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常染色体显性多囊肾病的预后评估及治疗

薛澄 周晨辰 梅长林

薛澄, 周晨辰, 梅长林. 常染色体显性多囊肾病的预后评估及治疗[J]. 协和医学杂志, 2018, 9(1): 75-80. doi: 10.3969/j.issn.1674-9081.2018.01.014
引用本文: 薛澄, 周晨辰, 梅长林. 常染色体显性多囊肾病的预后评估及治疗[J]. 协和医学杂志, 2018, 9(1): 75-80. doi: 10.3969/j.issn.1674-9081.2018.01.014
Cheng XUE, Chen-chen ZHOU, Chang-lin MEI. Prognostic Evaluation and Treatment of Autosomal Dominant Polycystic Kidney Disease[J]. Medical Journal of Peking Union Medical College Hospital, 2018, 9(1): 75-80. doi: 10.3969/j.issn.1674-9081.2018.01.014
Citation: Cheng XUE, Chen-chen ZHOU, Chang-lin MEI. Prognostic Evaluation and Treatment of Autosomal Dominant Polycystic Kidney Disease[J]. Medical Journal of Peking Union Medical College Hospital, 2018, 9(1): 75-80. doi: 10.3969/j.issn.1674-9081.2018.01.014

常染色体显性多囊肾病的预后评估及治疗

doi: 10.3969/j.issn.1674-9081.2018.01.014
基金项目: 

国家重点研发计划 2016YFC0901500

国家自然科学基金面上项目 81670612

上海地区慢性肾脏病早发现和诊疗体系建设与示范 GWIV-18

详细信息
    通讯作者:

    MEI Chang-lin Tel::021-81885411, E-mail:chlmei1954@126.com

  • 中图分类号: R692.1

Prognostic Evaluation and Treatment of Autosomal Dominant Polycystic Kidney Disease

  • 摘要: 常染色体显性多囊肾病(autosomal dominant polycystic kidney disease, ADPKD)患病率为1‰~2‰, 属于罕见病, 临床主要表现为双侧肾囊肿且逐渐发展, 肾脏体积进行性增大, 肾功能逐步降低。PKD1基因突变约占81%, PKD2基因突变约占10.5%~22%。血管加压素(arginine vasopressin, AVP)和环磷酸腺苷(cyclic adenosine monophosphate, cAMP)信号通路在ADPKD囊肿发展过程中发挥重要作用。近年来发表的梅奥风险评估模型和PROPKD(predicting renal outcome in polycystic kidney disease)评分是ADPKD较好的预后评估模型, 已成为临床医生决策的重要依据。通过拮抗AVP受体, 抑制cAMP通路的托伐普坦已成为ADPKD首个特异治疗药物, 可有效抑制总肾脏体积的增长和保护肾功能。药物的长期安全性仍需进一步研究。
  • 图  1  NICE指南制定的ADPKD诊疗决策流程图

    ADPKD、TKV、eGFR、ESRD:同表 1; MRI:磁共振成像;NICE:英国国家卫生保健优化研究所

    表  1  ADPKD的梅奥风险评估模型

    模型 1A 1B 1C 1D 1E
    TKV年增长率(%) <1.5 1.5~3.0 3.0~4.5 4.5~6.0 >6.0
    eGFR年下降值[ml/(min·1.73 m2)] 0.1 -1.2 -2.5 -3.4 -4.6
    10年ESRD发病风险(%) 2.4 11.0 37.8 47.1 66.9
    ADPKD:常染色体显性多囊肾病;TKV:总肾脏体积;eGFR:肾小球滤过率估计值;ESRD:终末期肾病
    下载: 导出CSV
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出版历程
  • 收稿日期:  2017-05-01
  • 刊出日期:  2018-01-30

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