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狼疮血清学检查阴性患者的满堂亮肾病

Full-house nephropathy in a patient with negative serology for lupus.

作者信息

Baskin Esra, Agras Pinar Isik, Menekşe Nurcan, Ozdemir Handan, Cengiz Nurcan

机构信息

Department of Pediatric Nephrology, Baskent University, 6, Cadde No: 72/3, O6490, Bahcelievler, Ankara, Turkey.

出版信息

Rheumatol Int. 2007 Jan;27(3):281-4. doi: 10.1007/s00296-006-0198-0. Epub 2006 Sep 14.

Abstract

A 10-year-old girl presented with a complaint of recurrent abdominal pain. Physical examination findings were unremarkable. Laboratory investigations revealed BUN of 17 mg/dl and creatinine of 1 mg/dl, and complement levels were normal. She had neither hematuria nor proteinuria, and glomerular filtration rate was 60.9 ml/min/1.73 m(2). ANA, anti-DNA, p-ANCA and c-ANCA were all negative. Renal biopsy revealed findings of class III lupus nephritis in light, "full-house" nephropathy in immune fluorescent and tubuloreticular inclusions in electron microscopic examinations. After 17 months of treatment, her last creatinine is 2.5 mg/dl and GFR is 17.9 ml/min/1.73 m(2) and ANA and anti-DNA remain still negative. This case presents an example that decreased GFR can be the first presenting symptom of full-house nephropathy. Those patients who have negative lupus serology and renal biopsy findings of full-house nephropathy and tubuloreticular inclusions may behave and should be treated as lupus nephritis.

摘要

一名10岁女孩因反复腹痛前来就诊。体格检查未发现异常。实验室检查显示血尿素氮为17mg/dl,肌酐为1mg/dl,补体水平正常。她既无血尿也无蛋白尿,肾小球滤过率为60.9ml/min/1.73m²。抗核抗体、抗双链DNA抗体、抗中性粒细胞胞浆抗体(p-ANCA)和抗中性粒细胞胞浆抗体(c-ANCA)均为阴性。肾活检在光学显微镜下显示为Ⅲ型狼疮性肾炎,免疫荧光检查显示为“满堂亮”肾病,电子显微镜检查显示有管网状包涵体。经过17个月的治疗,她最后的肌酐水平为2.5mg/dl,肾小球滤过率为17.9ml/min/1.73m²,抗核抗体和抗双链DNA抗体仍为阴性。该病例表明肾小球滤过率降低可能是“满堂亮”肾病的首发症状。那些狼疮血清学检查阴性但肾活检有“满堂亮”肾病和管网状包涵体表现的患者,其病情表现及治疗应按照狼疮性肾炎处理。

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