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女性多囊肾病的病理生理学与临床管理

Pathophysiology and clinical management of polycystic kidney disease in women.

作者信息

Wilson P D, Guay-Woodford L

机构信息

Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA.

出版信息

Semin Nephrol. 1999 Mar;19(2):123-32.

Abstract

Women of all geographic, racial, and ethnic groups are afflicted by autosomal dominant polycystic kidney disease (ADPKD), which is the most common, lethal genetic disease that is inherited as a dominant trait. General issues concerning epidemiology, diagnostic testing, clinical management of pain, hypertension, and intracranial aneurysms are discussed, as well as a short overview of our current understanding of the pathophysiological features, including some new molecular insights. Specific issues affecting the care of women are also discussed, including the management of liver cysts and fertility and pregnancy in ADPKD.

摘要

所有地理、种族和族裔群体的女性都会受到常染色体显性多囊肾病(ADPKD)的困扰,这是最常见的致死性遗传性疾病,以显性性状遗传。本文讨论了有关流行病学、诊断检测、疼痛、高血压和颅内动脉瘤的临床管理等一般问题,以及对我们目前对病理生理特征的理解的简要概述,包括一些新的分子见解。还讨论了影响女性护理的具体问题,包括肝囊肿的管理以及ADPKD患者的生育和妊娠问题。

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