End-stage renal disease in patients with Fabry disease: natural history data from the Fabry Registry

• Tipo: Artigo em Revista Científica Internacional
• Data: 2010

Autores

Ortiz, A (Autor) Outra; Cianciaruso, B (Autor) Outra; Cizmarik, M (Autor) Outra; Germain, DP (Autor) Outra; Mignani, R (Autor) Outra; João Paulo Oliveira (Autor) FMUP; Villalobos, J (Autor) Outra; Vujkovac, B (Autor) Outra; Waldek, S (Autor) Outra; Wanner, C (Autor) Outra; Warnock, DG (Autor) Outra

Revista

Título: Nephrology Dialysis Transplantation

Vol. 25 Nº 9

Páginas: 769-775

ISSN: 0931-0509

Editora: Oxford University Press

Indexação

ISI Web of Knowledge - 90 Citações

Scopus - 98 Citações

Classificação Científica

FOS: Ciências médicas e da saúde > Outras ciências médicas

Outras Informações

ID Authenticus: P-003-8XV

DOI: 10.1093/ndt/gfp554

Abstract (EN): Methods. Data from 2712 patients in the Fabry Registry were analysed to identify clinical characteristics of patients who received renal replacement therapy (RRT) during the natural history period (i.e. prior to any enzyme replacement therapy). Results. A total of 213 patients [186 of 1359 males (14%) and 27 of 1353 females (2%)] received RRT at a median age of 38 years in both males and females. Males who received RRT were diagnosed at a median age of 35 years, compared to 23 years for non-RRT males. Sixty-one males and 10 females were not diagnosed with Fabry disease until after they had received RRT. Compared to other Fabry Registry patients, a higher percentage of RRT patients also experienced either a serious cardiovascular event or a stroke. Ninety-two of 186 males who had RRT (50%) experienced a cardiac event or stroke, compared to 230 of 1173 non-RRT males (20%). Ten of 27 RRT females (37%) had experienced a cardiac event or stroke, compared to 226 of 1326 non-RRT females (17%). Patients who had RRT experienced cardiovascular events and strokes at earlier ages than did patients who had not received RRT, and most received RRT before having a cardiac event or stroke. Conclusions. While all Fabry patients are at risk of cardiovascular events and strokes, patients with Fabry nephropathy who develop kidney failure appear to have concurrent involvement of other major organ systems. It is important that Fabry patients are diagnosed early and that their renal function is monitored carefully.

Idioma: Inglês

Tipo (Avaliação Docente): Científica

Nº de páginas: 7