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成人起病的急性肾小管间质性肾炎、葡萄膜炎伴范科尼综合征。病例报告及文献复习

Adult-onset acute tubulointerstitial nephritis and uveitis with Fanconi syndrome. Case report and review of the literature.

作者信息

Koike K, Lida S, Usui M, Matsumoto Y, Fukami K, Ueda S, Tamaki K, Kato S, Okuda S

机构信息

Department of Medicine, Division of Nephrology, Kurume University School of Medicine, Kurume, Japan.

出版信息

Clin Nephrol. 2007 Apr;67(4):255-9. doi: 10.5414/cnp67255.

Abstract

We report a case of tubulointerstitial nephritis and uveitis (TINU syndrome) with full type Fanconi syndrome. A 32-year-old woman presented with fatigue, anorexia and weight loss. Laboratory findings showed anemia, polyclonal hypergammaglobulinemia and moderate renal dysfunction. Tubular function abnormalities were normoglycemic glucosuria, panaminoaciduria, phosphaturia and kaliuresis leading to hypokalemia. Renal tubular acidosis and hypouricemia were also evident. Serum antistreptolysin O titer was high. Ocular symptoms (bilateral anterior uveitis) emerged soon after admission. Renal biopsy showed diffuse tubulointerstitial infiltration by lymphocytes and plasma cells without granuloma. Treatment with systemic steroids was given and renal function, and ocular symptom returned to normal with 3 months. Although tubular abnormalities involving TINU syndrome has already been reported, the disease associated with full type Fanconi syndrome has rarely been seen, and systemic steroid may be beneficial in reducing the development of tubulointerstitial injury.

摘要

我们报告一例伴有完全型范科尼综合征的肾小管间质性肾炎和葡萄膜炎(TINU综合征)。一名32岁女性出现疲劳、厌食和体重减轻。实验室检查结果显示贫血、多克隆高球蛋白血症和中度肾功能不全。肾小管功能异常表现为血糖正常性糖尿、全氨基酸尿、磷酸盐尿和钾尿,导致低钾血症。肾小管酸中毒和低尿酸血症也很明显。血清抗链球菌溶血素O滴度升高。入院后不久出现眼部症状(双侧前葡萄膜炎)。肾活检显示淋巴细胞和浆细胞弥漫性浸润肾小管间质,无肉芽肿形成。给予全身类固醇治疗,3个月后肾功能和眼部症状恢复正常。虽然已经报道了涉及TINU综合征的肾小管异常,但与完全型范科尼综合征相关的疾病很少见,全身类固醇可能有助于减少肾小管间质损伤的发生。

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