Clinical characteristics and quality of life, depression, and anxiety in adults with neurofibromatosis type 1: A nationwide study.

Autor: Doser K; Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark.; Psychological Aspects of Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark., Andersen EW; Statistics and Pharmacoepidemiology, Danish Cancer Society Research Center, Copenhagen, Denmark., Kenborg L; Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark., Dalton SO; Survivorship and Inequality in Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark.; Department of Clinical Oncology and Palliative Care, Zealand University Hospital, Naestved, Denmark., Jepsen JRM; Center for Clinical Intervention and Neuropsychiatric Schizophrenia Research and Center for Neuropsychiatric Schizophrenia Research, Mental Health Services, Capital Region of Denmark, University of Copenhagen, Copenhagen, Denmark.; Child and Adolescent Mental Health Center, Mental Health Services Capital Region Copenhagen, University of Copenhagen, Copenhagen, Denmark., Krøyer A; Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark., Østergaard J; Center for Rare Disease, Aarhus University Hospital, Aarhus, Denmark., Hove H; The RAREDIS Database, Center for Rare Diseases, Copenhagen University Hospital and Aarhus University, Copenhagen, Denmark., Sørensen SA; Department of Neurogenetics, Institute of Cellular and Molecular Medicine, The Panum Institute, University of Copenhagen, Copenhagen, Denmark., Johansen C; Psychological Aspects of Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark.; Oncology Clinic, Finsen Center, Copenhagen, Denmark., Mulvihill J; Department of Pediatrics, University of Oklahoma, Norman, Oklahoma, USA., Winther JF; Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark.; Faculty of Health, Department of Clinical Medicine, Aarhus University and University Hospital, Aarhus, Denmark., Bidstrup PE; Psychological Aspects of Cancer, Danish Cancer Society Research Center, Copenhagen, Denmark.; Department of Psychology, University of Copenhagen, Copenhagen, Denmark.
Jazyk: angličtina
Zdroj: American journal of medical genetics. Part A [Am J Med Genet A] 2020 Jul; Vol. 182 (7), pp. 1704-1715. Date of Electronic Publication: 2020 Jun 02.
DOI: 10.1002/ajmg.a.61627
Abstrakt: Neurofibromatosis type 1 (NF1) is a genetic condition characterized by numerous somatic manifestations. The psychosocial burden in adults has rarely been studied. We examined the prevalence of self-reported impairment of quality of life (QoL), symptoms of anxiety and depression and need for support, associated with disease severity and visibility. We conducted a nationwide cross-sectional study of all 467 adults with NF1 diagnosed between 1977 and 2016 at one of the two national centers for rare diseases in Denmark. A total of 244 (56% response rate) completed a questionnaire that included standard measures of QoL, symptoms of depression and anxiety, indicators of disease-related severity, visibility, and need for professional support. Associations between disease severity and visibility and psychosocial burden were analyzed in descriptive and multivariate models. We observed impaired QoL (mean = 81.3; 95% CI, 76.2; 86.4); 19% reported symptoms of depression (mean = 5.7; SD = 5.4), and 15% reported anxiety (mean = 5.1; SD = 5.2) at a clinical level. Adults with NF1 also reported requiring professional support for physical, psychological, and work-related problems. Disease severity and (partly) visibility were significantly (p < .0001) associated with psychosocial well-being and a requirement for support. This study provides new understanding of the factors associated with impaired QoL, indicating that follow-up care should be optimized into adult life.
(© 2020 Wiley Periodicals, Inc.)
Databáze: MEDLINE
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