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Management of severe hypertriglyceridemia due to lipoprotein lipase deficiency in children

Sarah Poon1, Karen Leung1, Joanna Tung1

  • 1Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong

Endocrinology, Diabetes & Metabolism Case Reports
|July 29, 2019
PubMed

Insights

Severe hypertriglyceridemia in infants, often caused by lipoprotein lipase deficiency, can lead to acute pancreatitis. Prompt treatment with intravenous insulin and long-term dietary changes can ensure normal growth and development.

Area of Science:

  • Endocrinology
  • Pediatrics
  • Genetics

Background:

  • Severe hypertriglyceridemia is a critical endocrine condition linked to acute pancreatitis and hyperviscosity syndrome.
  • Lipoprotein lipase deficiency (LPLD) is a rare genetic disorder causing severe hypertriglyceridemia.

Purpose of the Study:

  • To describe the case of an infant with LPLD presenting with severe hypertriglyceridemia and acute pancreatitis.
  • To highlight the management and long-term outcomes of such a case.

Main Methods:

  • Acute management involved fasting and intravenous insulin infusion.
  • Long-term treatment focused on a low-fat diet without pharmacological agents.
  • Patient was monitored until age 5 years.

Main Results:

  • The infant's severe hypertriglyceridemia and acute pancreatitis were successfully managed.
  • Long-term follow-up demonstrated a satisfactory lipid profile.
  • The patient exhibited normal growth and development.

Conclusions:

  • Intravenous insulin is a safe and effective acute treatment for hypertriglyceridemia in infants.
  • Dietary modifications alone can be effective for long-term management of primary hypertriglyceridemia due to LPLD in early childhood.
Abstract

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