Abstract von Willebrand's disease (vWD) is an inherited disorder of bleeding, which is characterized by easy bruisability, mucosal hemorrhages, excessive posttraumatic bleeding, prolonged skin bleeding times, and quantitative or qualitative defects of the factor VIII molecular "complex." von Willebrand's disease and the structure and physiology of the factor VIII molecule have recently been reviewed.1-3 We studied a 7-month-old affected male infant whose father and paternal grandfather were known to have vWD. Because of his family history, the child's plasma was studied at birth, at 6 months of age, and after the intravenous (IV) administration of cryoprecipitate. Our findings emphasize the difficulty in establishing the diagnosis of vWD in the immediate postnatal period. Report of a Case.—The patient is a 7-month-old boy, product of an uncomplicated 40-week gestation, born to a 19-year-old, blood group O, Rh-positive, gravida 1, para 1, abortus 0, hemostatically normal woman. Labor was induced with IV References 1. Hoyer LW: von Willebrand's disease , in Spaet H (ed): Progress in Hemostasis and Thrombosis . Grune & Stratton Inc, New York, 1976, vol 3, pp 231-287. 2. Gralnick HR, Coller BS, Shulman NR, et al: Factor VIII . Ann Intern Med 86:598-616, 1977.Crossref 3. Ratnoff OD: Antihemophiliac factor (factor VIII) . Ann Intern Med 88:403-409, 1978.Crossref 4. Conlon CL, Weinger RS, Cimo PL, et al: Telangiectasia and von Willebrand's disease in two families . Ann Intern Med 89:921-924, 1978.Crossref 5. Ekert H, Firkin BG: Recent advances in haemophilia and von Willebrand's disease . Vox Sang 28:409-421, 1975.Crossref 6. Gralnick HR, Coller BS, Sultan Y: Studies of the human factor VIII/von Willebrand factor protein: III. Qualitative defects in von Willebrand's disease . J Clin Invest 56:814-827, 1975.Crossref 7. Sultan Y, Simeon J, Maisonneuve P, et al: Immunologic studies in von Willebrand's disease: Alteration of factor VIII/von Willebrand protein after transfusion with plasma concentrates in patients with von Willebrand's disease . Thromb Haemost 35:110-119, 1976. 8. Chediak JR, Telfer MC, Green D: Platelet function and immunologic parameters in von Willebrand's disease following cryoprecipitate and factor VIII concentrate infusion . Am J Med 62:369-376, 1977.Crossref 9. Fukui H, Takase T, Ikari H, et al: Factor VIII procoagulant activity, factor VIII related antigen and von Willebrand factor in newborn cord blood . Br J Haematol 42:637-646, 1979.Crossref 10. Holden KR, Young RB, Piland JH, et al: Plasma pressors in the normal and stressed newborn infant . Pediatrics 49:495-503, 1972.
American Journal of Diseases of Children – American Medical Association
Published: Aug 1, 1980