Board Review Week of July 13

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					Board Review Week of July 13
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Question 164
You are asked to consult on a 9-month-old boy who has been hospitalized five times for wheezing.
His history reveals occasional coughing with feedings, but results of a pH probe performed during
his last admission were normal. His weight and height are at the 50th percentile. Except for
scattered wheezes with good aeration bilaterally, results of his physical examination are normal.

Of the following, the test that is MOST likely to reveal the cause of his recurrent wheezing is
    a)   chest computed tomography scan
    b)   immunoglobulin panel
    c)   inspiratory and expiratory chest radiographs
    d)   pulmonary function testing
    e)   videofluoroscopic swallow study

Question 83
A 5-day-old term infant presents to the emergency department with a history of bile-stained
emesis. She is well nourished and hydrated and had an unremarkable course in the newborn
nursery. She was discharged at 48 hours after birth and was breastfeeding, but her mother states
the baby always has vomited. Physical examination reveals an afebrile infant who has normal vital
signs, but no audible bowel sounds on abdominal evaluation. A flat-plate abdominal radiograph
reveals a paucity of bowel gas (Item Q83A).




Of the following, the MOST likely diagnosis is
    a)   anorectal atresia
    b)   cystic fibrosis
    c)   malrotation of the bowel
    d)   septic ileus
    e)   tracheoesophageal fistula

Question 80
A 14-year-old boy presents to the office with a 4-hour history of severe scrotal pain after running in
a track meet. He is pale, nauseous, and in obvious pain. He denies trauma, dysuria, history of fever,
or penile discharge. He is sexually active. On physical examination, his penis appears normal and
without meatal discharge, but the scrotum is swollen bilaterally, and there is significant tenderness
to palpation of both testicles. The testicles appear high in the scrotum, and the cremasteric reflexes
are absent bilaterally.

Of the following, the next MOST appropriate step in the evaluation of this patient is
    a)   abdominal computed tomography scan
    b)   emergent surgical evaluation
    c)   polymerase chain reaction test for Chlamydia
    d)   radionuclide scan of the testicle
    e)   urinalysis

Question 192
You are evaluating a 1-month-old term infant who has persistent jaundice. The parents explain that
his stools were green 2 weeks ago and now are pale yellow. Physical examination findings are
unremarkable, except for a liver that is palpable 2 cm below the costal margin. The infant's total
bilirubin is 6.1 mg/dL (104.3 mcmol/L) and direct bilirubin is 4.2 mg/dL (71.8 mcmol/L). Alanine
aminotransferase is 240 U/L, and aspartate aminotransferase is 160 U/L. A hepatobiliary
iminodiacetic acid (HIDA) nuclear medicine scan demonstrates absence of excretion of tracer into
the bowel (Item Q192A).

Of the following, the MOST definitive diagnostic test to establish the diagnosis is
    a)   intraoperative cholangiography
    b)   magnetic resonance cholangiopancreatography
    c)   measurement of serum alpha-1-antitrypsin
    d)   sweat chloride test
    e)   ultrasonography of the biliary tree

Question 245
You are examining a young boy during a health supervision visit. His mother reports that he says
"mama, " "dada," "bye," "up," and "ball." Following the examination, he sits on the floor in front of
his mother while playing with a toy car. When he sees a jack-in-the-box on a shelf, he points to it.
After being instructed to do so by his mother, he brings the jack-in-the-box to her.

Of the following, these developmental milestones suggest that the child is CLOSEST to
    a)   12 months of age
    b)   15 months of age
    c)   18 months of age
    d)   21 months of age
    e)   24 months of age

Question 250
You observe a child entering the waiting room, accompanied by her mother. She looks at the
receptionist and says "Hi." While holding her doll, the child turns to her mother and says "juice."
The mother gives her a cup of juice, and the child says "doll" and tries to give the doll a drink. The
mother shakes her head, and the child says "no." The child then points to her own mouth, smiles,
and says "mouth." The mother takes a tissue to clean the doll's face. The child says "me" and
begins to imitate her mother's action with another wipe. The child looks at her mother, says "ma
ma," and gives her mother a hug.
Of the following, these developmental milestones are MOST typical for a child whose age is
    a)   12 months
    b)   15 months
    c)   18 months
    d)   24 months
    e)   30 months

Question 231
While examining an infant in the newborn nursery, you note that the pupil of one eye seems
abnormally large, and little of the iris is visible. The baby appears otherwise normal. A subsequent
ophthalmologic evaluation confirms the diagnosis of partial aniridia.

