Protein C (PROC) gene mutations in two Indian families with purpura fulminans.

摘要

Protein C is (PC) a vitamin K-dependent coagulation protein that serves a critical role in the regulation of thrombin by inactivation factor V and factor VIII [1]. The clinical manifestation of PC deficiency in neonates is generally disseminated intravascular coagulation and pur-pura fulminans [2], Severe homozygous PC deficiency with purpura fulminans is usually fatal and occurs with an incidence of approximately one in 500,000 to 750,000 live births worldwide.