دورية أكاديمية
Service evaluation suggests variation in clinical care provision in adults with congenital adrenal hyperplasia in the UK and Ireland.
| العنوان: | Service evaluation suggests variation in clinical care provision in adults with congenital adrenal hyperplasia in the UK and Ireland. |
|---|---|
| المؤلفون: | Doyle LM; Academic Division of Endocrinology, Department of Medicine, Royal College of Surgeons in Ireland (RCSI), Dublin, Ireland., Ahmed SF; Developmental Endocrinology Research Group, Royal Hospital for Children, University of Glasgow, Glasgow, UK., Davis J; Society for Endocrinology, Bristol, UK., Elford S; CAH Support Group, Living with CAH, Cambridge, UK., Elhassan YS; Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham, UK.; Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK.; Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK., James L; School of Medicine, University Hospital of Wales, Cardiff, UK., Lawrence N; Division of Clinical Medicine, School of Medicine and Population Health, University of Sheffield, Sheffield, UK., Llahana S; School of Health and Psychological Sciences, City, University of London, UK.; Department of Diabetes & Endocrinology, University College Hospital, London, UK., Okoro G; Society for Endocrinology, Bristol, UK., Rees DA; Neuroscience and Mental Health Innovation Institute, School of Medicine, Cardiff University, Cardiff, UK., Tomlinson JW; Oxford Centre for Diabetes, Endocrinology & Metabolism, NIHR Oxford Biomedical Research Centre, University of Oxford, Oxford, UK., O'Reilly MW; Academic Division of Endocrinology, Department of Medicine, Royal College of Surgeons in Ireland (RCSI), Dublin, Ireland., Krone NP; Division of Clinical Medicine, School of Medicine and Population Health, University of Sheffield, Sheffield, UK. |
| المصدر: | Clinical endocrinology [Clin Endocrinol (Oxf)] 2024 Mar 17. Date of Electronic Publication: 2024 Mar 17. |
| Publication Model: | Ahead of Print |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Blackwell Publishing Country of Publication: England NLM ID: 0346653 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2265 (Electronic) Linking ISSN: 03000664 NLM ISO Abbreviation: Clin Endocrinol (Oxf) Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: <2003->: Oxford : Blackwell Publishing Original Publication: Oxford, Blackwell Scientific Publications. |
| مستخلص: | Background: Congenital adrenal hyperplasia (CAH) encompasses a rare group of autosomal recessive disorders, characterised by enzymatic defects in steroidogenesis. Heterogeneity in management practices has been observed internationally. The International Congenital Adrenal Hyperplasia registry (I-CAH, https://sdmregistries.org/) was established to enable insights into CAH management and outcomes, yet its global adoption by endocrine centres remains unclear. Design: We sought (1) to assess current practices amongst clinicians managing patients with CAH in the United Kingdom and Ireland, with a focus on choice of glucocorticoid, monitoring practices and screening for associated co-morbidities, and (2) to assess use of the I-CAH registry. Measurements: We designed and distributed an anonymised online survey disseminated to members of the Society for Endocrinology and Irish Endocrine Society to capture management practices in the care of patients with CAH. Results: Marked variability was found in CAH management, with differences between general endocrinology and subspecialist settings, particularly in glucocorticoid use, biochemical monitoring and comorbidity screening, with significant disparities in reproductive health monitoring, notably in testicular adrenal rest tumours (TARTs) screening (p = .002), sperm banking (p = .0004) and partner testing for CAH (p < .0001). Adoption of the I-CAH registry was universally low. Conclusions: Differences in current management of CAH continue to exist. It appears crucial to objectify if different approaches result in different long-term outcomes. New studies such as CaHASE2, incorporating standardised minimum datasets including replacement therapies and monitoring strategies as well as longitudinal data collection, are now needed to define best-practice and standardise care. (© 2024 The Authors. Clinical Endocrinology published by John Wiley & Sons Ltd.) |
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| معلومات مُعتمدة: | Neurocrine Biosciences Inc |
| فهرسة مساهمة: | Keywords: I-CAH; congenital adrenal hyperplasia |
| تواريخ الأحداث: | Date Created: 20240317 Latest Revision: 20240317 |
| رمز التحديث: | 20240318 |
| DOI: | 10.1111/cen.15043 |
| PMID: | 38493480 |
| قاعدة البيانات: | MEDLINE |
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