AI Article Synopsis
- The study focused on a coagulation abnormality in 12 individuals from five related families who experienced easy bruising and excessive bleeding from minor injuries.
- The activated partial thromboplastin times (aPTT) were significantly prolonged, indicating a defect in the intrinsic pathway of coagulation, while other blood tests remained normal.
- Inheritance of this condition appears to follow an autosomal dominant pattern, and the defect, named after the first patient studied, is named the Passovoy syndrome.
Article Abstract
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.
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| http://dx.doi.org/10.1056/NEJM197805112981902 | DOI Listing |
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