ATI RN · Pediatric Endocrine System
Question 1 of 62
Matching: Primarily African-American
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ATI RN · Pediatric Endocrine System
Question 2 of 62
Sotos syndrome is characterized by all of the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 3 of 62
The MOST common cause of primary adrenal insufficiency in infancy is
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ATI RN · Pediatric Endocrine System
Question 4 of 62
Hunter disease is characterized by all the following features EXCEPT
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ATI RN · Pediatric Endocrine System
Question 5 of 62
Galactosemia is an autosomal recessive disease characterized by all the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 6 of 62
Type I diabetes mellitus is most often associated with
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ATI RN · Pediatric Endocrine System
Question 7 of 62
The following are counter regulatory hormones which are increased as blood glucose falls EXCEPT
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ATI RN · Pediatric Endocrine System
Question 8 of 62
Matching: Match the disease with its enzyme - Iduronidase
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ATI RN · Pediatric Endocrine System
Question 9 of 62
In insulin-dependent diabetes mellitus, hyperglycemia results when insulin secretion becomes inadequate to enhance peripheral glucose uptake and to suppress hepatic and renal glucose production. Of the following, the sign of more complete insulin deficiency is
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ATI RN · Pediatric Endocrine System
Question 10 of 62
Additional complications that may occur in the patient in the previous question include all of the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 11 of 62
Clinical manifestations of pheochromocytomas include the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 12 of 62
Matching: Lipidosis - Mucolipidosis I
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ATI RN · Pediatric Endocrine System
Question 13 of 62
Thyroid disorders consist of a variety of diseases that require different treatment according to the cause. Of the following, the thyroid disorder that does not require treatment is
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ATI RN · Pediatric Endocrine System
Question 14 of 62
A 9-month-old boy with exclusive breast feeding presented with jaundice, vomiting, lethargy, hepatomegaly, and hypoglycemic fits when sweetened cereal added. Laboratory findings showed prolonged clotting time, hypoalbuminemia, elevation of bilirubin and transaminase levels, and proximal tubular dysfunction. Of the following, the MOST likely diagnosis is
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ATI RN · Pediatric Endocrine System
Question 15 of 62
Matching: Platelet dysfunction
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ATI RN · Pediatric Endocrine System
Question 16 of 62
You suspect a metabolic problem in a 30-day-old girl presented with poor feeding, vomiting, lethargy, and convulsion. Previous sibling died with the same condition. Serum ammonia, pH, HCO3, and anion gap are normal. Of the following, the MOST likely diagnosis is
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ATI RN · Pediatric Endocrine System
Question 17 of 62
Guidelines suggest that diabetic patients should commence screening for celiac disease
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ATI RN · Pediatric Endocrine System
Question 18 of 62
A 5-year-old girl has been developed type 1 diabetes mellitus 6 months ago. She is on intensive control using multiple daily injections of insulin. A recent blood testing of HgbA1c is 6.5%. Of the following, the MOST frequent complication that may be encountered at this age is
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ATI RN · Pediatric Endocrine System
Question 19 of 62
The MOST common presentation of mitochondrial β-oxidation of fatty acids disorders is
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ATI RN · Pediatric Endocrine System
Question 20 of 62
All the following are clinical abnormalities of Zellweger syndrome EXCEPT
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ATI RN · Pediatric Endocrine System
Question 21 of 62
The thyroid hormones are transported in plasma bound to thyroxine-binding globulin (TBG), a glycoprotein synthesized in the liver. TBG binds approximately 70% of T4 and 50% of T3. TBG level increase with administration of
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ATI RN · Pediatric Endocrine System
Question 22 of 62
A relative parents brought their underweight 6-month-old female infant who has high foreheads, flat orbital ridges, widely open fontanelles, hepatomegaly, hypotonia, and nystagmus; she had history of recurrent seizures. Of the following, the MOST likely diagnosis is
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ATI RN · Pediatric Endocrine System
Question 23 of 62
Matching: Infantile multiple carboxylase deficiency
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ATI RN · Pediatric Endocrine System
Question 24 of 62
One of the following is a characteristic feature for both Infantile Sandhoff disease and Tay-Sachs disease
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ATI RN · Pediatric Endocrine System
Question 25 of 62
In children with Down syndrome (DS), the type of leukemia varies by age. In those younger than 2 years, it is generally
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ATI RN · Pediatric Endocrine System
Question 26 of 62
All the following are secondary causes of hypercholesterolemia EXCEPT
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ATI RN · Pediatric Endocrine System
Question 27 of 62
Which of the following porphyrias is NOT associated with cutaneous manifestations?
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ATI RN · Pediatric Endocrine System
Question 28 of 62
True gynecomastia is characterized by the presence of a palpable fibroglandular mass (located concentrically beneath the nipple and areolar region) at least
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ATI RN · Pediatric Endocrine System
Question 29 of 62
Any child with diabetic ketoacidosis (DKA) can be easily transitioned to oral intake and subcutaneous insulin when the following criteria are found EXCEPT
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ATI RN · Pediatric Endocrine System
Question 30 of 62
Hall Pallister syndrome includes all of the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 31 of 62
Which of the following mucopolysaccharidoses is susceptible to life-threatening atlantoaxial instability and dislocation?
