Literature DB >> 25074426

Quality of life in a large cohort of adult Brazilian patients with 46,XX and 46,XY disorders of sex development from a single tertiary centre.

Rita Cassia Amaral1, Marlene Inacio, Vinicius N Brito, Tania A S S Bachega, Ari A Oliveira, Sorahia Domenice, Francisco T Denes, Maria Helena Sircili, Ivo J P Arnhold, Guiomar Madureira, Larissa Gomes, Elaine M F Costa, Berenice B Mendonca.   

Abstract

OBJECTIVE: Few studies have focused on the quality of life (QoL) of patients with disorders of sex development (DSD). Our aim was to evaluate QoL in DSD patients with defined diagnoses followed until adulthood in a single tertiary centre. PATIENTS AND METHODS: Adult patients with DSD (56 patients with 46,XX DSD - 49 with female social sex and 7 with male social sex as well as 88 patients with 46,XY DSD - 54 with female social sex and 34 with male social sex). MEASUREMENTS: QoL using WHOQOL-Bref questionnaire.
RESULTS: Both patients with 46,XX DSD and patients with 46,XY DSD had similar QoL scores on the WHOQOL-Bref, comparable to the scores of the Brazilian general population. The chronological age at the start of treatment was negatively and significantly associated with general QoL score. Patients with male social sex DSD had better scores on the psychological domain than patients with female social sex DSD, as found in the Brazilian general population. In addition, among the 46,XY DSD group, the male social sex patients had better QoL compared with the female social sex patients. There was a positive and significant correlation between sexual performance and general QoL, although it explained only 4% of the variability of the general QoL score. The most influencing variables were general health, positive feelings and spirituality, religion and personal beliefs, each of them contributing with 18% of the variability of the general QoL score.
CONCLUSION: Our large cohort of adult patients with DSD, which was followed by a multidisciplinary team in a single tertiary centre, had good QoL in adulthood; in addition, late treatment compromised the QoL of patients with DSD, whereas sexual performance has little influence on QoL.
© 2014 John Wiley & Sons Ltd.

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Year:  2014        PMID: 25074426     DOI: 10.1111/cen.12572

Source DB:  PubMed          Journal:  Clin Endocrinol (Oxf)        ISSN: 0300-0664            Impact factor:   3.478


  9 in total

Review 1.  Disorders of sex development: effect of molecular diagnostics.

Authors:  John C Achermann; Sorahia Domenice; Tania A S S Bachega; Mirian Y Nishi; Berenice B Mendonca
Journal:  Nat Rev Endocrinol       Date:  2015-05-05       Impact factor: 43.330

2.  The influence of psychosocial and sexual wellbeing on quality of life in women with differences of sexual development.

Authors:  Ariane Liedmeier; David Jendryczko; Marion Rapp; Robert Roehle; Ute Thyen; Baudewijntje P C Kreukels
Journal:  Compr Psychoneuroendocrinol       Date:  2021-09-24

3.  Clinical profile of 93 cases of 46, XY disorders of sexual development in a referral center.

Authors:  Bianca Costa Mota; Luciana Mattos Barros Oliveira; Renata Lago; Paula Brito; Ana Karina Canguçú-Campinho; Ubirajara Barroso; Maria Betânia Pereira Toralles
Journal:  Int Braz J Urol       Date:  2015 Sep-Oct       Impact factor: 1.541

4.  Participation of adults with disorders/differences of sex development (DSD) in the clinical study dsd-LIFE: design, methodology, recruitment, data quality and study population.

Authors:  Robert Röhle; Katharina Gehrmann; Maria Szarras-Czapnik; Hedi Claahsen-van der Grinten; Catherine Pienkowski; Claire Bouvattier; Peggy Cohen-Kettenis; Anna Nordenström; Ute Thyen; Birgit Köhler
Journal:  BMC Endocr Disord       Date:  2017-08-18       Impact factor: 2.763

5.  Quality of Life in Men With Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

Authors:  Myrthe J M Verhees; Manon Engels; Paul N Span; Fred C G J Sweep; Antonius E van Herwaarden; Henrik Falhammar; Anna Nordenström; Emma A Webb; Annette Richter-Unruh; Claire Bouvattier; Aude Brac de la Perrière; Wiebke Arlt; Nicole Reisch; Birgit Köhler; Marion Rapp; Nike M M L Stikkelbroeck; Nel Roeleveld; Hedi L Claahsen-van der Grinten
Journal:  Front Endocrinol (Lausanne)       Date:  2021-03-19       Impact factor: 5.555

6.  Cohort profile: pathways to care among people with disorders of sex development (DSD).

Authors:  Michael Goodman; Rami Yacoub; Darios Getahun; Courtney E McCracken; Suma Vupputuri; Timothy L Lash; Douglas Roblin; Richard Contreras; Lee Cromwell; Melissa D Gardner; Trenton Hoffman; Haihong Hu; Theresa M Im; Radhika Prakash Asrani; Brandi Robinson; Fagen Xie; Rebecca Nash; Qi Zhang; Sadaf A Bhai; Kripa Venkatakrishnan; Bethany Stoller; Yijun Liu; Cricket Gullickson; Maaz Ahmed; David Rink; Ava Voss; Hye-Lee Jung; Jin Kim; Peter A Lee; David E Sandberg
Journal:  BMJ Open       Date:  2022-09-21       Impact factor: 3.006

Review 7.  Treatment of the Enlarged Clitoris.

Authors:  Martin Kaefer; Richard C Rink
Journal:  Front Pediatr       Date:  2017-08-28       Impact factor: 3.418

8.  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).

Authors:  Marion Rapp; Esther Mueller-Godeffroy; Peter Lee; Robert Roehle; Baudewijntje P C Kreukels; Birgit Köhler; Anna Nordenström; Claire Bouvattier; Ute Thyen
Journal:  Health Qual Life Outcomes       Date:  2018-04-03       Impact factor: 3.186

9.  Disorders of sex development: timing of diagnosis and management in a single large tertiary center.

Authors:  E Kohva; P J Miettinen; S Taskinen; M Hero; A Tarkkanen; T Raivio
Journal:  Endocr Connect       Date:  2018-03-26       Impact factor: 3.335

  9 in total

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