{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,25]],"date-time":"2026-06-25T16:45:42Z","timestamp":1782405942519,"version":"3.54.5"},"reference-count":0,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T00:00:00Z","timestamp":1750636800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T00:00:00Z","timestamp":1750636800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"abstract":"<jats:p>Objectives: Some children with classic congenital adrenal hyperplasia (CAH) achieve excellent control on very low glucocorticoid doses. We aimed to characterize these patients and assess the timing of their low-dose requirements.\nMethods: We reviewed charts of patients with salt-wasting CAH due to 21-hydroxylase deficiency, defining low-dose glucocorticoids as &lt;10 mg\/m\u00b2\/d. Demographic and growth data were compared with a matched group on standard doses.\nResults: Among 154 patients with CAH, 14 (9%) required low-dose glucocorticoid therapy (&lt;10 mg\/m\u00b2\/d), including 8 boys (57%) and 6 girls (43%). The average age at treatment initiation was 2.1 years, comparable to a matched group of 23 patients (48% boys). The low-dose group received 8.8 \u00b1 1.2 mg\/m\u00b2\/d versus 14.9 \u00b1 3.9 mg\/m\u00b2\/d in the matched group (P &lt; 0.001), with similar fludrocortisone doses (0.1 \u00b1 0.05 mg). No differences were observed in weight, height, or height velocity. Of the 14 patients on low-dose treatment, 3 experienced an increase in their glucocorticoid dose requirement above 10 mg\/m2\/d at ages 10.3, 10.8, and 8.5 years after being on 6.3 -9.8 mg\/m2\/d for 6.4-8.5 years. The remaining 11 patients are currently on 5.89-10 mg\/m2\/d with a duration on low-dose therapy ranging from 0.48 to 8.65 years.\nConclusions: Our findings highlight a subgroup of patients with 21-hydroxylase deficiency who achieve good control on low glucocorticoid doses from early childhood. The factors underlying this and the transient need for low doses in some remain unclear.\n<\/jats:p>","DOI":"10.1159\/000546883","type":"journal-article","created":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T16:00:49Z","timestamp":1750694449000},"page":"1-12","source":"Crossref","is-referenced-by-count":2,"title":["Clinical Variability in Congenital Adrenal Hyperplasia: A Distinct Subgroup with A Low Glucocorticoid Dose Requirement"],"prefix":"10.1159","author":[{"given":"Ala","family":"Ustyol","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6135-7474","authenticated-orcid":false,"given":"Erica A.","family":"Eugster","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2025,6,23]]},"container-title":["Hormone Research in Paediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000546883","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000546883","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,23]],"date-time":"2025-06-23T16:00:49Z","timestamp":1750694449000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000546883"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,23]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1159\/000546883","archive":["Portico"],"relation":{},"ISSN":["1663-2818","1663-2826"],"issn-type":[{"value":"1663-2818","type":"print"},{"value":"1663-2826","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,23]]}}}