Academic Journal
The impact of demographic and clinical characteristics on the trajectories of health-related quality of life among patients with Fabry disease
| العنوان: | The impact of demographic and clinical characteristics on the trajectories of health-related quality of life among patients with Fabry disease |
|---|---|
| المؤلفون: | Solrun Sigurdardottir, Birgitte Bjerkely, Trond G. Jenssen, Per Mathisen, Charlotte von der Lippe, Kristin Ørstavik, Ketil Heimdal, Dag Olav Dahle, Mina Susanne Weedon-Fekjær, Olga Solberg, Hege K. Pihlstrøm |
| المصدر: | Orphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-11 (2021) |
| بيانات النشر: | BMC, 2021. |
| سنة النشر: | 2021 |
| المجموعة: | LCC:Medicine |
| مصطلحات موضوعية: | Quality of life, HRQOL, SF-36, Longitudinal studies, Fabry disease, ERT, Medicine |
| الوصف: | Abstract Background Fabry disease (FD) is an X-linked lysosomal storage disorder characterized by multiorgan dysfunction. Since individuals with FD usually experience progressive clinical disease manifestations, their health-related quality of life (HRQOL) is expected to change over time. However, there is limited longitudinal research examining HRQOL outcomes in individuals with FD. We aimed to: assess longitudinal outcomes in HRQOL in adults with FD; examine the physical- and mental HRQOL trajectories at the initial registration (baseline), 3–5 year, and 7–13 year follow-ups; and evaluate the possible associations of age, sex and medical complications with the physical- and mental HRQOL trajectories. Methods Forty-three individuals with FD (53% female) who were aged 18 to 81 years at baseline attended clinical follow-up visits between 2006 and 2020. Medical records were extracted retrospectively. Demographics and the 36-item Short-Form Health Survey (SF-36) were recorded at scheduled visits, except for the last data collection which was prospectively obtained in 2020. The physical (PCS) and mental (MCS) composite scores (SF-36) were chosen as outcome measures. Results The eight SF-36 domain scores were stable over a span of 13 years, and only physical- and social functioning domains worsened clinically over this follow-up period. Mean baseline SF-36 domain scores were all significantly lower (decreased HRQOL) in the FD sample compared with Norwegian population norms. Two hierarchical linear models were run to examine whether demographics and medical complications (measured at the last clinical visit) predicted physical and mental HRQOL trajectories. Age above 47 years (p |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 1750-1172 |
| Relation: | https://doaj.org/toc/1750-1172 |
| DOI: | 10.1186/s13023-021-02066-y |
| URL الوصول: | https://doaj.org/article/6eff872ceb1a471793c5e8897534bfa1 |
| رقم الانضمام: | edsdoj.6eff872ceb1a471793c5e8897534bfa1 |
| قاعدة البيانات: | Directory of Open Access Journals |
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