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肾小管间质性肾炎相关范科尼综合征:一例病例报告及文献综述

TINU-associated Fanconi syndrome: a case report and review of literature.

作者信息

Vô Bernard, Yombi Jean Cyr, Aydin Selda, Demoulin Nathalie, Yildiz Halil

机构信息

Department of internal medicine and infectious diseases, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Av. Hippocrate, 1200, Brussels, Belgium.

Institut de Recherche Expérimentale et Clinique, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Av. Hippocrate, 1200, Brussels, Belgium.

出版信息

BMC Nephrol. 2018 Oct 19;19(1):274. doi: 10.1186/s12882-018-1077-0.

Abstract

BACKGROUND

Tubulo-interstitial Nephritis and Uveitis (TINU) syndrome is a rare oculo-renal inflammatory disease. Renal tubular defects are usually found, but full proximal tubular abnormalities have rarely been described.

CASE PRESENTATION

We report the case of a 55-year old woman, native from Morocco, presenting with bilateral, non-granulomatous, anterior uveitis, mild renal insufficiency, leucocyturia and glycosuria. Further work-up showed hypophosphatemia and hyperphosphaturia, hypouricemia and hyperuricosuria, and hyper aminoaciduria, consistent with Fanconi syndrome. A kidney biopsy was obtained and showed diffuse interstitial infiltrates with tubular necrosis. The patient improved after the initiation of a corticosteroid therapy, with tapering dose.

CONCLUSIONS

We reviewed the literature and found nine similar cases. This association mostly occurs in adult woman, without current evidence for an ethnic predilection, unlike previously reported. The renal prognosis seems favorable after corticosteroid therapy, even in case of severe renal injury. Nonetheless mild tubular defects may persist after treatment or spontaneous remission.

摘要

背景

肾小管间质性肾炎和葡萄膜炎(TINU)综合征是一种罕见的眼肾炎症性疾病。通常会发现肾小管缺陷,但很少描述完全的近端肾小管异常。

病例报告

我们报告了一名55岁摩洛哥籍女性的病例,该患者患有双侧非肉芽肿性前葡萄膜炎、轻度肾功能不全、白细胞尿和糖尿。进一步检查显示低磷血症和高磷尿症、低尿酸血症和高尿酸尿症以及高氨基酸尿症,符合范科尼综合征。进行了肾活检,结果显示弥漫性间质浸润伴肾小管坏死。患者在开始使用逐渐减量的皮质类固醇治疗后病情好转。

结论

我们查阅了文献,发现了9例类似病例。这种关联大多发生在成年女性中,与之前报道的不同,目前没有种族倾向的证据。即使在严重肾损伤的情况下,皮质类固醇治疗后的肾脏预后似乎良好。尽管如此,轻度肾小管缺陷可能在治疗后或自发缓解后持续存在。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4c9/6194638/c1e50e3f6c90/12882_2018_1077_Fig1_HTML.jpg

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