{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T09:47:56Z","timestamp":1779270476888,"version":"3.51.4"},"reference-count":28,"publisher":"The Endocrine Society","issue":"1","license":[{"start":{"date-parts":[[2019,9,16]],"date-time":"2019-09-16T00:00:00Z","timestamp":1568592000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"APHP","award":["AOR11111"],"award-info":[{"award-number":["AOR11111"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Non-classic 21-hydroxylase deficiency is usually diagnosed in post-pubertal women because of androgen excess. Indication of systematic steroid replacement therapy is controversial because the risk of acute adrenal insufficiency is unknown. In order to specify this risk we evaluated the cortisol and aldosterone secretions in response to appropriate pharmacologic challenges.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>In this prospective case\u2013control non-inferiority study we investigated 20 women with non-classic 21-hydroxylase deficiency carrying biallelic CYP21A2 mutations and with serum 17-hydroxyprogesterone (17OHP) &amp;gt;10 ng\/mL after stimulation with Synacthen\u00ae (tetracosactrin) and 20 age- and body mass index-matched healthy women with 17OHP after Synacthen\u00ae &amp;lt;2 ng\/mL. Each participant underwent sequentially an insulin tolerance test to evaluate cortisol secretion and a sodium depletion test, obtained by oral administration of 40 mg of furosemide under low sodium diet (&amp;lt;20 mmol during 24 hours), to evaluate renin and aldosterone secretion.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Findings<\/jats:title>\n                  <jats:p>The peak serum cortisol concentration after insulin hypoglycemia was lower in patients than in controls (mean difference \u201347 ng\/mL, 90% CI, \u201366, P = 0.0026). A peak serum cortisol above a cutoff value of 170 ng\/mL was obtained in all controls but only in 55% of patients (P = 0.0039). Twenty-four hours after sodium depletion, blood pressure, plasma sodium, potassium, and serum aldosterone concentrations were comparable between the two groups, but patients had higher stimulated renin concentrations than controls (P = 0.0044).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Interpretation<\/jats:title>\n                  <jats:p>Patients with non-classic 21-hydroxylase deficiency frequently display partial cortisol insufficiency and compensated defect in aldosterone secretion. Their clinical management should systematically include assessment of adrenal functions.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgz005","type":"journal-article","created":{"date-parts":[[2019,9,17]],"date-time":"2019-09-17T19:47:56Z","timestamp":1568749676000},"page":"55-64","source":"Crossref","is-referenced-by-count":10,"title":["Cortisol and Aldosterone Responses to Hypoglycemia and Na Depletion in Women With Non-Classic 21-Hydroxylase Deficiency"],"prefix":"10.1210","volume":"105","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1994-4226","authenticated-orcid":false,"given":"Peter","family":"Kamenick\u00fd","sequence":"first","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital de Bic\u00eatre, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Le Kremlin-Bic\u00eatre, France"},{"name":"Univ Paris-Sud, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (Inserm) UMR_S1185, Le Kremlin Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Blanchard","sequence":"additional","affiliation":[{"name":"Centre d\u2019Investigation Clinique, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonin","family":"Lamaziere","sequence":"additional","affiliation":[{"name":"Plateforme de Me\u0301tabolomique, Peptidomique et de Dosage de Me\u0301dicaments, H\u00f4pital Saint-Antoine, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9line","family":"Piedvache","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Recherche Clinique, H\u00f4pital de Bic\u00eatre, AP-HP, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno","family":"Donadille","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital Saint-Antoine, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lise","family":"Duranteau","sequence":"additional","affiliation":[{"name":"UF de Gyn\u00e9cologie Adolescente et Jeune Adulte, H\u00f4pital de Bic\u00eatre, AP-HP, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H\u00e9l\u00e8ne","family":"Bry","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital de Bic\u00eatre, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Fran\u00e7ois","family":"Gautier","sequence":"additional","affiliation":[{"name":"Centre Universitaire de Diab\u00e9tologie et de ses Complications, H\u00f4pital Lariboisi\u00e8re, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylvie","family":"Salenave","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital de Bic\u00eatre, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie-Laure","family":"Raffin-Sanson","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie, H\u00f4pital Ambroise Parr\u00e9, Boulogne-Billancourt, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seray","family":"Genc","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie, H\u00f4pital Jean Bernard, Valenciennes Cedex, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurence","family":"Pietri","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie, H\u00f4pital Saint-Joseph, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sophie","family":"Christin-Maitre","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital Saint-Antoine, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julien","family":"Thomas","sequence":"additional","affiliation":[{"name":"Plateforme de Me\u0301tabolomique, Peptidomique et de Dosage de Me\u0301dicaments, H\u00f4pital Saint-Antoine, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aurelien","family":"Lorthioir","sequence":"additional","affiliation":[{"name":"Centre d\u2019Investigation Clinique, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP, Paris, France"},{"name":"Unit\u00e9 d\u2019Hypertension, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michel","family":"Azizi","sequence":"additional","affiliation":[{"name":"Centre d\u2019Investigation Clinique, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP, Paris, France"},{"name":"Unit\u00e9 d\u2019Hypertension, H\u00f4pital Europ\u00e9en Georges Pompidou, AP-HP, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe","family":"Chanson","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital de Bic\u00eatre, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Le Kremlin-Bic\u00eatre, France"},{"name":"Univ Paris-Sud, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (Inserm) UMR_S1185, Le Kremlin Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yves","family":"Le Bouc","sequence":"additional","affiliation":[{"name":"Inserm UMR_S 938, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylvie","family":"Brailly-Tabard","sequence":"additional","affiliation":[{"name":"Univ Paris-Sud, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Service de G\u00e9n\u00e9tique Mol\u00e9culaire, Pharmacog\u00e9n\u00e9tique et Hormonologie, H\u00f4pital de Bic\u00eatre, AP-HP, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacques","family":"Young","sequence":"additional","affiliation":[{"name":"Service d\u2019Endocrinologie et des Maladies de la Reproduction, H\u00f4pital de Bic\u00eatre, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Le Kremlin-Bic\u00eatre, France"},{"name":"Univ Paris-Sud, 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