title: Prevalence of uncontrolled hypertension in patients with Fabry disease.
abstract: BACKGROUND
  Fabry disease is a rare X-linked disease arising from deficiency of alpha-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] >or=130 mm Hg or diastolic BP [DBP] >or=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.
authors.affiliation: Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria.
authors.forename: Julia
authors.initials: J
authors.lastname: Kleinert
authors.forename: François
authors.initials: F
authors.lastname: Dehout
authors.forename: Andreas
authors.initials: A
authors.lastname: Schwarting
authors.forename: Abelardo García
authors.initials: AG
authors.lastname: de Lorenzo
authors.forename: Roberta
authors.initials: R
authors.lastname: Ricci
authors.forename: Christoph
authors.initials: C
authors.lastname: Kampmann
authors.forename: Michael
authors.initials: M
authors.lastname: Beck
authors.forename: Uma
authors.initials: U
authors.lastname: Ramaswami
authors.forename: Ales
authors.initials: A
authors.lastname: Linhart
authors.forename: Andreas
authors.initials: A
authors.lastname: Gal
authors.forename: Gunnar
authors.initials: G
authors.lastname: Houge
authors.forename: Urs
authors.initials: U
authors.lastname: Widmer
authors.forename: Atul
authors.initials: A
authors.lastname: Mehta
authors.forename: Gere
authors.initials: G
authors.lastname: Sunder-Plassmann
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D001794:Blood Pressure; D003956:Dialysis; D000795:Fabry Disease / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D006973:Hypertension / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology* / Q000503:physiopathology; D007668:Kidney / Q000502:physiology; D016030:Kidney Transplantation; D008297:Male; D015995:Prevalence; D012744:Sex Ratio
chemical_list: D000959:Antihypertensive Agents