrated because they cannot believe him when
he says "I didn't know I had to go." He is otherwise normal; school is going
well, and his home life is stable. His only finding on examination is significant
for stool in the rectal vault. The plain radiograph of his abdomen is shown.
Initial management of this problem should include which of the following?
d. Clear fecal impaction and short-term stool softener use
14. A 2-year-old child presents to the office with a paternal complaint of
"bowlegs." The girl has always had bowlegs; her previous pediatrician told the
family she would grow out of it. Now, however, it seems to be worsening. Her
weight is greater than 95% for age, and she has significant bowing out of her legs
and internal tibial torsion; otherwise, her examination is normal. A radiograph
of her lower leg is shown. Which of the following is the most likely diagnosis?
e. Blount disease
15. A very concerned mother brings a 2-year-old child to your office
because of two episodes of a brief, shrill cry followed by a prolonged expiration
and apnea. You have been following this child in your practice since
birth and know the child to be a product of a normal pregnancy and delivery,
to be growing and developing normally, and to have no chronic medical
problems. The first episode occurred immediately after the mother refused to
give the child some juice; the child became cyanotic, unconscious, and had
generalized clonic jerks. A few moments later the child awakened and had no
residual effects. The most recent episode (identical in nature) occurred at the
grocery store when the child's father refused to purchase a toy for her. Your
physical examination reveals a delightful child without unexpected physical
examination findings. Which of the following is the most likely diagnosis?
e. Breath-holding spell
16. A 10-year-old child arrives with the complaint of new-onset bed-wetting.
He has had no fever, his urine culture is negative, and he has had no new
stresses in his life. He is well above the 95th percentile for weight as is much
of his family. Which of the following is most helpful in making a diagnosis?
a. Fasting plasma glucose of 135 mg/dL
17. You are called to the ER to see one of your patients. The father of
this 14-year-old mildly retarded child says that he found the child about
20 minutes ago in the neighbor's garden shed with an unknown substance
in his mouth. The child first had a headache, but then became agitated and
confused; while you are talking to the father in the ER the child begins to
have a seizure and dysrhythmia on the cardiac monitor. The blood gas
demonstrates a severe metabolic acidosis. Which of the following agents is
most likely the culprit?
c. Sodium cyanide
18. The mother of a 3-day-old infant brings her child to your office for an
early follow-up visit. The mom notes that the child has been eating well,
has had no temperature instability, and stools and urinates well. She notes
that over the previous 3 days the child has had a progressive "rash" on the
face as pictured here. Which of the following is the most likely diagnosis?
b. Neonatal acne
19. A 2-year-old child (A) presents with a 4-day history of a rash limited
to the feet and ankles. The papular rash is both pruritic and erythematous.
The 3-month-old sibling of this patient (B) has similar lesions also involving
the head and neck. The most appropriate treatment for this condition includes
which of the following?
20. An 8-hour-old infant develops increased respiratory distress, hypothermia,
and hypotension. A complete blood count (CBC) demonstrates a white blood
cell (WBC) count of 2500/μL with 80% bands. The chest radiograph is shown
below. Which of the following is the most likely diagnosis?
c. Group B streptococcal pneumonia
21. A 16-year-old arrives to your office soon after beginning basketball season.
He states that he has had progressive pain in his knees. A physical examination
reveals, in addition to tenderness, a swollen and prominent tibial
tubercle. Radiographs of the area are unremarkable. Which of the following
is the most likely diagnosis?
a. Osgood-Schlatter disease
22. You are performing a well-child examination on the 1-year-old child
shown in the picture. For this particular problem, which of the following is
the most appropriate next step in management?
e. Refer immediately to ophthalmology
23. You are seeing a 2-year-old child, brought by his father for a well-child
examination. In providing age-appropriate anticipatory guidance, you should
tell him which of the following?
b. Milk should be switched from whole to skim or low fat.
24. A child can walk well holding on to furniture but is slightly wobbly
when walking alone. She uses a neat pincer grasp to pick up a pellet, and
she can release a cube into a cup after it has been demonstrated to her. She
tries to build a tower of two cubes with variable success. She is most likely
at which of the following age?
e. 1 year
25. You are called by a general practitioner to consult on a patient admitted
to the hospital 4 days ago. The patient is a 7-month-old white boy with
poor weight gain for the past 3 months, who has not gained weight in the
hospital despite seemingly adequate nutrition. You take a detailed diet history
from his foster mother, and the amounts of formula and baby food
intake seem appropriate for age. Physical examination reveals an active,
alert infant with a strong suck reflex who appears wasted. You note generalized
lymphadenopathy with hepatomegaly. In addition, you find a severe
case of oral candidiasis that apparently has been resistant to treatment.
Which of the following is the most appropriate next step in the evaluation
or treatment of this child?
b. Order human immunodeficiency virus (HIV) polymerase chain reaction (PCR).
testing because this is likely the presentation of congenitally acquired HIV.
26. A 5-year-old boy presents with the severe rash shown in the photographs.
The rash is pruritic, and it is especially intense in the flexural
areas. The mother reports that the symptoms began in infancy (when it also
involved the face) and that her 6-month-old child has similar symptoms.
Which of the following is the most appropriate treatment of this condition?
d. Moisturizers and topical steroids
27. A 1-year-old presents for a well-child checkup, but the parents are
concerned about giving the child his immunizations. Which of the following
is a true contraindication to the administration of the fourth DTaP (diphtheria
and tetanus toxoid and acellular pertussis) vaccine?
e. Prolonged seizures 6 days after the last DTaP vaccine
28. A mother arrives to the clinic with her three children (ages 2 months,
18 months, and 36 months). The 18-month-old has an intensely pruritic
scalp, especially in the occipital region, with 0.5-mm lesions noted at the
base of hair shafts, as shown in the picture. Which of the following therapies
should be avoided in this situation?
a. Treatment of all household contacts with 1% lindane (Kwell)
29. A 2-year-old boy has been vomiting intermittently for 3 weeks and has
been irritable, listless, and anorectic. His use of language has regressed to speaking
single words. In your evaluation of this patient, which of the following
is the most reasonable diagnosis to consider?
c. Tuberculous meningitis
30. You find a discrete, whitish polyp that extends through the tympanic
membrane in a child with a history of recurrent otitis media. This most likely
represents which of the following?
a. A cholesteatoma
31. An 8-month-old infant arrives to the emergency department (ED) with
a 2-day history of diarrhea and poor fluid intake. Your quick examination
reveals a lethargic child; his heart rate is 180 beats per minute, his respiratory
rate is 30 breaths per minute, and his blood pressure is low for age. He has
poor skin turgor, 5-second capillary refill, and cool extremities. Which of the
following fluids is most appropriate management for his condition?
c. Normal saline
32. During the examination of a 2-month-old infant, you note that the infant's
umbilical cord is still firmly attached. This finding prompts you to suspect
which of the following?
b. Leukocyte adhesion deficiency
33. You are seeing an established patient, a 4-year-old girl brought in by
her mother for vaginal itching and irritation. She is toilet trained and has not
complained of frequency or urgency, nor has she noted any blood in her urine.
Her mother noted she has been afebrile and has not complained of abdominal
pain. Mom denies the risk of inappropriate contact; the girl also denies anyone
"touching her there." Your physical examination of the perineum is significant
for the lack of foul odor or discharge. You do note some erythema of the
vulvar area but no evidence of trauma. Which of the following is the most
appropriate course of action?
b. Counsel mother to stop giving the girl bubble baths, have the girl wear only cotton
underwear, and improve hygiene.
34. A 20-month-old child is brought to the ED because of fever and irritability
and refusal to move his right lower extremity. Physical examination
reveals a swollen and tender right knee that resists passive motion. Which
of the following is the most likely to yield the diagnosis in this patient?
a. Examination of joint fluid
35. A 14-year-old high school student arrives to your clinic for well-child
care. In reviewing his records you determine that his most recent immunization
for tetanus was at 4 years of age. Which of the following should you
e. Tetanus toxoid, reduced diphtheria toxoid, and acellular pertussis vaccine adsorbed
36. A 5-year-old boy is brought into the ER immediately after an unfortunate
altercation with a neighbor's immunized Chihuahua that occurred while
the child was attempting to dress the dog as a superhero. The fully immunized
child has a small, irregular, superficial laceration on his right forearm
that has stopped bleeding. His neuromuscular examination is completely
normal, and his perfusion is intact. Management should include which of
c. Copious irrigation
37. Aunt Mary is helping her family move to a new apartment. During the
confusion, 3-year-old Jimmy is noted to become lethargic. The contents of
Aunt Mary's purse are strewn about on the floor. In the ER, the lethargic
Jimmy is found to have miosis, bradycardia, and hypotension. He develops
apnea, respiratory depression, and has to be intubated. His condition would
most likely benefit from which of the following therapies?
b. Pediatric intensive care unit (PICU) support and trial of naloxone
38. As a city public health officer, you have been charged with the task of
screening high-risk children for lead poisoning. Which of the following is the
best screen for this purpose?
d. Blood lead level
39. A 15-year-old is participating in high school football practice in August
in Texas. He had complained of headache and nausea earlier in practice,
but kept playing after a cup of water. He is now confused and combative.
He is dizzy and sweating profusely. His temperature is 41°C (105.8°F).
Therapy should consist of which of the following?
d. Initiate whole body cold water immersion
40. As part of your anticipatory guidance to new parents of a healthy newborn,
you suggest putting the child in which of the following positions for
a. Supine position
41. A mentally retarded 14-year-old boy has a long face, large ears, micropenis,
and large testes. Chromosome analysis is likely to demonstrate which of the
d. Fragile X syndrome
42. A 5-month-old child with poor growth presents to the ER with generalized
tonic-clonic seizure activity of about 30-minute duration that stops
upon the administration of lorazepam. Which of the following historical bits
of information gathered from the mother is most likely to lead to the correct
diagnosis in this patient?
c. The mother has been diluting the infant's formula to make it last longer
Questions 43 to 48
43. A 1-week-old child's mother complains that the child has a transient
rash that has splotchy areas of erythema with a central clear pustule. Your
microscopic examination of the liquid in the pustule reveals eosinophils.
e. Erythema toxicum
44. An adolescent boy complains of a splotchy red rash on the nape of his
neck, discovered when he had his head shaved for football season. The rash
seems to become more prominent with exercise or emotion. His mother notes
that he has had the rash since infancy, but that it became invisible as hair grew.
He had a similar rash on his eyelids that resolved in the newborn period.
b. Salmon patch
45. A nurse calls you to evaluate an African American newborn whom she
thinks has a bacterial skin infection. The areas in question have many scattered
pustules full of a milky fluid. Upon examining pustules, they easily wipe
away, revealing a small hyperpigmented macule.
d. Pustular melanosis
46. The obstetrical resident on call asks you to evaluate an area of a newborn's
scalp that seems to have no hair and is scaly and yellowish
a. Sebaceous nevus
47. A newborn's mother complains that her infant seems to have very small
white dots all over his nose. The dots do not wipe off with bathing, but they
are also not erythematous
48. A newborn's father complains that his son has dandruff, with many waxy
flakes of skin on the scalp. When he scrapes the lesions, hair often comes
off with the flakes of skin. In addition, the baby has flaking of the eyebrows.
f. Seborrheic dermatitis
Questions 49 to 53
49. A 3-year-old boy awakens every night around 2:00 AM screaming incoherently.
His parents note that he is agitated, seems awake but unresponsive, and
goes back to sleep within a few minutes. He has no memory of the episodes
in the morning
a. Night terrors
50. A 15-month-old toddler continues to wake up crying every night. Her
parents give her a nighttime bottle, rock her, and sing to her to help her go
back to sleep. Her parents are exhausted and ask you if she is having bad
c. Learned behavior
51. Parents hear over their baby monitor that their 5-year-old girl regularly
calls out during the night. When the parents check on her, she is sleeping
comfortably and is in no apparent distress.
52. A 4-year-old boy occasionally wakes in the middle of the night crying.
When his parents check on him, he seems visibly frightened and tells his
parents that Chihuahuas were chasing him.
53. A 5-year-old child refuses to sleep in his bed, claiming there are monsters
in his closet and that he has bad dreams. The parents allow him to sleep
with them in their bed to avoid the otherwise inevitable screaming fit. The
parents note that the child sleeps soundly, waking only at sunrise.
c. Learned behavior
Questions 54 to 58
54. A 6-year-old returns from playing all day in the snow with several erythematous,
ulcerative lesions on his fingertips; he complains the lesions are
painful and itchy.
55. A teen, just back from a skiing trip, has blistering and peeling of several
areas on her face; she reports the lesions started as firm, cold, white areas that
felt stinging at the time and are now more sensitive than the surrounding skin.
56. A 9-year-old girl presents during summer break with an area of erythematous,
firm, and slightly swollen skin at the corner of her mouth and
extending to her cheek. The area is not tender.
d. Cold panniculitis
57. A 14-year-old on a mountain-climbing expedition in December becomes
tired, clumsy, and begins to hallucinate. His heart rate is 45 beats per minute
58. A skier recently rescued from a snowbank following an avalanche (caused
by a barking Chihuahua) complains about his feet. Upon rescue they were
whitish yellow and numb, but now they are blotchy and painful.
Questions 59 to 62
59. Megaloblastic anemia, growth failure, paresthesias, sensory defects, developmental
regression, weakness, and fatigue
f. Vitamin B12 deficiency
60. Photophobia, blurred vision, burning and itching of eyes, poor growth,
i. Riboflavin deficiency
Questions 63 to 66
63. A newborn infant is noted to have microcephaly with sloping forehead,
cutis aplasia on the scalp, microphthalmia, and cleft lip and palate. His echocardiogram
demonstrates a complex heart lesion including atrial septal defect
(ASD), ventricular septal defect (VSD), and dextrocardia.
a. Trisomy 13
64. A 17-year-old boy has an unusual gait, large mouth with tongue protrusion,
hypopigmentation with blond hair and pale blue eyes, and unprovoked
bursts of laughter
c. Angelman syndrome
65. A 6-week-old boy was small for his birth weight and had intrauterine
growth retardation. He is microcephalic, has a rounded face, hypertelorism,
and epicanthal folds. His cry is high-pitched
b. Cri du chat syndrome
66. A 3-day-old infant who was found at birth to have anal atresia also has
vertebral defects, a VSD, tracheoesophageal fistula, absent left kidney, and
Questions 67 to 70
67. An afebrile, obese 14-year-old boy has developed pain at the right
knee and a limp.
b. Slipped capital femoral epiphysis...
68. A 6-year-old boy has developed a limp and has limited mobility of the
hip, but denies pain and fever.
a. Legg-Calvé-Perthes disease
69. A 2-year-old refuses to walk, has fever, has significant pain with external
rotation of the right leg, and has an elevated WBC count.
d. Septic arthritis of the hip
70. A 3-year-old refuses to walk, is afebrile, had an upper respiratory tract
infection a week ago, has right hip pain with movement, and has a normal
e. Transient synovitis
Questions 71 to 75
71. Over the past several weeks, a 2-year-old girl has exhibited developmental
regression, abnormal sleep patterns, anorexia, irritability, and decreased activity.
These symptoms have progressed to acute encephalopathy with vomiting,
ataxia, and variable consciousness. The family recently moved, and they are
in the process of restoring the interior of their home.
c. Dimercaptosuccinic acid (DMSA, succimer)
72. After a fight with her boyfriend, a 16-year-old girl took "some pills." At
presentation she is alert and complains of emesis, diaphoresis, and malaise.
Her initial liver function tests, obtained about 12-hour postingestion, are
elevated. Repeat levels at 24-hours show markedly elevated aspartate aminotransferase
(AST) and alanine aminotransferase (ALT), along with abnormal
coagulation studies and an elevated bilirubin.
b. N-acetylcysteine (Mucomyst)
73. You are called to the delivery room. A newborn infant seems lethargic
and has poor tone with only marginal respiratory effort, but his heart rate
is above 100 beats per minute. The mother had an uncomplicated pregnancy,
and delivery was uncomplicated and vaginal 10 minutes after spontaneous
rupture of membranes. The mother received only pain medications while
d. Naloxone (Narcan)
74. A 4-year-old girl comes into the ER after eating a bottleful of small,
chewable pills she found while at her grandfather's house. She has an increased
respiratory rate, elevated temperature, vomiting, and is disoriented. She is
intermittently complaining that "a bell is ringing" in her ears. She has a metabolic
acidosis on an arterial blood gas.
e. Sodium bicarbonate
75. After helping his father in the yard, a 14-year-old boy complains of weakness
and feels like his muscles are twitching. He begins to drool, and then
collapses in a generalized tonic-clonic seizure. Upon the arrival of EMS, his
heart rate is found to be 40 beats per min and his pupils are pinpoint
a. Atropine and pralidoxime (2-PAM)
Questions 76 to 78
76. A 14-year-old male presents after taking a "happy pill" that his friend
gave him. He is alert and oriented, but complains of a muscle spasm in his
neck, making his head lean on his right shoulder. You also notice he is arching
his back in an unusual manner.
b. Diphenhydramine (Benadryl)
77. A 2-year-old boy found a bottle of his mother's prenatal vitamins and
consumed the majority of them. He now has hematemesis and abdominal
pain. He is febrile, and laboratory tests reveal a leukocytosis and hyperglycemia
a. Deferoxamine mesylate
78. A 17-year-old is brought into the ED by his friends at about 10:00 AM.
They were at a party the night before and drank some "homemade" alcohol.
