CLS Pediatric Lipid Disorders 3 — Questions and Answers
Question 1: Which secondary cause of hyperlipidemia is most common in overweight adolescents presenting with elevated triglycerides?
- Hypothyroidism
- Nephrotic syndrome
- Insulin resistance/metabolic syndrome (Correct answer)
- Cushing syndrome
Correct answer: Insulin resistance/metabolic syndrome
Insulin resistance associated with obesity and metabolic syndrome is the leading secondary cause of hypertriglyceridemia in adolescents.
Question 2: 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?
- Hepatic lipase deficiency
- Lipoprotein lipase deficiency (Correct answer)
- Cholesterol ester transfer protein deficiency
- Lecithin-cholesterol acyltransferase deficiency
Correct answer: Lipoprotein lipase deficiency
Lipoprotein lipase (LPL) deficiency causes severe chylomicronemia with massively elevated triglycerides, often presenting in childhood.
Question 3: Which dietary modification is most critical in managing a child with lipoprotein lipase deficiency?
- Reducing dietary cholesterol to <200 mg/day
- Eliminating dietary fat to <10-15% of calories (Correct answer)
- Increasing omega-3 fatty acids
- Restricting simple carbohydrates
Correct 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.
Question 4: Which condition should be excluded before initiating statin therapy in a hypercholesterolemic child due to the risk of myopathy?
- Iron deficiency anemia
- Hypothyroidism (Correct answer)
- Type 1 diabetes
- Celiac disease
Correct answer: Hypothyroidism
Untreated hypothyroidism increases the risk of statin-induced myopathy and can itself cause secondary hypercholesterolemia.
Question 5: In a child with nephrotic syndrome, which lipid abnormality is most characteristically elevated?
- HDL-C
- LDL-C and VLDL (Correct answer)
- Lipoprotein(a)
- Apolipoprotein A-I
Correct answer: LDL-C and VLDL
Nephrotic syndrome causes hepatic overproduction of VLDL and decreased LDL catabolism, markedly elevating LDL-C and VLDL.
Question 6: At what LDL-C threshold should pharmacological therapy be considered in a child aged 10 or older with no other cardiovascular risk factors?
- >130 mg/dL
- >160 mg/dL (Correct answer)
- >190 mg/dL
- >220 mg/dL
Correct 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.
Question 7: Which procedure is considered definitive treatment for homozygous familial hypercholesterolemia in pediatric patients when pharmacotherapy is insufficient?
- Plasma exchange
- LDL apheresis
- Liver transplantation (Correct answer)
- Gene silencing therapy
Correct answer: Liver transplantation
Liver transplantation can normalize LDL-C in homozygous FH by restoring functional LDLR expression in hepatocytes.
CLS Pediatric Lipid Disorders 3
Which secondary cause of hyperlipidemia is most common in overweight adolescents presenting with elevated triglycerides?