congenital plasminogen activator inhibitor type 1 deficiency
MeSH: C567640ORPHA: 465
Overview
Congenital plasminogen activator inhibitor type 1 (PAI-1) deficiency is a rare genetic bleeding disorder characterized by premature lysis of hemostatic clots and a moderate bleeding tendency
Available Treatments (0)
No treatments linked yet
Clinical trials or compassionate use may be available — consult a specialist.
Clinical Presentation
Signs and symptoms associated with congenital plasminogen activator inhibitor type 1 deficiency, sourced from HPO and Orphanet clinical annotations.
HemoperitoneumIntramuscular hematomaEpidural hemorrhagePersistent bleeding after traumaProlonged bleeding after surgeryDecreased level of plasminogenDecreased level of tissue plasminogen activatorReduced alpha-2-antiplasmin activityReduced plasminogen activator inhibitor 1 activityReduced plasminogen activator inhibitor 1 antigenMenorrhagiaPremature birthSubcutaneous hemorrhageEpistaxisIntracranial hemorrhageGastrointestinal hemorrhageProlonged bleeding after dental extractionPost-partum hemorrhageUmbilical cord hematomaOral bleedingPoor wound healingMyocardial fibrosisJoint hemorrhage
Classification & Codes
MeSH Code
C567640Orphanet Code
ORPHA:465congenital plasminogen activator inhibitor type 1 deficiency
| MeSH | C567640 |
| Orphanet | ORPHA:465 |
| Treatments | 0 drug(s) |
| Symptoms on record | 23 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO