Journal of the Korean Pediatric Society 1991;34(3):419-425.
Published online March 31, 1991.
Three cases of type I von Willebrand disease in a family.
Jie Sun Yoon1, Hey Sun Lee1, Young Min Ahn1, Myoung Sook Koo2, Sun Yang Park3
1Department of Pediatrics, Kangnam General Hospital Public Corporation, Seoul, Korea
2Department of Clinical Pathology, Kangnam General Hospital Public Corporation, Seoul, Korea
3Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
3모자에서 발견된 von Willebrand병
윤지선1, 이혜선1, 안영민1, 구명숙2, 박선양3
1지방공사 강남병원 소아과
2지방공사 강남병원 임상병리과
3서울대학교 의과대학 내과 암연구실
Received: 13 August 1990   • Accepted: 29 October 1990
Abstract
von Willebrand disease is a bleeding disorder characterized by a complex hemostatic defect. These abnormalities arise from quantitative and/or qualitative abnormalities of von Willebrand factor which circulates in plasma complexed with the factor VID procoagulant protein. We have experienced three cases of type I von Willebrand disease in two children and their mother. They had prolonged bleeding time and partial thromboplastin time, mildly decreased factor VIII coagulant activity, marked reduction of ristocetin induced platelet aggregation, low level of von Willebrand factor antigen and ristocetin cofactor activity, but normal multimeric structure of von Willebrand factor. Their bleeding time was corrected after intranasal l-deamino-8-d-arginine vasopressin administra- tion.
Key Words: von Willebrand disease




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