Management of severe hypertriglyceridemia due to lipoprotein lipase deficiency in children

Abstract

Summary Severe hypertriglyceridemia is an endocrine emergency and is associated with acute pancreatitis and hyperviscosity syndrome. We describe an infant with lipoprotein lipase deficiency with severe hypertriglyceridemia who presented with acute pancreatitis. She was managed acutely with fasting and intravenous insulin infusion, followed by low-fat diet with no pharmacological agent. Subsequent follow-up until the age of 5 years showed satisfactory lipid profile and she has normal growth and development. Learning points: Hypertriglyceridemia-induced acute pancreatitis has significant morbidity and mortality, and prompt treatment is imperative. When no secondary causes are readily identified, genetic evaluation should be pursued in hypertriglyceridemia in children. Intravenous insulin is a safe and effective acute treatment for hypertriglyceridemia in children, even in infants. Long-term management with dietary modifications alone could be effective for primary hypertriglyceridemia due to lipoprotein lipase deficiency, at least in early childhood phase.