遗传性出血性疾病：佩斯卡拉意大利地区血友病中心的研究结果。
Inherited bleeding disorders: results from the Italian Regional Haemophilia Centre of Pescara.
作者信息
Dragani Alfredo, Malizia Raniero, Iuliani Ornella, Di Marzio Ilaria, Davì Giovanni
机构信息
Dipartimento di Ematologia, Centro Regionale delle Malattie Rare del Sangue e degli Organi Emopoietici, Ospedale Santo Spirito, Pescara, Italy.
出版信息
Blood Transfus. 2008 Jul;6(3):136-42. doi: 10.2450/2008.0050-07.
BACKGROUND
Inherited bleeding disorders registries can be useful to improve health care of these rare disorders and document their natural history.
MATERIAL AND METHODS
We analysed the epidemiological, diagnostic and therapeutic aspects of patients managed at an Italian Regional Haemophilia Centre, based in Pescara (in the Region of Abruzzo).
RESULTS
This Regional Haemophilia Centre currently follows 376 patients: 248 with rare clotting factor defects, 33 with von Willebrand's disease, 75 with haemophilia A and 20 with haemophilia B. Three patients with severe haemophilia A have developed inhibitors. Among all the haemophiliacs, the prevalence of hepatitis C virus infection is 21% while the prevalence of human immunodeficiency virus infection is 5.3%. Among the whole haemophilic population referring to the Pescara Haemophilia Centre, 87.4% are treated with recombinant factors while 12 patients with severe haemophilia are receiving primary prophylaxis.
CONCLUSION
In brief, an analysis of the epidemiological, clinical and therapeutic data collected at the Regional Haemophilia Centre of Pescara is a useful tool for monitoring and continuously improving the quality of care of patients with inherited bleeding disorders.
背景
遗传性出血性疾病登记系统有助于改善这些罕见疾病的医疗保健状况并记录其自然病史。
材料与方法
我们分析了位于佩斯卡拉（阿布鲁佐大区）的意大利地区血友病中心所管理患者的流行病学、诊断及治疗方面的情况。
结果
该地区血友病中心目前跟踪376名患者：248名患有罕见凝血因子缺陷，33名患有血管性血友病，75名患有甲型血友病，20名患有乙型血友病。三名重度甲型血友病患者产生了抑制物。在所有血友病患者中，丙型肝炎病毒感染率为21%，而人类免疫缺陷病毒感染率为5.3%。在转诊至佩斯卡拉血友病中心的整个血友病患者群体中，87.4%接受重组因子治疗，12名重度血友病患者正在接受初级预防治疗。
结论
简而言之，对佩斯卡拉地区血友病中心收集的流行病学、临床和治疗数据进行分析，是监测和持续改善遗传性出血性疾病患者护理质量的有用工具。
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