Congenital plasminogen activator inhibitor type 1 deficiency (Q39607)

Congenital plasminogen activator inhibitor type 1 (PAI-1) deficiency is a disorder that causes premature lysis of haemostatic clots and a moderate bleeding syndrome. Spontaneous bleeding is rarely observed, whereas moderate haemorrhages of the knees, elbows, nose and gingiva are usually triggered by mild trauma. However, menstrual bleeding may be severe and a prolonged bleeding after surgery is common. The PAI-1 deficiency may be qualitative or quantitative, total or partial.