Summary
Some aspects of lipid metabolism were studied in 4 patients with a congenital lipoatrophic
diabetes (LAD) associated to a type IV hyperlipoproteinemia.
The analysis of lipoprotein composition, expressed as mg/dl, demonstrates a significant
increase of VLDL mass and a significant reduction of MDL mass. The analysis of lipoprotein
composition, expressed as per cent of total mass demonstrates an increase of the triglyceride
content in all fractions and a significant reduction of the cholesterol and phospholipid
content in HDL2 particles. Apo C-II, C-III0, C-III1 and C-III2 levels in lipoprotein fractions were normal in LAD patients. Lipoprotein lipase activity
in omental adipose tissue, collected during laparoscopy in one patient was undetectable.
The serum of this patient did not fully activate the lipoprotein lipase eluted from
normal adipose tissue.
In all patients the adipose tissue lipoprotein lipase activity in post-heparin plasma
was blunted or near absent. Thus a reduced peripheral clearance of triglyceride-rich
lipoprotein could be an important determinant of lipoprotein abnormalities in lipoatrophic
diabetes.
Key-Words
Lipoatrophic Diabetes
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Adipose Tissue Lipoprotein Lipase Activity
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Plasma Post-Heparin Lipolytic Activity
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Lipoprotein Composition