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RESEARCH PRODUCT
Prevalence of Uncontrolled Hypertension in Patients With Fabry Disease
Julia KleinertGunnar HougeGere Sunder-plassmannUma RamaswamiAleš LinhartAtul MehtaAndreas SchwartingUrs WidmerAndreas GalRoberta RicciF DehoutChristoph KampmannMichael BeckAbelardo García De Lorenzosubject
Malemedicine.medical_specialtymedicine.medical_treatmentDiastoleRenal functionBlood PressureKidneyInternal medicinePrevalenceInternal MedicinemedicineHumansSex Ratiocardiovascular diseasesAntihypertensive AgentsKidney transplantationDialysisbusiness.industryEnzyme replacement therapymedicine.diseaseKidney TransplantationFabry diseaseSurgeryBlood pressureHypertensionCardiologyFabry DiseaseFemalebusinessDialysiscirculatory and respiratory physiologyKidney diseasedescription
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.
year | journal | country | edition | language |
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2017-08-02 | American Journal of Hypertension |