ArticleOrphanet journal of rare diseases2020
Depressive symptoms in Fabry disease: the importance of coping, subjective health perception and pain.
Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 1 of them a synthesis that pooled it.
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Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 31 citations in OpenAlex.
- Neuropsychiatric Symptoms and Their Association With Sex, Age, and Enzyme Replacement Therapy in Fabry Disease: A Systematic Review.Frontiers in psychiatry · 2022Pooled it
- Venglustat, an orally administered glucosylceramide synthase inhibitor: Assessment over 3 years in adult males with classic Fabry disease in an open-label phase 2 study and its extension study.Molecular genetics and metabolism · 2023Trial
- Development and validation of a self-management efficacy questionnaire for patients with Fabry disease in China.Annals of medicine · 2026Article
- Fabry Disease: An Updated Perspective and Review of Treatment and Therapies.Advances in therapy · 2026Review
- Expert opinion on facilitating intrafamily communication in rare diseases-Lessons from Fabry disease.Genetics in medicine open · 2026Article
- Fabry disease in females: organ involvement and clinical outcomes compared with the general population (103/150 characters).Orphanet journal of rare diseases · 2025Review
- Characterizing pain in patients with Fabry disease: findings from a web-based cross-sectional survey in the US.Orphanet journal of rare diseases · 2025Article
- Impact of the Covid19 pandemic on health-related quality of life in patients with Fabry disease - implications for future care of patients with rare diseases.Molecular genetics and metabolism reports · 2024Article
- Measuring health related quality of life (HRQoL) in Lysosomal Storage Disorders (LSDs): a rapid scoping review of available tools and domains.Orphanet journal of rare diseases · 2024Article
- Fatigue as hallmark of Fabry disease: role of bioenergetic alterations.Frontiers in cardiovascular medicine · 2024Review
- Disruption of the microbiota-gut-brain axis is a defining characteristic of the α-Gal A (-/0) mouse model of Fabry disease.Gut microbes · 2023Article
- Brazilian registry of patients with porphyria: REBRAPPO study.Orphanet journal of rare diseases · 2023Article
- Palm-Plant Pain, Sign of a Severe Systemic Disease? Case Report and Review of Literature.Genes · 2023Review
- Estimation of Health State Utility Values in Fabry Disease Using Vignette Development and Valuation.Journal of health economics and outcomes research · 2023Article
- Quality of life in patients with Fabry's disease: a cross-sectional study of 86 adults.Cardiovascular diagnosis and therapy · 2022Article
- Association between social supports and negative emotions among pediatric residents in China: The chain-mediating role of psychological resilience and burnout.Frontiers in public health · 2022Article
- Health-related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress.JIMD reports · 2021Article
- Survey about the Quality of Life of Italian Patients with Fabry Disease.Diseases (Basel, Switzerland) · 2021Article
- Review of Mechanisms, Pharmacological Management, Psychosocial Implications, and Holistic Treatment of Pain in Fabry Disease.Journal of clinical medicine · 2021Review
- Attention Deficits and ADHD Symptoms in Adults with Fabry Disease-A Pilot Investigation.Journal of clinical medicine · 2021Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDespite the high prevalence of depressive symptoms in Fabry disease (FD), it is unclear which patient characteristics are important in relation to these symptoms. Additionally, the impact of coping styles in relation to depressive symptoms in FD has been unexplored. Determining the impact of different factors relating to depressive symptoms in FD can guide both prevention and treatment of these symptoms.
methodsDepressive symptoms (Center for Epidemiologic Studies Depression scale (CESD)) and coping styles (Utrecht Coping List) were assessed in a Dutch FD cohort. Other potentially important variables were identified from FD literature and assessed in this cohort. Relations were evaluated using multiple linear models.
resultsPotentially important variables in FD literature were: pain, unemployment, health perception, being single, comorbidities and stroke. Employed coping styles were "avoidance and brooding", "positivity and problem solving" and "seeking social support". Thirty-one of the 81 FD patients (38%) had depressive symptoms. CESD-scores were lower in patients with better health perception and more "positivity and problem solving" and higher in patients with more pain and "avoidance and brooding". The best model explained 70% (95%CI: 54-76%) of observed variance of the CESD.
conclusionsDepressive symptoms in FD are related to pain, negative health perception and use of specific coping styles. Psychological interventions could be employed to alter coping behavior and alleviate depressive symptoms.
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