He is disoriented and confused, and has an anion-gap acidosis. He begins
to have seizures
Questions 79 to 83
Excess vitamin intake has been shown to have deleterious effects. Match the
vitamin with the toxic effect. Each lettered option may be used once, more
than once, or not at all..
79. Vitamin A
d. An irritable 8-year-old child with headache, vomiting, alopecia, dry/itchy skin with
desquamation of the palms and soles, hepatosplenomegaly, and swelling of the bones.
80. Nicotinic acid
g. After getting into his mother's bottle of vitamins, a 3-year-old has burning, tingling,
and itching on his arms as well as a reddened face, arms, and chest.
81. Vitamin C
a. A 4-year-old with diarrhea, abdominal pain, and kidney stones which prove to
be caused by calcium oxalate.
82. Vitamin D
e. A 2-year-old presents with nausea, vomiting, poor feeding, abdominal pain, and
constipation. On electrocardiogram (ECG) he has decreased Q-T interval. He has
calcifications in his kidneys noted on a CT scan done for his abdominal pain
Questions 84 to 88
84. A 15-year-old with severe acne on an oral preparation of retinoic acid.
c. Bilateral microtia or anotia
85. A woman without prenatal care has a diet low in green vegetables and
enriched grain products.
d. Spina bifida
86. A woman with long-standing hypertension treated with angiotensinconverting
enzyme (ACE) inhibitors
f. Renal dysgenesis
88. A 23-year-old pregnant woman with manic-depressive disorder has
had poor prenatal care and was maintained on lithium.
e. Ebstein anomaly
Questions 89 to 92
89. An 18-year-old friend of the family returns from spring break from a
coastal town in Central America. He has an intensely pruritic lesion on his
foot. The lesion is raised, red, serpiginous, and has a few associated bullae.
90. A 2-week-old boy is brought by his mother to the clinic; he has scaly,
yellow patches on his scalp with associated hair loss.
f. Topical steroids or a selenium sulfide-containing product
91. Two days after a backyard party where the children enjoyed limeade and
the adults partook of margaritas, a father brings his 4-year-old child to your
office for a well-child checkup. The child is healthy other than a slight sunburn
and some hyperpigmentation around her face and on her chest. Her father
mentions that he, too, has some splotchy hyperpigmentation on his chest
d. Reassurance only
92. A 4-month-old presents with a dry, scaly rash on his cheeks, arms, and
upper chest. His 10-year-old sister had a similar rash when she was young,
but the rash is now confined to her antecubital and popliteal fossa; her rash
worsens in winter months
a. Mild cleansing cream, topical moisturizers, and topical steroids
Questions 93 to 95
93. A 6-month-old child has a loud systolic murmur at the left lower sternal
d. Ventricular septal defect (VSD)
94. A 14-year-old child has headache, hypertension, edema, and a change
in urine output and color
95. A 3-day-old infant was born to a mother with active systemic lupus
c. Vitamin A
Questions 103 to 107
103. A 1-year-old boy has been admitted three times in the past with
abscess formation requiring incision and drainage. He is now admitted for
surgical drainage of a hepatic abscess identified on ultrasound
c. Nitroblue tetrazolium (NBT) test
104. A 5-month-old infant is admitted with severe varicella infection. The
lesions cover the infant's entire body, and the infant is beginning to show
symptoms of respiratory distress. Past medical history is significant for a
history of atopic dermatitis. The family also notes frequent epistaxis; the
last episode required nasal packing in the ED.
f. Platelet count
105. A 3-year-old has had repeated episodes of sinusitis and otitis media.
He was recently admitted for osteomyelitis of his femur with Staphylococcus
aureus. The family notes that while his first 4 or 5 months of life were normal,
he has been persistently ill with multiple infections in the ensuing months.
The mother notes that her brother had similar problems with infections
and died at the age of 3 years from a "lung infection." Physical examination
is significant for the absence of lymph nodes and tonsillar tissue.
b. Serum immunoglobulin levels
106. A general practitioner refers to you for evaluation a 3-year-old boy
with frequent infections. You note the child to have a loud systolic murmur,
posteriorly rotated ears that are small and low-set, down-slanting and widely
spaced eyes, a small jaw, and an upturned nose. At birth the child spent
2 weeks in the nursery for "low calcium" and seizures, and he still receives
calcium supplementation, but the mother does not know why. You would
like to order a rapid diagnostic test for this child.
g. Intradermal skin test using Candida albicans
107. A 2-year-old girl has had two episodes of Neisseria meningitidis septicemia
and is now admitted for Streptococcus pneumoniae septicemia
e. CBC demonstrating Howell-Jolly bodies
108. The term infant pictured below weighs 2200 g (4 lb, 14 oz). He is
found to have a ventricular septal defect on cardiac evaluation. This infant
appears to have features consistent with which of the following?
d. Fetal alcohol syndrome
109. A newborn is noted to be quite jaundiced at 3 days of life. Laboratory
data demonstrate his total bilirubin to be 17.8 mg/dL (direct bilirubin is
0.3 mg/dL). Which of the following factors is associated with an increased
risk of neurologic damage in a jaundiced newborn?
d. Neonatal sepsis
110. A 2-hour-old full-term newborn infant is noted by the nursing staff to
be having episodes of cyanosis and apnea. Per nursery protocol they place an
oxygen saturation monitor on him. When they attempted to feed him, his
oxygen levels drop into the 60s. When he is stimulated and cries, his oxygen
levels increase into the 90s. Which of the following is the most important
next step to quickly establish the diagnosis?
c. Passage of catheter into nose
111. A mother calls you frantic because she has just been diagnosed with
chicken pox. She delivered 7 days ago a term infant that appears to be eating,
stooling, and urinating well. The child has been afebrile and seems to be doing
well. Which of the following is the most appropriate step in management?
e. Advise the mother to continue regular well-baby care for the infant.
112. A mother wishes to breast-feed her newborn infant, but is worried
about medical conditions that would prohibit her from doing so. You counsel
her that of her listed conditions, which of the following is a contraindication
e. HIV infection
113. A mother delivers a neonate with meconium staining and Apgar
scores of 3 at 1 and 5 minutes of life. She had no prenatal care and the delivery
was by emergency cesarean section for what the obstetricians report as
"severe fetal bradycardia." Which of the following sequelae could be expected
to develop in this intubated neonate with respiratory distress?
a. Sustained rise in pulmonary arterial pressure
114. A 2-year-old boy is being followed for congenital cytomegalovirus
(CMV) infection. He is deaf and developmentally delayed. The child's mother
informs you that she has just become pregnant and is concerned that the new
baby will be infected and may develop serious consequences. Which of the
following is true?
a. The mother has antibodies to CMV that are passed to the fetus
115. A full-term infant is born after a normal pregnancy; delivery, however,
is complicated by marginal placental separation. At 12 hours of age, the child,
although appearing to be in good health, passes a bloody meconium stool.
For determining the cause of the bleeding, which of the following diagnostic
procedures should be performed first?
b. An Apt test
116. As you are about to step out of a newly delivered mother's room, she
mentions that she wants to breast-feed her healthy infant, but that her
obstetrician was concerned about one of the medicines she was taking.
Which of the woman's medicines, listed below, is clearly contraindicated in
c. Amphetamines for her attention deficit disorder
117. A recovering premature infant who weighs 950 g (2 lb, 1 oz) is fed
breast milk to provide 120 cal/kg/d. Over the ensuing weeks, the baby is
most apt to develop which of the following?
118. A primiparous woman whose blood type is O positive gives birth at
term to an infant who has A-positive blood and a hematocrit of 55%. A total
serum bilirubin level obtained at 36 hours of age is 12 mg/dL. Which of the
following additional laboratory findings would be characteristic of ABO
hemolytic disease in this infant?
b. A positive direct Coombs test
119. The nurse from the level 2 neonatal intensive care nursery calls you to
evaluate a baby. The infant, born at 32 weeks' gestation, is now 1 week old and
had been doing well on increasing nasogastric feedings. This afternoon, however,
the nurse noted that the infant has vomited the last two feedings and
seems less active. Your examination reveals a tense and distended abdomen
with decreased bowel sounds. As you are evaluating the child, he has a grossly
bloody stool. The plain film of his abdomen is shown. The next step in your
management of this infant should include which of the following?
e. Removal of nasogastric tube, placement of a transpyloric tube and, after confirmation
via radiograph of tube positioning, switching feeds from nasogastric to
120. An infant weighing 1400 g (3 lb) is born at 32 weeks' gestation.
Initial evaluation was benign, and the infant was transferred to the level 2
nursery for prematurity. The nurse there calls at 1 hour of life and reports
the infant is tachypneic. Vital signs include a heart rate of 140 beats per
minute, a respiratory rate of 80 breaths per minute, a temperature of 35°C
(95°F), and a peripheral oxygen saturation of 98%. The lungs are clear
with bilateral breath sounds and there is no murmur; the infant is in no distress.
The child's chest radiograph is shown. Which of the following is the
most appropriate next step in evaluating the infant?
d. Place the infant under a warmer.
121. Two new mothers are discussing their infants outside the neonatal intensive
care unit. Both were born at 36 weeks' gestation. One infant weighs 2600 g
(5 lb, 12 oz) while the other infant weighs 1600 g (3 lb, 8 oz). The mother of
the second infant should be told that her child is more likely to have which of
the following conditions?
a. Congenital malformations
122. A 3-day-old infant, born at 32 weeks' gestation and weighing 1700 g
(3 lb, 12 oz), has three episodes of apnea, each lasting 20 to 25 seconds and
occurring after a feeding. During these episodes, the heart rate drops from
140 to 100 beats per minute, and the child remains motionless; between
episodes, however, the child displays normal activity. Blood sugar is 50 mg/dL
and serum calcium is normal. Which of the following is most likely true
regarding the child's apneic periods?
a. They are due to an immature respiratory center
123. You have an 11-day-old term infant in your office for a well-child
visit. The mother notes that she received a letter that day from the state's
Department of Health reporting that her child's newborn screen had come
back abnormal, indicating possible galactosemia. Which of the following is
the most appropriate management at this point?
d. Discontinue breast-feeding and initiate soy formula feedings.
124. The father of a 1-week-old infant comes to the office in a panic. He
has just noticed on his child a right anterior shoulder mass that seems tender.
The father is an osteosarcoma survivor and fears the child has the same
malignancy. In reviewing the baby's discharge papers, you note the child
was a term, appropriate-for-gestational-age vaginal delivery with a birth
weight of 3200 g (7 lb, 1 oz). Apgar scores were 9 at 1 and 5 minutes. Your
examination is significant for a large firm mass on the right clavicle; the rest
of the examination is normal. Management of this problem should include
which of the following?
b. Reassurance and supportive care
125. A 1-day-old infant who was born by a difficult forceps delivery is alert
and active. She does not move her left arm spontaneously or during a Moro
reflex. Rather, she prefers to maintain it internally rotated by her side with
the forearm extended and pronated. The rest of her physical examination is
normal. This clinical scenario most likely indicates which of the following?
c. Left-sided Erb-Duchenne paralysis
126. You are examining a newborn infant in the well-baby nursery. The
infant was the product of a benign pregnancy and vaginal delivery; he
appears to be in no distress. Interestingly, your measurement of frontooccipital
head circumference is about 2 cm larger than the initial measurement
done several hours before. Your examination otherwise is significant
for tachycardia and a "squishy" feel to the entire scalp. You can elicit a fluid
wave over the scalp. Management of this condition should include which
of the following?
a. Transfer to the newborn ICU
127. A 19-year-old primiparous woman develops toxemia in her last trimester
of pregnancy and during the course of her labor is treated with magnesium
sulfate. At 38 weeks' gestation, she delivers a 2100-g (4-lb, 10-oz) infant with
Apgar scores of 1 at 1 minute and 5 at 5 minutes. Laboratory studies at
18 hours of age reveal a hematocrit of 79%, platelet count of 100,000/μL,
glucose 41 mg/dL, magnesium 2.5 mEq/L, and calcium 8.7 mg/dL. Soon
after, the infant has a generalized convulsion. Which of the following is the
most likely cause of the infant's seizure?
128. An infant who appears to be of normal size is noted to be lethargic
and somewhat limp after birth. The mother is 28 years old, and this is her
fourth delivery. The pregnancy was uncomplicated, with normal fetal monitoring
prior to delivery. Labor was rapid, with local anesthesia and intravenous
meperidine (Demerol) administered for maternal pain control. Which
of the following therapeutic maneuvers is likely to improve this infant's
condition most rapidly?
b. Administration of naloxone (Narcan)
129. At 43 weeks' gestation, a long, thin infant is delivered. The infant is
apneic, limp, pale, and covered with "pea soup" amniotic fluid. Which of the
following is the best first step in the resuscitation of this infant at delivery?
a. Intubation and suction of the trachea; provision of oxygen
130. The newborn pictured below was born at home and has puffy, tense eyelids;
red conjunctivae; a copious amount of purulent ocular discharge; and
chemosis 2 days after birth. Which of the following is the most likely diagnosis?
d. Gonococcal ophthalmia
131. After an uneventful labor and delivery, an infant is born at 32 weeks'
gestation weighing 1500 g (3 lb, 5 oz). Respiratory difficulty develops
immediately after birth and increases in intensity thereafter. At 6 hours of
age, the child's respiratory rate is 60 breaths per minute. Examination reveals
grunting, intercostal retraction, nasal flaring, and marked cyanosis in room
air. Auscultation reveals poor air movement. Physiologic abnormalities compatible
with these data include which of the following?
b. Decreased lung compliance, reduced lung volume, right-to-left shunt of blood
132. A term infant is born to a known HIV-positive mother. She has been
taking antiretroviral medications for the weeks prior to the delivery of her
infant. Routine management of the healthy infant should include which of the
c. A course of zidovudine for the infant
133. Initial examination of a full-term infant weighing less than 2500 g (5 lb,
8 oz) shows edema over the dorsum of her hands and feet. Which of the following
findings would support a diagnosis of Turner syndrome?
c. Redundant skin folds at the nape of the neck
134. You have been recently named as the medical director of the normal
newborn nursery in your community hospital and have been asked to
write standardized admission orders for all pediatricians to follow. Which
of the following vaccines will you include on these orders?
b. Hepatitis B vaccine
135. A 1-week-old black infant presents to you for the first time with a
large, fairly well-defined, purple lesion over the buttocks bilaterally, as shown
in the photograph. The lesion is not palpable, and it is not warm nor tender.
The mother denies trauma and reports that the lesion has been present since
birth. This otherwise well-appearing infant is growing and developing normally
and appears normal upon physical examination. Which of the following
is the most appropriate course of action in this infant?
b. Reassurance of the normalcy of the condition
136. A newborn infant develops respiratory distress immediately after
birth. His abdomen is scaphoid. No breath sounds are heard on the left side
of his chest, but they are audible on the right. Immediate intubation is successful
with little or no improvement in clinical status. Emergency chest
x-ray is shown (A) along with an x-ray 2 hours later (B). Which of the following
is the most likely explanation for this infant's condition?
c. Diaphragmatic hernia
137. Shortly after birth, an infant develops abdominal distention and begins
to drool. When she is given her first feeding, it runs out the side of her mouth,
and she coughs and chokes. Physical examination reveals tachypnea, intercostal
retractions, and bilateral pulmonary rales. The esophageal anomaly that
most commonly causes these signs and symptoms is illustrated by which of the
following? (ישנו ציור שאני לא יכולתי להעתיק***)
d. Figure D
138. You are advised by the obstetrician that the mother of a baby she has
delivered is a carrier of hepatitis B surface antigen (HBsAg-positive). Which
of the following is the most appropriate action in managing this infant?
d. Administer hepatitis B immune globulin and hepatitis B vaccine to the infant.
