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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Asakura, A. Articles by Abildgaard, C. Search for Related Content PubMed PubMed Citation Articles by Asakura, A. Articles by Abildgaard, C. Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Type IIA von Willebrand disease with apparent recessive inheritance A Asakura, J Harrison, E Gomperts and C Abildgaard Type IIA von Willebrand's Disease (vWD) is the most common type II variant, and all reported cases (56 individuals in 26 families) have had autosomal dominant inheritance. An eight-year-old female with an increased bleeding tendency since infancy was found to have laboratory values typical of type IIA vWD, but her parents and siblings were asymptomatic. With the exception of uniformly decreased levels of ristocetin cofactor in relation to von Willebrand factor antigen, the results of family studies were normal including the presence of large multimeric forms of von Willebrand factor antigen. These findings are consistent with the propositus having the homozygous state of an autosomal recessive trait. Desmopressin infusion in the propositus was followed by a significant increase of factor VIII coagulant and von Willebrand factor antigen but a limited change in ristocetin cofactor with no development of large multimers. Volume 69, Issue 5, pp. 1419-1420, 05/01/1987 Copyright © 1987 by The American Society of Hematology CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. I. Pareti, A. Lattuada, C. Bressi, M. Zanobini, A. Sala, A. Steffan, and Z. M. Ruggeri Proteolysis of von Willebrand Factor and Shear Stress-Induced Platelet Aggregation in Patients With Aortic Valve Stenosis Circulation, September 12, 2000; 102(11): 1290 - 1295. [Abstract] [Full Text] [PDF]

	Copyright © 1987 by American Society of Hematology         Online ISSN: 1528-0020