Kidney function as an underestimated factor for reduced health related quality of life in patients with Fabry disease

BMC Nephrology

Christoph Wanner⁴  Frank Breunig¹  Frank Weidemann³  Dorothee Vergho¹  Elisabeth Blohm²  Johannes Krämer³  Martin Wagner⁴ 

[1] Department of Medicine I, Division of Nephrology, University Hospital Würzburg, Oberdürrbacherstr. 6, Würzburg 97080, Germany;Department of Anaesthesia and Critical Care, Zentrum Innere Medizin, University Hospital Würzburg, Oberdürrbacherstr. 6, Würzburg 97080, Germany;Department of Medicine I, Division of Cardiology, University Hospital Würzburg, Oberdürrbacherstr. 6, Würzburg 97080, Germany;Comprehensive Heart Failure Center, University of Würzburg, Oberdürrbacherstr. 6, Würzburg 97080, Germany

关键词: Fabry disease;    Chronic kidney disease;    SF-36;    Quality of life;   

Others  :  1082552
DOI  :  10.1186/1471-2369-15-188

 received in 2014-06-26, accepted in 2014-11-19,  发布年份 2014

【 摘 要 】

Background

Impairments of health related quality of life (HRQoL) are frequently observed in Fabry disease (FD) and are known to be related to neuropathic pain and cardiovascular events. This study aimed to explore the role of chronic kidney disease (CKD) in a large cohort of patients with FD.

Methods

In 96 patients (53% female; age 40 ± 12 yrs) with genetically proven FD, HRQoL was assessed by the Medical Outcomes Study (SF-36) questionnaire. All patients were naïve to enzyme replacement therapy. Three categories for kidney dysfunction were chosen, eGFR ≥/<60 ml/min/1.73 m² or need of renal replacement therapy (RRT). Minor (e.g. arrhythmia, angina pectoris, etc.) and major (e.g. myocardial infarction, coronary artery bypass, stroke or implantable cardioverter-defibrillator) vascular events as well as pain and pain therapy were considered in linear regression analyses with the dimensions of HRQoL.

Results

Ten patients (10%) had impaired kidney function and a further nine were on RRT (9.4%). Kidney function and pain emerged as the main factors associated with lower scores on the SF 36, in particular on physical components (PCS beta-coefficients for CKD −6.2, for RRT −11.8, for pain −9.1, p < 0.05, respectively), while controlling for gender, vascular event and pain-therapy. Relationships were found for mental aspects of HRQoL. Age and history of vascular events were not related to HRQoL.

Conclusion

Cardiovascular events and pain are important factors related to HRQoL, social functioning and depression. Our study highlights impaired chronic kidney disease, in particular after initiation of RRT, as a strong determinant of reduced HRQoL in FD.

【 授权许可】

   
2014 Wagner et al.; licensee BioMed Central Ltd.

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