{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T08:29:53Z","timestamp":1792225793298,"version":"4.4.0"},"reference-count":26,"publisher":"S. Karger AG","issue":"2","license":[{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Horm Res Paediatr"],"published-print":{"date-parts":[[2011]]},"abstract":"<i>Objective:<\/i> Glucocorticoid-remediable aldosteronism (GRA) is caused by the presence of a chimeric gene originating from an unequal cross-over between the <i>CYP11B1<\/i> and <i>CYP11B2<\/i> genes. Aldosterone suppression by dexamethasone and high 18-hydroxycortisol (18-OHF) levels have been used to differentiate GRA from the other forms of primary aldosteronism. <i>Methods:<\/i> A dexamethasone suppression test including serum 18-OHF determination and the measurement of urinary excretion of aldosterone, its metabolites and 18-OHF were performed in 3 children of a family with primary aldosteronism. Polymerase chain reactions were performed to identify the chimeric gene. <i>Results:<\/i> The chimeric gene was identified in 2 children, their mother and grandmother. The affected children had an aldosterone-to-plasma renin activity ratio >30, elevated serum 18-OHF concentration and increased urinary excretion of aldosterone, its metabolites, and 18-OHF. Post-dexamethasone concentrations of serum aldosterone and 18-OHF concentrations were suppressed. <i>Conclusion:<\/i> Although very rare, the possible diagnosis of GRA should be considered in all children or young adults with low-renin hypertension. Since genetic testing is more specific than biochemical testing, a definitive diagnosis can only be obtained by identification of the <i>CYP11B1\/CYP11B2<\/i> chimeric gene.<\/jats:p>","DOI":"10.1159\/000326524","type":"journal-article","created":{"date-parts":[[2011,5,27]],"date-time":"2011-05-27T06:42:23Z","timestamp":1306478543000},"page":"93-98","source":"Crossref","is-referenced-by-count":15,"title":["Diagnosis of Glucocorticoid-Remediable Aldosteronism in Hypertensive Children"],"prefix":"10.1159","volume":"76","author":[{"given":"Clemens","family":"Kamrath","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christiane","family":"Maser-Gluth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Haag","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Egbert","family":"Schulze","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2011,5,27]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1210%2Fjcem-27-2-300"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1097%2F00060793-199606000-00012"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.82.5.1507"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1038%2F355262a0"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1038%2Fng0992-66"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1073%2Fpnas.89.17.8327"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1038%2Fnsb0197-32"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.83.11.3996"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.82.11.3570"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.83.7.2573"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.85.5.1863"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016%2Fj.mce.2003.10.056"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1016%2FS0022-4731%2883%2980046-6"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1038%2Fsj.jhh.1000918"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1161%2F01.HYP.0000107251.49515.c2"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1038%2Fsj.jhh.1001821"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1097%2F00004872-200311000-00025"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1530%2Feje.0.1500329"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.2003-031337"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1291%2Fhypres.30.111"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1210%2Fjc.2008-0104"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1440-1681.1996.tb02789.x"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1530%2Feje.1.01922"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1210%2Fjcem-65-2-310"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.2165%2F00003088-200140060-00004"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1067%2Fmpd.2001.112648"}],"container-title":["Hormone Research in Paediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/326524","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/326524","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T19:04:59Z","timestamp":1745435099000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000326524"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011]]},"references-count":26,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2011,8,1]]},"published-print":{"date-parts":[[2011,8,12]]}},"URL":"https:\/\/doi.org\/10.1159\/000326524","archive":["Portico"],"relation":{},"ISSN":["1663-2818","1663-2826"],"issn-type":[{"value":"1663-2818","type":"print"},{"value":"1663-2826","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011]]}}}