Congenital plasminogen activator inhibitor type 1 deficiency

Description

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.

Index terms

Congenital plasminogen activator inhibitor type 1 deficiency
Congenital PAI-1 - [plasminogen activator inhibitor type 1] deficiency

Exclusions from above levels

JA43.3 – Postpartum coagulation defects
04 – Diseases of the immune system
05 – Endocrine, nutritional or metabolic diseases

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18 – Complications of pregnancy, childbirth or the puerperium
19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations or chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes
http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

3B50.0 – Congenital alpha-2 antiplasmin deficiency
3B50.Y – Other specified inherited fibrinolytic defects
3B50.Z – Inherited fibrinolytic defects, unspecified

Foundation entity

Congenital plasminogen activator inhibitor type 1 deficiency – http://id.who.int/icd/entity/428643962

Part of: 3B50: Inherited fibrinolytic defects › 03: Diseases of the blood or blood-forming organs