Quality of Life in Children and Young People With Congenital Adrenal Hyperplasia-UK Nationwide Multicenter Assessment.

Autor:	Lawrence NR; Department of Oncology & Metabolism, University of Sheffield, Sheffield S10 2RX, UK., Bacila I; Department of Oncology & Metabolism, University of Sheffield, Sheffield S10 2RX, UK., Dawson J; Department of Oncology & Metabolism, University of Sheffield, Sheffield S10 2RX, UK., Mahdi S; Department of Oncology & Metabolism, University of Sheffield, Sheffield S10 2RX, UK., Alvi S; Department of Paediatric Endocrinology and Diabetes, Leeds Children's Hospital, Leeds LS2 3AX, UK., Cheetham TD; Department of Paediatric Endocrinology, Great North Children's Hospital, University of Newcastle, Newcastle NE1 4LP, UK., Crowne E; Department of Paediatric Endocrinology & Diabetes, Bristol Royal Hospital for Children, University Hospitals Bristol Foundation Trust, Bristol BS2 8BJ, UK., Das U; Department of Paediatric Endocrinology, Alder Hey Children's Hospital, Liverpool L14 5AB, UK., Dattani MT; Department of Paediatric Endocrinology and Diabetes, Great Ormond Street Hospital, London WC1N 1LE, UK., Davies JH; Department of Paediatric Endocrinology, University Hospital Southampton, Southampton SO16 6YD, UK.; Department of Human Development & Health, University of Southampton, Southampton SO17 1BJ, UK., Gevers E; Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London and Barts Health NHS Trust-The Royal London Hospital, London E1 1BB, UK., Krone RE; Department of Paediatric Endocrinology, Birmingham Women's & Children's Hospital, Birmingham B4 6NH, UK., Patel L; Paediatric Endocrine Service, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester M13 9WL, UK., Randell T; Department of Paediatric Diabetes and Endocrinology, Nottingham Children's Hospital, Nottingham NG7 2UH, UK., Ryan FJ; Department of Paediatric Endocrinology and Diabetes, Oxford Children's Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford OX3 9DU, UK., Keevil B; Department of Biochemistry, Manchester University NHS Foundation Trust, Manchester M13 9PL, UK., Ahmed SF; Developmental Endocrinology Research Group, University of Glasgow, Glasgow G12 8QQ, UK., Krone NP; Department of Oncology & Metabolism, University of Sheffield, Sheffield S10 2RX, UK.
Jazyk:	angličtina
Zdroj:	The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2023 Dec 21; Vol. 109 (1), pp. e336-e346.
DOI:	10.1210/clinem/dgad405
Abstrakt:	Context: Quality of life (QoL) has been inconsistently reported in children and young people (CYP) with congenital adrenal hyperplasia (CAH). Objective: Assess QoL in CYP with CAH in the UK alongside biometric and androgen profiles. Design: To define the evidence base for health care delivery, we conducted a cross-sectional study in CYP with CAH in the UK. Questionnaire results were compared with normative data and between groups, and modelled for association with sex, height, weight, body mass index, or steroid biomarkers of CAH control. Setting: Tertiary care in 14 UK centers. Patients: Results from 104 patients, 55% female, mean age 12.7 years (SD 3.0), paired responses from parents. Interventions: Strengths and Difficulties questionnaire (SDQ) and pediatric QoL questionnaire. Main Outcome Measure: Total QoL scores as assessed by SDQ and a pediatric QoL questionnaire in comparison to normative data. Results: Total scores were worse in parents than normative data, but similar in patients. Patient QoL was rated better in social functioning but worse in emotional, school, and peer domains by patients, and worse in total scores and domains of peer problems, and psychosocial, emotional, and school functioning by parents. Parents consistently scored QoL of their children lower than their child. Larger height-SD score and lower weight-SD score were associated with better QoL. Girls with lower steroid biomarkers had worse SDQ scores. Conclusions: In CYP with CAH, reduced height, increased weight, and hormonal biomarkers consistent with overtreatment were associated with worse QoL; addressing these problems should be prioritized in clinical management.Clinical Trials Registration Number: SCH/15/088. (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu