{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,24]],"date-time":"2024-07-24T11:54:40Z","timestamp":1721822080130},"reference-count":26,"publisher":"The Endocrine Society","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1997,11,1]]},"abstract":"<jats:p>Glucocorticoid-remediable aldosteronism (GRA) is a rare form of inherited hypertension caused by a characteristic gene duplication. With the advent of definitive genetic testing for GRA, the performance of the traditional screening test for GRA, the dexamethasone suppression test (DST), can be evaluated. We compared the DST to direct genetic testing in 24 patients referred for genetic screening for GRA (12 GRA positive and 12 GRA negative) based on clinical and biochemical findings, DST, and family history. Plasma aldosterone was measured before and after oral dexamethasone administration to determine the extent to which aldosterone was suppressed by glucocorticoids in each patient group. The results of the DST in these subjects were also compared to those in 19 historical patients with primary aldosteronism[ 4 bilateral hyperplasia and 15 aldosterone-producing adenoma (APA)] reported previously. The DST differentiated GRA-positive from GRA-negative patients with 92% sensitivity and 100% specificity. Cutoffs based on the post-DST plasma aldosterone level (&amp;lt;4 ng\/dL) or percent suppression compared to baseline (&amp;gt;80%) were equally effective in correctly diagnosing GRA (only one GRA-positive patient would have been incorrectly diagnosed). However, DST in 15 APA patients revealed that 33% had greater than 80% suppression of aldosterone, and 1 had aldosterone levels below 4 ng\/dL.<\/jats:p>\n               <jats:p>We conclued that a post-DST aldosterone level below 4 ng\/dL will correctly diagnose GRA patients with high sensitivity and specificity. Suppression compared to baseline can be misleading, as evidenced by the results in APA patients and referred subjects who genetically screened negative.<\/jats:p>","DOI":"10.1210\/jcem.82.11.4381","type":"journal-article","created":{"date-parts":[[2014,1,8]],"date-time":"2014-01-08T17:01:10Z","timestamp":1389200470000},"page":"3570-3573","source":"Crossref","is-referenced-by-count":3,"title":["Evaluation of the Dexamethasone Suppression Test for the Diagnosis of Glucocorticoid-Remediable Aldosteronism1"],"prefix":"10.1210","volume":"82","author":[{"given":"W. Reid","family":"Litchfield","sequence":"first","affiliation":[{"name":"Department of Medicine, Brigham and Women\u2019s Hospital and Harvard Medical School (W.R.L., C.C., R.G.D.), Boston, Massachusetts 02115"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria I.","family":"New","sequence":"additional","affiliation":[{"name":"the Department of Medicine, New York Hospital and Cornell Medical Center (M.I.N.), New York, New York 10021"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Coolidge","sequence":"additional","affiliation":[{"name":"Department of Medicine, Brigham and Women\u2019s Hospital and Harvard Medical School (W.R.L., C.C., R.G.D.), Boston, Massachusetts 02115"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard P.","family":"Lifton","sequence":"additional","affiliation":[{"name":"the Departments of Medicine and Genetics, Boyer Center for Molecular Medicine (R.P.L.), Yale University School of Medicine, New Haven, Connecticut 06510"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert G.","family":"Dluhy","sequence":"additional","affiliation":[{"name":"Department of Medicine, Brigham and Women\u2019s Hospital and Harvard Medical School (W.R.L., C.C., R.G.D.), Boston, Massachusetts 02115"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","reference":[{"key":"2020071618332819900_R1","first-page":"265","volume":"3","author":"Litchfield","year":"1996"},{"key":"2020071618332819900_R2","first-page":"553","article-title":"Glucocorticoid-remediable aldosteronism.","volume":"21","author":"Litchfield","year":"1995","journal-title":"Comp Ther"},{"key":"2020071618332819900_R3","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1038\/ng0992-66","article-title":"Hereditary hypertension caused by chimaeric gene duplications and ectopic expression of aldosterone synthase.","volume":"2","author":"Lifton","year":"1992","journal-title":"Nat Genet"},{"key":"2020071618332819900_R4","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1038\/355262a0","article-title":"A chimaeric 11 beta-hydroxylase\/aldosterone synthase gene causes glucocorticoid-remediable aldosteronism and human hypertension.","volume":"355","author":"Lifton","year":"1992","journal-title":"Nature"},{"key":"2020071618332819900_R5","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1210\/jcem-44-4-775","article-title":"Transient fall and subsequent return of high aldosterone secretion by adrenal adenoma during continued dexamethasone administration.","volume":"44","author":"Ganguly","year":"1977","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R6","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1210\/jcem-46-2-326","article-title":"ACTH-dependent aldosterone excess due to adrenocortical adenoma: a variant of primary aldosteronism.","volume":"46","author":"Wenting","year":"1978","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R7","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1210\/jcem-51-6-1330","article-title":"Nocturnal, daytime, and postural changes of plasma aldosterone before and during dexamethasone in adenomatous and idiopathic aldosteronism.","volume":"51","author":"Hoefnagels","year":"1980","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R8","doi-asserted-by":"crossref","first-page":"5759","DOI":"10.1073\/pnas.78.9.5759","article-title":"Polymorphic DNA region adjacent to the 5\u2032endo of the human insulin gene.","volume":"78","author":"Bell","year":"1981","journal-title":"Proc Natl Acad Sci USA"},{"key":"2020071618332819900_R9","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1210\/jcem-20-12-1539","article-title":"Tests of pituitary-adrenal suppressibility in the diagnosis of Cushing\u2019s