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大学・研究所にある論文を検索できる 「Improvement in Decline Rate of Estimated Glomerular Filtration Rate after Febuxostat Treatment in a Fabry Disease Patient with Enzyme Replacement Therapy-resistant Proteinuria.」の論文概要。リケラボ論文検索は、全国の大学リポジトリにある学位論文・教授論文を一括検索できる論文検索サービスです。
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Improvement in Decline Rate of Estimated Glomerular Filtration Rate after Febuxostat Treatment in a Fabry Disease Patient with Enzyme Replacement Therapy-resistant Proteinuria.

KUME Shinji 00452235 0000-0001-6937-9715 YASUDA-YAMAHARA Mako 70731941 IMAMURA-UEHARA Yoshimi KUWAGATA Shogo 00791617 YAMAHARA Kosuke 50731915 TAKEDA Naoko 20737655 CHIN-KANASAKI Masami 30402720 KATO Koichi 70736983 0000-0002-6125-0789 OHNO Seiko 20610025 0000-0003-1209-8896 NAKAGAWA Yoshihisa 00378613 MAEGAWA Hiroshi 00209363 0000-0002-4611-8149 滋賀医科大学

2022.10.15

概要

Fabry disease is an inherited lysosomal disorder caused by mutations in the alpha-galactosidase A gene. We herein report a Fabry disease patient with enzyme replacement therapy (ERT)-resistant proteinuria who showed improvement in the estimated glomerular filtration rate (eGFR) decline rate after uric acid (UA)-lowering therapy. The patient was diagnosed with Fabry disease at 36 years old. After that, even under ERT, proteinuria and eGFR decline persisted. During the clinical course, serum UA levels were elevated with increases in renal tubular damage markers. Febuxostat administration immediately improved tubular damage and prevented further eGFR decline. UA-mediated tubulopathy may become an additional therapeutic target for eGFR decline in Fabry disease.

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参考文献

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