{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T05:35:07Z","timestamp":1777095307903,"version":"3.51.4"},"reference-count":34,"publisher":"The Endocrine Society","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Treatment of children with classic congenital adrenal hyperplasia (CAH) with glucocorticoids is a difficult balance between hypercortisolism and hyperandrogenism. Biochemical monitoring of treatment is not well defined. Achievement of a normal growth rate is the most important therapeutic goal.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We retrospectively evaluated 123 24-hour gas chromatography\u2013mass spectrometry urinary steroid metabolome analyses together with their corresponding 1-year height velocity (HV) z scores in 63 prepubertal children aged 7.2 \u00b1 1.6 years with classic CAH due to 21-hydroxylase deficiency treated with hydrocortisone and fludrocortisone.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Multivariate linear mixed effects model analysis revealed a positive influence of CAH-specific z scores of summed urinary androgen metabolites (B = 0.97 \u00b1 0.20, t = 4.87, P &lt; 0.0001) and a negative influence of the cortisol metabolite tetrahydrocortisol (B = \u22121.75 \u00b1 0.79, t = \u22122.20, P = 0.03) on HV z scores. Receiver operating characteristic analysis demonstrated that adrenal androgen excess, defined as HV &gt;1.5 z, was best determined by a z score of all urinary androgen metabolites of &gt;0.512 [accuracy, 66.2%; sensitivity, 57.1%; specificity, 74.4%; positive prediction value (PPV), 66.7%; negative prediction value (NPV), 65.9%]. Tetrahydrocortisol excretion &gt;1480 \u00b5g\/m2 BSA\/d in conjunction with suppressed urinary androgen metabolites &lt;0.163 z indicated overtreatment, defined as HV &lt; \u22121.5 z (accuracy, 79.6%; sensitivity, 40.0%; specificity, 94.9%; PPV, 75.0%; NPV, 80.4%).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>We established target values for urinary steroid metabolite excretions in children with CAH based on their growth rate. Urinary steroid metabolome analysis represents a highly suitable method for monitoring metabolic control in children with CAH.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/jc.2019-00438","type":"journal-article","created":{"date-parts":[[2019,5,21]],"date-time":"2019-05-21T09:31:06Z","timestamp":1558431066000},"page":"4214-4224","source":"Crossref","is-referenced-by-count":18,"title":["Height Velocity Defined Metabolic Control in Children With Congenital Adrenal Hyperplasia Using Urinary Steroid GC-MS Analysis"],"prefix":"10.1210","volume":"104","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8241-4105","authenticated-orcid":false,"given":"Clemens","family":"Kamrath","sequence":"first","affiliation":[{"name":"Division of Pediatric Endocrinology and Diabetology, Laboratory for Translational Hormone Analysis in Pediatric Endocrinology, Steroid Research & Mass Spectrometry Unit, Center of Child and Adolescent Medicine, Justus Liebig University, Giessen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa","family":"Wettstaedt","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology and Diabetology, Laboratory for Translational Hormone Analysis in Pediatric Endocrinology, Steroid Research & Mass Spectrometry Unit, Center of Child and Adolescent Medicine, Justus Liebig University, Giessen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michaela F","family":"Hartmann","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology and Diabetology, Laboratory for Translational Hormone Analysis in Pediatric Endocrinology, Steroid Research & Mass Spectrometry Unit, Center of Child and Adolescent Medicine, Justus Liebig University, Giessen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan A","family":"Wudy","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology and Diabetology, 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