Hemophilia in Children

S. Islam, A. Morshed, Z. J. Khan
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引用次数: 2

Abstract

Hemophilia is the most common serious congenital coagulation factor deficiencies. The prevalence of hemophilia is estimated to be about 1:10,000 birth and that of the severe form of the disease to be about 6% per 1,00,000 population. The most pathetic part of this disease is that even medical personnel are sometimes not familiar with its diagnosis and management. There is obviously a need to establish facilities and treatment options that will help the patient with hemophilia to manage their life with ease. As this is a genetic disorder no complete cure is possible as of now. The only available treatment option is the infusion of factors and some adjuvant therapies depending upon the bleeding conditions .Hemophilia, if not properly managed, can lead to chronic disease and lifelong disabilities. The challenges and issues in infants/young children are different from those in older children and adults although episodes of bleeding still predominate as the diagnostic trigger. Currently, inhibitor development is a challenging complication of pediatric hemophilia and prophylaxis is emerging as the optimal preventive care strategy. In this section we will review some important aspects of hemophilia in children including early prophylaxis, current evidence relating to inhibitor development. J. Paediatr. Surg. Bangladesh 6 (2): 54-63, 2015 (July)
儿童血友病
血友病是最常见的严重先天性凝血因子缺乏。血友病的患病率估计约为1:10 000名新生儿,严重形式的血友病患病率约为每10万人中6%。这种疾病最可悲的部分是,即使是医务人员有时也不熟悉它的诊断和治疗。显然,有必要建立设施和治疗方案,以帮助血友病患者轻松地管理他们的生活。由于这是一种遗传性疾病,目前还不可能完全治愈。唯一可用的治疗选择是根据出血情况注射因子和一些辅助治疗。血友病如果管理不当,可能导致慢性疾病和终身残疾。婴儿/幼儿的挑战和问题不同于年龄较大的儿童和成人,尽管出血发作仍然是主要的诊断触发因素。目前,抑制剂的发展是儿童血友病的一个具有挑战性的并发症,预防正在成为最佳的预防保健策略。在本节中,我们将回顾儿童血友病的一些重要方面,包括早期预防,目前与抑制剂发展有关的证据。j . Paediatr。孟加拉外科6 (2):54-63,2015 (7)
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