Article ; Online: Treatment of Fabry Nephropathy: A Literature Review.
2023 Volume 59, Issue 8
Abstract: Fabry disease is an X-linked inherited lysosomal storage disorder with a deficiency of α-galactosidase A activity, which results in the intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in various organs. Fabry ... ...
Abstract | Fabry disease is an X-linked inherited lysosomal storage disorder with a deficiency of α-galactosidase A activity, which results in the intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in various organs. Fabry nephropathy is one of the major complications of Fabry disease, and kidney damage is often related to cardiovascular disease and mortality. The treatment of Fabry nephropathy thus helps prolong life expectancy. Two treatment options for Fabry nephropathy and cardiopathy are now commercially available: enzyme replacement therapy (agalsidase α agalsidase β, and a biosimilar of agalsidase β) and pharmacological chaperone therapy (migalastat). In this review, we summarize the efficacy of these treatment options for Fabry nephropathy with respect to renal function, proteinuria, and renal pathological findings. We also describe the importance of adjunctive therapy for Fabry nephropathy. |
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MeSH term(s) | Humans ; Fabry Disease/complications ; Fabry Disease/drug therapy ; Kidney Diseases ; Dental Care ; Kidney ; Cardiovascular Diseases |
Language | English |
Publishing date | 2023-08-17 |
Publishing country | Switzerland |
Document type | Journal Article ; Review |
ZDB-ID | 2188113-3 |
ISSN | 1648-9144 ; 1010-660X |
ISSN (online) | 1648-9144 |
ISSN | 1010-660X |
DOI | 10.3390/medicina59081478 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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