Lecithin-cholesterol acyltransferase deficiency presenting with acute pancreatitis: effect of infusion of normal plasma on triglyceride-rich lipoproteins

J Intern Med. 1995 Aug;238(2):137-41. doi: 10.1111/j.1365-2796.1995.tb00911.x.

Abstract

A 38-year-old Asian man presented with acute pancreatitis, marked hypertriglyceridaemia and macroproteinuria, 20 years after the diagnosis of lecithin-cholesterol acyltransferase (LCAT) deficiency. After recovery, he exhibited macroproteinuria and chylomicronaemia despite treatment with a very-low-fat diet. Infusion of normal plasma significantly increased the proportion of cholesterol esters in the patient's plasma and significantly lowered chylomicron-triglyceride levels, but not proteinuria. We conclude that renal dysfunction may be a late manifestation of LCAT deficiency and that it may lead to severe chylomicronaemia and acute pancreatitis. Infusion of normal plasma corrects the dyslipidaemia in LCAT deficiency, but in the short term does not improve renal function.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Adult
  • Blood Component Transfusion
  • Diagnosis, Differential
  • Humans
  • Lecithin Cholesterol Acyltransferase Deficiency / blood
  • Lecithin Cholesterol Acyltransferase Deficiency / complications
  • Lecithin Cholesterol Acyltransferase Deficiency / diagnosis*
  • Lecithin Cholesterol Acyltransferase Deficiency / therapy
  • Lipoproteins / blood
  • Male
  • Pancreatitis / blood
  • Pancreatitis / etiology*

Substances

  • Lipoproteins