139. You are called to a delivery of a term infant, about to be born via
cesarean section to a mother with multiple medical problems, including a
1-month history of a seizure disorder, for which she takes phenytoin;
rheumatic heart disease, for which she must take penicillin daily for life;
hypertension, for which she takes propranolol; acid reflux, for which she
takes aluminum hydroxide; and a deep venous thrombosis in her left calf
diagnosed 2 days ago, for which she was started on a heparin infusion. The
obstetrician is concerned about the possible effects of the mother's multiple
medications on the newborn infant. Which of the following medications is
most likely to cause harm in this newborn infant at delivery?
140. Your older sister, her husband, their 2-day-old infant, and their pet
Chihuahua arrive at your door. The parents of the child are concerned because
the pediatrician noted the child was "yellow" and ordered some studies. They
produce a wad of papers for you to review. Both the mother and baby have
O-positive blood. The baby's direct serum bilirubin is 0.2 mg/dL, with a total
serum bilirubin of 11.8 mg/dL. Urine bilirubin is positive. The infant's white
blood cell count is 13,000/μL with a differential of 50% polymorphonuclear
cells, 45% lymphocytes, and 5% monocytes. The hemoglobin is 17 g/dL, and
the platelet count is 278,000/μL. Reticulocyte count is 1.5%. The peripheral
smear does not show fragments or abnormal cell shapes. Which of the following
is the most likely explanation for this infant's skin color?
b. Physiologic jaundice
141. At the time of delivery, a woman is noted to have a large volume of
amniotic fluid. At 6 hours of age, her baby begins regurgitating small
amounts of mucus and bile-stained fluid. Physical examination of the infant
is normal, and an abdominal x-ray is obtained (see below). Which of the
following is the most likely diagnosis of this infant's disorder?
d. Duodenal atresia
142. The mother and father of a newborn come in for the 2-week
checkup. The mother complains of "colic" and asks if she can switch to
goat's milk instead of breast milk. Which of the following should be your
main concern about using goat's milk instead of breast milk or cow's milk?
b. It has insufficient folate.
143. You see the newborn baby shown below for the first time in the nursery.
You consult plastic and reconstructive surgeon as well as the hospital's
speech therapist. Understandably, the parents have many questions. Which of
the following statements is appropriate anticipatory guidance for this family?
d. Recurrent otitis media and hearing loss are likely complications.
144. The mother of a 2-week-old infant reports that since birth, her infant
sleeps most of the day; she has to awaken her every 4 hours to feed, and she
will take only an ounce of formula at a time. She also is concerned that the
infant has persistently hard, pellet-like stools. On your examination you
find an infant with normal weight and length, but with an enlarged head.
The heart rate is 75 beats per minute and the temperature is 35°C (95°F).
The child is still jaundiced. You note large anterior and posterior fontanelles,
a distended abdomen, and an umbilical hernia. This clinical presentation is
likely a result of which of the following?
a. Congenital hypothyroidism
145. A routine prenatal ultrasound reveals a male fetus with meningomyelocele.
The 24-year-old primigravid mother is told the infant will require surgery
shortly after birth. You counsel her about the etiology of this defect and the risk
of further pregnancies being similarly affected, and state which of the following?
d. Supplementation of maternal diet with folate leads to a decrease in incidence of
146. A term, 4200-g (9-lb, 4-oz) female infant is delivered via cesarean
section because of cephalopelvic disproportion. The amniotic fluid was
clear, and the infant cried almost immediately after birth. Within the first
15 minutes of life, however, the infant's respiratory rate increased to 80 breaths
per minute, and she began to have intermittent grunting respirations. The
infant was transferred to the level 2 nursery and was noted to have an oxygen
saturation of 94%. The chest radiograph is shown. Which of the following is
the most likely diagnosis?
e. Transient tachypnea of the newborn
147. The infant in the following picture presents with hepatosplenomegaly,
anemia, persistent rhinitis, and a maculopapular rash. Which of the following
is the most likely diagnosis for this child?
d. Congenital syphilis
148. A well-appearing, 3200-g (7-lb, 1-oz) black infant is noted to have fifth
finger (postaxial) polydactyly. The extra digit has no skeletal duplications and
is attached to the rest of the hand by a threadlike soft tissue pedicle (see
photograph). Appropriate treatment for this condition includes which of the
b. Excision of extra digit
149. An infant born at 35 weeks' gestation to a mother with no prenatal care
is noted to be jittery and irritable, and is having difficulty feeding. You note
coarse tremors on examination. The nurses report a high-pitched cry and note
several episodes of diarrhea and emesis. You suspect which of the following?
c. Heroin withdrawal syndrome
150. A previously healthy full-term infant has several episodes of duskiness
and apnea during the second day of life. Diagnostic considerations
should include which of the following?
b. Congenital heart disease
151. The signs and symptoms of meningitis in an infant can be different
than those in an adult. Which of the following signs and symptoms of
meningitis is more helpful in an adult patient than in a 4-month-old?
d. Brudzinski sign
152. A woman gives birth to twins at 38 weeks' gestation. The first twin
weighs 2800 g (6 lb, 3 oz) and has a hematocrit of 70%; the second twin
weighs 2100 g (4 lb, 10 oz) and has a hematocrit of 40%. Which of the following
statements is correct?
b. The first twin is more likely to have hyperbilirubinemia and convulsions
153. Parents bring a 5-day-old infant to your office. The mother is O negative
and was Coombs positive at delivery. The term child weighed 3055 g
(6 lb, 1 oz) at birth and had measured baseline hemoglobin of 16 g/dL and
a total serum bilirubin of 3 mg/dL. He passed a black tarlike stool within
the first 24 hours of life. He was discharged at 30 hours of life with a stable
axillary temperature of 36.5°C (97.7°F). Today the infant's weight is 3000 g,
his axillary temperature is 35°C (95°F), and he is jaundiced to the chest.
Parents report frequent yellow, seedy stool. You redraw labs and find his
hemoglobin is now 14 g/dL, and his total serum bilirubin is 13 mg/dL. The
change in which of the following parameters is of most concern?
154. You are called to a delivery of a woman with no prenatal care; she is
in active labor but has no history of amniotic rupture. The biophysical profile
done in the emergency center revealed severe oligohydramnios. When
you get this infant to the nursery, you should carefully evaluate him for
which of the following?
c. Renal agenesis
155. A newborn infant becomes markedly jaundiced on the second day of
life, and a faint petechial eruption, first noted at birth, is now a generalized
purpuric rash. Hematologic studies for hemolytic diseases are negative. Acute
management should include which of the following?
b. Isolation of the infant from pregnant hospital personnel
Questions 156 to 159
156. A newborn has bilateral cataracts and microphthalmia, intrauterine
growth retardation, hemorrhagic skin lesions scattered throughout the body,
and a harsh systolic murmur heard at the left sternal border and radiating to
the lung fields.
c. Congenital rubella
158. A newborn has hydrocephalus, chorioretinitis, intracranial calcifications,
a. Congenital toxoplasmosis
159. A newborn has microcephaly, intracranial calcifications, hepatosplenomegaly,
and marked hyperbilirubinemia and thrombocytopenia
d. Congenital cytomegalovirus
Questions 160 to 163
160. A large-for-gestation-age term infant is delivered via scheduled
cesarean section develops, at 15 minutes of age, tachypnea, grunting, flaring,
and retractions. The child does not move his left arm well, but you find
no clavicular fracture. A chest radiograph shows the left diaphragm to be
markedly higher than the right.
i. Phrenic nerve paralysis
161. A postterm infant is born at home after a prolonged and difficult labor.
The maternal grandmother brings the infant to the hospital at 1 hour of life
because of fast breathing. Grandmother notes that the child seemed well for
a while, but then developed increased work of breathing. Physical examination
reveals an infant in moderate respiratory distress with diminished breath
sounds on the left. Chest radiograph reveals the heart to be pushed to the
right side and loss of lung markings in the left lung field.
162. An infant of uncertain but seemingly term dates is born via emergent
cesarean section for nonreassuring heart tones; the obstetrician has noted
little or no amniotic fluid. The infant is small, has abnormally shaped limbs,
and an unusual facies. The child has immediate respiratory distress. A chest
radiograph reveals a poorly developed chest with little lung tissue.
e. Primary pulmonary hypoplasia
163. A preterm infant is now 7 weeks old. She was intubated for 2 weeks and
was weaned off oxygen at 3 weeks of age. You are about to leave your office for
Thanksgiving holiday when the emergency room calls to tell you she has new
hypoxia, respiratory distress, wheezes, and runny nose. A chest radiograph
reveals patchy infiltrates and hyperexpansion in both lung fields. The newborn's
2-year-old sibling has an upper respiratory infection.
Questions 164 to 167
Blood samples of a 3-day-old full-term infant are sent for screening to identify
diseases that would have serious, permanent consequences without prompt
and appropriate treatment. Select the most appropriate treatment for each
disease below. Each lettered option may be used once, more than once, or
not at all.
a. Special infant formula
Questions 168 to 171
168. A child's left arm and leg seem bigger than those on the right. In
addition, the child has aniridia. None of the family members have aniridia
or hemihypertrophy, nor do they know of anyone else in the family with
c. Wilms tumor
169. An infant has fusion of the eyebrows, heterochromic irises, a broad
nasal root with lateral displacement of the medial canthi, and a white forelock.
170. An infant presents with a large, flat vascular malformation over the
left face and scalp. The mother notes that her other child was born with a
capillary hemangioma on his arm and asks if this is the same thing. You
explain that this vascular malformation is different, and that you will want
to monitor him for another condition.
171. A new mother points out several hypopigmented oval macules over
the child's trunk and extremities. She notes that these have been present
since birth. She is concerned because she had a brother with the same spots
whom she thinks had "growths" in his brain.
Questions 172 to 174
172. A 1-day-old infant has a fronto-occipital head circumference that is 2 cm
larger than the initial measurement done several hours before, the scalp has a
"squishy" feel to it, and the infant has developed tachycardia.
e. Subgaleal hemorrhage
173. A 6-month-old comatose infant has multiple broken bones in various
stages of healing, a bulging anterior fontanelle, and retinal hemorrhages.
c. Subdural hemorrhage
174. Previous premature infant born at 27 weeks' gestation and now
6 months of age presents with macrocephaly and hydrocephalus on ultrasonogram
a. Intraventricular hemorrhage
175. A 14-year-old girl, angry at her mother for taking away her MP3
player, takes an unknown quantity of a friend's pills. Within the first hour
she is sleepy, but in the emergency center she develops a widened QRS complex
on her electrocardiogram (ECG), hypotension, and right bundle
branch block. The therapy you would initiate for this ingestion is which of
c. Intensive care unit (ICU) admission, close monitoring, and possible Fab antibody
176. A 10-year-old boy, the star pitcher for the Salt Lake City Little League
baseball team, had a sore throat about 2 weeks ago but did not tell anyone
because he was afraid he would miss the play-offs. Since several children
have been diagnosed with rheumatic fever in the area, his mother is worried
that he may be at risk as well. You tell her that several criteria must be
met to make the diagnosis but the most common finding is which of the
177. You are seeing a 2-year-old boy for the first time. His father denies
any past medical or surgical history, but does note that the child's day care
recently sent a note home asking about several episodes, usually after the
child does not get what he wants, when he "breathes funny" and sits in a
corner with his knees under his chin for a few minutes. The day-care
staffers think this "self-imposed time-out" is a good thing, but they worry
about the breathing. One teacher even thought he once looked blue, but
decided that it was probably because of the finger paints he had been using.
On examination, you identify a right ventricular impulse, a systolic thrill
along the left sternal border, and a harsh systolic murmur (loudest at the
left sternal border but radiating through the lung fields). His chest radiograph
and ECG are shown. Which of the following congenital cardiac
lesions would you expect to find in this child?
אין תשובה שלך ואין תשובות של הספר
178. The parents of a 2-month-old baby boy are concerned about his risk
of coronary artery disease because of the recent death of his 40-year-old
maternal uncle from a myocardial infarction. Which of the following is the
most appropriate management in this situation?
a. Screen the parents for total cholesterol.
179. For the past year, a 12-year-old boy has had recurrent episodes of swelling
of his hands and feet, which has been getting worse recently. These episodes
occur following exercise and emotional stress, last for 2 to 3 days, and resolve
spontaneously. The last episode was accompanied by abdominal pain, vomiting,
and diarrhea. The results of routine laboratory workup are normal. An older
sister and a maternal uncle have had similar episodes, but they were not given a
diagnosis. He presents today with another episode as shown in the photographs
on the next page. Which of the following is the most likely diagnosis?
d. Hereditary angioedema
180. A 15-year-old girl with short stature, neck webbing, and sexual infantilism
is found to have coarctation of the aorta. A chromosomal analysis likely
would demonstrate which of the following?
c. XO karyotype
181. A newborn is diagnosed with congenital heart disease. You counsel the
family that the incidence of heart disease in future children is which of the
b. 2% to 6%
182. During a regular checkup of an 8-year-old child, you note a loud first
heart sound with a fixed and widely split second heart sound at the upper
left sternal border that does not change with respirations. The patient is
otherwise active and healthy. Which of the following heart lesions most
likely explains these findings?
a. Atrial septal defect (ASD)
183. A 2-year-old boy is brought into the emergency room with a complaint
of fever for 6 days and the development of a limp. On examination, he is found to
have an erythematous macular exanthem over his body as shown in image A, ocular
conjunctivitis, dry and cracked lips, a red throat, and cervical lymphadenopathy.
There is a grade 2/6 vibratory systolic ejection murmur at the lower left sternal
border. A white blood cell (WBC) count and differential show predominant
neutrophils with increased platelets on smear. Later, he develops the findings as
seen in image B. Which of the following is the most likely diagnosis?
c. Kawasaki disease
184. An ill-appearing 2-week-old baby girl is brought to the emergency room.
She is pale and dyspneic with a respiratory rate of 80 breaths per minute. Heart
rate is 195 beats per minute, heart sounds are distant, a gallop is heard, and she
has cardiomegaly on x-ray. An echocardiogram demonstrates poor ventricular
function, dilated ventricles, and dilation of the left atrium. An ECG shows ventricular
depolarization complexes that have low voltage. Which of the following
is the most likely diagnosis based on this clinical picture?
185. A newborn infant has mild cyanosis, diaphoresis, poor peripheral
pulses, hepatomegaly, and cardiomegaly. Respiratory rate is 60 breaths per
minute, and heart rate is 250 beats per minute. The child most likely has
congestive heart failure caused by which of the following?
e. Paroxysmal atrial tachycardia
186. A 3-month-old infant is brought to your office for pallor and listlessness.
Your physical examination reveals tachycardia that is constant and
does not vary with crying. He has no hepatomegaly and the lungs are clear.
His ECG is shown. Which of the following is the most appropriate initial
management of this patient?
c. Carotid massage
187. A 2-year-old child with minimal cyanosis has an S3 and S4 (a quadruple
rhythm), a systolic murmur in the pulmonic area, and a middiastolic murmur
along the lower left sternal border. An ECG shows right atrial hypertrophy
and a ventricular block pattern in the right chest leads. Which of the following
is the most likely diagnosis?
d. Ebstein anomaly
188. A 4-year-old girl is brought to the pediatrician's office. Her father
reports that she suddenly became pale and stopped running while he had
been playfully chasing her and her pet Chihuahua. After 30 minutes, she
was no longer pale and wanted to resume the game. She has never had a
previous episode and has never been cyanotic. Her physical examination
was normal, as were her chest x-ray and echocardiogram. An ECG showed
the pattern seen on the next page, which indicates which of the following?
c. Wolff-Parkinson-White syndrome
189. A child has a 2-week history of spiking fevers, which have been as
high as 40°C (104°F). She has spindle-shaped swelling of finger joints and
complains of upper sternal pain. When she has fever, the parents note a
faint salmon-colored rash that resolves with the resolution of the fever. She
has had no conjunctivitis or mucositis, but her heart sounds are muffled
and she has increased pulsus paradoxus. Which of the following is the
most likely diagnosis?
b. Juvenile rheumatoid arthritis
190. A cyanotic newborn is suspected of having congenital heart disease. He
has an increased left ventricular impulse and a holosystolic murmur along the
left sternal border. The ECG shows left-axis deviation and left ventricular
hypertrophy (LVH). Which of the following is the most likely diagnosis?
c. Tricuspid atresia
191. A 3-day-old infant with a single second heart sound has had progressively
deepening cyanosis since birth but no respiratory distress. Chest radiography
demonstrates no cardiomegaly and normal pulmonary vasculature.
