Expression of components of the renin-angiotensin system in autosomal recessive polycystic kidney disease
Expression of components of the renin-angiotensin system in autosomal recessive polycystic kidney disease
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DOI：
10.1369/jhc.4a6494.2005
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发表时间：
2005-08-01
影响因子：
3.2
通讯作者：
Sotelo-Avila, C
中科院分区：
文献类型：
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作者：
Loghman-Adham, M;Soto, CE;Sotelo-Avila, C
高血压是常染色体隐性遗传性多囊肾病 (ARPKD) 新生儿期存活儿童的常见并发症。没有关于这种情况下高血压机制的信息。肾素-血管紧张素系统（RAS）被认为在与更常见的常染色体显性多囊肾病（ADPKD）相关的高血压中发挥作用。偶尔有报告记录 ADPKD 中肾内 RAS 活性增加，囊肿和扩张的肾小管内有异位肾素表达。由于 ARPKD 和 ADPKD 之间的相似性，我们假设 ARPKD 中也可能发现肾内 RAS 活性增加。我们对两名患有 ARPKD 的婴儿和两个对照肾脏的肾脏组织进行了免疫组织化学研究。囊性扩张的小管显示出花生凝集素花生凝集素（远端小管和集合管的标记物）染色，但没有被莲花四角叶（近端小管的标记物）染色。在 ARPKD 肾脏的许多囊肿和肾小管中观察到强烈的肾素染色，但仅在正常对照肾脏的传入小动脉中观察到。在一些囊肿和近端小管中也观察到血管紧张素原染色。许多囊性扩张小管中存在血管紧张素转换酶、血管紧张素II I型受体和血管紧张素II肽的染色。这些免疫组织化学研究首次记录了 ARPKD 囊性扩张小管中 RAS 成分的异位表达，并表明 RAS 过度活跃可能导致肾内血管紧张素 II 产生增加，这可能导致 ARPKD 发生高血压。
Hypertension is a common complication in children with autosomal recessive polycystic kidney disease (ARPKD) who have survived the neonatal period. No information is available regarding the mechanism of hypertension in this condition. The renin-angiotensin system (RAS) is thought to play a role in hypertension associated with the more common autosomal dominant polycystic kidney disease (ADPKD). Occasional reports have documented increased activity of the intrarenal RAS in ADPKD, with ectopic renin expression within cysts and dilated tubules. Because of similarities between ARPKD and ADPKD, we hypothesized that increased intrarenal RAS activity might also be found in ARPKD. We performed immunohistochemical studies on kidney tissues from two infants with ARPKD and two control kidneys. The cystic dilated tubules showed staining with the peanut lectin arachis hypogaea, a marker of distal tubules and collecting ducts, but not with lotus tetragonolobus, a marker of proximal tubules. Strong renin staining was seen in many cysts and tubules of ARPKD kidneys, but only in the afferent arterioles of the normal control kidneys. Angiotensinogen staining was also observed in some cysts and in proximal tubules. Staining for angiotensin-converting enzyme, angiotensin II type I receptor, and angiotensin II peptide was present in many cystic dilated tubules. These immunohistochemical studies document for the first time ectopic expression of components of the RAS in cystic-dilated tubules of ARPKD and suggest that overactivity of RAS could result in increased intrarenal angiotensin II production, which may contribute to the development of hypertension in ARPKD.