syndrome.","volume":"20","year":"1960","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R10","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1210\/jcem-19-8-875","article-title":"Clinical application of a new test of pituitary reserve.","volume":"19","author":"Liddle","year":"1959","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R11","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1530\/acta.0.1130564","article-title":"Plasma aldosterone-plasma renin activity ratio. A simple test to identify patients with primary aldosteronism.","volume":"113","author":"Lins","year":"1986","journal-title":"Acta Endocrinol (Copenh)"},{"key":"2020071618332819900_R12","first-page":"1610","article-title":"Clinical, biochemical, and genetic approaches to the detection of familial hyperaldosteronism type I.","volume":"13","author":"Stowasser","year":"1995","journal-title":"J Hypertens"},{"key":"2020071618332819900_R13","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1006\/bbrc.1995.1225","article-title":"A new genetic test for familial hyperaldosteronism type I aids in the detection of curable hypertension.","volume":"207","author":"Jonsson","year":"1995","journal-title":"Biochem Biophys Res Commun"},{"key":"2020071618332819900_R14","first-page":"1109","article-title":"Hypertension, increased aldosterone secretion, and low plasma renin activity relieved by dexamethasone.","volume":"95","author":"Sutherland","year":"1966","journal-title":"Can Med Assoc J"},{"key":"2020071618332819900_R15","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1210\/jcem-36-4-715","article-title":"A kindred with familial glucocorticoid suppressible aldosteronism.","volume":"36","author":"Giebink","year":"1973","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R16","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1210\/jcem-53-1-158","article-title":"Adrenal glomerulosa function in patients with dexamethasone-suppressible hyperaldosteronism.","volume":"53","author":"Oberfield","year":"1981","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R17","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1111\/j.1440-1681.1985.tb02640.x","article-title":"Hypertension corrected and aldosterone responsiveness to renin-angiotensin restored by long-term dexamethasone in glucocorticoid-suppressible hyperaldosteronism.","volume":"12","author":"Woodland","year":"1985","journal-title":"Clin Exp Pharmacol Physiol"},{"key":"2020071618332819900_R18","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1507\/endocrj1954.35.311","article-title":"A case of glucocorticoid-suppressible hyperaldosteronism with aldosterone producing adenoma.","volume":"35","author":"Kato","year":"1988","journal-title":"Endocrinol Jpn"},{"key":"2020071618332819900_R19","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1210\/jcem-53-5-1040","article-title":"Genetic and pathophysiologic studies of a new kindred with glucocorticoid-suppressible hyperaldosteronism manifest in three generations.","volume":"53","author":"Ganguly","year":"1981","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R20","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1210\/jcem-64-1-22","article-title":"Long term evolution of glucocorticoid-suppressible hyperaldosteronism.","volume":"64","author":"Stockigt","year":"1987","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R21","doi-asserted-by":"crossref","first-page":"1014","DOI":"10.1210\/jcem-28-7-1014","article-title":"Effects of glucocorticoid administration on aldosterone excretion and plasma renin in normal subjects, in essential hypertension and in primary aldosteronism.","volume":"28","author":"Newton","year":"1968","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R22","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1210\/jcem-43-1-115","article-title":"Circadian rhythm and effect of posture on plasma aldosterone concentration in primary aldosteronism.","volume":"43","author":"Schambelan","year":"1976","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R23","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1210\/jcem-29-2-239","article-title":"Stimulation and suppression of aldosterone secretion in patients with an aldosterone producing adenoma.","volume":"29","author":"Slaton","year":"1969","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R24","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1210\/jcem-64-1-22","article-title":"Long term evolution of glucocorticoid-suppressible hyperaldosteronism.","volume":"64","author":"Stockigt","year":"1987","journal-title":"J Clin Endocrinol Metab"},{"key":"2020071618332819900_R25","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1161\/01.HYP.8.8.669","article-title":"Dexamethasone-suppressible hyperaldosteronism. Adrenal transition cell hyperplasia?","volume":"8","author":"Connell","year":"1986","journal-title":"Hypertension"},{"key":"2020071618332819900_R26","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1210\/jcem-71-5-1151","article-title":"Defective fasciculata zone function as the mechanism of glucocorticoid-remediable aldosteronism.","volume":"71","author":"Ulick","year":"1990","journal-title":"J Clin Endocrinol Metab"}],"container-title":["The Journal of Clinical Endocrinology &amp; Metabolism"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/jcem\/article-pdf\/82\/11\/3570\/13134606\/jcem3570.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/jcem\/article-pdf\/82\/11\/3570\/13134606\/jcem3570.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,7,17]],"date-time":"2020-07-17T03:57:59Z","timestamp":1594958279000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcem\/article\/82\/11\/3570\/2865930"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1997,11,1]]},"references-count":26,"journal-issue":{"issue":"11","published-print":{"date-parts":[[1997,11,1]]}},"URL":"https:\/\/doi.org\/10.1210\/jcem.82.11.4381","relation":{},"ISSN":["0021-972X","1945-7197"],"issn-type":[{"value":"0021-972X","type":"print"},{"value":"1945-7197","type":"electronic"}],"subject":[],"published":{"date-parts":[[1997,11,1]]}}}