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常染色体显性遗传多囊肾病的治疗研究进展
引用本文:高晋生,杨宝学. 常染色体显性遗传多囊肾病的治疗研究进展[J]. 中国药理学通报, 2009, 25(2)
作者姓名:高晋生  杨宝学
作者单位:北京大学医学部基础医学院药理系,北京,100191
基金项目:面向21世纪教育振兴行动计划(985计划),国家自然科学基金 
摘    要:常染色体显性遗传多囊肾病是一种较常见的单基因遗传病,病变以双肾多发性进行性充液囊泡为主要特征。囊泡损伤肾组织,引起肾功能改变,最终导致肾衰竭。除透析和肾移植治疗终末期肾衰竭,尚无有效疗法延缓多囊肾病程进展。抑制囊泡液体分泌,抗囊泡上皮细胞生长和减轻继发病变是治疗该病的主要策略。

关 键 词:常染色体显性遗传多囊肾病  多囊肾病治疗  多囊蛋白  抗细胞增殖  抑制囊液分泌

Progress in treatment of autosomal dominant polycystic kidney disease
GAO Jin-sheng,YANG Bao-xue. Progress in treatment of autosomal dominant polycystic kidney disease[J]. Chinese Pharmacological Bulletin, 2009, 25(2)
Authors:GAO Jin-sheng  YANG Bao-xue
Abstract:Autosomal dominant polycystic kidney disease(ADPKD),a common inherited disease,is characterized by massive enlargement of fluid-filled renal cysts.Progressively enlarging cysts compromise normal renal parenchyma,reduce renal function and lead to renal failure.Up to now,the treatment options for ADPKD have been limited to renal replacement therapy by dialysis or by transplantation for patients with end-stage renal failure.Inhibition of cyst fluid secretion,suppression of cyst epithelial cell growth and prevention of renal failure are new approaches to treat PKD.
Keywords:ADPKD  treatment of PKD  polycystin  suppression of cell growth  inhibition of cyst fluid secretion
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