An ECG shows an axis of 120° and right ventricular prominence. Which of
the following congenital cardiac malformations is most likely responsible for
b. Transposition of the great vessels
192. During a physical examination for participation in a sport, a 16-yearold
girl is noted to have a late apical systolic murmur, which is preceded by a
click. The rest of the cardiac examination is normal. She states that her mother
also has some type of heart "murmur" but knows nothing else about it. Which
of the following is the most likely diagnosis?
d. Mitral valve prolapse
193. A previously normal newborn infant in a community hospital nursery
is noted to be cyanotic at 14 hours of life. She is placed on a face mask with
oxygen flowing at 10 L/min. She remains cyanotic, and her pulse oximetry
reading does not change. An arterial blood gas shows her PaO2 to be 23 mm Hg.
Bilateral breath sounds are present, and she has no murmur. She is breathing
deeply and quickly, but she is not retracting. While you are waiting for the transport
team from the nearby children's hospital, you should initiate which of the
d. Prostaglandin E1 infusion
Questions 194 to 196
194. An infant in the neonatal intensive care unit (NICU) has shortened
lower distal arms but with thumbs. Her platelet count is profoundly low.
195. A 15-year-old boy comes to your office for a Special Olympics sports
physical. His height is in the 3rd to 5th percentile and his weight is in the 50th
percentile. Physical examination reveals a young man with shieldlike chest,
cryptorchidism, low-set and malformed ears, ptosis, and pectus excavatum.
d. Pulmonic stenosis
196. A new patient to your practice, a happy 10-year-old girl has a history
of hyperextensible skin and easy bruising. She often has sprains and joint
dislocations when she exercises.
c. Mitral valve prolapse
Questions 197 to 200
197. A 15-year-old adolescent male presents to the office for a sports physical.
In his screening questionnaire, he notes that he occasionally gets short
of breath and dizzy during exercise, with occasional chest pain. He lost consciousness
once last summer during football practice, but attributed it to the
heat. His grandfather died suddenly at the age of 35 of unknown etiology,
but otherwise the family is healthy.
b. Hypertrophic cardiomyopathy
198. A 15-year-old adolescent female comes to be evaluated for syncopal
episodes. Her only other medical problem is congenital deafness. She notes
through a signing interpreter that syncopal episodes happen during stressful
or emotional situations and that they started only within the past year
or so. Her mother and father are from the same small farming town and are
c. Prolonged QT syndrome
199. You are called to the nursery to evaluate a newborn. The mother has
a history of systemic lupus erythematosus and gestational diabetes. The
nurses are concerned because the baby has developed petechiae and bruising
after his bath. Vital signs have been stable, with a heart rate in the 60 beats
per minute range and a respiratory rate in the 40 breaths per minute range.
You note a large liver, scattered petechiae, and an erythematous rash on the
cheeks and on the bridge of the nose.
a. Congenital heart block
200. An infant previously diagnosed with a large muscular VSD comes to
the office with complaints from the mother of fatigue and poor feeding over
the past month. You note the child has not gained weight since the previous
visit 2 months ago. The child is apathetic, tachypneic, and has wheezes
and crackles on lung auscultation.
d. Congestive heart failure
201. You are asked to evaluate an infant born vaginally 3 hours previously to
a mother whose only pregnancy complication was poorly controlled gestational
diabetes. The nursing staff noticed that the infant was breathing abnormally.
On examination, you find that the infant is cyanotic, has irregular,
labored breathing, and has decreased breath sounds on the right side. You also
note decreased tone in the right arm. You provide oxygen and order a stat
portable chest radiograph, which is normal. Which of the following studies is
most likely to confirm your diagnosis?
d. Chest ultrasound
202. A 10-month-old infant has poor weight gain, a persistent cough, and
a history of several bouts of pneumonitis. The mother describes the child as
having very large, foul-smelling stools for months. Which of the following
diagnostic maneuvers is likely to result in the correct diagnosis of this child?
e. Sweat chloride test
203. A previously well 1-year-old infant has had a runny nose and has been
sneezing and coughing for 2 days. Two other members of the family had similar
symptoms. Four hours ago, his cough became much worse. On physical
examination, he is in moderate respiratory distress with nasal flaring, hyperexpansion
of the chest, and easily audible wheezing without rales. His chest
radiographs are shown. Which of the following is the appropriate next course
a. Monitoring oxygenation and fluid status alone
204. A 6-year-old girl presents with a 2-day history of cough and fever. At
your office she has a temperature of 39.4°C (103°F), a respiratory rate of
45 breaths per minute, and decreased breath sounds on the left side. Her
chest x-ray is shown below. Which of the following is the most appropriate
205. A 2-year-old girl is playing in the garage with her Chihuahua, only partially
supervised by her father, who is weed-whacking around the garden
gnomes in the front yard. He finds her in the garage, gagging and vomiting.
She smells of gasoline. In a few minutes she stops vomiting, but later that day
she develops cough, tachypnea, and subcostal retractions. She is brought to
your emergency center. Which of the following is the most appropriate first
step in management?
d. Perform pulse oximetry and arterial blood gas
206. A 3-year-old girl is admitted with the x-ray shown below. The child lives
with her parents and a 6-week-old brother. Her grandfather stayed with the
family for 2 months before his return to the West Indies 1 month ago. The
grandfather had a 3-month history of weight loss, fever, and hemoptysis.
Appropriate management of this problem includes which of the following?
d. Treating the 3-year-old patient with isoniazid (INH) and rifampin
207. You are asked to evaluate a 4-year-old boy admitted to your local children's
hospital with a diagnosis of pneumonia. The parents state that the
child has had multiple, intermittent episodes of fever and respiratory difficulty
over the past 2 years, including cyanosis, wheezing, and dyspnea; each
episode lasts for about 3 days. During each event he has a small amount of
hemoptysis, is diagnosed with left lower lobe pneumonia, and improves
upon treatment. Repeat radiographs done several days after each event are
reportedly normal. His examination on the current admission is significant
for findings similar to those described above, as well as digital clubbing.
Which of the following is the most appropriate primary recommendation?
e. Bronchoalveolar lavage
208. A previously healthy, active, 18-month-old African American child presents
with unilateral nasal obstruction and foul-smelling discharge. The child's
examination is otherwise unremarkable. Which of the following is the most
a. Foreign body
209. A 7-year-old child is brought by his mother for a school physical. His
growth parameters show his height to be 50th percentile and his weight to
be significantly higher than 95th percentile. His mother complains that he
always seems sleepy during the day and that he has started complaining of
headaches. His second-grade teacher has commented that he has difficulty
staying awake in class. His mother complains that he wakes up the whole
house with his snoring at night. Which of the following is the most appropriate
next step in evaluating and managing this condition?
e. Arrange for polysomnography
210. You admitted to the hospital the previous evening a 1-year-old boy
who presented with cough, fever, and mild hypoxia. At the time of his
admission, he had evidence of a right upper lobe consolidation on his chest
radiograph. A blood culture has become positive in less than 24 hours for
Staphylococcus aureus. Approximately 20 hours into his hospitalization, the
nurse calls you because the child has acutely worsened over the previous
few minutes, with markedly increased work in breathing, increasing oxygen
requirement, and hypotension. As you move swiftly to the child's hospital
room, you tell the nurse to order which of the following?
b. A large-bore needle and chest tube kit for aspiration of a probable tension
211. A fully immunized 2-year-old presents to the emergency room with
several days of low-grade fever, barking cough, and noisy breathing. Over
the past few hours he has developed a fever of 40°C (104°F) and looks
toxic. He has inspiratory and expiratory stridor. The family has not noticed
drooling, and he seems to be drinking without pain. Direct laryngoscopy
reveals a normal epiglottis. The management of this disease process includes
which of the following?
a. Intubation and intravenous antibiotics
212. A 6-year-old, fully immunized boy is brought to the emergency room
with a 3-hour history of fever to 39.5°C (103.1°F) and sore throat. The child
appears alert, but anxious and toxic. He has mild inspiratory stridor and is
drooling. He is sitting on the examination table leaning forward with his neck
extended. A lateral radiograph of his neck is shown below. Which of the following
is the most appropriate immediate management of this patient?
d. Prepare to establish an airway in the operating room.
213. A 4-year-old boy was admitted to the hospital last night with the
complaint of "difficulty breathing." He has no past history of lung infection,
no recent travel, and no day-care exposure; he does, however, have an
annoying tendency to eat dirt. In the emergency center he was noted to be
wheezing and to have hepatomegaly. He is able to talk, relaying his concern
about his 6-week-old Chihuahua being left alone at home. Laboratory
studies revealed marked eosinophilia (60% eosinophils). Which of the following
tests is most likely to produce a specific diagnosis?
c. ELISA for Toxocara
214. A 10-year-old girl has had a "cold" for 14 days. In the 2 days prior to the
visit to your office, she has developed a fever of 39°C (102.2°F), purulent
nasal discharge, facial pain, and a daytime cough. Examination of the nose
after topical decongestants shows pus in the middle meatus. Which of the following
is the most likely diagnosis?
b. Maxillary sinusitis
215. You are awakened in the night by your 2-year-old son, who has developed
noisy breathing on inspiration, marked retractions of the chest wall, flaring
of the nostrils, and a barking cough. He has had a mild upper respiratory
infection (URI) for 2 days. Which of the following therapies is indicated?
d. Inhaled epinephrine and a dose of steroids
216. An 8-year-old girl presents with well-controlled, moderately persistent
asthma. Her therapies consist of occasional use of short-acting β-agonists,
daily inhaled steroids, and a leukotriene inhibitor. She presents with white
patches on her buccal mucosa. You recommend which of the following?
e. Rinse her mouth after use of her inhaled medications
217. A 13-year-old develops fever, malaise, sore throat, and a dry, hacking
cough over several days. He does not appear to be particularly sick, but his
chest examination is significant for diffuse rales and rhonchi. The chest radiograph
is shown below. Which of the following is the most likely pathogen?
e. Mycoplasma pneumoniae
218. You have just given a 10-year-old boy an injection of pollen extract
as prescribed by his allergist. You are about to move on to the next patient
when the boy starts to complain about nausea and a funny feeling in his
chest. You note that his face is flushed and his voice sounds muffled and
strained. Which of the following is the first priority in managing this
episode of anaphylaxis?
a. Preparation for endotracheal intubation
219. A previously healthy 18-month-old has been in a separate room from
his family. The family notices the sudden onset of coughing, which resolves
in a few minutes. Subsequently, the patient appears to be normal except for
increased amounts of drooling and refusal to take foods orally. Which of
the following is the most likely explanation for this toddler's condition?
e. Foreign body in the esophagus
220. You receive a telephone call from the mother of a 4-year-old child
with sickle-cell anemia. She tells you that the child is breathing fast, coughing,
and has a temperature of 40°C (104°F). Which of the following is the
most conservative, prudent course of action?
e. Admit the child to the hospital
221. You are asked by a colleague to evaluate a 5-year-old boy as a second
opinion. He has a history of chronic and recurrent upper respiratory tract
infections, several admissions to the hospital for pneumonia, and three surgeries
for PE tubes for chronic otitis media. Of note is a right-sided heart on
repeated radiographs. Convinced you know the diagnosis based on history
alone, you confirm your diagnosis with a biopsy of the nasal mucosa. You
expect to find which of the following?
d. Random orientation of cilia
222. A 13-year-old patient with sickle-cell anemia presents with respiratory
distress; she has an infiltrate on chest radiograph. The laboratory workup of
the patient reveals the following: hemoglobin 5 g/dL; hematocrit 16%; white
blood cell count 30,000/μL; and arterial blood (room air) pH 7.1, PO2 35 mm
Hg, and PaCO2 28 mm Hg. These values indicate which of the following?
a. Acidemia, metabolic acidosis, respiratory alkalosis, and hypoxia
223. A 24-year-old woman arrives in the emergency center in active labor.
She is at term, but received no prenatal care after 16 weeks of gestation
when she lost her insurance coverage. The mother has an uncomplicated
vaginal delivery. You are paged shortly after birth when the baby is noted to
have respiratory distress. The infant has diminished breath sounds on the
left, and the PMI is shifted toward the right. A chest radiograph is shown.
The NG tube you placed earlier reveals the stomach to be below the
diaphragm. Which of the following is the most likely diagnosis at this point?
a. Congenital cystic adenomatoid malformation
224. A 13-year-old boy has a 3-day history of low-grade fever, symptoms of
upper respiratory infection, and a sore throat. A few hours before his presentation
to the emergency room, he has an abrupt onset of high fever, difficulty
swallowing, and poor handling of his secretions. He indicates that he has a
marked worsening in the severity of his sore throat. His pharynx has a fluctuant
bulge in the posterior wall. A soft tissue radiograph of his neck is shown.
Which of the following is the most appropriate initial therapy for this patient?
c. Surgical consultation for incision and drainage under general anesthesia
225. A 6-week-old child arrives with a complaint of "breathing fast" and a
cough. On examination you note the child to have no temperature elevation,
a respiratory rate of 65 breaths per minute, and her oxygen saturation
to be 94%. Physical examination also is significant for rales and rhonchi.
The past medical history for the child is positive for an eye discharge at
3 weeks of age, which was treated with a topical antibiotic drug. Which of
the following organisms is the most likely cause of this child's condition?
d. Chlamydia trachomatis
226. One of your asthmatic patients arrives for a checkup. The mother
reports that the child seems to need albuterol daily, especially when exercising,
and she has coughing fits that awaken her from sleep about twice a
week. Her grandmother had recommended a Chihuahua as a "cure" for her
asthma, but her mother has seen no difference since the arrival of the pet.
Appropriate treatment measures would include which of the following?
d. Medium-dose, inhaled corticosteroids with short-acting β-agonists
227. A previously healthy 2-year-old black child has developed a chronic
cough during the previous 6 weeks. He has been seen in different emergency
rooms on two occasions during this period and has been placed on
antibiotics for pneumonia. Upon auscultation, you hear normal breath
sounds on the left. On the right side, you hear decreased air movement
during inspiration but none upon expiration. Inspiratory (A) and expiratory
(B) radiographs of the chest are shown below. Which of the following
is the most appropriate next step in making the diagnosis in this patient?
b. Consult pediatric surgery for bronchoscopy
236. A 10-year-old boy has been having "bellyaches" for about 2 years.
They occur at night as well as during the day. Occasionally, he vomits after
the onset of pain. Occult blood has been found in his stool. His father also
gets frequent, nonspecific stomachaches. Which of the following is the
most likely diagnosis?
a. Peptic ulcer
237. An 8-year-old boy presents to your office for a second opinion. He
has a 2-year history of intermittent vomiting, dysphagia, and epigastric
pain. His father reports he occasionally gets food "stuck" in his throat. He
has been on a proton pump inhibitor for 18 months without symptom
relief. His past history is significant only for eczema and a peanut allergy.
Endoscopy was performed 6 months ago; no erosive lesions were noted
and a biopsy was not performed. You arrange for a repeat endoscopy with
biopsy. Microscopy on the biopsy sample reveals many eosinophils. Treatment
of this condition should include which of the following?
240. A 15-year-old girl is admitted to the hospital with a 6-kg weight
loss, bloody diarrhea, and fever that have occurred intermittently over the
previous 6 months. She reports cramping abdominal pain with bowel
movements. She also reports secondary amenorrhea during this time. Stool
cultures in her physician's office have shown only normal intestinal flora.
A urine pregnancy test was negative, while an erythrocyte sedimentation
rate (ESR) was elevated. Her examination is significant for the lack of oral
mucosal ulcerations and a normal perianal examination. Anti-Saccharomyces
cerevisiae antibodies (ASCA) are negative, while anti-neutrophil cytoplasm
antibodies (p-ANCA) are positive. You confirm your presumptive diagnosis
with a rectal biopsy. In counseling her about her disease, which of the
following statements would be true?
a. Inheritance is autosomal dominant
241. A 3-year-old child presents to your office for an evaluation of constipation.
The mother notes that since birth, and despite frequent use of stool
softeners, the child has only about one stool per week. He does not have
fecal soiling or diarrhea. He was born at term and without pregnancy complications.
The child stayed an extra day in the hospital at birth because
he did not pass stool for 48 hours, but has not been in the hospital since.
Initial evaluation of this child should include which of the following?
b. A barium enema and rectal manometry
243. A 17-year-old adolescent female is 6 weeks postpartum. She presents
to the emergency room with the complaints of increased jaundice, abdominal
pain, nausea, vomiting, and fever. Her examination is remarkable for
jaundice, pain of the right upper quadrant with guarding, and a clear chest.
Chest radiographs appear normal. Which of the following tests is most likely
to reveal the cause of this pain?
c. Ultrasound of the right upper quadrant
244. An 8-year-old is accidentally hit in the abdomen by a baseball bat. After
several minutes of discomfort, he seems to be fine. Over the ensuing 24 hours,
however, he develops a fever, abdominal pain radiating to the back, and persistent
vomiting. On examination, the child appears quite uncomfortable. The
abdomen is tender, with decreased bowel sounds throughout, but especially
painful in the midepigastric region with guarding. Which of the following
tests is most likely to confirm the diagnosis?
a. Serum amylase levels
245. A 10-month-old baby boy, recently adopted from Guyana, has a 5-hour
history of crying, with intermittent drawing up of his knees to his chest.
