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肾小球囊性肾病谱:一例病例报告及文献综述

Spectrum of glomerulocystic kidneys: a case report and review of the literature.

作者信息

Dedeoglu I O, Fisher J E, Springate J E, Waz W R, Stapleton F B, Feld L G

机构信息

Department of Pathology, Children's Hospital of Buffalo, NY 14222, USA.

出版信息

Pediatr Pathol Lab Med. 1996 Nov-Dec;16(6):941-9. doi: 10.1080/15513819609168717.

Abstract

An 8-year-old boy developed end-stage renal disease 7 years after the in utero diagnosis of bilateral cystic kidneys. There was no history of cystic renal disease in the family. Initial ultrasonographic screening of the parents failed to reveal cysts in the kidneys. Pathological evaluation of the kidney biopsy findings was consistent with the glomerulocystic kidney disease. He had bilateral nephrectomies in preparation for a living related renal transplant at 7 years of age. At that time, a repeated renal ultrasound examination of the mother showed bilateral cystic kidneys. Pathological evaluation of the nephrectomy specimens confirmed the diagnosis of autosomal dominant polycystic kidney disease. In this report, a discussion of the differential diagnosis of glomerular cysts and the relationship of glomerulocystic kidney disease and autosomal dominant polycystic kidney disease is provided.

摘要

一名8岁男孩在子宫内被诊断为双侧肾囊肿7年后发展为终末期肾病。其家族中无肾囊肿疾病史。对父母进行的初步超声筛查未发现肾脏囊肿。肾活检结果的病理评估与肾小球囊肿性肾病一致。他在7岁时接受了双侧肾切除术,为亲属活体肾移植做准备。当时,对其母亲进行的重复肾脏超声检查显示双侧肾囊肿。肾切除标本的病理评估证实了常染色体显性遗传性多囊肾病的诊断。在本报告中,对肾小球囊肿的鉴别诊断以及肾小球囊肿性肾病与常染色体显性遗传性多囊肾病的关系进行了讨论。

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