Variant von Willebrand’s Disease
| Autor: | K. J. Philip, David Green |
|---|---|
| Rok vydání: | 1980 |
| Předmět: | |
| Zdroj: | Thrombosis and Haemostasis. 43:002-005 |
| ISSN: | 2567-689X 0340-6245 |
| DOI: | 10.1055/s-0038-1649998 |
| Popis: | Summary30 members of an Illinois kindred were studied with a battery of haemostatic tests including the template bleeding time, platelet retention by glass beads (PRGB), measurement of activities related to factor VIII, and crossed-immunoelectrophoresis (CIEP). 9 family members had a history of excessive bleeding, and all 9 had prolonged bleeding times and increased migration of their factor VIII-related antigen (VIIIR:Ag) on CIEP. Of the other tests performed, the VIII: Ristocetin Cofactor and the PRGB showed the best correlation with the bleeding time. 3 subjects who were not bleeders, but who came from a branch of the family where there were several affected members, also had an abnormal VIIIR: Ag. The pattern of inheritance of the altered VIIIR: Ag in this family was one of autosomal dominance with full penetrance. The CIEP is a valuable screening test for the detection of variant von Willebrand’s disease and the recognition of silent heterozygotes. |
| Databáze: | OpenAIRE |
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