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

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Harvard

Doser, K, Andersen, EW, Kenborg, L, Dalton, SO, Jepsen, JRM, Krøyer, A, Østergaard, J, Hove, H, Sørensen, SA, Johansen, C, Mulvihill, J, Winther, JF & Bidstrup, PE 2020, 'Clinical characteristics and quality of life, depression, and anxiety in adults with neurofibromatosis type 1: A nationwide study', American Journal of Medical Genetics. Part A, vol. 182, no. 7, pp. 1704-1715. https://doi.org/10.1002/ajmg.a.61627

APA

Doser, K., Andersen, E. W., Kenborg, L., Dalton, S. O., Jepsen, J. R. M., Krøyer, A., Østergaard, J., Hove, H., Sørensen, S. A., Johansen, C., Mulvihill, J., Winther, J. F., & Bidstrup, P. E. (2020). Clinical characteristics and quality of life, depression, and anxiety in adults with neurofibromatosis type 1: A nationwide study. American Journal of Medical Genetics. Part A, 182(7), 1704-1715. https://doi.org/10.1002/ajmg.a.61627

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Author

Doser, Karoline ; Andersen, Elisabeth Wreford ; Kenborg, Line ; Dalton, Susanne Oksbjerg ; Jepsen, Jens Richardt Møllegaard ; Krøyer, Anja ; Østergaard, John ; Hove, Hanne ; Sørensen, Sven Asger ; Johansen, Christoffer ; Mulvihill, John ; Winther, Jeanette Falck ; Bidstrup, Pernille Envold. / Clinical characteristics and quality of life, depression, and anxiety in adults with neurofibromatosis type 1 : A nationwide study. In: American Journal of Medical Genetics. Part A. 2020 ; Vol. 182, No. 7. pp. 1704-1715.

Bibtex

@article{e381d53450be4acf889370928db875c8,
title = "Clinical characteristics and quality of life, depression, and anxiety in adults with neurofibromatosis type 1: A nationwide study",
abstract = "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.",
keywords = "adults, nationwide, need for support, neurofibromatosis type 1, population-based, psychosocial burden",
author = "Karoline Doser and Andersen, {Elisabeth Wreford} and Line Kenborg and Dalton, {Susanne Oksbjerg} and Jepsen, {Jens Richardt M{\o}llegaard} and Anja Kr{\o}yer and John {\O}stergaard and Hanne Hove and S{\o}rensen, {Sven Asger} and Christoffer Johansen and John Mulvihill and Winther, {Jeanette Falck} and Bidstrup, {Pernille Envold}",
note = "{\textcopyright} 2020 Wiley Periodicals, Inc.",
year = "2020",
month = jul,
doi = "10.1002/ajmg.a.61627",
language = "English",
volume = "182",
pages = "1704--1715",
journal = "American Journal of Medical Genetics, Part A",
issn = "1552-4825",
publisher = "JohnWiley & Sons, Inc",
number = "7",

}

RIS

TY - JOUR

T1 - Clinical characteristics and quality of life, depression, and anxiety in adults with neurofibromatosis type 1

T2 - A nationwide study

AU - Doser, Karoline

AU - Andersen, Elisabeth Wreford

AU - Kenborg, Line

AU - Dalton, Susanne Oksbjerg

AU - Jepsen, Jens Richardt Møllegaard

AU - Krøyer, Anja

AU - Østergaard, John

AU - Hove, Hanne

AU - Sørensen, Sven Asger

AU - Johansen, Christoffer

AU - Mulvihill, John

AU - Winther, Jeanette Falck

AU - Bidstrup, Pernille Envold

N1 - © 2020 Wiley Periodicals, Inc.

PY - 2020/7

Y1 - 2020/7

N2 - 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.

AB - 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.

KW - adults

KW - nationwide

KW - need for support

KW - neurofibromatosis type 1

KW - population-based

KW - psychosocial burden

U2 - 10.1002/ajmg.a.61627

DO - 10.1002/ajmg.a.61627

M3 - Journal article

C2 - 32484306

VL - 182

SP - 1704

EP - 1715

JO - American Journal of Medical Genetics, Part A

JF - American Journal of Medical Genetics, Part A

SN - 1552-4825

IS - 7

ER -

ID: 60068557