Renal histopathology of stone-forming patients with distal renal tubular acidosis
Renal histopathology of stone-forming patients with distal renal tubular acidosis
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DOI：
10.1038/sj.ki.5002113
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发表时间：
2007-04-01
影响因子：
19.6
通讯作者：
Worcester, E.
中科院分区：
文献类型：
--
作者：
Evan, A. P.;Lingeman, J.;Worcester, E.
为了明确远端肾小管酸中毒（dRTA）结石形成患者的肾组织变化，我们对5例患者进行了术中乳头和皮质活检。主要异常是内髓集合管（IMCD）和Bellini管（BD）被磷灰石形式的磷酸钙沉积物堵塞;上皮细胞损伤和丢失明显。堵塞的导管被间质纤维化包围，但纤维化也是普遍的，即使不存在堵塞，纤维化也是组织病理学的主要特征。与此相反，常见的特发性草酸钙结石（SF）患者从不出现小管内晶体或间质纤维化。透钙磷石（磷酸一氢钙）结石和胱氨酸结石患者的IMCD和BD被磷灰石（或胱氨酸，在胱氨酸尿症中）堵塞的情况要少得多，间质纤维化仅限于堵塞导管周围的区域。dRTA患者通常会出现肾钙质沉着的影像学表现。我们的直接手术观察显示，这些可能是手术可取的结石，特别是在肾功能保留良好的患者中。总之，dRTA SF比其他研究的SF具有更弥漫的乳头状肾病，并且在间质纤维化程度方面也是不寻常的。
To define the renal tissue changes in stone-forming patients with distal renal tubular acidosis (dRTA), we performed intra-operative papillary and cortical biopsies in five patients. The main abnormalities were plugging of inner medullary collecting ducts (IMCD) and Bellini ducts (BD) with deposits of calcium phosphate in the form of apatite; epithelial cell injury and loss was marked. Plugged ducts were surrounded by interstitial fibrosis, but the fibrosis was generalized, as well, and was a main feature of the histopathology even when plugging was not present. In contrast, common idiopathic calcium oxalate stone formers (SF) never manifest intratubule crystals or interstitial fibrosis. Patients with brushite ( calcium monohydrogen phosphate) stones and those with cystine stones have many fewer IMCD and BD plugged with apatite ( or cystine, in cystinuria), and interstitial fibrosis is limited to the regions around plugged ducts. Patients with dRTA often present a radiographic picture of nephrocalcinosis. Our direct surgical observations reveal that these may be surgically removable stones, especially in patients with well preserved renal function. In all, dRTA SF have a more diffuse papillary renal disease than other SF thus studied, and are also unusual for the degree of interstitial fibrosis.