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Severe hereditary thrombophilia due to congenital protein C deficiency

ORPHA:745DiseaseAutosomal dominant, Autosomal recessiveNeonatal

Ассоциированные гены (1)

PROC
protein C, inactivator of coagulation factors Va and VIIIa
Disease-causing germline mutation(s) in
OMIM: 612283

Фенотипы (10)

Частый (30–79%)4
HP:0000963Thin skin
HP:0000979Purpura
HP:0004936Venous thrombosis
HP:0008065Aplasia/Hypoplasia of the skin
Периодический (5–29%)6
HP:0001000Abnormality of skin pigmentation
HP:0001038Warfarin-induced skin necrosis
HP:0002204Pulmonary embolism
HP:0005293Venous insufficiency
HP:0100659Abnormality of the cerebral vasculature
HP:0100758Gangrene

Эпидемиология (2)

Prevalence at birth
1-9 / 1 000 000
Worldwide
Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы