Quality of life and its predictors in adults with tuberous sclerosis complex (TSC): a multicentre cohort study from Germany
| Autor: | Christoph Hertzberg, Felix von Podewils, Matthias Sauter, Felix Rosenow, Susanne Schubert-Bast, Anna H. Noda, Thomas E. Mayer, Laurent M. Willems, Klaus Marquard, Jens-Peter Reese, Ilka Immisch, Bianca Zukunft, Johann Philipp Zöllner, Janina Grau, Nadine Conradi, Susanne Knake, Astrid Bertsche, Markus Knuf, Karl Martin Klein, Sascha Meyer, Gerhard Kurlemann, Hannah Schäfer, Charlotte Thiels, Adam Strzelczyk |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Quality of life
medicine.medical_specialty Organ manifestations Neurology mTOR inhibitor Neurosciences. Biological psychiatry. Neuropsychiatry Disease 03 medical and health sciences Epilepsy 0302 clinical medicine EQ-5D Internal medicine medicine 030212 general & internal medicine QOLIE-31 Adverse effect RC346-429 Depression (differential diagnoses) TSC business.industry medicine.disease Seizure humanities Tuberous sclerosis complex Neurology. Diseases of the nervous system business Rare disease 030217 neurology & neurosurgery Cohort study RC321-571 Research Article |
| Zdroj: | Neurological Research and Practice Neurological Research and Practice, Vol 3, Iss 1, Pp 1-18 (2021) |
| ISSN: | 2524-3489 |
| Popis: | Background Tuberous sclerosis complex (TSC) is a monogenetic, multisystemic disease characterised by the formation of benign tumours that can affect almost all organs, caused by pathogenic variations in TSC1 or TSC2. In this multicentre study from Germany, we investigated the influence of sociodemographic, clinical, and therapeutic factors on quality of life (QoL) among individuals with TSC. Methods We assessed sociodemographic and clinical characteristics and QoL among adults with TSC throughout Germany using a validated, three-month, retrospective questionnaire. We examined predictors of health-related QoL (HRQoL) using multiple linear regression analysis and compared the QoL among patients with TSC with QoL among patients with other chronic neurological disorders. Results We enrolled 121 adults with TSC (mean age: 31.0 ± 10.5 years; range: 18–61 years, 45.5% [n = 55] women). Unemployment, a higher grade of disability, a higher number of organ manifestations, the presence of neuropsychiatric manifestations or active epilepsy, and a higher burden of therapy-related adverse events were associated with worse QoL, as measured by two QoL instruments (EuroQoL-5 dimensions [EQ-5D] and Quality of Life in Epilepsy Patients [QOLIE-31]). Neuropsychiatric and structural nervous system manifestations, the number of affected organs, and therapy-related adverse events were also associated with higher depression, as measured by the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E). In multiple regression analysis, more severe therapy-related adverse events (large effect, p p p = 0.003) were independently associated with worse HRQoL, explaining 65% of the variance (p p Conclusions Active epilepsy, neuropsychiatric manifestations (such as anxiety and depression), and therapy-related adverse events are important independent predictors of worse quality of life among adults with TSC. Generic quality of life in TSC with several manifestations is similar to uncontrolled severe chronic diseases and significantly negatively correlates with TSC severity. Trial registration DRKS, DRKS00016045. Registered 01 March 2019. |
| Databáze: | OpenAIRE |
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