Patients with Fabry disease on dialysis in the United States

Thadhani, Ravi; Wolf, Myles; West, Michael L.; Tonelli, Marcello; Ruthazer, Robin; Pastores, Gregory M.; Obrador, Gregorio

doi:10.1046/j.1523-1755.2002.00097.x

Patients with Fabry disease on dialysis in the United States

Journal

Kidney International

ISSN

0085-2538

Date Issued

2002

Author(s)

Thadhani, Ravi

Wolf, Myles

West, Michael L.

Tonelli, Marcello

Ruthazer, Robin

Pastores, Gregory M.

Obrador, Gregorio

Facultad de Ciencias de la Salud - CampCM

Type

text::journal::journal article

DOI

10.1046/j.1523-1755.2002.00097.x

URL

https://scripta.up.edu.mx/handle/20.500.12552/2524

Abstract

Background: Fabry disease results from an X-linked deficiency of lysosomal alpha-galactosidase A and is a rare cause of end-stage renal disease. Little is known about the characteristics of patients with Fabry disease that initiate dialysis in the United States, although data from Europe suggests these individuals have a poor survival. Methods: Using the United States Renal Disease System database, we first studied in detail 42 Fabry patients who initiated dialysis between April 1995 (following the introduction of the new detailed HCFA 2728 form) and July 1998. To examine crude survival in a larger cohort, 95 Fabry patients were studied who initiated dialysis between 1985 and 1993, similar to the European Registry. Diabetic and non-diabetic controls matched by age, gender, race, year of dialysis initiation, and initial dialysis modality were examined for comparison. ©Copyright © Elsevier Inc.,