Of the following, the MOST accurate statement regarding the diagnosis is that
    a) aniridia is associated with hepatoblastoma
    b) few individuals who have aniridia have an affected parent
    c) isolated aniridia has an autosomal recessive inheritance pattern
    d) molecular testing is available to determine risk for Wilms tumor
    e) routine abdominal ultrasonography should be performed every 3 months until age 5 years
         in affected individuals

Question 26
You are seeing a 10-year-old girl for her yearly health supervision visit. On physical examination,
you palpate a smooth and symmetric thyroid that seems twice normal size (Item Q26A). There are
no palpable nodules. Serum free thyroxine and thyroid-stimulating hormone (TSH) values are both
normal. Serum thyroperoxidase antibody concentrations are elevated.

Of the following, the initial BEST approach to management is to
    a)   obtain a 123-I thyroid scan
    b)   obtain thyroid ultrasonography
    c)   recheck TSH concentration in 6 months
    d)   start treatment with triiodothyronine
    e)   start treatment with TSH

Question 181
An 8 yo girl is brought in to see you because she is sleeping more, and her grandmother things she might
have a thyroid problem. The grandmother says that the girl’s mother and aunt have both underactive
thyroids that were noticed when they were young adults, and she things her granddaughter might have
the same problem. You measure free thyroxine and TSH levels, which are normal, and anti-
thyroperoxidase antibodies, which are positive.

Of the following, this girl is MOST likely to have
     a) cervical lymphadenopathy
     b) difficulty swallowing
     c) hypoactive reflexes
     d) thyroid pain
     e) thyromegaly

Question 55
A 15 yo boy who has cystic acne has experienced a frontal headache for 1 week. He reports that the only
drug he takes is isotretinoin. Last night he presented to the emergency department for headache. CT of
the head was obtained and was normal; he was given meperidine and discharged home. He presents now
to your office for follow-up. The boy has papilledema, but his physical examination findings are otherwise
normal.

Of the following, the MOST appropriate next step in the evaluation of this patient is
     a) lumbar puncture
     b) magnetic resonance imaging of the brain with gadolinium contrast
     c) neurosurgery consultation
     d) ophthalmology consultation
     e) urine toxicology screen
Question 21
Your region performs newborn screening for congenital adrenal hyperplasia using an assay for 17-
hydroxyprogesterone. You receive a report on a Friday that a 6-day old male infant in your practice has a
newborn screening value for 17-hydroxyprogesterone that is 5 times the normal range. You immediately
call the mother and tell her to bring the baby into your office. On exam, the child has normal genitalia
with descended testes. He has good color and tone and seems to be feeding reasonably well. His
birthweight was 8lbs 6 oz (3.8kg) and now he weights 8 lbs 1 oz (3.6 kg). In addition to serum
electrolytes, the BEST diagnostic test that should be done at this time is
      a) Chromosome analysis
      b) Measurement of serum testosterone
      c) Measurement of urine 17-ketosteroids
      d) Repeat of the 17-hydroxyprogesterone measurement
      e) Ultrasonography of the adrenals



Question 36
A 3 yo girl who has congenital adrenal hyperplasia has been staying with an aunt of the weekend while
her parents are out of town. The aunt calls you because her niece has a temperature of 101F and is
vomiting. The aunt has tried to give her a double dose of her hydrocortisone medication, but she does not
keep it down, and she looks pale and sweaty and has a rapid pulse. The aunt knows that her sister
sometimes needs to use injectable hydrocortisone, but she was left with no supplies. You instruct her to
come to the emergency department and meet her there. The girl is barely responsive by this time. Her
pulse is 120 beats/min, blood pressure is 60/40 mm Hg, and capillary refill is poor. You start IV infusion
with 0.9% NaCL 5% dextrose and order measurements of electrolytes.

Of the following, the MOST definitive therapy is to administer
     a) Cortisone acetate intramuscularly
     b) Dopamine intravenously
     c) Hydrocortisone hemisuccinate and aldosterone intravenously
     d) Hydrocortisone hemisuccinate intravenously
     e) Potassium chloride 40meq/L and hydrocortisone hemisuccinate intravenously

Question 74
A couple seeks genetic counseling after having lost a sone due to salt-losing 21-hydroxylase deficiency
(21-OHD). They ask about prenatal diagnosis and management of this condition.
Of the following the MOST accurate response is that
     a) Dexamethasone administration to the mother should be initiated after the fetus is determined to
         be female
     b) Fetal cells are human lymphocyte antigen typed routinely for linkage analysis
     c) Fetoscopy is suggested for visualization of the fetal genitalia
     d) Measurement of 17-hydroxyprogesterone in amniotic fluid is recommended
     e) Molecular genetic testing of fetal cells is preferred

				
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