A 16-year-old boy has delayed puberty and absence of secondary sexual characteristics; he has decreased sense of smell. Examination reveals shortening of the fourth finger. MRI of the brain shows absence of the olfactory bulbs and sulci; abdominal ultrasonography shows absence of the right kidney. Of the following, the MOST likely diagnosis is
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Question 2 of 62
Episodic symptomatic hypocalcemia occurs in the Kenny-Caffey syndrome, the latter is characterized by the following EXCEPT
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Question 3 of 62
A previously healthy 4-month-old now manifests increasing hypotonia and poor feeding. Physical examination reveals macroglossia, a gallop rhythm and tachycardia, marked flaccidity, but normal mental status. Laboratory studies reveal a blood glucose level of 85 mg/dL and sinus tachycardia with a shortened PR interval on an electrocardiogram. The most helpful diagnostic study would be
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Question 4 of 62
The best diagnostic test for the patient in the previous question is
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Question 5 of 62
The upper-to-lower segment ratio is one of methods for growth assessment that changes with age. Of the following, this ratio becomes 1:1 at the age of
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Question 6 of 62
The normal level of serum thyroid-stimulating hormone in first week of term infants is up to
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Question 7 of 62
The plasma amino acids in the patient in the previous two questions reveal a marked elevation of leucine, isoleucine, and valine. The most likely diagnosis is
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Question 8 of 62
Many patients with Turner syndrome are recognizable at birth because of
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Question 9 of 62
In the classic phenylketonurea, the affected infant is normal at birth, intellectual disability develops gradually if the infant remains untreated, and cognitive delay may not be evident for the first few months. All the following are true about neonatal screening program of this condition EXCEPT
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Question 10 of 62
Metachromatic leukodystrophy, an autosomal recessive white matter disease, is characterized by all of the following EXCEPT
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Question 11 of 62
The antimicrosomal antibodies noted in lymphocytic thyroiditis are central to its etiology but have been renamed
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Question 12 of 62
The glycoproteinoses are a class of disorders that result from the deficiency of specific lysosomal enzymes that are required for the degradation of glycoproteins. Each statement about the glycoproteinoses is true EXCEPT
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Question 13 of 62
A 7-month-old white female presents with severe developmental delay and episodes of vomiting. Physical examination reveals blue eyes, light skin with an eczematoid rash, and hyperactive deep tendon reflexes. There is failure to thrive and microcephaly. The most likely diagnosis is
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Question 14 of 62
Hyponatremia in cerebral salt wasting is accompanied by the following EXCEPT
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Question 15 of 62
In older infants and children, hypoglycemia is defined as whole blood glucose concentration less than
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Question 16 of 62
Matching: Disorder of peroxisomal import
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Question 17 of 62
A 3-year-old girl presented with growth retardation, recurrent attacks of otitis media, diarrhea, and hypoglycemic seizures. On examination, there is hepatomegaly and her absolute neutrophil count (ANC) is 600/μL. Of the following, the MOST likely diagnosis is
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Question 18 of 62
A 15-year-old Ashkenazi Jewish girl is seen because of chronic fatigue. On examination, she seems pale and thin and has a somewhat large abdomen. Her spleen is felt in the iliac fossa. She is mentally alert and has a history of normal development and normal school performance. Her blood count shows hemoglobin of 9.0 g/dL and a white blood cell count of 3000 with normal differential and no abnormal cells. Platelet count is 60,000. The likely diagnosis is
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Question 19 of 62
Acquired hypogonadotropic hypogonadism include
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Question 20 of 62
In mucopolysaccharidosis, disproportionate severe CNS involvement with mild physical features is unique to
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Question 21 of 62
Matching: Giant granules in granulocytes
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Question 22 of 62
Physical findings in Graves disease include all of the following EXCEPT
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Question 23 of 62
Potential causes of central diabetes insipidus include all of the following EXCEPT
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Question 24 of 62
Irreversible complication of untreated galactosemia is
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Question 25 of 62
You are examining a 10-month-old male infant during a routine visit; you find that he has wide wrists. He is still breast fed. X-ray of the wrist joint shows signs of rickets. Of the following, the BEST reflection of vitamin D deficiency is
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Question 26 of 62
Williams syndrome is characterized by
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Question 27 of 62
The MOST common cause of acquired hypopituitarism is
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Question 28 of 62
Enzyme replacement is the main therapy of Hurler disease, it improve all the following EXCEPT
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Question 29 of 62
The normally functioning organ in Hutchinson-Gilford progeria syndrome (progeria) is
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Question 30 of 62
Adverse reaction reported exclusively with propylthiouracil is
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Question 31 of 62
The prognosis of glycogen storage diseases is good in
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Question 32 of 62
All the following are widely used modalities for treatment of inborn errors of metabolism (IEM) EXCEPT
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Question 33 of 62
High-dose glucocorticoids (the equivalent of >10 times physiologic cortisol secretion) can be administered without requiring a subsequent tapering for
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Question 34 of 62
Lipidoses affect many solid organs, optic atrophy is seen in
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Question 35 of 62
The diagnosis of homocystinuria is usually made after 3 yr of age when the ophthalmologist found
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Question 36 of 62
Disorder of carbohydrate-deficient glycoprotein structure are characterized by all of the following EXCEPT
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Question 37 of 62
Transient tyrosinemia of the newborn responds to treatment with
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Question 38 of 62
Autosomal recessive PKD is characterized by which of the following?
