Preferred Label : Familial partial lipodystrophy associated with PPARG mutations;
ICD-11 definition : Familial partial lipodystrophy associated with PPARG (peroxisome proliferative activated
receptor gamma) mutations belongs to the group of lipodystrophy syndromes characterised
by anomalies of adipose tissue distribution and often associated with severe insulin
resistance manifested by hyperinsulinaemia (insulin levels above 20mUI/l), carbohydrate
intolerance or diabetes, hypertriglyceridaemia with low levels of high-density lipoproteins
(HDL) and arterial hypertension. Acanthosis nigricans, hepatic steatosis and polycystic
ovary syndrome are also frequent.;
ICD-11 synonym : FPLD3 - [Familial partial lipodystrophy type 3]; Familial partial lipodystrophy type 3;
ICD-11 acronym : FPLD3;
Origin ID : 2042807932;
Automatic exact mappings (from CISMeF team)
Familial partial lipodystrophy associated with PPARG (peroxisome proliferative activated
receptor gamma) mutations belongs to the group of lipodystrophy syndromes characterised
by anomalies of adipose tissue distribution and often associated with severe insulin
resistance manifested by hyperinsulinaemia (insulin levels above 20mUI/l), carbohydrate
intolerance or diabetes, hypertriglyceridaemia with low levels of high-density lipoproteins
(HDL) and arterial hypertension. Acanthosis nigricans, hepatic steatosis and polycystic
ovary syndrome are also frequent.
