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, from the Inserm; Faculte de medecine, universite Pierre-et-Marie-Curie-Paris-VI; Service de biochimie, APHP, hopital Tenon, Paris, France. Primary lipodystrophies represent a heterogeneous group of very rare diseases with a prevalence of less than 1 case for 100.000, inherited or acquired (for eg. Barraquer-Simons syndrome), caracterized by a loss of body fat either generalized or localized. All lipodystrophies are associated with dysmetabolic alterations with insulin resistance, altered glucose tolerance or diabetes and hypertriglyceridemia leading to a risk of acute pancreatitis. Chronic complications are those resulting from diabetes involving the retina, kidney and nerves, cardiovascular complications and steatotic liver lesions that could result in cirrhosis. Treatment of diabetes, dyslipidemia and complications involves the classical intervention strategies. Therapeutic trials with recombinant human leptin in patients with very low leptin levels reported good results with respect to the metabolic and liver alterations. The prognosis is linked to the precocity and severity of the diabetic, cardiovascular and liver complications. To access the full abstract of the article, click here.
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