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Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)

BACKGROUND: Previous studies in quality of life (QOL) in individuals with disorders/differences of sex development (DSD) have been restricted to subpopulations of the condition. We describe QOL in adult persons with DSD compared to country specific references and assess the impact of diagnosis. METH...

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Autores principales: Rapp, Marion, Mueller-Godeffroy, Esther, Lee, Peter, Roehle, Robert, Kreukels, Baudewijntje P. C., Köhler, Birgit, Nordenström, Anna, Bouvattier, Claire, Thyen, Ute
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5883311/
https://www.ncbi.nlm.nih.gov/pubmed/29615040
http://dx.doi.org/10.1186/s12955-018-0881-3
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author Rapp, Marion
Mueller-Godeffroy, Esther
Lee, Peter
Roehle, Robert
Kreukels, Baudewijntje P. C.
Köhler, Birgit
Nordenström, Anna
Bouvattier, Claire
Thyen, Ute
author_facet Rapp, Marion
Mueller-Godeffroy, Esther
Lee, Peter
Roehle, Robert
Kreukels, Baudewijntje P. C.
Köhler, Birgit
Nordenström, Anna
Bouvattier, Claire
Thyen, Ute
author_sort Rapp, Marion
collection PubMed
description BACKGROUND: Previous studies in quality of life (QOL) in individuals with disorders/differences of sex development (DSD) have been restricted to subpopulations of the condition. We describe QOL in adult persons with DSD compared to country specific references and assess the impact of diagnosis. METHODS: The multicentre cross-sectional clinical evaluation (dsd-LIFE) took place in 14 specialized clinics in six European countries. Adolescents (≥16 years) and adults having a DSD condition were included from 02/2014 to 09/2015. The main outcome QOL was measured by the WHOQOL-BREF (domains of physical health, psychological, social relationships, and environment). QOL was compared to country specific reference populations by using unpaired t-tests. Linear regression models explained the additional variance of the diagnosis on QOL. RESULTS: Three hundred one individuals with Turner Syndrome, 219 with Klinefelter Syndrome (including XYY), 226 with 46,XX CAH and 294 with rare DSD conditions (gonadal dysgenesis, androgen insensitivity syndrome, severe hypospadias, and androgen synthesis errors or other diagnosis) took part. Compared to healthy European populations, QOL was similar in psychological, slightly worse in physical health, and slightly better in environment. In social relationships, QOL was significantly poorer compared to healthy and non-healthy reference populations. In linear regression models health status was the most important predictor of QOL; additional variance was explained by feelings about household’s income in all domains, and the relationship status in social relationships. Diagnosis explained nearly no additional variance. CONCLUSIONS: Except for social relationships, most people with DSD adapt well to their life circumstances and report a good QOL. Not diagnosis, but the individual’s health status is much more important than previously thought. Therefore care for people with DSD should focus more on chronic physical or mental health problems both related and unrelated to the diagnosis itself. TRIAL REGISTRATION: German Clinical Trials Register DRKS00006072.
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spelling pubmed-58833112018-04-10 Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE) Rapp, Marion Mueller-Godeffroy, Esther Lee, Peter Roehle, Robert Kreukels, Baudewijntje P. C. Köhler, Birgit Nordenström, Anna Bouvattier, Claire Thyen, Ute Health Qual Life Outcomes Research BACKGROUND: Previous studies in quality of life (QOL) in individuals with disorders/differences of sex development (DSD) have been restricted to subpopulations of the condition. We describe QOL in adult persons with DSD compared to country specific references and assess the impact of diagnosis. METHODS: The multicentre cross-sectional clinical evaluation (dsd-LIFE) took place in 14 specialized clinics in six European countries. Adolescents (≥16 years) and adults having a DSD condition were included from 02/2014 to 09/2015. The main outcome QOL was measured by the WHOQOL-BREF (domains of physical health, psychological, social relationships, and environment). QOL was compared to country specific reference populations by using unpaired t-tests. Linear regression models explained the additional variance of the diagnosis on QOL. RESULTS: Three hundred one individuals with Turner Syndrome, 219 with Klinefelter Syndrome (including XYY), 226 with 46,XX CAH and 294 with rare DSD conditions (gonadal dysgenesis, androgen insensitivity syndrome, severe hypospadias, and androgen synthesis errors or other diagnosis) took part. Compared to healthy European populations, QOL was similar in psychological, slightly worse in physical health, and slightly better in environment. In social relationships, QOL was significantly poorer compared to healthy and non-healthy reference populations. In linear regression models health status was the most important predictor of QOL; additional variance was explained by feelings about household’s income in all domains, and the relationship status in social relationships. Diagnosis explained nearly no additional variance. CONCLUSIONS: Except for social relationships, most people with DSD adapt well to their life circumstances and report a good QOL. Not diagnosis, but the individual’s health status is much more important than previously thought. Therefore care for people with DSD should focus more on chronic physical or mental health problems both related and unrelated to the diagnosis itself. TRIAL REGISTRATION: German Clinical Trials Register DRKS00006072. BioMed Central 2018-04-03 /pmc/articles/PMC5883311/ /pubmed/29615040 http://dx.doi.org/10.1186/s12955-018-0881-3 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
Rapp, Marion
Mueller-Godeffroy, Esther
Lee, Peter
Roehle, Robert
Kreukels, Baudewijntje P. C.
Köhler, Birgit
Nordenström, Anna
Bouvattier, Claire
Thyen, Ute
Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)
title Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)
title_full Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)
title_fullStr Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)
title_full_unstemmed Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)
title_short Multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (DSD) compared to country specific reference populations (dsd-LIFE)
title_sort multicentre cross-sectional clinical evaluation study about quality of life in adults with disorders/differences of sex development (dsd) compared to country specific reference populations (dsd-life)
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5883311/
https://www.ncbi.nlm.nih.gov/pubmed/29615040
http://dx.doi.org/10.1186/s12955-018-0881-3
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