On the way to the emergency room he passes a loose, bloody stool. He has
had no vomiting and has refused his bottle since the crying began. Physical
examination is noteworthy for an irritable infant whose abdomen is very
difficult to examine because of constant crying. His temperature is 38.8°C
(101.8°F). The rectal ampulla is empty, but there is some gross blood on the
examining finger. Which of the following studies would be most helpful in
the immediate management of this patient?
c. Air contrast enema
246. A 12-month-old girl has been spitting up her meals since 1 month of
age. Her growth is at the 95th percentile, and she is otherwise asymptomatic
and without findings on physical examination. Which of the following is the
most likely diagnosis?
d. Gastroesophageal reflux
247. A 14-year-old girl has a 9-month history of diarrhea, abdominal pain
(usually periumbilical and postprandial), fever, and weight loss. She has had
several episodes of blood in her stools. Which of the following is the most
c. Crohn disease
248. A 4-year-old boy, recently adopted through an international adoption
service, is noted to have intermittent watery diarrhea, nausea, belching, and
abdominal pain. His weight is less than the fifth percentile for his age. Which
of the following studies would be most helpful in making the diagnosis?
e. Stool microscopy for ova and parasites
249. A 5-month-old child regularly regurgitates a large portion of her feeds.
A pH probe study showed significant periods of low esophageal pH. The
child has normal growth and no other significant past medical history. Which
of the following is the best management at this point?
d. Close observation only
250. A 2-year-old presents to the emergency center with several days of rectal
bleeding. The mother first noticed reddish-colored stools 2 days prior to
arrival and has since changed several diapers with just blood. The child is
afebrile, alert, and playful, and is eating well without emesis. He is slightly
tachycardic, and his abdominal examination is normal. Which of the following
is the best diagnostic study to order to confirm the diagnosis?
d. Radionucleotide scan
251. A 15-year-old otherwise healthy boy presents with a complaint of
intermittent abdominal distention, crampy abdominal pain, and excessive
flatulence. He first started noticing these symptoms when he moved into
his father's house, and his stepmother insisted on milk at dinner every night.
He has normal growth, has not lost weight, and has no travel history. Which of
the following is the most appropriate study to diagnose his condition?
b. Hydrogen excretion in breath after oral administration of lactose
252. A 6-week-old infant is admitted to the hospital with jaundice. Her outpatient
blood work demonstrated a total bilirubin of 12 mg/dL with a direct
portion of 3.5 mg/dL. Which of the following disorders is most likely to be
b. Choledochal cyst
253. A 2-year-old arrives in the emergency center after having swallowed
a button battery from one of her toys. She is breathing comfortably, without
stridor. Radiographs show the battery to be lodged in the esophagus. Which
of the following is the correct next step?
d. Immediate removal of the battery via endoscopy.
254. An awake, alert infant with a 2-day history of diarrhea presents with
a depressed fontanelle, tachycardia, sunken eyes, and the loss of skin elasticity.
Which of the following is the correct percentage of dehydration?
c. 5% to 9%
255. A 9-month-old is brought to the emergency center by ambulance.
The child had been having emesis and diarrhea with decreased urine output
for several days, and the parents noted that she was hard to wake up
this morning. Her weight is 9 kg, down from 11 kg the week prior at her
9-month checkup. You note her heart rate and blood pressure to be normal.
She is lethargic, and her skin is noted to be "doughy." After confirming that
her respiratory status is stable, you send electrolytes, which you expect to
be abnormal. You start an IV. Which of the following is the best solution for
an initial IV bolus?
c. Normal saline (154 mEq sodium/L)
256. You are admitting to the hospital a 3-month-old infant who has been
having poor feeding, emesis, and diarrhea for 3 days. In the emergency center,
her electrolytes were found to be: sodium 157 mEq/L, potassium 2.6 mEq/L,
chloride 120 mEq/L, bicarbonate 14 mEq/L, creatinine 1.8 mEq/L, blood urea
nitrogen (BUN) 68 mEq/L, and glucose 195 mEq/L. She was given a fluid bolus
in the emergency center and has subsequently produced urine. Which of
the following is the most appropriate next step in her management?
a. Slow rehydration over 48 hours
257. The mother of a 6-month-old infant is concerned that her baby may be
teething. You explain to her that the first teeth to erupt in most children are
which of the following?
a. Mandibular central incisors
258. An 8-year-old boy is brought to your office with the complaint of abdominal
pain. The pain is worse during the week and seems to be less prominent
during the weekends and during the summer. The patient's growth and development
are normal. The physical examination is unremarkable. Laboratory
screening, including stool for occult blood, CBC, urinalysis, and chemistry
panel, yields normal results. Which of the following is the best next step in
the care of this patient?
d. Observe the patient and reassure the patient and family
260. A 12-year-old girl was hit in the face by a baseball 15 minutes earlier
and has had her mandibular incisors knocked out. Which of the following
represents the best plan of action?
d. Avulsed teeth can be transported in the mouth of the parent or a cooperative
262. An 18-month-old infant is found with the contents of a bottle of drain
cleaner in his mouth. Which of the following treatment options is most
b. Endoscopic examination within the first 12 to 24 hours
263. A 16-year-old male, despondent over a recent breakup, tries to commit
suicide by taking an unknown quantity of an unknown material he
found at home. He is brought to the emergency center by his parents within
30 minutes of the ingestion. For which of the following household materials
and medications should he be given activated charcoal as part of his emergency
Questions 264 to 268
264. A newborn infant, the product of an emergency cesarean section, is
24 hours old and has a grossly bloody stool. She looks well otherwise.
c. Apt test
265. A 7-day-old premature infant born at 26 weeks of gestation now has
a grossly bloody stool, abdominal distention, and increasing oxygen requirements.
a. Abdominal series
266. A 2-year-old has crampy abdominal pain and grossly bloody diarrheal
stool, but no fever. His abdominal examination reveals no masses. A family
member, who ate at the same local hamburger shop the night prior, has the
b. Fiberoptic endoscopy
267. A 7-year-old has been vomiting for 2 days and has had diarrhea for
1 day. He now notes that he has small streaks of blood in his emesis. The rest
of his family has had similar symptoms
f. No immediate intervention
268. A 10-year-old has complained for 1 month of intermittent epigastric
pain that awakens him from sleep. He notes that eating food sometimes
helps. He reports black stools during the prior week, and also admits that
he has occasionally vomited frank blood.
b. Fiberoptic endoscopy
Questions 269 to 274
269. A 15-year-old vegetarian being treated for tuberculosis develops peripheral
b. Vitamin B6
270. A 9-month-old infant, who has been fed cow's milk exclusively for
4 months, is tachycardic and pale.
271. A 3-day-old infant born at home is brought to the emergency center with
bloody stools, hematemesis, and purpura. His circumcision is oozing blood.
g. Vitamin K
272. A 17-month-old toddler has been irritable over the past month. She
now refuses to walk and seems to have tenderness in both of her legs. She
has had a low-grade fever, and she has petechiae on her skin and mucous
membranes. She has a small cut that has not healed well. Radiographs of the
legs reveal generalized bony atrophy with epiphyseal separation.
c. Vitamin C
273. A 4-year-old whose diet consists mostly of cheese puffs and cola begins
to have problems walking at night, complaining that he cannot see well. In addition,
his skin has become dry and scaly, and he has complained of headache
for a month.
a. Vitamin A
274. An exclusively breast-fed 2-year-old is brought to the emergency
center with pain in his right leg after a fall. Physical examination reveals a
small child with a 3-cm anterior fontanelle, a flattened occiput, a prominent
forehead, significant dental caries, bumpy ribs, and bowed extremities.
Radiographs reveal a greenstick fracture at the site of pain, along with
fraying at the distal ends of the femur.
d. Vitamin D
Questions 275 to 277
275. A 3-week-old child currently admitted to the hospital for pneumonia
who gags and chokes during feedings
e. Modified barium swallow
277. A newborn with arching of the back temporally related to feeds but no
b. 24-hour pH probe
279. At the 2-week checkup of a term female infant, the mother reports a
grayish and sometimes bloody vaginal discharge since birth. The infant's
mother and grandmother are the only caretakers. Examination of the external
genitalia reveals an intact hymen with a thin grayish mucous discharge.
Which of the following is the most appropriate next step?
a. Parental reassurance
281. A 17-year-old boy is brought to the emergency department by his parents
with the complaint of coughing up blood. He is stabilized, and his hemoglobin
and hematocrit levels are 11 mg/dL and 33%, respectively. During
his hospitalization, he is noted to have systolic blood pressure persistently
greater than 130 mm Hg and diastolic blood pressure greater than 90 mm Hg.
His urinalysis is remarkable for hematuria and proteinuria. You are suspicious
the patient has which of the following?
b. Goodpasture syndrome
282. A 1-year-old child presents with failure to thrive, frequent large voids of
dilute urine, excessive thirst, and three episodes of dehydration not associated
with vomiting or diarrhea. Over the years, other family members reportedly
have had similar histories. Which of the following is the most likely diagnosis?
c. Diabetes insipidus
283. A 6-month-old infant has poor weight gain, vomiting, episodic fevers,
and chronic constipation. Laboratory studies reveal a urinalysis with a pH
of 8.0, specific gravity of 1.010, 1+ glucose, and 1+ protein. Urine anion gap
is normal. Serum chemistries show a normal glucose and a normal albumin
with a hyperchloremic metabolic acidosis. Serum phosphorus and calcium
are low. What is the best diagnosis to explain these findings?
d. Hereditary Fanconi syndrome
284. The mother of a 2-year-old male child states that she has noticed white,
cheeselike material arising from his foreskin and also that he cannot fully
retract the foreskin behind the glans penis. Which of the following is the correct
advice for this parent?
c. The child is normal.
287. A 5-year-old girl without past history of UTI is in the hospital on antibiotics
for Escherichia coli pyelonephritis. She is still febrile after 4 days of appropriate
antibiotics. A renal ultrasound revealed no abscess, but a focal enlargement
of one of the lobes of the right kidney. CT of the abdomen reveals a wedgeshaped
area in the right kidney distinct from the normal tissue with minimal
contrast enhancement. Appropriate management of this patient includes which
of the following interventions?
a. Prolonged antibiotic therapy
288. A 4-year-old boy, whose past medical history is positive for three urinary
tract infections, presents with a blood pressure of 135/90 mm Hg. He is likely
to exhibit which of the following symptoms or signs?
290. A 6-week-old child is being evaluated for a fever of unknown etiology.
As part of the laboratory evaluation, a urine specimen was obtained that grew
E coli with a colony count of 2000/μL. These findings would be definite evidence
of a urinary tract infection if which of the following is true about the
d. It is from a suprapubic tap
292. A 4-year-old boy and his family have recently visited a local amusement
park. Several of the family members developed "gastroenteritis" with fever
and diarrhea, but the 4-year-old's stool was slightly different, as it contained
blood. His mother reports that in the past 24 hours he developed pallor and
lethargy; she relates that his face looks swollen and that he has been urinating
very little. Laboratory evaluation reveals a hematocrit of 28% and a platelet
count of 72,000/μL. He has blood and protein in the urine. Which of the
following diagnoses is most likely to explain these symptoms?
e. Hemolytic-uremic syndrome
293. A 6-year-old girl is brought to the emergency room because her urine
is red. She has been healthy her whole life, and has recently returned from
an outing with her grandmother to a local amusement park. Her urine dip for
heme is positive, suggesting which of the following is a possibility?
d. Presence of myoglobin
295. A 12-year-old boy comes to the emergency department at midnight
with a complaint of severe scrotal pain since 7 PM. There is no history of trauma.
Which of the following is the most appropriate first step in management?
a. Order a surgical consult immediately.
296. The mother of a 2-year-old girl reports that her daughter complains
of burning when she urinates and that she has foul-smelling discharge from
her vagina. She has some slight staining on the front of her underwear, but
denies fever, nausea, vomiting, or other constitutional signs. The child does
not attend day care, and she has demonstrated no change in behavior. The
physical examination is normal with an intact hymen, but the child's vulva
is reddened and with a malodorous scent noted. Her urinalysis and culture
are normal. Management of this condition includes which of the following?
c. Advice to stop taking prolonged bubble baths
297. A 7-year-old boy has cramping abdominal pain and a rash mainly on
the back of his legs and buttocks as well as on the extensor surfaces of his
forearms. Laboratory analysis reveals proteinuria and microhematuria. You
diagnose Henoch-Schönlein, or anaphylactoid, purpura. In addition to his
rash and abdominal pain, what other finding is he likely to have?
b. Arthritis or arthralgia
300. A 2-year-old patient arrives late to your office with his father and a
sign-language translator. They are very apologetic, but the father communicates
that he had car trouble at his dialysis center and thus was late picking
up the child from day care. The father is concerned about his child's having
intermittent red, bloody-looking urine. A gross inspection of the child's
urine in your office looks normal, but the dipstick demonstrates 3+ blood.
Which of the following is the most likely cause of this child's hematuria?
a. Alport syndrome
301. A 9-year-old boy comes to the office for a pre-participation physical
examination for summer camp. His parents report that he still has episodes of
bed-wetting. The boy's father confides that he also had bed-wetting until he
was 10. They are concerned about the bed-wetting, but they are more concerned
about their son's upcoming week at summer camp and that the
other boys may harass him for wetting the bed. Which of the following
statements about nocturnal enuresis is correct?
c. Spontaneous cure rates are high regardless of therapy.
302. A 21-year-old woman presents to the emergency room in active labor.
She has had no prenatal care, but her last menstrual period was approximately
9 months prior. Her membranes are artificially ruptured, yielding no amniotic
fluid. She delivers an 1800-g (4-lb) term infant who develops significant
respiratory distress immediately at birth. The first chest radiograph on this
infant demonstrates hypoplastic lungs. After this infant is stabilized, which
of the following is the most appropriate next step for this infant?
b. Renal ultrasound
Questions 303 to 306
303. An 8-year-old boy with the intermittent complaint of "burning"
when he urinates and who has trace blood on his urine dip test.
f. Idiopathic hypercalciuria
304. A 6-year-old girl with a complaint of "dark urine"; she has a blood
pressure of 120/80 mm Hg.
d. Acute glomerulonephritis
305. Elevated levels of cholesterol and triglycerides found in a 6-year-old
boy whose mother reports that he has been awakening with puffy eyes each
morning. On your physical examination you determine that he has had
unexpected weight gain and has scrotal edema
a. Nephrotic syndrome
306. A 12-month-old girl whose height and weight are less than the fifth
percentile; she has had several bouts of constipation and two previous
admissions for dehydration. She is again admitted for dehydration and is
noted to have serum potassium of 2.7 mEq/L.
c. Bartter syndrome
307. A 6-year-old child has had repeated episodes of otitis media. She
undergoes an uneventful surgical placement of pressure-equalization (PE)
tubes. In the recovery room she develops a fever of 40°C (104°F), rigidity
of her muscles, and metabolic and respiratory acidosis. Which of the following
is the most likely explanation for her condition?
c. Malignant hyperthermia
308. The 7-year-old boy now in your office was last seen 2 weeks ago with
a mild viral upper respiratory tract infection. Today, however, he presents with
fever, ataxia, weakness, headache, and emesis. In the office he has a 3 minute
left-sided tonic-clonic seizure. You send him to the hospital and order a
magnetic resonance imaging (MRI) of the brain, the results of which show
disseminated multifocal white matter lesions that enhance with contrast.
This boy's likely diagnosis is which of the following?
b. Acute disseminated encephalomyelitis
310. A 6-month-old child was noted to be normal at birth, but over the
ensuing months you have been somewhat concerned about his slowish
weight gain and his mild delay in achieving developmental milestones. The
family calls you urgently at 7:00 AM noting that their child seems unable to
move the right side of his body. Which of the following conditions might
explain this child's condition?
311. On a newborn boy's first examination, you note a prominent occiput,
a broad forehead, and an absent anterior fontanelle. The baby's head is long
and narrow. The remainder of the physical examination, including a careful
neurological evaluation, is normal. You note that the baby was born via
cesarean section for cephalopelvic disproportion. When you enter the
mother's room, the first question she asks is about her baby's head shape.
Which of the following is the most appropriate statement to the mother
about this infant's condition?
e. The condition requires referral to a surgeon.
