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[威尔逊氏病(肝豆状核变性,肝脑变性)的诊断、治疗及门诊观察。本指南于2015年经俄罗斯第十五届胃肠病学科学协会批准]

[WILSON'S DISEASE (HEPATOLENTICULAR DEGENERATION, DYSTROPHIA HEPATOCEREBRALIS) DIAGNOSIS, TREATMENT AND DISPENSARY OBSERVATION. GUIDELINES WERE APPROVED BY THE XV GASTROENTEROLOGICAL SCIENTIFIC SOCIETY OF RUSSIA IN 2015].

作者信息

Golovanova E V, Lazebnik L B, Konev Yu V, Shaposhnikova N A

出版信息

Eksp Klin Gastroenterol. 2015(7):108-11.

Abstract

Wilson's disease (hepatolenticular degeneration, dystrophia hepatocerebralis)--a chronic, progressive disease with a genetically determined autosomal recessive mode of inheritance. The violence in metabolism of copper with its excessive accumulation in visceral organs and central nervous system is the basis of the disease. The guidelines on clinical diagnosis and management of patients based on a Review and analysis of recent publications on this topic in the global and Domestic literature and on the authors' experience in treating patients with Wilson's disease.

摘要

威尔逊病(肝豆状核变性,脑肝营养不良症)——一种慢性进行性疾病,具有由基因决定的常染色体隐性遗传模式。铜代谢紊乱及其在内脏器官和中枢神经系统中的过度积累是该疾病的基础。基于对全球和国内文献中有关该主题的近期出版物的综述和分析以及作者治疗威尔逊病患者的经验,制定了患者临床诊断和管理指南。

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