ATI RN
Endocrine System Pediatric Questions
Question 1 of 5
Signs of insulin resistance or conditions associated with insulin resistance in T2 DM in children include the following EXCEPT
Correct Answer: D
Rationale: PCOS (E) is associated but not a direct sign of insulin resistance; others (A-D) are.
Question 2 of 5
A 6-year-old boy with eczematoid rash, abnormal face, and prominent maxilla complains from deterioration in school performance, hyperactivity, and seizures for the last 2 years. Of the following, the MOST likely enzymatic deficiency is
Correct Answer: D
Rationale: Phenylalanine hydroxylase deficiency (PKU) causes mental decline, seizures, rash, and facial changes from phenylalanine buildup, unlike lysosomal deficiencies (A, B, C) or tyrosinemia (E).
Question 3 of 5
A 15-month-old boy had recurrent hospital admissions because of fever, jaundice, subcutaneous bleeding, and hypoglycemic fits; on examination, there is hepatomegaly. In the last attack, the child developed severe legs pain associated with retraction of the neck and trunk. Of the following, the MOST likely diagnosis is
Correct Answer: D
Rationale: Tyrosinemia type 1 fits with recurrent fever, jaundice, bleeding, hypoglycemia, hepatomegaly, and neurological symptoms (pain, retraction) from tyrosine accumulation, unlike other conditions (A, B, C, E).
Question 4 of 5
A 6-year-old girl that looks tall and thin with light skin. On examination she has a peculiar malar flush, subluxation of the ocular lens, developmental delay, and severe hypertension. Of the following, the MOST likely cause of hypertension is
Correct Answer: C
Rationale: Homocystinuria’s hypertension often stems from thromboembolism due to homocysteine’s vascular effects, fitting this presentation, unlike atherosclerosis (A), hyperthyroidism (B), coarctation (D), or renal stenosis (E).
Question 5 of 5
A 9-year-old boy admitted to the hospital because of fracture of left femur due to a minor trauma. Past history revealed recurrent attacks of epistaxis and intermittent generalized bone pain. On examination, there is massive splenomegaly. Of the following, the MOST appropriate practical treatment of this disease is
Correct Answer: C
Rationale: Enzyme replacement therapy (ERT) is the practical, standard treatment for Gaucher disease, reducing splenomegaly and bone issues, more widely used than gene therapy (A), transplantation (B, E), or substrate reduction (D).