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波斯猫和波斯杂交猫的常染色体显性多囊肾病

Autosomal dominant polycystic kidney disease in Persian and Persian-cross cats.

作者信息

Eaton K A, Biller D S, DiBartola S P, Radin M J, Wellman M L

机构信息

Department of Veterinary Biosciences, College of Veterinary Medicine, Ohio State University, Columbus, USA.

出版信息

Vet Pathol. 1997 Mar;34(2):117-26. doi: 10.1177/030098589703400204.

DOI:10.1177/030098589703400204
PMID:9066078
Abstract

A form of autosomal dominant polycystic kidney disease (ADPKD) similar in clinical features to human ADPKD occurs in the Persian cat. We characterized the morphologic and immunohistochemical features of this disease in a colony of affected cats. Complete postmortem examinations were performed on 11 normal and 22 affected cats ranging in age from 3 months to 10 years. Kidneys were evaluated by gross and histologic examinations, ultrastructure, lectin staining, bromodeoxyuridine immunochemistry for labeling index and immunochemistry for distribution of Na/K ATPase. Feline ADPKD was characterized by variable numbers of cysts in the renal cortex and medullar. Ultrastructural examination and lectin staining suggested that cysts arose from proximal and distal nephron segments. Bromodeoxyuridine labeling demonstrated increased proliferation of epithelium lining some cysts in young cats. Immunohistochemical staining showed variable translocation of Na/K ATPase from the basolateral membranes of cyst-lining cells to the cytoplasm or luminal membranes. Cystic renal disease commonly was associated with chronic tubulointerstitial nephritis and hepatobiliary hyperplasia and fibrosis. Focal hyperplasia of renal tubular epithelium, hepatic cysts, and cardiac lesions were present in some cats. Feline ADPKD shares many morphologic and pathogenetic features with human ADPKD.

摘要

波斯猫会出现一种常染色体显性多囊肾病(ADPKD),其临床特征与人类的ADPKD相似。我们对一群患病猫的这种疾病的形态学和免疫组化特征进行了描述。对11只正常猫和22只患病猫进行了完整的尸检,这些猫的年龄从3个月到10岁不等。通过大体和组织学检查、超微结构、凝集素染色、用于标记指数的溴脱氧尿苷免疫化学以及用于Na/K ATP酶分布的免疫化学对肾脏进行评估。猫ADPKD的特征是肾皮质和髓质中有数量不等的囊肿。超微结构检查和凝集素染色表明囊肿起源于近端和远端肾单位节段。溴脱氧尿苷标记显示幼猫中一些囊肿内衬上皮细胞的增殖增加。免疫组化染色显示Na/K ATP酶从囊肿内衬细胞的基底外侧膜向细胞质或腔膜发生不同程度的易位。囊性肾病通常与慢性肾小管间质性肾炎以及肝胆增生和纤维化有关。一些猫存在肾小管上皮细胞局灶性增生、肝囊肿和心脏病变。猫ADPKD与人类ADPKD具有许多形态学和发病机制特征。

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