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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아혈액종양학회 Clinical Pediatric Hematology-Oncology Clinical Pediatric Hematology-Oncology Vol.28 No.1
- 발행연도
- 2021.1
- 수록면
- 54 - 57 (4page)
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초록· 키워드
오류제보하기
von Willebrand disease (VWD) is the most common hereditary bleeding disorder. The treatment of VWD consists mainly of desmopressin and plasma-derived von Willebrand factor (pd-VWF) concentrate. We report on the first patient with VWD to be treated with recombinant VWF (rVWF) concentrate in Korea. Our patient was diagnosed with type 2 VWD at 10 months of age and suffered persistent severe epistaxis despite therapeutic levels of VWF activity and factor VIII (FVIII). At 34 months of age, rVWF was initiated and administered a total of 15 times in the following 8 months. No drug-related adverse events were observed and the patient was neither admitted nor given any transfusions during this period. Unlike pd-VWF/FVIII concentrates, rVWF did not increase FVIII to the excessively high levels that constitute a risk factor for thromboembolism, and was also preferable to pd-VWF/FVIII concentrates in that it contains ultra-large multimers of VWF. This is the first reported case in Korea in which rVWF was used to treat VWD. rVWF may be well tolerated and effective in VWD patients, especially those with refractory bleeding.
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등록된 정보가 없습니다.
참고문헌 (15)
참고문헌 신청
Yee A / 2014 / von Willebrand factor : form for function / Semin Thromb Hemost 40:17~27
Mannucci PM / 2013 / Pharmacokinetics and safety of a novel recombinant human von Willebrand actor manufactured with a plasma-free method : a pro-spective clinical trial / Blood 122:648~657
Mannucci PM / 2004 / Treatment of von Willebrand's disease / N Engl J Med 351:683~694
Fischer BE / 1999 / Recombinant von Willebrand factor : potential therapeutic use / J Thromb Thrombolysis 8:197~205
Castaman G / 2013 / Principles of care for the diagnosis and treatment of von Willebrand disease / Haematologica 98:667~674
Mannucci PM / 2013 / Pharmacokinetics and safety of a novel recombinant human von Willebrand actor manufactured with a plasma-free method : a pro-spective clinical trial / Blood 122:648~657
Mannucci PM / 2004 / Treatment of von Willebrand's disease / N Engl J Med 351:683~694
Fischer BE / 1999 / Recombinant von Willebrand factor : potential therapeutic use / J Thromb Thrombolysis 8:197~205
Castaman G / 2013 / Principles of care for the diagnosis and treatment of von Willebrand disease / Haematologica 98:667~674
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