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Pediatric Nursing Cardiac Practice Questions Questions

Question 1 of 5

Hereditary methaemoglobinaemia is most commonly due to deficiency of which red cell enzyme?

Correct Answer: A

Rationale: Hereditary methemoglobinemia is most commonly due to cytochrome b5 reductase deficiency (
Choice
A), impairing reduction of methemoglobin to hemoglobin, causing cyanosis. Glutathione peroxidase (
Choice
B) protects against oxidative stress, not methemoglobin. Heme oxygenase (
Choice
C) degrades heme, unrelated here. Methylenetetrahydrofolate reductase (
Choice
D) affects folate metabolism. Cytochrome b5 reductase is the key enzyme.

Question 2 of 5

Which first-line agents are preferred in treating seizures secondary to hypoxic-ischemic encephalopathy (HIE):

Correct Answer: D

Rationale: Phenobarbital is the first-line treatment for neonatal HIE seizures, per AAP and WHO guidelines, due to efficacy and safety. Benzodiazepines are adjunctive, paraldehyde is obsolete, phenytoin is second-line, and propofol is contraindicated in neonates.

Question 3 of 5

In newborns with polycythemia, highly elevated viscosity is due to:

Correct Answer: A

Rationale: Polycythemia increases viscosity primarily via a high RBC count , raising hematocrit (AAP). Plasma volume contributes less, and capillary factors (C-E) are secondary.

Question 4 of 5

Most common cause of severe mental retardation is:

Correct Answer: A

Rationale: Chromosomal disorders , like Down syndrome, are the top cause of severe mental retardation due to prevalence and impact (CD
C). B-E are less frequent.

Question 5 of 5

Best diagnostic study to diagnose patients with adrenoleukodystrophy is:

Correct Answer: A

Rationale: Elevated VLCFA in plasma and RBCs confirms adrenoleukodystrophy diagnosis (NOR
D). B-E are incorrect.

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