312. A 4-year-old child is observed to hold his eyelids open with his fingers
and to close one eye periodically, especially in the evening. He has some
trouble swallowing his food. He usually appears sad, although he laughs
often enough. He can throw a ball, and he runs well. Which of the following
is most likely to aid in the diagnosis?
c. Effect of a test dose of edrophonium
313. A 14-year-old girl with a history of seizures is admitted to the hospital
with the diagnosis of status epilepticus. Her valproic acid level is in the therapeutic
range. You arrange a 24-hour video electroencephalogram (EEG).
During the EEG, she has several episodes of tonic and clonic movements
with moaning and crying, with no loss of bowel or bladder control. The
neurologist tells you that during the events the EEG had excessive muscle
artifact but no epileptiform discharges. Which of the following treatments
is the most appropriate for this condition?
e. Request a psychiatric evaluation
314. A previously healthy 7-year-old child suddenly complains of a headache
and falls to the floor. When examined in the emergency room (ER), he is
lethargic and has a left central facial weakness and left hemiparesis with conjugate
ocular deviation to the right. Which of the following is the most likely
e. Acute infantile hemiplegia
315. A 5-month-old child was normal at birth, but the family reports that
the child does not seem to look at them any longer. They also report the
child seems to "startle" more easily than he had before. Testing of his white
blood cells (WBCs) identifies the absence of β-hexosaminidase A activity,
confirming the diagnosis of which of the following?
c. Tay-Sachs disease
316. The family of a 4-year-old boy has just moved into your area. The
child was recently brought to the emergency department (ED) for an evaluation
of abdominal pain. Although appendicitis was ruled out in the ED
and the child's abdominal pain has resolved, the ED physician requested
that the family follow up in your office to evaluate an incidental finding of
an elevated creatine kinase. The family notes that he was a late walker
(began walking independently at about 18 months of age), that he is more
clumsy than their daughter was at the same age (especially when trying to
hold onto small objects), and that he seems to be somewhat sluggish when
he runs, climbs stairs, rises from the ground after he sits, and rides his tricycle.
A thorough history and physical examination are likely to reveal
which of the following?
317. A 9-year-old girl is brought by her sister to her pediatrician with the
complaint of severe, intermittent headaches for the past several months,
one of which resulted in her going to the ER. The physical examination
today, including a careful neurologic examination, is normal. The headache
is diffuse, throbbing, lasts several hours, and is not associated with vomiting
or other symptoms. The child cannot feel the headaches coming on; they
appear on all days of the week; and usually the headaches are gone when
she awakens from a nap. The child reports that she is doing well in school,
plays clarinet in the school band, and has "lots of friends." The sister is not
sure, but she thinks their father, who lives in another state, may have
headaches. The most likely explanation for this girl's headache is which of
318. Examination of the cerebrospinal fluid (CSF) of an 8-year-old, mildly
febrile child with nuchal rigidity and intermittent stupor shows the following:
WBCs 85/μL (all lymphocytes), negative Gram stain, protein 150 mg/dL, and
glucose 15 mg/dL. A computed tomographic (CT) scan with contrast shows
enhancement of the basal cisterns by the contrast material. Which of the following
is the most likely diagnosis?
b. Tuberculous meningitis
319. An irritable 6-year-old child has a somewhat unsteady but nonspecific
gait. Physical examination reveals a very mild left facial weakness, brisk
stretch reflexes in all four extremities, bilateral extensor plantar responses
(Babinski reflex), and mild hypertonicity of the left upper and lower extremities;
there is no muscular weakness. Which of the following is the most likely
a. Pontine glioma
320. A 2-year-old boy has been doing well despite his diagnosis of tetralogy
of Fallot. He presented to an outside ER a few days ago with a complaint of an
acute febrile illness for which he was started on a "pink antibiotic." His mother
reports that for the past 12 hours or so he has had a headache and is more
lethargic than normal. On your examination he seems to have a severe
headache, nystagmus, and ataxia. Which of the following would be the most
appropriate first test to order?
d. CT or MRI of the brain
321. A 6-year-old child is hospitalized for observation because of a short
period of unconsciousness after a fall from a playground swing. He has developed
unilateral pupillary dilatation, focal seizures, recurrence of depressed
consciousness, and hemiplegia. Which of the following is the most appropriate
management at this time?
b. CT scan
322. A 6-year-old boy is seen in the office for evaluation of polyuria. Further
questioning reveals several months of headache with occasional emesis.
Your physical examination reveals a child who is less than 5% for weight. He
has mild papilledema. His glucose is normal, and his first urine void specific
gravity after a night without liquids is 1.005 g/mL. Which of the following
might also be expected to be seen in this patient?
e. Bitemporal hemianopsia
323. A 6-year-old boy had been in his normal state of good health until
a few hours prior to presentation to the ER room. His mother reports that
he began to have difficulty walking, and she noticed that he was falling and
unable to maintain his balance. Which of the following is the most likely
cause for his condition?
a. Drug intoxication
324. A 9-year-old child has developed headaches that are more frequent in
the morning and are followed by vomiting. Over the previous few months,
his family has noted a change in his behavior (generally more irritable than
usual) and his school performance has begun to drop. Imaging of this child
is most likely to reveal a lesion in which of the following regions?
325. A young infant is noted to have developed constipation over the past
week, and then facial diplegia and difficulty sucking and swallowing. The
child has been colicky, and the maternal grandmother has been treating the
child with a mixture of weak tea, rice water, and honey. Which of the following
disorders is the most likely culprit in this child?
326. At birth, an infant is noted to have an abnormal neurologic examination.
Over the next few weeks he develops severe progressive central nervous system
(CNS) degeneration, an enlarged liver and spleen, macroglossia, coarse facial
features, and a cherry-red spot in the eye. Which of the following laboratory
findings most likely explains this child's problem?
b. Deficient activity of acid β-galactosidase
327. The parents of a 2-year-old bring her to the emergency center after
she had a seizure. Although the parents report she was in a good state of
health, the vital signs in the emergency center reveal a temperature of 39°C
(102.2°F). She is now running around the room. Which part of the story
would suggest the best outcome in this condition?
b. Otitis media on examination
328. About 12 days after a mild upper respiratory infection, a 12-year-old
boy complains of weakness in his lower extremities. Over several days, the
weakness progresses to include his trunk. On physical examination, he has
the weakness described and no lower extremity deep tendon reflexes, muscle
atrophy, or pain. Spinal fluid studies are notable for elevated protein only.
Which of the following is the most likely diagnosis in this patient?
c. Guillain-Barré syndrome
331. A newborn infant has respiratory distress and trouble feeding in the
nursery. The mother has no significant medical history, but the pregnancy
was complicated by decreased fetal movement. On physical examination,
you note that aside from shallow respirations and some twitching of the
fingers and toes, the infant is not moving, and is very hypotonic. In the
mouth there is pooled saliva and you note tongue fasciculations. Deep tendon
reflexes are absent. Spinal fluid is normal. Appropriate statements about
this condition include which of the following statements?
b. The condition is caused by the degeneration of anterior horn cells in the spinal
332. A 3-year-old boy's parents complain that their child has difficulty
walking. The child rolled, sat, and first stood at essentially normal ages and
first walked at 13 months of age. Over the past several months, however, the
family has noticed an increased inward curvature of the lower spine as he
walks and that his gait has become more "waddling" in nature. On examination,
you confirm these findings and also notice that he has enlargement
of his calves. Which of the following is the most likely diagnosis?
b. Muscular dystrophy
333. Your 6-year-old son awakens at 1:00 AM screaming. You note that he
is hyperventilating, is tachycardic, and has dilated pupils. He cannot be consoled,
does not respond, and is unaware of his environment. After a few
minutes, he returns to normal sleep. He recalls nothing the following morning.
Which of the following is the most likely diagnosis?
b. Night terrors
334. A previously healthy 16-year-old girl presents to the emergency center
with the complaint of "falling out." She was with her friends at a local
fast food restaurant when she felt faint and, according to her friends, lost
consciousness for about a minute. There was no seizure activity noted, but
the friends did notice her arms twitching irregularly. She is now acting normally.
She denies chest pain or palpitations, and her electrocardiogram
(ECG) is normal. Further management of this patient should include
which of the following?
d. Encourage adequate fluid and salt intake
Questions 335 to 337
335. A 15-year-old girl has an acute, recurrent, pulsatile headache localized
behind the eyes that tends to occur more frequently around menses. She has
no symptoms that occur prior to the headache; her neurologic examination
c. Vascular headache (migraine)
336. A 7-year-old boy has chronic, worsening headache without preceding
symptoms. He complains of emesis in the morning before breakfast for the
last 2 weeks.
d. Increased intracranial pressure
337. A 12-year-old boy has chronic headache that worsens during the school
day. These headaches are not associated with nausea or emesis, and he does
not have any symptoms prior to the headache.
a. Tension headache
Questions 338 to 340
338. Eye blinking or throat-clearing noises in an otherwise healthy 8-yearold
a. Transient tic disorder of childhood
340. An 8-year-old hospitalized boy with unusual "spasms" of his neck and
arms shortly after receiving Phenergan for nausea caused by his chemotherapy.
Questions 341 to 343
341. Neurofibromatosis type 1
c. An 18-year-old with a history of fractures and optic gliomas, who now has developed
a malignant schwannoma
343. Tuberous sclerosis
b. An infant with infantile spasms, a hypsarrhythmic EEG pattern, and ash-leaf
depigmentation on her back
345. A 2-month-old child of an HIV-positive mother is followed in your
pediatric practice. Which of the following therapies should be considered for
b. Prophylaxis against Pneumocystis jiroveci pneumonia (Pneumocystis carinii)
346. A 10-month-old infant on long-term aspirin therapy for Kawasaki
disease develops sudden onset of high fever, chills, diarrhea, and irritability.
A rapid swab in your office identifies influenza A, adding her to the long list
of influenza patients you have seen this December. Over the next few days,
she slowly improves and becomes afebrile. However, 5 days after your last
encounter you hear from the hospital that she has presented to the emergency
center obtunded and posturing with evidence of liver dysfunction. Which
of the following statements about her current condition is correct?
e. Death is usually associated with increased intracranial pressures and herniation.
347. An 18-month-old child presents to the emergency center having had
a brief, generalized tonic-clonic seizure. He is now postictal and has a temperature
of 40°C (104°F). During the lumbar puncture (which ultimately
proves to be normal), he has a large, watery stool that has both blood and mucus
in it. Which of the following is the most likely diagnosis in this patient?
348. A 2-year-old child is admitted to your hospital team. The child's primary
care doctor has been following the child for several days and has noted her
to have had high fever, peeling skin, abdominal pain, and a bright red throat.
You are concerned because two common pediatric problems that could explain
this child's condition have overlapping presenting signs and symptoms. Which
of the following statements comparing these two diseases in your differential
d. Pharyngeal culture aids in the diagnosis of one of the conditions.
350. An 8-year-old sickle-cell patient arrives at the emergency room (ER)
in respiratory distress. Over the previous several days, the child has become
progressively tired and pale. The child's hemoglobin concentration in the
ER is 3.1 mg/dL. Which of the following viruses commonly causes such a
b. Parvovirus B19
351. A 10-year-old boy was healthy until about 10 days ago when he developed
7 days of fever, chills, severe muscle pain, pharyngitis, headache, scleral
injection, photophobia, and cervical adenopathy. After 7 days of symptoms
he seemed to get better, but yesterday he developed fever, nausea, emesis,
headache and mild nuchal rigidity. Cerebrospinal fluid (CSF) shows 200 white
blood cells (WBC) per microliter (all monocytes) and an elevated protein.
Correct statements about this infection include which of the following?
c. Most cases are mild or subclinical
352. A previously healthy 8-year-old boy has a 3-week history of low-grade
fever of unknown source, fatigue, weight loss, myalgia, and headaches. On
repeated examinations during this time, he is found to have developed a heart
murmur, petechiae, and mild splenomegaly. Which of the following is the
most likely diagnosis?
353. A 15-month-old boy is brought to the ER because of fever and a rash.
Six hours earlier he was fine, except for tugging on his ears; another physician
diagnosed otitis media and prescribed amoxicillin. During the interim period,
the child has developed an erythematous rash on his face, trunk, and extremities.
Some of the lesions, which are of variable size, do not blanch on pressure.
The child is now very irritable, and he does not interact well with the
examiner. Temperature is 39.5°C (103.1°F). He continues to have injected,
immobile tympanic membranes, but you are concerned about his change
in mental status. Which of the following is the most appropriate next step
in the management of this infant?
e. Perform a lumbar puncture
354. The 3-year-old sister of a newborn baby develops a cough diagnosed
as pertussis by nasopharyngeal culture. The mother gives a history of having
been immunized as a child. Which of the following is a correct statement
regarding this clinical situation?
d. Erythromycin should be administered to the infant.
355. A 14-year-old boy is seen in the ER because of a 3-week history of fever
between 38.3°C and 38.9°C (101°F and 102°F), lethargy, and a 2.7-kg (6-lb)
weight loss. Physical examination reveals marked cervical and inguinal adenopathy,
enlarged tonsils with exudate, small hemorrhages on the soft palate,
a WBC differential that has 50% lymphocytes (10% atypical), and a palpable
spleen 2 cm below the left costal margin. Which of the following therapies
should be initiated?
e. Avoidance of contact sports
357. A 16-day-old infant presents with fever, irritability, poor feeding, and
a bulging fontanelle. Spinal fluid demonstrates gram-positive cocci. Which
of the following is the most likely diagnosis?
c. Group B streptococci
358. A 16-year-old boy presents to the emergency center with a 2-day history
of an abscess with spreading cellulitis. While in the emergency center, he
develops a high fever, hypotension, and vomiting with diarrhea. On examination
you note a diffuse erythematous macular rash, injected conjunctiva
and oral mucosa, and a strawberry tongue. He is not as alert as when he first
arrived. This rapidly progressive symptom constellation is likely caused by
which of the following disease processes?
b. TSST-1-secreting S aureus
359. A 14-month-old infant suddenly develops a fever of 40.2°C (104.4°F).
Physical examination shows an alert, active infant who drinks milk eagerly.
No physical abnormalities are noted. The WBC count is 22,000/μL with
78% polymorphonuclear leukocytes, 18% of which are band forms. Which
of the following is the most likely diagnosis?
a. Pneumococcal bacteremia
360. A 21-year-old woman has just delivered a term infant. She has had
only one visit to her obstetrician, and that was at about 6 weeks of pregnancy.
She provides her laboratory results from that visit. The delivered infant is
microcephalic, has cataracts, a heart murmur, and hepatosplenomegaly. Your
further evaluation of the child demonstrates thrombocytopenia, mild hemolytic
anemia, and, on the echocardiogram, patent ductus arteriosus and peripheral
pulmonary artery stenosis. Which of the following maternal laboratory tests
done at 6 weeks gestation is likely to explain the findings in this child?
c. Negative rubella titer
361. The parents of a 7-day-old infant bring her to your office for a swollen
eye. Her temperature has been normal, but for the last 2 days she has had
progressive erythema and swelling over the medial aspect of the right lower
lid near the punctum. Her sclera and conjunctiva are clear. Gentle pressure
extrudes a whitish material from the punctum. Which of the following
ophthalmic conditions is the correct diagnosis?
362. The parents of a 3-year-old patient followed in your clinic recently
took their child on quickly planned 5-day trip to Africa to visit an ill grandparent.
Everyone did well on the trip, but since their return about 10 days
ago the boy has been having intermittent, spiking fevers associated with
headache, sweating, and nausea. The parents had not been too concerned
since he was relatively well, except for being tired, between the fevers. Today,
however, they feel that he looks a bit pale and his eyes appear "yellow."
Which of the following is likely to reveal the source of his problem?
b. Complete blood count (CBC) with smear
364. A 14-year-old girl awakens with a mild sore throat, low-grade fever,
and a diffuse maculopapular rash. During the next 24 hours, she develops
tender swelling of her wrists and redness of her eyes. In addition, her physician
notes mild tenderness and marked swelling of her posterior cervical and
occipital lymph nodes. Four days after the onset of her illness, the rash has
vanished. Which of the following is the most likely diagnosis?
365. A 4-year-old child presents in the clinic with an illness notable for swelling
in front of and in back of the ear on the affected side, as well as altered taste sensation.
Correct statements about this condition include which of the following?
c. Involvement of the central nervous system (CNS) may occur 10 days after the
resolution of the swelling.
366. A bat is found in the bedroom of a 4-year-old patient while the boy
is sleeping. The family and the patient deny close contact with or bites from
the bat. Which of the following is a correct statement regarding this situation?
c. The patient should be started on the rabies vaccine series.
