All the following are common features between hereditary fructose intolerance and galactosemia EXCEPT

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Endocrine System in Pediatrics Questions

Question 1 of 5

All the following are common features between hereditary fructose intolerance and galactosemia EXCEPT

Correct Answer: D

Rationale: HFI and galactosemia share failure to thrive (A), renal (B) and hepatic (C) impairment, and reducing substances (D). Intellectual disability (E) is specific to untreated galactosemia, not HFI.

Question 2 of 5

The characteristic radiographic finding of Hurler disease is

Correct Answer: D

Rationale: Hook-shaped L1 vertebra is a characteristic Hurler (MPS I) finding from GAG deposition, distinct from other features (A, B, C, E).

Question 3 of 5

The MOST common electrolyte abnormality in acute intermittent porphyria is

Correct Answer: B

Rationale: Hyponatremia is common in AIP from SIADH or GI losses, more frequent than other imbalances (A, C, D, E).

Question 4 of 5

A 3-year-old boy presented to emergency unit at 9:00 AM with history of difficult arousal from sleep, flue like illness, and low grade fever; the mother stated that he had slept without having his supper. Fasting blood sugar was 42 mg/dL. The following metabolic changes are seen in this condition EXCEPT

Correct Answer: C

Rationale: Ketotic hypoglycemia from fasting and illness shows ketonuria (A), low insulin (B), and elevated cortisol (D) and GH (E). High alanine (C) is not typical, as it’s consumed for gluconeogenesis.

Question 5 of 5

All the following drugs are secondary causes of hypertriglyceridemia EXCEPT

Correct Answer: A

Rationale: Estrogen (B), thiazides (C), β-blockers (D), and steroids (E) increase triglycerides. Tegretol (carbamazepine, A) does not, primarily affecting neurology.

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