Preferred Label : Familial partial lipodystrophy, Köbberling type;
ICD-11 definition : Familial partial lipodystrophy, Köbberling type 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 : Familial partial lipodystrophy type 1; FPLD1 - [Familial partial lipodystrophy type 1];
ICD-11 acronym : FPLD1;
Origin ID : 1790224502;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Familial partial lipodystrophy, Köbberling type 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.
