A SURGICAL OPTION FOR FAMILIAL CHYLOMICRONEMIA ASSOCIATED WITH INSULIN-RESISTANT DIABETES-MELLITUS

Background: The goal of the present work is to present an effective su rgical approach for the treatment of a medically-resistant form of hyp erlipidemia. Methods: Two siblings with familial lipoprotein-lipase de ficiency and subsequent hyperchylomicronemia, widespread skin xanthoma s and severe insulin-resistant diabetes mellitus came to our observati on after several unsuccessful attempts at medical treatment. In order to lower plasma lipids through lipid malabsorption, a modified bilio-p ancreatic diversion operation was employed. The rationale in deciding to use this surgical approach was based also on the likely hypothesis that diabetes, in these subjects, was secondary to high circulating an d tissue levels of lipids. Insulin sensitivity in the two treated subj ects, as well as in 24 healthy volunteers constituting the control gro up, was assessed by euglycemic hyperinsulinemic clamp and indirect cal orimetry, obtaining total end-clamp glucose uptake (M) and end-clamp g lucose oxidation (ECGO) rates. Results: Within 3 weeks of surgery, pla sma triglycerides and cholesterol levels had decreased from 4500 and 5 00 mg/dl (with dietary restrictions) to lower than 450 and 150 mg/dl ( on a free, lipid-rich diet) respectively. Pasting plasma glucose level s had decreased from above 300 (under daily repeated subcutaneous inje ctions of insulin) to 80-100 mg/dl (without administration of insulin or oral hypoglycemic agents). Body weight and fat free mass were maint ained in both subjects after surgery. In both patients, before surgery M and ECGO were significantly lower than in normal subjects, while af ter surgery they were not significantly different from normal subjects , confirming the positive metabolic effect of the operation. Conclusio n: The surgical option used in these patients may represent an interes ting and effective new possibility for treatment of those severe cases of hyperlipemia leading otherwise to metabolic complications and a lo w quality of life. (C) 1998 Lippincott-Raven Publishers.