Thelansis

  Familial Partial Lipodystrophy (FPLD) syndromes are a heterogeneous group of genetically inherited or acquired conditions characterized by dysfunctional white adipose tissue. It is characterized by selective loss of adipose tissue in the extremities and gluteal region without changing abdominal and visceral fat. In most cases, abnormal fat distribution becomes apparent at puberty. This selective deficiency of metabolically active adipose tissue is tightly linked with a wide range of metabolic complications, such as insulin resistance, lipoatrophic diabetes, dyslipidemia with severe hypertriglyceridemia, hypertension, or hepatic steatosis. Moreover, female patients often develop hyperandrogenism, hirsutism, polycystic ovaries, and infertility. The extent of fat loss often determines the severity of metabolic consequences. There are seven subtypes of FPLD caused by mutations in various genes (LMNA, PPARG, PLIN1, CIDEC, LIPE, AKT2 or CAV1); the condition can be inherited in either...

 Familial Partial Lipodystrophy (FPLD) syndromes are a heterogeneous group of genetically inherited or acquired conditions characterised by dysfunctional white adipose tissue. It is characterised by selective loss of adipose tissue in the extremities and gluteal region without changing abdominal and visceral fat. In most cases, abnormal fat distribution becomes apparent at puberty. This selective deficiency of metabolically active adipose tissue is tightly linked with a wide range of metabolic complications, such as insulin resistance, lipoatrophic diabetes, dyslipidemia with severe hypertriglyceridemia, hypertension or hepatic steatosis. Moreover, female patients often develop hyperandrogenism, hirsutism, polycystic ovaries and infertility. The extent of fat loss often determines the severity of metabolic consequences. There are seven subtypes of FPLD caused by mutations in various genes (LMNA, PPARG, PLIN1, CIDEC, LIPE, AKT2 or CAV1); the condition can be inherited in either a do...