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两例先天性肾病综合征。

Two cases of congenital nephrotic syndrome.

作者信息

Iitaka Kikuo, Motoyama Osamu, Hojo Midori, Iwanami Naomi, Koshino Hiroe, Nakamura Shinya, Moriya Shunsuke

机构信息

Department of Pediatrics, Yamato City Hospital, 8-3-6 Fukaminishi, Yamato, Kanagawa 242-8602, Japan.

出版信息

Clin Exp Nephrol. 2004 Jun;8(2):146-9. doi: 10.1007/s10157-003-0273-8.

Abstract

The clinical course and renal histological changes in two patients with congenital nephrotic syndrome are presented. Both patients developed edema and proteinuria at 2 days of age. Steroids and immunosuppressive drugs were not used in either patient. One patient showed gradual disappearance of nephrotic syndrome and proteinuria during the 16-month follow-up. In the other patient, the nephrotic state persisted and nephrotic complications necessitated bilateral nephrectomy when she was 12 months old. Renal biopsy showed minimal change lesions in one patient, while the nephrectomized kidneys showed mild mesangial proliferation and matrix increase in the glomeruli. No tubular dilatation or microcysts were observed in either patient.

摘要

本文介绍了两名先天性肾病综合征患者的临床病程及肾脏组织学变化。两名患者均在出生2天时出现水肿和蛋白尿。两名患者均未使用类固醇和免疫抑制药物。一名患者在16个月的随访期间肾病综合征和蛋白尿逐渐消失。另一名患者肾病状态持续存在,12个月大时因肾病并发症而行双侧肾切除术。肾活检显示一名患者为微小病变,而切除的肾脏显示肾小球系膜轻度增生和基质增加。两名患者均未观察到肾小管扩张或微囊肿。

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