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CIDEC-related familial partial lipodystrophy

ORPHA:435651DiseaseAutosomal recessiveAdolescent

Ассоциированные гены (1)

CIDEC
cell death inducing DFFA like effector c
Disease-causing germline mutation(s) (loss of function) in
OMIM: 612120

Фенотипы (16)

Облигатный (100%)2
HP:0003635Loss of subcutaneous adipose tissue in limbs
HP:0009125Lipodystrophy
Очень частый (80–99%)13
HP:0000147Polycystic ovaries
HP:0000831Insulin-resistant diabetes mellitus
HP:0000876Oligomenorrhea
HP:0000956Acanthosis nigricans
HP:0001397Hepatic steatosis
HP:0001733Pancreatitis
HP:0002155Hypertriglyceridemia
HP:0002240Hepatomegaly
HP:0003292Decreased serum leptin
HP:0003712Skeletal muscle hypertrophy
HP:0008981Calf muscle hypertrophy
HP:0009017Loss of gluteal subcutaneous adipose tissue
HP:0030685Decreased adiponectin level
Исключён (0%)1
HP:0000292Loss of facial adipose tissue

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы