{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,9]],"date-time":"2026-05-09T09:32:41Z","timestamp":1778319161846,"version":"3.51.4"},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T00:00:00Z","timestamp":1768348800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Diurnal Ltd and research"},{"DOI":"10.13039\/501100000265","name":"Medical Research Council United Kingdom","doi-asserted-by":"publisher","award":["G1100236"],"award-info":[{"award-number":["G1100236"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004963","name":"Seventh Framework Program","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004963","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000780","name":"European Union","doi-asserted-by":"publisher","award":["201444"],"award-info":[{"award-number":["201444"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010381","name":"European Society for Paediatric Endocrinology","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010381","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,2,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Management of 21-hydroxylase deficiency (21-OHD) congenital adrenal hyperplasia (CAH) in early infancy is challenging, with extent of variation in management unclear.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Design and Methods<\/jats:title>\n                    <jats:p>Using the I-CAH Registry, we retrospectively reviewed management over the first 90 days of life of 154 infants with 21-OHD born in 2018-2023, across 33 centers in 18 countries.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 154 infants (92 female, 62 male), 136 were diagnosed postnatally, with median (10th centile, 90th centile) presentation age of Day 4 (0, 20.8). At initial hospital discharge, median doses of hydrocortisone (HC), fludrocortisone (FC), and salt were 17 (11.4, 39.6) mg\/m2\/day, 100 (50, 200) mcg\/day and 3.5 (1.6, 8.7) mmol\/kg\/day, and at Day 90 (D90) 14.5 (8.7, 24.1) mg\/m2\/day, 100 (50, 200) mcg\/day, and 2.1 (1.0, 5.2) mmol\/kg\/day, respectively. Hyponatremia, hyperkalemia, and hypoglycemia were reported in 70.0%, 71.9%, and 13.0% of infants, respectively. At D90, hyponatremia and hyperkalemia were reported in 7.4% and 28.6%, respectively. At D90, BP measurements were recorded in 30.5%, amongst whom 31.9% had hypertension reported. Median total hospitalization duration over 90 days was 9 days (2, 24). Adrenal crises were associated with 40. 6% of hospitalization episodes. Percentages (males:females) of cases seen by a pediatric endocrinologist, psychologist, pediatric endocrine nurse specialist, and surgeon by D90 were 95.9% (58:84), 33.3% (9:35), 42.1% (20:36), and 23.8% (0:35), respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Contemporary management of CAH in early infancy varies considerably. Hypertension and hyperkalemia are frequently reported. Our data may help inform development of quality indicators for benchmarking CAH care in infancy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejendo\/lvag004","type":"journal-article","created":{"date-parts":[[2026,1,13]],"date-time":"2026-01-13T12:29:22Z","timestamp":1768307362000},"page":"123-135","source":"Crossref","is-referenced-by-count":1,"title":["Contemporary global management of 21-hydroxylase deficiency congenital adrenal hyperplasia in early infancy: a multi-national registry study"],"prefix":"10.1093","volume":"194","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3174-5051","authenticated-orcid":false,"given":"David B N","family":"Lim","sequence":"first","affiliation":[{"name":"Department of Paediatric Endocrinology, University Hospital Southampton , Southampton SO16 6YD ,","place":["United Kingdom"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jillian","family":"Bryce","sequence":"additional","affiliation":[{"name":"Office for Rare Conditions, Royal Hospital for Children & Queen Elizabeth University Hospital , Glasgow G51 4TF ,","place":["United Kingdom"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2178-269X","authenticated-orcid":false,"given":"Salma R","family":"Ali","sequence":"additional","affiliation":[{"name":"Office for Rare Conditions, Royal Hospital for Children & Queen Elizabeth University Hospital , Glasgow G51 4TF ,","place":["United Kingdom"]},{"name":"Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow , Glasgow G12 8QQ ,","place":["United Kingdom"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3608-8695","authenticated-orcid":false,"given":"Xanthippi","family":"Tseretopoulou","sequence":"additional","affiliation":[{"name":"Office for Rare Conditions, Royal Hospital for Children & Queen Elizabeth University Hospital , Glasgow G51 4TF ,","place":["United Kingdom"]},{"name":"Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow , Glasgow G12 8QQ ,","place":["United Kingdom"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niels H","family":"Birkebaek","sequence":"additional","affiliation":[{"name":"Department of Paediatrics and Adolescent Medicine and Department of Clinical Medicine, Aarhus University Hospital , 8200 Aarhus ,","place":["Denmark"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sabine E","family":"Hannema","sequence":"additional","affiliation":[{"name":"Department of Paediatric Endocrinology, Amsterdam UMC Location Vrije Universiteit , 1081 HV, Amsterdam ,","place":["Netherlands"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ariadna","family":"Campos-Martorell","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology Section, Vall d\u2019Hebron Hospital; Growth and Development Research Group, Vall d\u2019Hebron Research Institute (VHIR), Universitat Aut\u00f2noma de Barcelona, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III , 08035 Barcelona ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Clemente","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology Section, Vall d\u2019Hebron Hospital; Growth and Development Research Group, Vall d\u2019Hebron Research Institute (VHIR), Universitat Aut\u00f2noma de Barcelona, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III , 08035 Barcelona ,","place":["Spain"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3892-0577","authenticated-orcid":false,"given":"Uta","family":"Neumann","sequence":"additional","affiliation":[{"name":"Clinic for Paediatric Endocrinology and Diabetes, Charit\u00e9 Universit\u00e4tsmedizin Berlin , 10117 Berlin ,","place":["Germany"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4568-5504","authenticated-orcid":false,"given":"Christa E","family":"Fl\u00fcck","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology and Diabetology, University Children's Hospital Bern , 3010 Bern ,","place":["Switzerland"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Metzger","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology and Diabetology, University Children's Hospital Bern , 3010 Bern ,","place":["Switzerland"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruth E","family":"Krone","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology, Birmingham Women's and Children's Hospital , Birmingham B4 6NH ,","place":["United Kingdom"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0934-9420","authenticated-orcid":false,"given":"Alina","family":"German","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology and Diabetes, Haemek Medical Center , Afula, 1834111 ,","place":["Israel"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federico","family":"Baronio","sequence":"additional","affiliation":[{"name":"Paediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico di S. 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