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

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 Mor...

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Autores principales: Vô, Bernard, Yombi, Jean Cyr, Aydin, Selda, Demoulin, Nathalie, Yildiz, Halil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6194638/
https://www.ncbi.nlm.nih.gov/pubmed/30340545
http://dx.doi.org/10.1186/s12882-018-1077-0
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author Vô, Bernard
Yombi, Jean Cyr
Aydin, Selda
Demoulin, Nathalie
Yildiz, Halil
author_facet Vô, Bernard
Yombi, Jean Cyr
Aydin, Selda
Demoulin, Nathalie
Yildiz, Halil
author_sort Vô, Bernard
collection PubMed
description 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.
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spelling pubmed-61946382018-10-25 TINU-associated Fanconi syndrome: a case report and review of literature Vô, Bernard Yombi, Jean Cyr Aydin, Selda Demoulin, Nathalie Yildiz, Halil BMC Nephrol Case Report 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. BioMed Central 2018-10-19 /pmc/articles/PMC6194638/ /pubmed/30340545 http://dx.doi.org/10.1186/s12882-018-1077-0 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Vô, Bernard
Yombi, Jean Cyr
Aydin, Selda
Demoulin, Nathalie
Yildiz, Halil
TINU-associated Fanconi syndrome: a case report and review of literature
title TINU-associated Fanconi syndrome: a case report and review of literature
title_full TINU-associated Fanconi syndrome: a case report and review of literature
title_fullStr TINU-associated Fanconi syndrome: a case report and review of literature
title_full_unstemmed TINU-associated Fanconi syndrome: a case report and review of literature
title_short TINU-associated Fanconi syndrome: a case report and review of literature
title_sort tinu-associated fanconi syndrome: a case report and review of literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6194638/
https://www.ncbi.nlm.nih.gov/pubmed/30340545
http://dx.doi.org/10.1186/s12882-018-1077-0
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