367. An 8-year-old Cub Scout who returned from an outing 9 days ago is
brought to the clinic with the rapid onset of fever, headache, muscle pain,
and rash. The maculopapular rash began on the flexor surfaces of the wrist
and has become petechial as it spread inward to his trunk. Which of the
following is the most likely diagnosis?
e. Rocky Mountain spotted fever
368. The parents of a 7-month-old boy arrive in your office with the child
and a stack of medical records for a second opinion. The boy first started
having problems after his circumcision in the nursery when he had prolonged
bleeding. Studies were sent at the time for hemophilia, but factor
VIII and IX activity were normal. At 2 months he developed bloody diarrhea,
which his doctor assumed was a milk protein allergy and changed
him to soy; his parents note he still has occasional bloody diarrhea. He has
seen a dermatologist several times for eczema, and he has been admitted to
the hospital twice for pneumococcal bacteremia. During both admissions,
the parents were told that the infant's platelet count was low, but they have
yet to attend the hematology appointment arranged for them. The child's
WBC count and differential were normal. Which of the following is the most
likely diagnosis in this child?
b. Wiskott-Aldrich syndrome
369. A 10-year-old boy from the Connecticut coast is seen because of discomfort
in his right knee. He had a large, annular, erythematous lesion on
his back that disappeared 4 weeks prior to the present visit. His mother recalls
pulling a small tick off his back. Which of the following is a correct statement
about this child's likely illness?
c. In addition to skin and joint involvement, CNS and cardiac abnormalities may
370. Two weeks ago, a 5-year-old boy developed diarrhea, which has persisted
to the present time despite dietary management. His stools have been watery,
pale, and frothy. He has been afebrile. Microscopic examination of his stools
is likely to show which of the following?
372. An 8-year-old immigrant from rural Central America presents with
complaints of weakness, facial swelling, muscle pain, and fever. A CBC reveals
marked eosinophilia. Which of the following parasites is most likely to be
e. Trichinella spiralis
373. A patient presents to the emergency center with a 6-hour history of
fever to 38.9°C (102°F). Her mother reports that the patient appeared to
be feeling poorly, that she had been eating less than normal, and that she
vomited once. About 2 hours prior to arrival at the ER, the mother states
that she noted a few purple spots scattered about the body on the patient,
especially on the buttocks and legs. On the 30-minute ride to the ER, the
purple areas spread rapidly and became coalesced in areas, and the patient
is now obtunded. Which of the following is the most likely diagnosis?
374. A 2-month-old infant comes to the emergency center with fever for
2 days, emesis, a petechial rash, and increasing lethargy. In the ambulance
he had a 3-minute generalized tonic/clonic seizure that was aborted with
lorazepam. He does not respond when blood is drawn or when an IV is
placed, but he continues to ooze blood from the skin puncture sites. On
examination, his anterior fontanelle is open and bulging. His CBC shows a
WBC of 30,000 cells/μL with 20% band forms. Which of the infant's problems
listed below is a contraindication to lumbar puncture?
a. Uncorrected bleeding diathesis
375. The mother of one of your regular patients calls your office. She reports
that her daughter has a 3-day history of subjective fever, hoarseness, and a
bad barking cough. You arrange for her to be seen in your office that morning.
Upon seeing this child, you would expect to find which of the following?
c. Infection with parainfluenza virus
Questions 380 to 385
380. An 18-year-old college girl with an extremely sore throat and high
fever who develops a rash upon administration of ampicillin
i. Epstein-Barr virus
381. Foot puncture wound through a tennis shoe of an adolescent exploring
a construction site
382. Warm, red, tender axillary lymph nodes in a 5-year-old girl; she has
a few red papules on her hand at the site of a feline scratch
a. Bartonella henselae
383. The appearance of an evanescent, erythematous, maculopapular rash
following the rapid defervescence of several days of high fever in a 9-monthold
d. Human herpesvirus 6
385. Several weeks' history of epigastric and periumbilical pain that worsens
with fasting and is relieved by eating, in a 9-year-old with guaiac-positive stools
f. Helicobacter pylori
Questions 386 to 388
386. Culture on sorbitol MacConkey medium
d. A toddler with bloody diarrhea, anemia, and acute renal failure
387. Culture on modified Thayer-Martin medium
c. An infant born at home 2 days ago who has profuse purulent drainage from
both eyes, lid edema, and chemosis
388. Dark field microscopy
a. A 15-year-old sexually active girl with a single non-tender lesion on her labia
and regional nontender lymphadenopathy
Questions 389 to 392
389. A 6-week-old infant with tachypnea and history of eye discharge at
2 weeks of age.
c. Chlamydial pneumonia
390. A 14-year-old girl with low-grade fever, cough of 3 weeks' duration,
and interstitial infiltrate.
a. Mycoplasmal pneumonia
391. A 2-month-old boy with a 3-day history of upper respiratory infection who
suddenly develops high fever, cough, and respiratory distress; within 48 hours,
the patient has developed a pneumatocele and a left-sided pneumothorax.
g. Staphylococcal pneumonia
392. An 8-year-old girl with fever, tachypnea, and lobar infiltrate. She has
failed outpatient therapy of amoxicillin, has developed empyema, and has
had to have chest tubes placed.
b. Pneumococcal pneumonia
393. Two weeks after a viral syndrome, a 2-year-old child develops bruising
and generalized petechiae, more prominent over the legs. No hepatosplenomegaly
or lymph node enlargement is noted. The examination is otherwise
unremarkable. Laboratory testing shows the patient to have a normal hemoglobin,
hematocrit, and white blood cell (WBC) count and differential. The
platelet count is 15,000/μL. Which of the following is the most likely diagnosis?
c. Idiopathic (immune) thrombocytopenic purpura (ITP)
394. An 11-month-old African American boy has a hematocrit of 24% on
a screening laboratory done at his well-child checkup. Further testing demonstrates:
hemoglobin 7.8 g/dL; hematocrit 22.9%; leukocyte count 12,200/μL
with 39% neutrophils, 6% bands, 55% lymphocytes; hypochromia on smear;
free erythrocyte protoporphyrin (FEP) 114 μg/dL; lead level 6 μg/dL whole
blood; platelet count 175,000/μL; reticulocyte count 0.2%; sickle-cell preparation
negative; stool guaiac-negative; and mean corpuscular volume (MCV)
64 fL. Which of the following is the most appropriate recommendation?
b. Oral ferrous sulfate
395. The parents of a previously healthy 2-year-old child note her to be
pale and bring her to your clinic for evaluation. She currently has no fever,
nausea, emesis, bone pain, or other complaints. Her examination is significant
for pallor, tachycardia, and a systolic ejection murmur, but she has no
organomegaly. Her complete blood count (CBC) reveals a hemoglobin of 4 g/dL,
normal indices for age, a WBC count of 6.5/μL, and a platelet count of
750,000/μL. Her reticulocyte count is 0%. Coombs test is negative. Her
peripheral blood smear shows no blast forms and no fragments. Red blood
cell (RBC) adenosine deaminase levels are normal. A bone marrow reveals
markedly decreased erythroid precursors. Which of the following is this child's
e. Transient erythroblastopenia of childhood
396. On a routine-screening CBC, a 1-year-old is noted to have a microcytic
anemia. A follow-up hemoglobin electrophoresis demonstrates an increased
concentration of hemoglobin A2. The child is most likely to have which of
b. β-Thalassemia trait
397. After being delivered following a benign gestation, a newborn infant is
noted to have a platelet count of 35,000/μL, decreased fibrinogen, and elevated
fibrin spilt products. On examination you note a large cutaneous hemangioma
on the abdomen that is purple and firm. Which of the following
anomalies might also be expected in this infant?
a. Kaposiform hemangioendothelioma
398. A 4-year-old previously well African American boy is brought to the
office by his aunt. She reports that he developed pallor, dark urine, and
jaundice over the past few days. He stays with her, has not traveled, and has
not been exposed to a jaundiced person, but he is taking trimethoprim sulfamethoxazole
for otitis media. The CBC in the office shows a low hemoglobin
and hematocrit, while his "stat" serum electrolytes, blood urea nitrogen (BUN),
and chemistries are remarkable only for an elevation of his bilirubin levels.
His aunt seems to recall his 8-year-old brother having had an "allergic reaction"
to aspirin, which also caused a short-lived period of anemia and jaundice.
Which of the following is the most likely cause of this patient's symptoms?
e. Glucose-6-phosphate dehydrogenase deficiency
399. Your sister who lives in another state sends via e-mail photographs of
her 6-month-old infant. You note the child has a white reflection from one of
his eyes. You hastily assist in arranging an urgent pediatric ophthalmologic
evaluation. Your sister immediately accesses the Internet and begins to ask
questions of you. Which of the following statements found by your sister is
b. Cure rates for retinoblastoma treated in the United States exceed 90%.
400. A 2950-g (6.5-lb) black baby boy is born at home at term. On arrival at
the hospital, he appears pale, but the physical examination is otherwise normal.
Laboratory studies reveal the following: mother's blood type A, Rh-positive;
baby's blood type O, Rh-positive; hematocrit 38%; and reticulocyte count
5%. Which of the following is the most likely cause of the anemia?
a. Fetomaternal transfusion
401. A father brings his 3-year-old daughter to the emergency center after
noting her to be pale and tired and with a subjective fever for several days.
Her past history is significant for an upper respiratory infection 4 weeks prior,
but she had been otherwise healthy. The father denies emesis or diarrhea,
but does report his daughter has had leg pain over the previous week, waking
her from sleep. He also reports that she has been bleeding from her gums
after brushing her teeth. Examination reveals a listless pale child. She has
diffuse lymphadenopathy with splenomegaly but no hepatomegaly. She has
a few petechiae scattered across her face and abdomen and is mildly tender
over her shins, but does not have associated erythema or joint swelling.
A CBC reveals a leukocyte count of 8,000/μL with a hemoglobin of 4 g/dL and
a platelet count of 7,000/μL. The automated differential reports an elevated
number of atypical lymphocytes. Which of the following diagnostic studies
is the most appropriate next step in the management of this child?
e. Bone marrow biopsy
402. While bathing her newly-received 2-year-old son, a foster mother feels a
mass in his abdomen. A thorough medical evaluation of the child reveals
aniridia, hypospadias, horseshoe kidney, and hemihypertrophy. Which of
the following is the most likely diagnosis for this child?
b. Wilms tumor
403. A healthy 1-year-old child comes to your office for a routine checkup
and for immunizations. His parents have no complaints or concerns. The next
day, the CBC you performed as customary screening for anemia returns with
the percentage of eosinophils on the differential to be 30%. Which of the
following is the most likely explanation?
d. Helminth infestation
405. A male infant was found to be jaundiced 12 hours after birth. At 36 hours
of age, his serum bilirubin was 18 mg/dL, hemoglobin concentration was
12.5 g/dL, and reticulocyte count 9%. Many nucleated RBCs and some spherocytes
were seen in the peripheral blood smear. The differential diagnosis
should include which of the following?
b. Hereditary spherocytosis
406. On a routine well-child examination, a 1-year-old boy is noted to be
pale. He is in the 75th percentile for weight and the 25th percentile for length.
Results of physical examination are otherwise normal. His hematocrit is 24%.
The answer to which of the following questions is most likely to be helpful
in making a diagnosis?
a. What is the child's usual daily diet?
407. A 10-year-old boy is admitted to the hospital because of bleeding.
Pertinent laboratory findings include a platelet count of 50,000/μL, prothrombin
time (PT) of 15 seconds (control 11.5 seconds), activated partial thromboplastin
time (aPTT) of 51 seconds (control 36 seconds), thrombin time (TT)
of 13.7 seconds (control 10.5 seconds), and factor VIII level of 14% (normal
38%-178%). Which of the following is the most likely cause of his bleeding?
c. Disseminated intravascular coagulation (DIC)
408. A 17-year-old adolescent comes to your office seeking help for "heavy"
menses. Your review of systems also reveals weekly epistaxis. Her only significant
past history includes a tonsillectomy at age 6 after which she required
blood transfusion for excessive bleeding. Her family history includes several
people who seem to bleed and bruise more easily than others. The patient's
mother required a hysterectomy after child birth for excessive hemorrhage.
You order a variety of laboratory tests. The patient has a hemoglobin of
6.5 mg/dL with an MCV of 60%; her platelet count is 350,000/μL. Her von
Willebrand antigen and her von Willebrand factor (vWF) activity (ristocetin
cofactor activity) are decreased. Her vWF is reported as normal but in
decreased amounts. You have been unable to reach her to report the findings,
but when she calls about 1 week later she reports she is having a mild to
moderate nosebleed. You initiate therapy with which of the following?
d. Desmopressin (DDAVP)
410. An otherwise healthy 17-year-old complains of swollen glands in his
neck and groin for the past 6 months and an increasing cough over the previous
2 weeks. He also reports some fevers, especially at night, and possibly some
weight loss. On examination, you notice that he has nontender cervical,
supraclavicular, axillary, and inguinal nodes, no hepatosplenomegaly, and
otherwise looks to be fairly healthy. Which of the following would be the
appropriate next step?
d. Chest radiograph
416. A 6-month-old infant has been exclusively fed a commercially available
infant formula. Upon introduction of fruit juices, however, the child
develops jaundice, hepatomegaly, vomiting, lethargy, irritability, and seizures.
Tests for urine-reducing substances are positive. Which of the following is
likely to explain this child's condition?
c. Hereditary fructose intolerance
417. A 12-year-old healthy girl has some dizziness at synagogue. In your
office you find her to have a hemoglobin of 8 mg/dL, a white blood cell
(WBC) count of 4000/μL, and a platelet count of 98,000/μL. Physical examination
reveals an enlarged spleen. An x-ray of the femur is described as
"appearing to be an Erlenmeyer flask." Bone marrow examination shows
abnormal cells. The diagnosis can be confirmed by measurement of activity
of which of the following?
418. An infant is born to a woman who has received very little prenatal
care. The mother is anxious, complains of heat intolerance and fatigue, and
reports that she has not gained much weight despite having an increased
appetite. On examination the mother is tachycardic, has a tremor, and has
fullness in her neck and in her eyes. The infant is most likely at risk for
development of which of the following?
b. Heart failure
419. An otherwise healthy 7-year-old girl is brought to your office by her
father because she has some acne, breast development, and fine pubic hair.
Which of the following is the most likely etiology for her condition?
e. Early onset of "normal" puberty (constitutional)
420. The parents of a 14-year-old boy are concerned about his short
stature and lack of sexual development. By history, you learn that his birth
weight and length were 3 kg and 50 cm, respectively, and that he had a normal
growth pattern, although he was always shorter than children his age.
The physical examination is normal and his growth curve is shown on the
next page. His upper-to-lower segment ratio is 0.98. A small amount of fine
axillary and pubic hair is present. There is no scrotal pigmentation; his
testes measure 4.0 cm3 and his penis is 6 cm in length. In this situation,
which of the following is the most appropriate course of action?
e. Reassure the parents that the boy is normal
421. Friends are considering adopting a "special needs" child from
another country. The family has few details, but the information they have
received so far suggests the 4-year-old child has had surgery for an endocardial
cushion defect, is short for his age, and had a history of what
sounds like surgically repaired duodenal atresia at birth. You are suspicious
this child may have which of the following syndromes?
423. During a routine well-child examination a 10-year-old girl reports
that she has occasional headache, "racing heart," abdominal pain, and dizziness.
Her mother states that she has witnessed one of the episodes, which
occurred during an outing at the mall, and reported the child to be pale and
to have sweating as well. Other than some hypertension, she has a normal
physical examination. Evaluation of this child is most likely to result in
which of the following diagnoses?
425. A 6-year-old boy is brought to your practice by his paternal grandmother
for his first visit. She has recently received custody of him after his
mother entered the penal system in another state; she does not have much
information about him. You note that the child is short for his age, has
downslanting palpebral fissures, ptosis, low-set and malformed ears, a broad
and webbed neck, shield chest, and cryptorchidism. You hear a systolic ejection
murmur in the pulmonic region. His grandmother reports that he does
well in regular classes, but has been diagnosed with learning disabilities and
receives speech therapy for language delay. His constellation of symptoms is
suggestive of which of the following?
a. Noonan syndrome
426. The parents of a 1-month-old infant bring him to the emergency center
in your local hospital for emesis and listlessness. Both of his parents
wanted a natural birth, so he was born at home and has not yet been to see
a physician. On examination, you find a dehydrated, listless, and irritable
infant. Although you don't have a birth weight, the parents do not feel that
he has gained much weight. He has significant jaundice. His abdominal
examination is significant for both hepatomegaly and splenomegaly. Laboratory
values include a total bilirubin of 15.8 mg/dL and a direct bilirubin
of 5.5 mg/dL. His liver function tests are elevated and his serum glucose is
38 mg/dL. Serum ammonia is normal. A urinalysis is negative for glucose,
but it has a "mouse-like" odor. These findings are consistent with which of
the following conditions?
