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Prevalence of Uncontrolled Hypertension in Patients With Fabry Disease

Kleinert, Julia ; Dehout, François ; Schwarting, Andreas ; de Lorenzo, Abelardo García ; Ricci, Roberta ; Kampmann, Christoph ; Beck, Michael ; Ramaswami, Uma ; Linhart, Ales ; Gal, Andreas ; Houge, Gunnar ; Widmer, Urs ; Mehta, Atul ; Sunder-Plassmann, Gere

In: American Journal of Hypertension, 2006, vol. 19, no. 8, p. 782-787

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    Title
    • Prevalence of Uncontrolled Hypertension in Patients With Fabry Disease
    Author
    • Kleinert, Julia. Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria
    • Dehout, François. Department of Nephrology, CHU de Charleroi, Charleroi, Belgium
    • Schwarting, Andreas. Department of Nephrology, University of Mainz, Mainz, Germany
    • de Lorenzo, Abelardo García. Formación Médica Continuada Hospital Universitario La Paz, Madrid, Spain
    • Ricci, Roberta. Institute of Clinical Pediatrics, UCSC, Rome, Italy
    • Kampmann, Christoph. Department of Pediatrics, University of Mainz, Mainz, Germany
    • Beck, Michael. Department of Pediatrics, University of Mainz, Mainz, Germany
    • Ramaswami, Uma. Department of Paediatrics, University of Cambridge, Cambridge, England
    • Linhart, Ales. Second Department of Internal Medicine, Charles University, Prague, Czech Republic
    • Gal, Andreas. Institute of Human Genetics, University Hospital Eppendorf, Hamburg, Germany
    • Houge, Gunnar. Department of Paediatrics, University Hospital Haukeland, Bergen, Norway
    • Widmer, Urs. Department of Medicine, University of Zürich, Zürich, Switzerland
    • Mehta, Atul. Department of Haematology, Royal Free Hospital, London, England.
    • Sunder-Plassmann, Gere. Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria
    Document Type
    • Postprint
    OAI-PMH Identifier
    • oai:doc.rero.ch:298362
    Summary
    Background: Fabry disease is a rare X-linked disease arising from deficiency of α-galactosidase A. It results in early death related to renal, cardiac, and cerebrovascular disease, which are also important outcomes in patients with elevated blood pressure (BP). The prevalence of uncontrolled hypertension, as well as the effect of enzyme replacement therapy on BP, in patients with Fabry disease is unknown. Methods: We examined uncontrolled hypertension (systolic BP [SBP] ≥130 mm Hg or diastolic BP [DBP] ≥80 mm Hg) among 391 patients with Fabry disease who were participating in the Fabry Outcome Survey (FOS). Results: Uncontrolled hypertension was present in 57% of men and 47% of women. In patients with chronic kidney disease (CKD) stage 1 (n100), median SBP was 120 mm Hg and median DBP was 74 mm Hg. In patients with CKD stage 2 (n172), median SBP was 125 mm Hg and median DBP was 75 mm Hg. In patients with CKD stage 3 (n63), median SBP was 130 mm Hg and median DBP was 75 mm Hg. There was a significant decrease in both SBP and DBP during a 2-year course of enzyme replacement therapy. Conclusions: This study revealed a high prevalence of uncontrolled hypertension among patients with Fabry disease. Thus there is a need to improve BP control and renoprotection in patients with Fabry disease