Determinants of impaired quality of life in patients with fibrous dysplasia
Autor: | Jens Bruggemann, Cornelie D. Andela, B.C.J. Majoor, Neveen A. T. Hamdy, Natasha M. Appelman-Dijkstra, P. D. Sander Dijkstra, Michiel A. J. van de Sande, Ad A. Kaptein |
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Jazyk: | angličtina |
Rok vydání: | 2017 |
Předmět: |
Adult
Male 0301 basic medicine Quality of life medicine.medical_specialty Adolescent lcsh:Medicine Pain 030209 endocrinology & metabolism Fibrous Dysplasia Polyostotic Fibrous dysplasia McCune–Albright syndrome Young Adult 03 medical and health sciences 0302 clinical medicine Surveys and Questionnaires Internal medicine Biochemical markers of bone turnover Humans Medicine Pharmacology (medical) Brief Pain Inventory Young adult Child Genetics (clinical) Disease burden Aged business.industry Research lcsh:R McCune-Albright syndrome Infant General Medicine Middle Aged medicine.disease Monostotic fibrous dysplasia 030104 developmental biology Endocrinology Child Preschool Cohort Female business Fibrous Dysplasia Monostotic |
Zdroj: | Orphanet Journal of Rare Diseases, 12 Orphanet Journal of Rare Diseases Orphanet Journal of Rare Diseases, Vol 12, Iss 1, Pp 1-8 (2017) |
Popis: | Background Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life. In this study we evaluate quality of life in patients with fibrous dysplasia using the Short Form-36 and the Brief Pain Inventory questionnaires. Data were compared with those of the general Dutch population. Results Out of 138 patients from a cohort of 255 patients with fibrous dysplasia that were sent questionnaires assessing quality of life and pain, the response rate was 70.3%, with 97 patients, predominantly female (65%), completing the questionnaires. Monostotic fibrous dysplasia was predominant (n = 62, 64%). Fibrous dysplasia patients had significantly lower quality of life outcome scores than the general Dutch population for all tested domains of the Short Form-36 except for the “Mental health” and the “Role emotional” domains. More severe forms of fibrous dysplasia, had the more severe Short-Form-36 quality of life outcomes, but there was no significant difference in Brief Pain Inventory domains between different subtypes of fibrous dysplasia. Quality of life was lower in patients with higher disease burden, as reflected by high skeletal burden scores (p = 0.003) and high levels of P1NP (p = 0.002). Conclusion We demonstrate impairments in all domains of quality of life, except for ‘Mental health’ and ‘Role emotional’ domains, across the wide spectrum of fibrous dysplasia including its milder forms. We identified high skeletal burden scores, reflecting disease severity, as the most consistent predictor of impaired quality of life. Our findings hold significant clinical implications as they draw attention to the clinically unmet need to address quality of life issues in the management of patients with all subtypes of fibrous dysplasia, including its milder forms. |
Databáze: | OpenAIRE |
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