Primary type V hyperlipoproteinemia in childhood.
Metabolic studies of a 9-year-old girl with primary type V hyperlipoproteinemia demonstrated normal glucose tolerance, plasma insulin and glucagon responses to stimuli, and serum uric acid level. Fasting plasma triglyceride levels rapidly increased when the patient received a diet containing 40% of the total calories as fat and rapidly decreased on a 10% fat diet. Hepatic and nonhepatic lipase activities in postheparin plasma were normal, thus excluding type I hyperlipoproteinemia. Because of the potential complication of acute pancreatitis in this disorder, early diagnosis and prompt institution of diet therapy is important.
D000367:Age Factors; D002648:Child; D002784:Cholesterol / Q000097:blood*; D004041:Dietary Fats / Q000008:administration & dosage; D005260:Female; D006493:Heparin / Q000008:administration & dosage; D006529:Hepatomegaly / Q000175:diagnosis; D006801:Humans; D006949:Hyperlipidemias / Q000145:classification / Q000178:diet therapy / Q000378:metabolism*; D008071:Lipoprotein Lipase / Q000097:blood; D013163:Splenomegaly / Q000175:diagnosis; D014280:Triglycerides / Q000097:blood*
D004041:Dietary Fats; D014280:Triglycerides; D006493:Heparin; D002784:Cholesterol; D008071:Lipoprotein Lipase