All issues > Volume 34(3); 1991

Original Article

J Korean Pediatr Soc. 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

Received: August 13, 1990;  Accepted: October 29, 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.

Keywords :von Willebrand disease