Pediatric Lipid Disorders Flashcards

Which secondary cause of hyperlipidemia is most common in overweight adolescents presenting with elevated triglycerides?

Answer: Insulin resistance/metabolic syndrome

Insulin resistance associated with obesity and metabolic syndrome is the leading secondary cause of hypertriglyceridemia in adolescents.

A 7-year-old with type 1 diabetes presents with lipemia retinalis and TG of 2,800 mg/dL. Which enzyme deficiency is most likely?

Answer: Lipoprotein lipase deficiency

Lipoprotein lipase (LPL) deficiency causes severe chylomicronemia with massively elevated triglycerides, often presenting in childhood.

Which dietary modification is most critical in managing a child with lipoprotein lipase deficiency?

Answer: Eliminating dietary fat to <10-15% of calories

Severe restriction of total dietary fat to less than 10-15% of calories is the cornerstone of therapy for LPL deficiency.

Which condition should be excluded before initiating statin therapy in a hypercholesterolemic child due to the risk of myopathy?

Answer: Hypothyroidism

Untreated hypothyroidism increases the risk of statin-induced myopathy and can itself cause secondary hypercholesterolemia.

In a child with nephrotic syndrome, which lipid abnormality is most characteristically elevated?

Answer: LDL-C and VLDL

Nephrotic syndrome causes hepatic overproduction of VLDL and decreased LDL catabolism, markedly elevating LDL-C and VLDL.

At what LDL-C threshold should pharmacological therapy be considered in a child aged 10 or older with no other cardiovascular risk factors?

Answer: >160 mg/dL

Statin therapy is recommended when LDL-C remains ≥160 mg/dL after dietary intervention in low-risk children aged 10 and older.

Which procedure is considered definitive treatment for homozygous familial hypercholesterolemia in pediatric patients when pharmacotherapy is insufficient?

Answer: Liver transplantation

Liver transplantation can normalize LDL-C in homozygous FH by restoring functional LDLR expression in hepatocytes.