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常染色体显性遗传性多囊肾引起的高血压

Authors
Arlene B Chapman, MD
Frederic F Rahbari-Oskoui, MD, MSCR
William M Bennett, MD
Section Editor
Ronald D Perrone, MD
Deputy Editor
Alice M Sheridan, MD
Translators
韩劲松, 副主任医师

引言

大多数慢性进行性肾脏疾病患者常见高血压。然而，常染色体显性遗传性多囊肾(autosomal dominant polycystic kidney disease, ADPKD)引起高血压的发病机制有些不同。

高血压是ADPKD中常见的早期表现，50%-70%的病例发生在肾小球滤过率(glomerular filtration rate, GFR)明显降低之前，平均发病年龄为30岁[1-3]。然而，发展成为高血压和出现高血压并发症(例如左心室肥大)的趋势出现的更早[4]。与年龄匹配的对照组比较，受累的年轻成人动态血压和左心室质量指数更高，即使数值仍在正常范围内[5,6]。这就提出一个可能性，即治疗血压正常的ADPKD患者可能是有益的。

发病机制

肾素-血管紧张素系统(renin-angiotensin system, RAS)的活性升高和细胞外容积扩张常出现在ADPKD早期(即在血清肌酐升高之前)，对血压升高起到重要作用[7]。囊肿扩张可导致肾脏局部区域缺血并使肾素释放增加，研究表明这至少对初始血压增高起主要作用[1,2]。下面两个观察研究结果符合这一假设：

●含肾素细胞存在于囊肿壁较细的动脉内和囊肿周围结缔组织细胞内[8,9]。囊肿内衬的上皮细胞也可以产生肾素，囊肿液中常存在活性肾素[9]。由于血管紧张素Ⅱ是一种生长因子，肾素可直接促进上皮细胞增生和囊肿生长。

●早期高血压的程度因结构改变的程度不同而有所差异，因为与血压正常的患者相比，血清肌酐正常但血压升高的患者往往囊肿总体积更大[10]。

           

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Literature review current through: 2017-06 . | This topic last updated: 2015-09-04.
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