427. A 2-month-old boy is admitted to the hospital for failure to thrive.
You note him to have fatty stools, and consider cystic fibrosis in your differential
diagnosis. You order pilocarpine iontophoresis, but the laboratory
calls to say they could not collect enough sweat from the infant. Which of
the following is another way to make the diagnosis of cystic fibrosis?
d. DNA testing for cystic fibrosis transmembrane regulator (CFTR) mutations
428. An otherwise healthy 7-year-old child is brought to you to be evaluated
because he is the shortest child in his class. Careful measurements of
his upper and lower body segments demonstrate normal body proportions
for his age. Which of the following disorders of growth should remain in
d. Growth hormone deficiency
429. The state laboratory calls your office telling you that a newborn
infant, now 8 days old, has an elevated thyroid stimulating hormone (TSH)
and low thyroxin (T4) on his newborn screen. If this condition is left
untreated, the infant is likely to demonstrate which of the following in the
first few months of life?
d. Prolonged jaundice
430. A 1-year-old boy presents with the complaint from his parents of
"not developing normally." He was the product of an uneventful term pregnancy
and delivery, and reportedly was normal at birth. His previous
health-care provider noted his developmental delay, and also noted that the
child seemed to have an enlarged spleen and liver. On your examination,
you confirm the developmental delay and the hepatosplenomegaly, and
also notice that the child has short stature, macrocephaly, hirsutism, a
coarse facies, and decreased joint mobility. Which of the following is the
most likely etiology of his condition?
e. Hurler syndrome
431. A 13-year-old comes to your office expressing concern about his height.
He had first seen you a year prior for his routine checkup and a preparticipation
sports physical for soccer (see growth curve). Now in the eighth grade, all
of his friends are taller than he is, and he is at a disadvantage on the soccer
field playing against much larger boys. After obtaining height information
from his parents shown here, you order a skeletal bone age radiograph. Which
of the following results would allow you to assure him of an excellent prognosis
for normal adult height?
a. A bone age of 9 years
433. A 12-year-old girl has a solitary thyroid nodule found on routine
examination; she has no symptoms. Which of the following is the most
appropriate next step for this patient?
c. Serum thyroid function tests
435. A 4-year-old child has mental retardation, shortness of stature, brachydactyly
(especially of the fourth and fifth digits), and obesity with round
facies and short neck. The child is followed by an ophthalmologist for subcapsular
cataracts, and has previously been noted to have cutaneous and
subcutaneous calcifications, as well as perivascular calcifications of the basal
ganglia. This patient is most likely to have which of the following features?
c. Elevated concentrations of parathyroid hormone
436. A 15-year-old boy has been immobilized in a double hip spica cast
for 6 weeks after having fractured his femur in a skiing accident. He has
become depressed and listless during the past few days and has complained
of nausea and constipation. He is found to have microscopic hematuria
and a blood pressure of 150/100 mm Hg. Which of the following is
the most appropriate course of action?
c. Collect urine for measurement of the calcium to creatinine ratio
437. An adolescent with type 1 diabetes returns for a follow-up visit after
his annual checkup last week. You note that his serum glucose is elevated,
and his glycosylated hemoglobin (hemoglobin A1C) is 16.7%. This finding
suggests poor control of his diabetes over at least which of the following
d. 2 months
438. A 7-day-old boy is admitted to a hospital for evaluation of vomiting
and dehydration. Physical examination is otherwise normal except for minimal
hyperpigmentation of the nipples. Serum sodium and potassium concentrations
are 120 mEq/L and 9 mEq/L (without hemolysis), respectively;
serum glucose is 40 mg/dL. Which of the following is the most likely diagnosis?
b. Congenital adrenal hyperplasia
439. A 14-year-old boy presents with the complaint of "breast swelling."
The boy reports that he has been in good health and without other problems,
but has noticed over the past month or so that his left breast has been
"achy" and that he has now noticed some mild swelling under the nipple.
He has never seen discharge; the other breast has not been swelling; and he
denies trauma. Your examination demonstrates a quarter-sized area of breast
tissue under the left nipple that is not tender and has no discharge. The right
breast has no such tissue. He has a normal genitourinary examination, and
is Tanner stage 3. Which of the following is the best next course of action?
d. Reassurance of the normalcy of the condition
440. An infant is brought to a hospital because her wet diapers turn black
when they are exposed to air. Physical examination is normal. Urine is positive
both for reducing substance and when tested with ferric chloride. This
disorder is caused by a deficiency of which of the following?
a. Homogentisic acid oxidase
441. An 18-year-old girl has hepatosplenomegaly, an intention tremor,
dysarthria, dystonia, and deterioration in her school performance. She also
developed abnormal urine with excess glucose, protein, and uric acid. She
has a several-year history of elevated liver enzymes of unknown etiology.
Which of the following best explains her condition?
e. Wilson disease
442. A 3-month-old infant without significant past history was brought to
the emergency center by her mother with a generalized tonic-clonic
seizure. She is found to have glucose of 5 mg/dL. After correction of her
hypoglycemia, she is admitted to your service for further evaluation. Several
hours later, her nurse calls to tell you that her bedside glucose check
was now 10 mg/dL. You order laboratory work suggested by the pediatric
endocrinology team and again correct the infant's hypoglycemia. The results
of the laboratory tests you drew include an elevated serum insulin level of
50 μU/mL, and a low IGFBP-1 (plasma insulin-like growth factor binding
protein-1). C-peptide levels are not detectable. Which of the following is
the likely cause of this child's recurrent hypoglycemia?
e. Factitious hypoglycemia
443. An 11-year-old is the tallest child in his sixth grade class. He is thin,
has a high arched palate, mild scoliosis, and joint hyperextensibility. He is
an honor student, and enjoys basketball but must use prescription sports
goggles so he can see the ball on the court. He is likely to have which of the
d. Marfan syndrome
445. A small-for-gestational-age infant is born at 30 weeks' gestation. At
1 hour of age, his serum glucose is noted to be 20 mg/dL (normally greater
than 40 mg/dL). Which of the following is the most likely explanation for
hypoglycemia in this infant?
a. Inadequate stores of nutrients
446. A 1-day-old normal-appearing infant develops tetany and convulsions.
He was born at 34 weeks' gestation with Apgar scores of 2 and 4
(at 1 and 5 minutes, respectively) to a woman whose pregnancy was complicated
by diabetes mellitus and pregnancy-induced hypertension. Which
of the following serum chemistry values is likely to be the explanation for
b. Serum calcium of 6.2 mg/dL
Questions 447 to 451
447. Three boys in a family with hypodense calcification of their bones
and a history of fractures; their condition is unresponsive to dietary changes
or vitamin supplementation
a. Low PO4, normal Ca
448. A short, 4-year-old, mentally retarded child with brachydactyly of
the fourth and fifth digits; obesity with round facies; short neck; subcapsular
cataracts; and cutaneous, subcutaneous, and perivascular calcifications
of the basal ganglia
e. High PO4, low Ca
449. A 4-year-old child with blue sclera and a history of multiple fractures
with minimal trauma
d. Normal PO4, normal Ca
450. A 6-year-old child who complains of numbness and tingling of the
hands, and later develops tonic-clonic seizures
e. High PO4, low Ca
451. An otherwise healthy 18-year-old girl with medullary thyroid carcinoma
d. Normal PO4, normal Ca
Questions 452 to 457
452. A 15-year-old defensive lineman for his high school football team
whose mother reports that his shoulder pads have permanently stained his
g. Type 2 diabetes
457. A 14-year-old boy with hypogonadism and night blindness with
b. Laurence-Moon-Biedl syndrome
Questions 458 to 463
458. A 4-month-old boy with salt-losing 21-hydroxylase deficiency
a. Na+ 118, K+ 7.5
459. An 11-year-old boy with central diabetes insipidus secondary to an
e. Na+ 155, K+ 5.5
463. A 1-year-old girl with glucose-6-phosphatase deficiency (von Gierke
b. Na+ 125, K+ 3.0
Questions 464 to 470
464. A 10-year-old boy with hypermobile joints and poor wound healing
g. Ehlers-Danlos syndrome
465. A 6-year-old girl with cognitive delay, a blowing systolic heart murmur,
short stature, round face, bilateral transpalmar crease, upslanting palpebral
fissures, small ears, and epicanthal folds.
a. Trisomy 21 (Down syndrome)
466. A newborn has low sloping shoulders, right hand attached at elbow
with agenesis of the forearm, cardiac abnormalities, missing chest wall musculature,
and a bifid thumb.
c. Holt-Oram syndrome
467. A 2-year-old boy, less than the 5% for weight and height, is in early
childhood intervention for developmental delay. He has a short nose, thin
upper lip with thin vermillion border, a VSD, and short palpebral fissures
e. Fetal alcohol syndrome
468. A 14-year-old girl with short stature, thick neck, minimal pubertal
development, repaired coarctation of the aorta, and normal intelligence.
f. Turner syndrome
469. A small newborn with a large ventricular septal defect (VSD), clenched
hands, cleft palate, rounded heels, and a horseshoe kidney.
b. Trisomy 18 (Edwards syndrome)
470. A newborn with hypoglycemia, hypocalcemia, and hypoplastic lower
d. Diabetic embryopathy
Questions 471 to 474
471. A 3-year-old boy with myoclonus, ataxia, weakness, and seizures
who has cytochrome oxidase-negative ragged red fibers noted on muscle
a. Mitochondrial inheritance
472. A mentally retarded 4-year-old boy who was noted to be hypotonic
at birth and had failure to thrive in infancy now has a tremendous appetite,
obesity, hypogonadism, and small hands and feet
c. Genomic imprinting
473. A previously normal father of one of your patients, from a family
with members known to have fragile X syndrome, develops ataxia and
e. Triplet repeat expansion disorder
474. A developmentally delayed 2-year-old has bilateral hypopigmented
whorls on the upper extremities
475. A 15-year-old female presents to your office with secondary amenorrhea.
As part of your evaluation, you find that she is pregnant. After informing
her of the pregnancy, you continue to explain that young mothers have a
higher risk of several pregnancy-related complications, including which of
b. Low-birthweight infants
476. A 12-year-old boy has scant, long, slightly pigmented pubic hairs; slight
enlargement of his penis, and a pink, textured, and enlarged scrotum. He is
most likely at which sexual maturation rating (SMR, also called Tanner) stage?
b. SMR 2
477. A 16-year-old boy who is the backup quarterback for the local high
school team is in your office complaining of worsening acne. For the last
few months he has noted more acne and more oily hair. On his examination,
you note gynecomastia and small testicular volume. He is SMR 5. Which of
the following drugs of abuse is the likely explanation for all of his findings?
478. A 15-year-old girl is brought to the pediatric emergency room by the
lunchroom teacher, who observed her sitting alone and crying. On questioning,
the teacher learned that the girl had taken five unidentified tablets after having
had an argument with her mother about a boyfriend of whom the mother
disapproved. Toxicology studies are negative, and physical examination is
normal. Which of the following is the most appropriate course of action?
a. Hospitalize the teenager on the adolescent ward.
479. A 15-year-old girl is seen in your clinic with a sprained ankle, which
occurred the previous day while she was exercising in her room. You realize
that you have not seen her for quite some time, and begin to expand your
examination beyond the ankle. You find relatively minimal swelling on her
right ankle. She has dental decay, especially of anterior teeth and a swollen,
reddened, irritated uvula. She seems to be somewhat hirsute on her arms and
legs, but has thinning of her hair of the head. She has a resting heart rate of
60 beats per minute, and her oral temperature is 35.5°C (96°F). Further questioning
suggests that she has developed secondary amenorrhea. Which of the
following is the most appropriate next step in the management of this girl?
d. Comparison of current and past weights
480. A 17-year-old sexually active girl comes to your office complaining of
acne that is unresponsive to the usual treatment regimen. Physical examination
reveals severe nodulocystic acne of her face, upper chest, and back.
You consider prescribing isotretinoin (Accutane), but you are concerned
about side effects. Reviewing the literature, you find which of the following
to be true about isotretinoin?
a. Its efficacy can be profound and permanent.
482. A 15-year-old athlete is in your office for his annual physical examination
before the start of football season. He has no complaints, has suffered no
injuries, and appears to be physically fit. On his heart examination, you note
a heart rate of 100 beats per minute, and a diffuse point of maximal impulse
(PMI) with a prominent ventricular lift. He has a normal S1 and S2, with an
S4 gallop. He has no murmur sitting, but when he stands you clearly hear
a systolic ejection murmur along the lower left sternal edge and the apex.
For which of the following conditions is this examination most consistent?
e. Hypertrophic cardiomyopathy
483. You are the sideline physician for a local high school football team.
During a district playoff game, the starting quarterback is sacked for a loss
on third down. As the punter heads out onto the field, the quarterback is
slow to come to the sidelines. He seems confused and dazed. Aside from
his confusion, his examination is normal. After 10 minutes, he is lucid and
wants to get back into the game. Based on published guidelines, which of
the following is your correct course of action?
a. Allow the player back in the game
484. A 16-year-old girl, accompanied by her mother, is in your office for a
well-adolescent visit. The mother asks about drug and alcohol abuse. You
explain that the warning signs of abuse include which of the following?
d. Deterioration in personal habits, hygiene, dress, grooming, speech patterns, and
fluency of expression
485. The recent suicide of a well-known high school cheerleader in your
community has generated an enormous amount of community concern
and media coverage. A girl who was close friends with the deceased makes
an appointment and comes in to your office to discuss the event with you.
You ask, and she denies suicidal ideation, but she has many questions about
suicide. Correct statements about adolescent suicide include which of the
c. Those who are successful have a history of a prior attempt or prior serious suicidal
486. A 16-year-old girl is in your office for a preparticipation sports examination.
She plans to play soccer in the fall, and needs her form filled out.
Which of the following history or physical examination findings is usually
considered a contraindication to playing contact sports?
d. Absence of a single eye
487. A 15-year-old boy is in the office for a preparticipation sports physical
examination before he begins playing with the varsity football team at his
school. Although he is a skilled receiver, he will be one of the smallest players
on the field and is concerned about the potential for injury. He asks how to
bulk up. Appropriate advice to increase muscle mass includes which of the
d. Increasing muscle work
488. An 18-year-old male college student is seen in the student health clinic
for urinary frequency, dysuria, and urethral discharge. Which of the following
is likely to explain his condition?
c. Chlamydial urethritis
489. A 19-year-old male college student returns from spring break in Fort
Lauderdale, Florida, with complaints of acute pain and swelling of the scrotum.
Physical examination reveals an exquisitely tender, swollen right testis that
is rather hard to examine. The cremasteric reflex is absent, but there is no
swelling in the inguinal area. The rest of his genitourinary examination appears
to be normal. A urine dip is negative for red and white blood cells. Which
of the following is the appropriate next step in management?
d. Ultrasound of the scrotum
490. A 16-year-old girl presents with lower abdominal pain and fever. On
physical examination, a tender adnexal mass is felt. Further questioning in
private reveals the following: she has a new sexual partner; her periods are
irregular; she has a vaginal discharge. Which of the following is the most likely
b. Tubo-ovarian abscess
491. The parents of a 16-year-old girl complain that she does not get
enough sleep. They recently discovered that she stays awake most nights
until 1:00 AM reading and text messaging her friends. She wakes at 6:30 AM
for school, and complains of sleepiness during the day. On weekends she
sleeps until noon. Her parents have tried taking away her computer and
phone, but she still would go to bed at the same time. The parents are looking
for advice in dealing with their "night owl" daughter. Which of the following
is appropriate advice for this family?
b. Effects of puberty on melatonin cause a phase delay with later sleep onset.
Questions 492 to 495
492. A 16-year-old female has a positive rapid plasma reagin (RPR) of
1:64 and an ulcer on her labia.
a. Dark-field microscopic examination
493. A 19-year-old male has recurrent episodes of painful, erythematous,
small vesicles, and ulcers on his glans penis.
d. Tzanck preparation for multinucleated giant cells
494. A 15-year-old male had a painless papule on his genitals that resolved,
but he has now developed a unilateral draining inguinal lymphadenitis
e. Chlamydia culture
495. A 19-year-old female has a few small papules on her labia and perineum.
The papules become pustular, eroded, and ulcerated over the next
few days; at the same time, the patient develops painful, tender inguinal
b. Special chocolate agar culture
Questions 496 to 500
496. Patellar tendinitis and Osgood-Schlatter disease
c. A 17-year-old starting center for a high school basketball team
497. Injuries almost exclusively related to the shoulder, including rotator
a. A 15-year-old competitive swimmer
499. Hyperextension of the thumb and sprains of the anterior cruciate ligament
g. A 14-year-old snow skier
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