Clinical Nephrology – Epidemiology – Clinical Trials
Kidney International (2005) 67, 1955–1960; doi:10.1111/j.1523-1755.2005.00294.x
Anemia is a new complication in Fabry disease: Data from the Fabry Outcome Survey
JULIA KLEINERT, FRANÇOIS DEHOUT, ANDREAS SCHWARTING, ABELARDO GARCÍA DE LORENZO, ROBERTA RICCI, CHRISTOPH KAMPMANN, MICHAEL BECK, UMA RAMASWAMI, ALES LINHART, ANDREAS GAL, GUNNAR HOUGE, URS WIDMER , ATUL MEHTA and GERE SUNDER-PLASSMANN
Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria; Department of Nephrology, CHU de Charleroi, Charleroi, Belgium; Department of Nephrology, University of Mainz, Mainz, Germany; Formación Médica Continuada Hospital Universitario La Paz, Madrid, Spain; Institute of Clinical Pediatrics, UCSC, Rome, Italy; Department of Pediatrics, University of Mainz, Mainz, Germany; Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom; Second Department of Internal Medicine, Charles University, Prague, Czech Republic; Institute of Human Genetics, University Hospital Eppendorf, Hamburg, Germany; Department of Paediatrics, University Hospital Haukeland, Bergen, Norway; Department of Medicine, University of Zürich, Zürich, Switzerland; and Department of Haematology, Royal Free Hospital, London, United Kingdom
Correspondence: Gere Sunder-Plassmann, M.D., Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Währinger Gürtel 18–20 A-1090 Wien, Austria. E-mail: Gere.Sunder-Plassmann@meduniwien.ac.at
Received 3 August 2004; Revised 19 September 2004; Re-revised 4 November 2004; Accepted 23 November 2004.
Abstract
Anemia is a new complication in Fabry disease: Data from the Fabry Outcome Survey.
Background
The prevalence and causes of anemia among patients with Fabry disease are unknown.
Methods
In a cross-sectional study we examined hemoglobin concentrations of patients with Fabry disease using a large international database, the Fabry Outcome Survey (FOS), and analyzed the association of renal function, heart failure, gastrointestinal symptoms, and inflammation, with anemia (hemoglobin <12 g/dL in females and <13 g/dL in males).
Results
Anemia was present in 34% of 345 patients with Fabry disease. Median hemoglobin in 158 females was 12.9 g/dL and the median hemoglobin of 187 male patients was 13.2 g/dL. The prevalence of anemia among females was 20%, and among males 47%. Among patients with normal renal function [estimated glomerular filtration rate (GFR) >90 mL/min/1.73 m2] and anemia, heart failure [New York Heart Association (NYHA) class II to IV] and/or elevated C-reactive protein (CRP) levels were documented in 82% of patients. Up to 67% of patients with decreased estimated GFR presented with anemia. There was also a trend for lower hemoglobin levels among patients with signs of inflammation (defined by an elevated CRP level). We observed no association of the presence of gastrointestinal symptoms with anemia. Analyses in 53 patients receiving enzyme replacement therapy for up to 2 years, suggest no effect on anemia.
Conclusion
The results of this study point to a high prevalence of anemia among patients with Fabry disease that is in most instances related to impaired renal function, heart failure, and inflammation. This finding may be of clinical relevance, because anemia is a major risk factor for patients with kidney disease, heart failure, or stroke, which are important manifestations of Fabry disease.
Keywords:
Fabry disease, anemia, renal failure, heart failure, inflammation, enzyme replacement therapy
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