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戈谢病:治疗与病因学的新进展

Gaucher disease: new developments in treatment and etiology.

作者信息

Harmanci Ozgur, Bayraktar Yusuf

机构信息

Department of Gastroenterology, Hacettepe University Faculty of Medicine, Sihhiye 06100, Ankara, Turkey.

出版信息

World J Gastroenterol. 2008 Jul 7;14(25):3968-73. doi: 10.3748/wjg.14.3968.

Abstract

Gaucher disease (GD) is an autosomal recessive disease which if undiagnosed or diagnosed late results in devastating complications. Because of the heterozygous nature of GD, there is a wide spectrum of clinical presentation. Clinicians should be aware of this rare but potentially treatable disease in patients who present with unexplained organomegaly, anemia, massive splenomegaly, ascites and even cirrhosis of unknown origin. The treatment options for adult type GD include enzyme replacement treatment (ERT) and substrate reduction treatment (SRT) depending on the status of the patient. Future treatment options are gene therapy and "smart molecules" which provide specific cure and additional treatment options. In this review, we present the key issues about GD and new developments that gastroenterologists should be aware of.

摘要

戈谢病(GD)是一种常染色体隐性疾病,若未被诊断或诊断较晚,会导致严重的并发症。由于戈谢病的杂合性质,其临床表现具有广泛的谱系。临床医生应在出现不明原因的器官肿大、贫血、巨脾、腹水甚至不明原因肝硬化的患者中,意识到这种罕见但有可能治疗的疾病。成人型戈谢病的治疗选择包括根据患者状况进行酶替代疗法(ERT)和底物减少疗法(SRT)。未来的治疗选择是基因疗法和“智能分子”,它们可提供特异性治愈方法及其他治疗选择。在本综述中,我们介绍了关于戈谢病的关键问题以及胃肠病学家应了解的新进展。

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