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Fabry disease: progression of nephropathy, and prevalence of cardiac and cerebrovascular events before enzyme replacement therapy

Nephrology Dialysis Transplantation · 2009 · Vol. 24(7) · pp. 2102–2111

Raphael Schiffmann✉(National Institute of Neurological Disorders and Stroke)David G. Warnock(University of Alabama at Birmingham)Maryam Banikazemi(Icahn School of Medicine at Mount Sinai)Jan Bultas(Charles University)Gabor E. Linthorst(University of Amsterdam)Seymour Packman(University of California, San Francisco)S. A. Sørensen(University of Copenhagen)William R. Wilcox(Cedars-Sinai Medical Center)Robert J. Desnick(Icahn School of Medicine at Mount Sinai)

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Abstract

Baseline proteinuria, reduced baseline eGFR, hypertension and male gender were associated with more rapid progression of Fabry nephropathy. The eGFR progression rate may increase with advancing nephropathy, and may differ between subgroups of patients with Fabry disease.

Lysosomal Storage Disorders Research Carbohydrate Chemistry and Synthesis Glycogen Storage Diseases and MyoclonusMedicineFabry diseaseEnzyme replacement therapyInternal medicineProteinuriaRenal functionNephropathyCardiologyStroke (engine)Renal replacement therapy

MeSH terms

AdolescentAdultAgedFabry DiseaseCerebrovascular DisordersChildChild, PreschoolFemaleHeart DiseasesHumansKidney DiseasesMaleMiddle AgedRetrospective StudiesPrevalence