Congenital and acquired lipodystrophies / 中华实用儿科临床杂志

ABSTRACT

Lipodystrophies represent a heterogeneous group of diseases characterized by varying degrees of body fat loss and predisposition to insulin resistance and metabolic complications such as diabetes mellitus,hypertrigly-ceridemia and hepatic steatosis. The lipodystrophies can be divided into generalized,partial or local,depending on the degree and locality of the observable fat loss;moreover,the generalized and partial divisions can be partitioned further into congenital or acquired forms. Until now,11 genetic factors including AGPAT2,BSCL2,CAVI,PTRF,PPARG, LMNA,ZMPSTE24,AKT2,CIDEC,PLINI and WRN were reported to be involved in congenital lipodystrophies. The most prevalent subtype of acquired lipodystrophy currently occurs with prolonged duration of protease inhibitor - contai-ning,highly - active antiretroviral therapy in human immunodeficiency virus(HIV)- infected patients. Other types of acquired lipodystrophies are mainly autoimmune in origin and display complement abnormalities. The current manage-ment includes cosmetic surgery and early identification and treatment of metabolic and other complications with diet, exercise,hypoglycemic drugs,and lipid - lowering agents. Metreleptin treatment demonstrated remarkable clinical effect and good tolerance.