{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T08:21:34Z","timestamp":1740126094422,"version":"3.37.3"},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,3,17]],"date-time":"2024-03-17T00:00:00Z","timestamp":1710633600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Endocrinology"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>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\u2010CAH, <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"https:\/\/sdmregistries.org\/\">https:\/\/sdmregistries.org\/<\/jats:ext-link>) was established to enable insights into CAH management and outcomes, yet its global adoption by endocrine centres remains unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>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\u2010morbidities, and (2) to assess use of the I\u2010CAH registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Measurements<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>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 (<jats:italic>p<\/jats:italic>\u2009=\u2009.002), sperm banking (<jats:italic>p<\/jats:italic>\u2009=\u2009.0004) and partner testing for CAH (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.0001). Adoption of the I\u2010CAH registry was universally low.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Differences in current management of CAH continue to exist. It appears crucial to objectify if different approaches result in different long\u2010term 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\u2010practice and standardise care.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cen.15043","type":"journal-article","created":{"date-parts":[[2024,3,17]],"date-time":"2024-03-17T17:54:19Z","timestamp":1710698059000},"page":"386-396","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Service evaluation suggests variation in clinical care provision in adults with congenital adrenal hyperplasia in the UK and Ireland"],"prefix":"10.1111","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-6878-8013","authenticated-orcid":false,"given":"Lauren Madden","family":"Doyle","sequence":"first","affiliation":[{"name":"Academic Division of Endocrinology, Department of Medicine Royal College of Surgeons in Ireland (RCSI) Dublin Ireland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0689-5549","authenticated-orcid":false,"given":"S. Faisal","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Developmental Endocrinology Research Group, Royal Hospital for Children University of Glasgow Glasgow UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessica","family":"Davis","sequence":"additional","affiliation":[{"name":"Society for Endocrinology Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sue","family":"Elford","sequence":"additional","affiliation":[{"name":"CAH Support Group Living with CAH Cambridge UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasir S.","family":"Elhassan","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Queen Elizabeth Hospital Birmingham Birmingham UK"},{"name":"Centre for Endocrinology, Diabetes and Metabolism Birmingham Health Partners Birmingham UK"},{"name":"Institute of Metabolism and Systems Research University of Birmingham Birmingham UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lynette","family":"James","sequence":"additional","affiliation":[{"name":"School of Medicine University Hospital of Wales Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neil","family":"Lawrence","sequence":"additional","affiliation":[{"name":"Division of Clinical Medicine, School of Medicine and Population Health University of Sheffield Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sofia","family":"Llahana","sequence":"additional","affiliation":[{"name":"School of Health and Psychological Sciences City, University of London  UK"},{"name":"Department of Diabetes &amp; Endocrinology University College Hospital London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grace","family":"Okoro","sequence":"additional","affiliation":[{"name":"Society for Endocrinology Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. Aled","family":"Rees","sequence":"additional","affiliation":[{"name":"Neuroscience and Mental Health Innovation Institute, School of Medicine Cardiff University Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3170-8533","authenticated-orcid":false,"given":"Jeremy W.","family":"Tomlinson","sequence":"additional","affiliation":[{"name":"Oxford Centre for Diabetes, Endocrinology &amp; Metabolism, NIHR Oxford Biomedical Research Centre University of Oxford Oxford UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9108-9105","authenticated-orcid":false,"given":"Michael W.","family":"O'Reilly","sequence":"additional","affiliation":[{"name":"Academic Division of Endocrinology, Department of Medicine Royal College of Surgeons in Ireland (RCSI) Dublin Ireland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3402-4727","authenticated-orcid":false,"given":"Nils P.","family":"Krone","sequence":"additional","affiliation":[{"name":"Division of Clinical Medicine, School of Medicine and Population Health University of Sheffield Sheffield UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,3,17]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1210\/endrev\/bnab016"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2018-01865"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-20-1249"},{"key":"e_1_2_6_5_1","first-page":"CD012517","article-title":"Glucocorticoid replacement regimens in the treatment of 21\u2010hydroxylase deficiency congenital adrenal hyperplasia","volume":"184","author":"Ng SM","year":"2017","journal-title":"Cochrane Database Syst 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