Orphanet: Congenital plasminogen activator inhibitor type 1 deficiency

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Congenital plasminogen activator inhibitor type 1 deficiency

Disease definition

A rare hemorrhagic disorder due to a constitutional haemostatic factors defect characterized by premature lysis of hemostatic clots and a moderate bleeding tendency.


Classification level: Disorder
  • Synonym(s):
    • Congenital PAI-1 deficiency
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: Elderly, Childhood, Adolescent, Adult
  • ICD-10: D68.8
  • OMIM: 613329
  • UMLS: C2750067
  • MeSH: -
  • GARD: 4381
  • MedDRA: -

Detailed information


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