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Question 39 of 62
You are going to treat a 7-year-old boy with type 1 diabetes mellitus who develops DKA due to a recent infection. Examination reveals severe dehydration and deep acidotic breathing. Lab investigations shows: blood glucose, 450 mg/dL; blood pH, 7.2; and serum bicarbonate concentration, 14 mEq/L. Which of the following should be avoided during the treatment of this girl?
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Question 40 of 62
All the following tests are of diagnostic utility in the diagnosis of Cushing syndrome EXCEPT
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Question 41 of 62
The MOST accurate test of thyroid function is
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Question 42 of 62
The treatment of the girl in the previous two questions is best accomplished with
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Question 43 of 62
You are evaluating a newborn baby with cleft palate; his mother asks you about any risk of associated congenital hypopituitarism. Of the following, this baby is MOST likely at risk for
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Question 44 of 62
During acute metabolic decompensation, all of the following are appropriate therapy EXCEPT
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Question 45 of 62
The first clinical manifestation of acquired hypothyroidism is
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Question 46 of 62
Noonan syndrome is associated with all of the following EXCEPT
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Question 47 of 62
Microvascular complications of DM include
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Question 48 of 62
The following features suggest constitutional delay as a cause of short stature EXCEPT
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Question 49 of 62
The current criteria for the diagnosis of type 1 and type 2 DM is a fasting blood glucose that exceeds
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Question 50 of 62
Complications of tyrosinemia include all of the following EXCEPT
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Question 51 of 62
The MOST specific and important laboratory finding in X-linked adrenoleukodystrophy (ALD) is elevated level of
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Question 52 of 62
A 10-day-old boy presented with lethargy, poor feeding, repeated vomiting, and recurrent hypoglycemic fits not responding to IV glucose. On examination, he was hypertonic with severe opisthotonos alternating with flaccidity. Of the following, the MOST likely diagnosis is
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Question 53 of 62
A 3-year-old boy presented to emergency unit at 10:00 AM with history of difficult arousal from sleep, lethargy, vomiting, and seizures; the mother stated that he had slept without having his supper. Fasting blood sugar was 38 mg/dL and urinary ketone was low. Of the following, the MOST likely diagnosis is
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Question 54 of 62
Treatment for the infant described in the previous question should include which of the following (choose as many as are appropriate)?
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Question 55 of 62
All the following are recognized feature of Niemann-pick disease type B EXCEPT
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Question 56 of 62
A 7-year-old boy is going to undergo orthopedic surgery for right total hip replacement; he has been diagnosed with Addison disease 2 years ago and he is on replacement therapy by hydrocortisone since that time. Of the following, the MOST important step in the management of this boy is
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Question 57 of 62
Sweaty feet odor is seen in untreated infants with
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Question 58 of 62
All the following drugs are unsafe in acute intermittent porphyria EXCEPT
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Question 59 of 62
Donohue syndrome is characterized by the following EXCEPT
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Question 60 of 62
Matching: Lipidosis - Niemann-Pick
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Question 61 of 62
Glucocorticoids readily penetrate the blood-brain barrier and have direct effects on brain metabolism. They have the following actions EXCEPT
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Question 62 of 62
A 10-day-old manifests profound coma one day after an illness characterized by emesis, poor oral intake, and hypotonia. Laboratory findings reveal 4+ ketonuria, an arterial blood gas of 6.9, PCO2 19, PO2 95, an anion gap of 37, and an absolute neutrophil count of 400. The most likely diagnosis is
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