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ATI RN · Pediatric Endocrine System
Question 32 of 62
A 16-year-old male has a tall stature but no facial, axillary, or pubic hair. His penis and scrotum are obscured by pubic adipose tissue, but they appear infantile. Chromosome analysis reveals XY karyotype. His serum follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels are elevated. The most likely diagnosis is
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ATI RN · Pediatric Endocrine System
Question 33 of 62
Nocturnal enuresis is best described as
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ATI RN · Pediatric Endocrine System
Question 34 of 62
Chronic lymphocytic thyroiditis is the most common cause of acquired hypothyroidism, with or without goiter. All the following are true EXCEPT
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ATI RN · Pediatric Endocrine System
Question 35 of 62
Parathyroid crisis is manifested by the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 36 of 62
You are discussing short stature (SS) with medical students. You mention that congenital growth hormone deficiency is one of common causes of SS. An important statement that should be included with your discussion is
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ATI RN · Pediatric Endocrine System
Question 37 of 62
Reported side effects of GH treatment include the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 38 of 62
Diabetes insipidus is associated with all of the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 39 of 62
The following criteria are needed for the diagnosis of syndrome of inappropriate secretion of antidiuretic hormone (SIADH) EXCEPT
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ATI RN · Pediatric Endocrine System
Question 40 of 62
The 47,XXX (trisomy) chromosomal constitution is the most frequent extra-X chromosome abnormality in females, occurring in almost 1 in 1,000 liveborn females. It is characterized by the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 41 of 62
A 3-week-old neonate presented with poor feeding, vomiting, and tachypnea with profound depression of the central nervous system, associated with alternating hypotonia and hypertonia, opisthotonos, and seizures. Laboratory manifestations reveal hypoglycemia and metabolic acidosis with high anion gap. Of the following, the MOST likely diagnosis is
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ATI RN · Pediatric Endocrine System
Question 42 of 62
Precocious puberty in girls is defined by the onset of secondary sexual characteristics before the age of
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ATI RN · Pediatric Endocrine System
Question 43 of 62
All of the following are present in children with homozygous familial hypercholesterolemia EXCEPT
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ATI RN · Pediatric Endocrine System
Question 44 of 62
When diabetes and thyroid disease coexist, the possibility of autoimmune adrenal insufficiency should be considered. It may be heralded by the followings EXCEPT
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ATI RN · Pediatric Endocrine System
Question 45 of 62
Hypocalcemia is common in neonates between 12 and 72 hr of life, especially in infants with
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ATI RN · Pediatric Endocrine System
Question 46 of 62
A 10-month-old Ashkenazi Jewish female manifests hypotonia and macrocephaly. By 18 months of age, she has hyperreflexia and optic atrophy. Additional problems include failure to thrive and swallowing difficulties. MRI demonstrates diffuse white matter degeneration of the cerebral cortex. The most likely diagnosis is
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ATI RN · Pediatric Endocrine System
Question 47 of 62
All of the characteristics of congenital hypothyroidism are true EXCEPT
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ATI RN · Pediatric Endocrine System
Question 48 of 62
The least common presentation of acute intermittent porphyria is
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ATI RN · Pediatric Endocrine System
Question 49 of 62
During a routine physical examination, a solitary thyroid nodule is palpated in asymptomatic 10-year-old child. Of the following, the MOST likely cause is
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ATI RN · Pediatric Endocrine System
Question 50 of 62
Levothyroxine (I-T4) given orally is the treatment of choice in congenital hypothyroidism. The recommended initial starting dose is
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ATI RN · Pediatric Endocrine System
Question 51 of 62
The following factors are implicated in the pathogenesis of type 1 DM EXCEPT
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ATI RN · Pediatric Endocrine System
Question 52 of 62
The following are ocular manifestations of albinism EXCEPT
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ATI RN · Pediatric Endocrine System
Question 53 of 62
Dietary restriction of phenylalanine during pregnancy to serum levels of 10 mg/dL prevents all of the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 54 of 62
Niemann-pick disease type A is characterized by all the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 55 of 62
Rickets is defined as decreased bone mineralization in growing children. Hypocalcemia may accompany rickets. Of the following, the condition that causes hypocalcemia rather than rickets is
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ATI RN · Pediatric Endocrine System
Question 56 of 62
The parents of a 2-year-old boy with Tay-Sachs disease ask you about the availability of prenatal testing in their pregnancy. When counseling this couple about prenatal diagnosis, it would be most appropriate to include which one of the following statements:
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ATI RN · Pediatric Endocrine System
Question 57 of 62
In von Gierke disease; treatment is designed to maintain normal blood glucose levels which is achieved by all the following EXCEPT
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ATI RN · Pediatric Endocrine System
Question 58 of 62
A 4-month-old boy presented with failure to thrive, rapid breathing, and repeated vomiting. On examination, there is hepatomegaly and abdominal sonography revealed renal calcification. Of the following, the MOST likely urine odor of this infant is
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ATI RN · Pediatric Endocrine System
Question 59 of 62
Matching: Glycogen storage diseases - Pompe
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ATI RN · Pediatric Endocrine System
Question 60 of 62
The signs and symptoms of congenital adrenal hyperplasia (salt wasting form of the disease) caused by 21-hydroxylase deficiency typically first develop in affected infants at approximately
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ATI RN · Pediatric Endocrine System
Question 61 of 62
A 1-day-old presents with failure to suck, hypotonia, lethargy, and refractile myoclonic seizures. The infant is normocephalic and laboratory studies reveal normal serum pH and glucose, calcium, ammonia, and electrolyte levels. The birth history is unremarkable, and the Apgar score was 9 and 9. Hyperglycinemia is present in a mild to moderate range. The next most appropriate test is
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ATI RN · Pediatric Endocrine System
Question 62 of 62
The initial treatment of choice for Graves disease is
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