ATI RN
Endocrine System in Pediatrics Questions
Question 1 of 5
Therapy with one of the following drugs usually results in significant insulin resistance leading to glucose intolerance and overt diabetes
Correct Answer: C
Rationale: High-dose steroids (C) commonly cause insulin resistance and diabetes.
Question 2 of 5
A 10-month-old boy presented with failure to thrive, fever, jaundice, hepatomegaly, and severe rickets. Investigations revealed hypoglycemia and normal anion gap metabolic acidosis. Of the following, the MOST likely enzyme deficiency is
Correct Answer: D
Rationale: Fumarylacetoacetate hydrolase deficiency (tyrosinemia type 1) causes failure to thrive, jaundice, hepatomegaly, rickets, hypoglycemia, and normal anion gap acidosis from renal tubular dysfunction, unlike other deficiencies (A-D).
Question 3 of 5
A 6-year-old tall and thin boy who had failed preparatory exam for school entry found to have myopia and subluxation of the ocular lens. Of the following, the MOST important challenge is to know whether he is responsive to
Correct Answer: D
Rationale: Homocystinuria, suggested by tall stature, lens subluxation, and myopia, responds to vitamin B6 (pyridoxine) in some cases, critical for management, unlike other vitamins (A, B, C, E).
Question 4 of 5
A 12-year-old boy presented with epistaxis, mild bruising, intermittent bone pain, and massive splenomegaly. Complete blood count shows thrombocytopenia and mild anemia. Radiological study revealed lytic lesions and Erlenmeyer flask deformity of the distal femur. Of the following, the MOST likely diagnosis is
Correct Answer: A
Rationale: Gaucher disease presents with splenomegaly, bone pain, cytopenias, and Erlenmeyer flask deformity from glucocerebroside accumulation, unlike others (B, C, D, E).
Question 5 of 5
A 7-month-old boy presented with recurrent attacks of hypoglycemic seizures especially during an acute illness. On examination, there are thin extremities, protuberant abdomen, and hepatomegaly. All the following are biochemical hallmarks of this disease EXCEPT
Correct Answer: D
Rationale: Von Gierke disease (GSD I) causes lactic acidosis (A), hypoglycemia (B), hyperuricemia (C), and hyperlipidemia (D). Elevated transaminases (E) are less prominent, more typical of other liver disorders.