Biopsy proven medullary sponge kidney: clinical findings, histopathology, and role of osteogenesis in stone and plaque formation.
Biopsy proven medullary sponge kidney: clinical findings, histopathology, and role of osteogenesis in stone and plaque formation.
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DOI：
10.1002/ar.23105
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发表时间：
2015-05
影响因子：
2
通讯作者：
Coe, Fredric L.
中科院分区：
文献类型：
--
作者：
Evan, Andrew P.;Worcester, Elaine M.;Williams, James C., Jr.;Sommer, Andre J.;Lingeman, James E.;Phillips, Carrie L.;Coe, Fredric L.
关键词：
延髓海绵肾(MSK)与复发性结石形成有关，但临床表型尚不清楚，因为其他疾病的患者可能被错误地标记为MSK。我们研究了12例MSK的病理组织学表现。所有患者都有内窥镜下可识别的乳头畸形类型，可能是节段性的或弥漫性的。由于内髓集合管(IMCD)明显增大，其中含有小的、可移动的导管结石，受影响的乳头增大和波状。患者有频繁的贝里尼导管扩张，偶尔还会有矿物质堵塞。结石可能形成在白色(兰德尔氏)斑块上，但大多数肾盆结石没有附着，其形态与导管结石相似，后者是草酸钙和磷灰石的混合物。患者无尿酸或酸排泄异常；最常见的代谢异常是特发性高钙尿症。虽然Runx2和Osterix在MSK患者的乳头中都有表达，但在基因表达部位没有发现矿物质沉积，这证明这些基因在这一过程中发挥了作用。在特发性钙结石患者中的类似研究表明，这些基因在兰德尔斑块的部位没有表达。MSK中最可能的结石形成机制似乎是由于扩张的IMCD中的尿滞留导致的结晶，随后导管结石进入肾盆，在那里它们可能是结石形成的核心。
Medullary sponge kidney (MSK) is associated with recurrent stone formation, but the clinical phenotype is unclear because patients with other disorders may be incorrectly labeled MSK. We studied 12 patients with histologic findings pathognomonic of MSK. All patients had an endoscopically recognizable pattern of papillary malformation, which may be segmental or diffuse. Affected papillae are enlarged and billowy, due to markedly enlarged inner medullary collecting ducts (IMCD), which contain small, mobile ductal stones. Patients had frequent dilation of Bellini ducts, with occasional mineral plugs. Stones may form over white (Randall’s) plaque, but most renal pelvic stones are not attached, and have a similar morphology as ductal stones, which are a mixture of calcium oxalate and apatite. Patients had no abnormalities of urinary acidification or acid excretion; the most frequent metabolic abnormality was idiopathic hypercalciuria. Although both Runx2 and Osterix are expressed in papillae of MSK patients, no mineral deposition was seen at the sites of gene expression, arguing against a role of these genes in this process. Similar studies in idiopathic calcium stone formers showed no expression of these genes at sites of Randall’s plaque. The most likely mechanism for stone formation in MSK appears to be crystallization due to urinary stasis in dilated IMCD with subsequent passage of ductal stones into the renal pelvis where they may serve as nuclei for stone formation.
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影响因子：
19.6
作者：
HIGASHIHARA, E;NUTAHARA, K;NIIJIMA, T
通讯作者：
NIIJIMA, T
影响因子：
19.6
作者：
Evan, Andrew P.;Lingeman, James E.;Worcester, Elaine M.;Bledsoe, Sharon B.;Sommer, Andre J.;Williams, James C., Jr.;Krambeck, Amy E.;Philips, Carrie L.;Coe, Fredric L.
通讯作者：
Coe, Fredric L.
影响因子：
2.1
作者：
McPhail, E. Fred;Gettman, Matthew T.;Krambeck, Amy E.
通讯作者：
Krambeck, Amy E.
影响因子：
19.6
作者：
Gambaro, G;Feltrin, G;Antonello, A
通讯作者：
Antonello, A
影响因子：
19.6
作者：
Evan, A. P.;Lingeman, J.;Worcester, E.
通讯作者：
Worcester, E.