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The Passovoy Defect

1978; Massachusetts Medical Society; Volume: 298; Issue: 19 Linguagem: Inglês

10.1056/nejm197805112981902

1533-4406

Cecil Hougie, Richard A. McPherson, James E. Brown, Patricia L. Lakin‐Thomas, Anthony J. Melaragno, Linda Aronson, Robert F. Baugh,

Body Contouring and Surgery

We studied a coagulation abnormality present in 12 members of five kindreds who bruised easily and bled excessively after minor trauma. Their activated partial thromboplastin times were between 32 and 39 seconds (normal, 22.8 to 28.8 seconds). Prothrombin times, thrombin times, platelet-function tests and the levels of factors XII, XI, IX, VIII, prekallikrein and high-molecular-weight kininogen were normal. Within these kindreds inheritance of prolonged partial thromboplastin times followed an autosomal and probably dominant pattern. The prolonged thromboplastin times were corrected by normal plasma and by normal plasma adsorbed with celite, but there was no mutual correction between plasmas of the patients. These subjects shared a common defect in the intrinsic pathway of coagulation that we designate by the proband's surname, Passovoy. (N Engl J Med 298:1045–1048, 1978)