{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T01:52:28Z","timestamp":1791337948088,"version":"4.3.1"},"reference-count":36,"publisher":"The Endocrine Society","issue":"2","funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","award":["1K08DK109116"],"award-info":[{"award-number":["1K08DK109116"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Context<\/jats:title>\n                    <jats:p>Serum 17-hydroxyprogesterone (17OHP) and androstenedione (A4) are the conventional biomarkers used to assess disease control in patients with 21-hydroxylase deficiency (21OHD). However, discrepancy between the two is not uncommon, limiting interpretation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To evaluate 11-oxyandrogens in discriminating good versus poor disease control in 21OHD in the setting of discrepant 17OHP and A4.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Retrospective analysis of 2738 laboratory assessments obtained as part of Natural History Study of congenital adrenal hyperplasia (CAH) at the National Institutes Health Clinical Center. Patients with discrepant 17OHP and A4 and available sera were selected. A 15-steroid mass-spectrometry panel was performed in sera from patients with 21OHD and age- and sex-matched controls. Patients were categorized in \u201cgood\u201d or \u201cpoor\u201d control based on clinical assessment (bone age advancement, signs and symptoms of precocious puberty, menstrual irregularity, hirsutism, or hypogonadotrophic hypogonadism).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Discrepant 17OHP and A4 was found in 469 (17%) laboratory assessments. Of these, 403 (86%) had elevated 17OHP with A4 in reference range. Of 46 patients with available sera, 30 (65%) were in good control. Median fold elevation relative to controls was higher in patients with poor versus good control for 11-hydroxytestosterone (median [interquartile range], 2.82 [1.25-5.43] vs 0.91 [0.49- 2.07], P\u2005=\u2005.003), and 11-ketotestosterone (3.57 [2.11-7.41] vs 1.76 [1.24-4.00], P\u2005=\u2005.047). Fold elevation of 11-hydroxytestosterone between 3.48 (sensitivity 97%, specificity 47%) and 3.88 (sensitivity 100%, specificity 40%) provided the best discrimination between poor vs good control.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>11-Oxyandrogens, especially 11-hydroxytestosterone, may be useful in the management of CAH when conventional biomarkers are inconclusive.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/jendso\/bvaa192","type":"journal-article","created":{"date-parts":[[2020,12,12]],"date-time":"2020-12-12T02:14:27Z","timestamp":1607739267000},"source":"Crossref","is-referenced-by-count":37,"title":["11-Oxygenated Androgens Useful in the Setting of Discrepant Conventional Biomarkers in 21-Hydroxylase Deficiency"],"prefix":"10.1210","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9201-3340","authenticated-orcid":false,"given":"Smita","family":"Jha","sequence":"first","affiliation":[{"name":"Section on Congenital Disorders, National Institutes of Health Clinical Center, Bethesda, MD, USA"},{"name":"Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adina F","family":"Turcu","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ninet","family":"Sinaii","sequence":"additional","affiliation":[{"name":"Biostatistics and Clinical Epidemiology Service, National Institutes of Health, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7372-9340","authenticated-orcid":false,"given":"Brittany","family":"Brookner","sequence":"additional","affiliation":[{"name":"Section on Congenital Disorders, National Institutes of Health Clinical Center, Bethesda, MD, USA"}],"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":"Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, MI, 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":"Section on Congenital Disorders, National Institutes of Health Clinical Center, Bethesda, MD, USA"},{"name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2020,12,11]]},"reference":[{"issue":"11","key":"2021121113050937500_CIT0001","doi-asserted-by":"crossref","first-page":"4043","DOI":"10.1210\/jc.2018-01865","article-title":"Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an endocrine society clinical practice guideline","volume":"103","author":"Speiser","year":"2018","journal-title":"J Clin Endocrinol Metab."},{"issue":"13","key":"2021121113050937500_CIT0002","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 Med."},{"issue":"11","key":"2021121113050937500_CIT0003","first-page":"810","article-title":"Correlation between androstenedione and 17-hydroxyprogesterone levels in the saliva and plasma of patients with congenital adrenal hyperplasia","volume":"52","author":"Juniarto","year":"2011","journal-title":"Singapore Med J."},{"issue":"10","key":"2021121113050937500_CIT0004","doi-asserted-by":"crossref","first-page":"4679","DOI":"10.1210\/jcem.86.10.7972","article-title":"Serum cortisol and 17-hydroxyprogesterone interrelation in classic 21-hydroxylase deficiency: is current replacement therapy satisfactory?","volume":"86","author":"Charmandari","year":"2001","journal-title":"J Clin Endocrinol Metab."},{"issue":"2","key":"2021121113050937500_CIT0005","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1203\/00006450-198302000-00010","article-title":"Disproportionate suppression of dehydroepiandrosterone sulfate (DHEAS) in treated patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency","volume":"17","author":"Rezvani","year":"1983","journal-title":"Pediatr 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