ATI RN
Endocrine System in Pediatrics Questions
Question 1 of 5
Transient elevation of urinary albumin can occur in the following conditions EXCEPT
Correct Answer: A
Rationale: Hypoglycemia (A) does not typically elevate urinary albumin; others (B-E) do.
Question 2 of 5
A 3-year-old boy presented with history of hyperactivity and mild mental retardation. Past history revealed repeated projectile vomiting with normal abdominal sonography. On examination, there are eczematoid rash with lighter skin, microcephaly, and mild spasticity with exaggerated tendon reflexes. Of the following, the MOST likely urine odor of this baby is
Correct Answer: A
Rationale: Phenylketonuria (PKU) matches this presentation (mental retardation, rash, spasticity, vomiting) and produces a musty urine odor due to phenylacetate, unlike other odors linked to different disorders (B, C, D, E).
Question 3 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 4 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 5 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).