{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T10:08:52Z","timestamp":1792663732148,"version":"4.5.0"},"reference-count":210,"publisher":"The Endocrine Society","issue":"9","license":[{"start":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T00:00:00Z","timestamp":1679529600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,8,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The most common form of congenital adrenal hyperplasia is 21-hydroxylase deficiency (21OHD), which in the classic (severe) form occurs in roughly 1:16 000 newborns worldwide. Lifelong treatment consists of replacing cortisol and aldosterone deficiencies, and supraphysiological dosing schedules are typically employed to simultaneously attenuate production of adrenal-derived androgens. Glucocorticoid titration in 21OHD is challenging as it must balance the consequences of androgen excess vs those from chronic high glucocorticoid exposure, which are further complicated by interindividual variability in cortisol kinetics and glucocorticoid sensitivity. Clinical assessment and biochemical parameters are both used to guide therapy, but the specific purpose and goals of each biomarker vary with age and clinical context. Here we review the approach to medication titration for children and adults with classic 21OHD, with an emphasis on how to interpret adrenal biomarker values in guiding this process. In parallel, we illustrate how an understanding of the pathophysiologic and pharmacologic principles can be used to avoid and to correct complications of this disease and consequences of its management using existing treatment options.<\/jats:p>","DOI":"10.1210\/clinem\/dgad134","type":"journal-article","created":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T02:36:37Z","timestamp":1679538997000},"page":"2154-2175","source":"Crossref","is-referenced-by-count":32,"title":["Interpretation of Steroid Biomarkers in 21-Hydroxylase Deficiency and Their Use in Disease Management"],"prefix":"10.1210","volume":"108","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5741-3629","authenticated-orcid":false,"given":"Kyriakie","family":"Sarafoglou","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Endocrinology, University of Minnesota Medical School , Minneapolis, MN 55454 , USA"},{"name":"Department of Experimental and Clinical Pharmacology, University of Minnesota College of Pharmacy , Minneapolis, MN 55455 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3746-0460","authenticated-orcid":false,"given":"Deborah P","family":"Merke","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, National Institutes of Health Clinical Center , Bethesda, MD 20892 , USA"},{"name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development , Bethesda, MD , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7469-6069","authenticated-orcid":false,"given":"Nicole","family":"Reisch","sequence":"additional","affiliation":[{"name":"Medizinische Klinik and Poliklinik IV, Klinikum der Universit\u00e4t M\u00fcnchen , 80336 Munich , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0181-0403","authenticated-orcid":false,"given":"Hedi","family":"Claahsen-van der Grinten","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center , 6500 HB, Nijmegen , The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5622-6987","authenticated-orcid":false,"given":"Henrik","family":"Falhammar","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet , SE-17176, Stockholm , Sweden"},{"name":"Department of Endocrinology, Karolinska University Hospital , SE-17176, Stockholm , Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6815-6181","authenticated-orcid":false,"given":"Richard J","family":"Auchus","sequence":"additional","affiliation":[{"name":"Departments of Pharmacology and Internal Medicine, Division of Metabolism, Endocrinology and Diabetes, University of Michigan Medical School , Ann Arbor, MI 48109 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2023,3,23]]},"reference":[{"issue":"1","key":"2023081819563237400_dgad134-B1","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1210\/er.2010-0013","article-title":"The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders","volume":"32","author":"Miller","year":"2011","journal-title":"Endocr Rev"},{"issue":"13","key":"2023081819563237400_dgad134-B2","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1056\/NEJMra1909786","article-title":"Congenital adrenal hyperplasia due to 21-hydroxylase deficiency","volume":"383","author":"Merke","year":"2020","journal-title":"N Engl J 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