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Hypercholesterolemia due to cholesterol 7alpha-hydroxylase deficiency

ORPHA:209902DiseaseSemi-dominantAdult

Ассоциированные гены (1)

CYP7A1
cytochrome P450 family 7 subfamily A member 1
Disease-causing germline mutation(s) in
OMIM: 118455

Фенотипы (15)

Очень частый (80–99%)9
HP:0001396Cholestasis
HP:0001397Hepatic steatosis
HP:0001403Macrovesicular hepatic steatosis
HP:0002155Hypertriglyceridemia
HP:0003124Hypercholesterolemia
HP:0003141Increased LDL cholesterol concentration
HP:0006573Acute hepatic steatosis
HP:0011980Cholesterol gallstones
HP:0012115Hepatitis
Частый (30–79%)6
HP:0001513Obesity
HP:0001677Coronaryartery atherosclerosis
HP:0004943Accelerated atherosclerosis
HP:0008372Abnormality of vitamin A metabolism
HP:0012397Aortic atherosclerosis
HP:0100514Abnormality of vitamin E metabolism

Эпидемиология (2)

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

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы