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按需治疗下中、重型血友病患儿关节及颅内出血现状

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摘要 血友病是先天性凝血因子Ⅷ／Ⅸ缺乏或合成障碍的伴性遗传性疾病，据统计报道我国血友病患病率为2．73／10万人口^[1]。血友病中、重型患者出血情况重^[2]，出血症状从学步期开始并逐步增多。血友病的出血80％发生在关节腔内^[3]，反复关节出血可造成血友病性关节病变，是最常见的并发症和最主要的致残原因。而颅内出血（intracranial heamorrahge，ICH）作为血友病最严重的出血类型H0，可危及生命^[5]。
作者 何雯雯 吴润晖
机构地区 首都医科大学附属北京儿童医院血液病中心
出处 《中华儿科杂志》 CAS CSCD 北大核心 2012年第8期636-638,共3页 Chinese Journal of Pediatrics
关键词 血友病性 颅内出血 关节病变 重型患者 按需治疗 患儿 遗传性疾病 凝血因子Ⅷ
分类号 R725.5 [医药卫生—儿科]
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参考文献41

  • 1张之南,郝玉书,赵永强,等.血液病学.2版.北京:人民卫生出版社,2011.1350-1352.
  • 2den Uijl IE, Fischer K, Van Der Born JG, et al. Clinical outcome of moderate haemophilia compared with severe and mild haemophilia. Haemophilia,2009,15 : 83-90.
  • 3Rodriguez-Merchan EC, Heim M. Hemophilia orthopedic management with emphasis on developing countries. SeminThromb Hemost,2005,31 : 518-526.
  • 4Antunes SV, Vicari P, Cavalheiro S, et al. Intracranial haemorrhage among a population of haemophilic patients in Brazil. I-Iaemophilia, 2003,9 : 573 -577.
  • 5Stiehjes N, Calvez T, Demiguel V, et al. French ICH Study Group. Intracranial haemorrhages in French haemophilia patients ( 1991-2001 ) : clinical presentation, management and prognosis factors for death. Haemophilia, 2005,11:452-458.
  • 6Fischer K, van der Bom JG, Mauser-Bunschoten EP, et al. Changes in treatment strategies for severe haemophilia over the last 3 decades: effects on clotting factor consumption and arthropathy. Hnemophilia,2001,7 : 446-452.
  • 7Coppola A, Franchini M, Tagliaferri A. Prophylaxis in people with haemophilia- Thromb Haemost, 2009,101 : 674-681.
  • 8血友病诊断与治疗中国专家共识[J].中华血液学杂志,2011,32(3):212-213. 被引量：43
  • 9Fischer K, van der Bom JG, Mauser-Bunsehoten EP, et al. The effects of postponing prophylaxisctic treatment on long-term outcome in patients with severe hemophilia. Blood, 2002,99: 2337-2341.
  • 10Pollmann H, Richter H, Ringkamp H, et al. When are children diagnosed as having severe haemophilia and when do they start to bleed? A 10-year single-centre PUP study. Eur J Pediatr, 1999, 158 Suppl 3 : S166-170.

二级参考文献38

  • 1郭晔,张磊,竺晓凡,杨仁池.儿童血友病429例临床回顾分析[J].中国实用儿科杂志,2006,21(4):292-295. 被引量：37
  • 2RolfCRL.Intracranial haemorrhage in haemophilia A and B.British Journal of Haematology,2007,140:378-384.
  • 3World Federation of Hemophilia.Guidelines for the management of hemophilia,2005.
  • 4王振义等.血友病.上海:上海科学技术出版社,2007.
  • 5David L.Extending half-life in coagulation factors:where do we stand? Thrombosis Research,2008,122:S2-S8.
  • 6Robert TP,Susan CL,George DK,et al.Prolonged activity of factor IX as a monomeric Fc fusion protein.Blood,Mar 2010,115:2057-2064.
  • 7Thomas W,Wilffied W,Ulrich K,et al.Prolonged in-vivo half-life of factor VIIa by fusion to albumin.Thromb Haemost,2008,99:659-667.
  • 8Samuel L,Murphy,Katherine AH.Gene therapy for haemophilia.British Journal of Haematology,2008,40:479-487.
  • 9Rodriguez-Merchan EC, Heim M. Hemophilia orthopedic manage- ment with emphasis on developing countries. Semin Thromb Hem- ost,2005 ,31:518-526.
  • 10Antunes SV, Vicari P, Cavalheiro S, et al. Intracranial haemor- rhage among a population of haemophilic patients in Brazil. Hae- mophilia. 2003,9:573-577.

共引文献105

中华儿科杂志
2012年 第8期

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