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儿童肝豆状核变性的长期治疗与随访管理
Long-term treatment and follow-up management of children with hepatolenticular degeneration
文章发布日期:2017年09月07日  来源:  作者:方峰  点击次数:242次  下载次数:56次

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【摘要】:肝豆状核变性是儿童期最为常见且又是为数不多的可治疗性的常染色体隐性遗传病,若能早期诊断和早期启动终身低铜饮食和排铜治疗,可不发病(症状前诊断者)或实现疾病长期缓解,并可获得良好生活质量和与正常人近似的生存期。可见坚持有效的终身治疗对于改善本病患者的预后至关重要。参照国内外临床指南,以临床病例的实际治疗问题为切入点,从治疗理念与重要性、不同临床类型患者疾病期和维持治疗期及症状前诊断者的治疗方案与随访管理等方面进行讨论。
【Abstract】:Hepatolenticular degeneration is the most common autosomal recessive disorder in childhood and is one of the few diseases that can be treated. Early diagnosis and initiation of lifelong low-copper diet and copper removal therapy can help to reduce the risk of disease onset (for patients who are diagnosed before symptoms appear), achieve long-term remission, and achieve good quality of life and a similar survival time as healthy people. Therefore, sticking to effective lifelong treatment is critical to improving the prognosis of such patients. With reference to clinical guidelines in China and foreign countries and a focus on actual problems in clinical treatment, this article discusses treatment concepts and their importance, as well as treatment regimens and follow-up management for patients with different clinical types in the stages of disease onset and maintenance treatment and patients who are diagnosed before symptoms appear.
【关键字】:肝豆状核变性; 儿童; 治疗; 随访研究
【Key words】:hepatolenticular degeneration; child; thearapy; follow-up studies
【引证本文】:方峰. 儿童肝豆状核变性的长期治疗与随访管理[J]. 临床肝胆病杂志, 2017, 33(10): 1936-1938.

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