Background: Impairments of health related quality of life (HRQoL) are frequently observed in Fabry disease (FD)\nand are known to be related to neuropathic pain and cardiovascular events. This study aimed to explore the role of\nchronic kidney disease (CKD) in a large cohort of patients with FD.\nMethods: In 96 patients (53% female; age 40 �± 12 yrs) with genetically proven FD, HRQoL was assessed by the\nMedical Outcomes Study (SF-36) questionnaire. All patients were na�¯ve to enzyme replacement therapy. Three\ncategories for kidney dysfunction were chosen, eGFR ?/<60 ml/min/1.73 m2 or need of renal replacement therapy\n(RRT). Minor (e.g. arrhythmia, angina pectoris, etc.) and major (e.g. myocardial infarction, coronary artery bypass,\nstroke or implantable cardioverter-defibrillator) vascular events as well as pain and pain therapy were considered in\nlinear regression analyses with the dimensions of HRQoL.\nResults: Ten patients (10%) had impaired kidney function and a further nine were on RRT (9.4%). Kidney function\nand pain emerged as the main factors associated with lower scores on the SF 36, in particular on physical\ncomponents (PCS beta-coefficients for CKD ?6.2, for RRT ?11.8, for pain ?9.1, p < 0.05, respectively), while\ncontrolling for gender, vascular event and pain-therapy. Relationships were found for mental aspects of HRQoL.\nAge and history of vascular events were not related to HRQoL.\nConclusion: Cardiovascular events and pain are important factors related to HRQoL, social functioning and\ndepression. Our study highlights impaired chronic kidney disease, in particular after initiation of RRT, as a strong\ndeterminant of reduced HRQoL in FD.
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