Encephalopathy in type I hyperlipidemia

Indian Pediatr. 2007 Apr;44(4):306-8.

Abstract

Familial chylomicronemia syndrome is a group of rare genetic disorders characterized by deficient activity of an enzyme lipoprotein lipase or apo-protein C-II deficiency. In this paper we present an infant with massive hyperchylomicronemia and severe pancreatitis. Exchange transfusion for controlling hypertriglyceridemia and pancreatitis led to an increase in hyperviscosity which resulted in encephalopathy.

Publication types

  • Case Reports

MeSH terms

  • Apolipoprotein C-II / deficiency*
  • Blood Viscosity
  • Brain Diseases, Metabolic / diagnosis*
  • Brain Diseases, Metabolic / etiology
  • Humans
  • Hyperlipoproteinemia Type I / complications*
  • Hyperlipoproteinemia Type I / diagnosis*
  • Hyperlipoproteinemia Type I / etiology
  • Hypertriglyceridemia / complications*
  • Infant
  • Lipoprotein Lipase / deficiency
  • Lipoproteins
  • Male
  • Pancreatitis / diagnosis*
  • Pancreatitis / etiology
  • Plasma Exchange*

Substances

  • Apolipoprotein C-II
  • Lipoproteins
  • Lipoprotein Lipase