Diabetic Lipemia with Maturity-Onset Diabetes of the Young.

Abstract

A 19-year-old womanwith diabetic lipemia and maturity-onset diabetes of the young (MODY) is reported. Though her insulin secretory activity was preserved, she fell into mild diabetic ketoacidosis (DKA) and showed type V hyperlipidemia. Post-heparin plasma activity oflipoprotein lipase (LPL) was decreased even 10 days after initiating insulin injection but not deficient. The abnormalities in lipid metabolism were improved by long-term insulin treatment. Though the contribution of the genetic background to the lipid abnormalities is not clear, the characteristics of MODY in this patient including insulin secretory capacity under stress conditions such as DKA might play a role in the development of diabetic lipemia. (Internal Medicine 36: 571-574, 1997)

Severe hypertriglyceridaemia in diabetic ketoacidosis: clinical and genetic study

“…It has been suggested that genetic studies might be necessary in patients with DKA in order to rule out the presence of co‐existing mutations in LPL gene [6–9]. Although LPL mass measurements in the patient under good glycaemic control were within normal range, it is possible that subtle abnormality in lipid metabolism in usual conditions may contribute to the development of hyperlipidaemia under stress such as DM.…”

Type V Hyperlipidemia Associated with Diabetic Ketoacidosis

“…On the other hand, in NIDDMboth overproduction and decreased clearance are observed. The MODY case described by Shinozaki et al (5) in this issue of the Journal is a unique example. Extreme increases in serum VLDL-TG and chylomicron levels are a reflection of both overproduction and decreased clearance.…”

Very Severe Hypertriglyceridemia in Children with Diabetic Ketoacidosis

“…It has been suggested that genetic studies might be necessary in patients with hypertriglyceridemia in DKA to rule out the presence of coexisting mutations in LPL gene. 18 Mutation in LPL gene can lead to severe and persistent hypertriglyceridemia in DKA that does not improve with insulin alone. 30 On the other hand, a severe but not persistent hypertriglyceridemia may be caused by abnormality in lipoprotein cofactors.…”