{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T03:45:29Z","timestamp":1792899929163,"version":"4.5.0"},"reference-count":64,"publisher":"Oxford University Press (OUP)","issue":"3","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,3]]},"abstract":"\nObjective<\/jats:title>\nTriple-A or Allgrove syndrome is an autosomal recessive disorder due to mutations in the AAAS<\/jats:italic> gene, which encodes a nucleoporin named ALADIN. It is characterized by a classical clinical triad: alacrima, achalasia and adrenal insufficiency, the canonic symptoms that are associated with progressive peripheral neuropathy. Only a few cohorts have been reported. The objective of the present study was to characterize the various spectra of adrenal function in Triple-A patients.<\/jats:p>\n<\/jats:sec>\n\nMethods<\/jats:title>\nA retrospective clinical and biological monitoring of 14 patients (10 families) was done in a single multidisciplinary French center. All had AAAS<\/jats:italic> gene sequenced and adrenal function evaluation.<\/jats:p>\n<\/jats:sec>\n\nResults<\/jats:title>\nNine different AAAS<\/jats:italic> mutations were found, including one new mutation: c.755G>C, p.(Trp252Ser). Regarding adrenal function, defects of the zona fasciculata and reticularis were demonstrated by increased basal ACTH levels and low DHEAS levels in all cases regardless of the degree of glucocorticoid deficiency. In contrast, mineralocorticoid function was always conserved: i.e., normal plasma renin level associated with normal aldosterone level. The main prognostic feature was exacerbation of neuropathy and cognitive disorders.<\/jats:p>\n<\/jats:sec>\n\nConclusions<\/jats:title>\nThese data suggest that, in Triple-A patients, adrenal function can be deficient, insufficient or compensated. In our cohort after the first decade of life, there does not appear to be any degradation of adrenal function over time. However, patients with compensated adrenal function should be informed and educated to manage a glucocorticoid replacement therapy in case of stressful conditions, with no need for systematic long-term treatment.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-17-0642","type":"journal-article","created":{"date-parts":[[2017,12,14]],"date-time":"2017-12-14T01:25:42Z","timestamp":1513214742000},"page":"199-207","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":55,"title":["Triple-A syndrome: a wide spectrum of adrenal dysfunction"],"prefix":"10.1093","volume":"178","author":[{"given":"Florence","family":"Roucher-Boulez","sequence":"first","affiliation":[{"name":"1Laboratoire de Biochimie et Biologie Mol\u00e9culaire Grand Est, UM Pathologies Endocriniennes R\u00e9nales Musculaires et Mucoviscidose, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France"},{"name":"2Univ Lyon, Universit\u00e9 Claude Bernard Lyon 1, Lyon, France"},{"name":"3Centre de R\u00e9f\u00e9rence du D\u00e9veloppement G\u00e9nital: du F\u0153tus \u00e0 l\u2019Adulte, Fili\u00e8re Maladies Rares Endocriniennes, Bron, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aude","family":"Brac de la Perriere","sequence":"additional","affiliation":[{"name":"3Centre de R\u00e9f\u00e9rence du D\u00e9veloppement G\u00e9nital: du F\u0153tus \u00e0 l\u2019Adulte, Fili\u00e8re Maladies Rares Endocriniennes, Bron, France"},{"name":"4F\u00e9d\u00e9ration d\u2019Endocrinologie, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aude","family":"Jacquez","sequence":"additional","affiliation":[{"name":"2Univ Lyon, Universit\u00e9 Claude Bernard Lyon 1, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Chau","sequence":"additional","affiliation":[{"name":"2Univ Lyon, Universit\u00e9 Claude Bernard Lyon 1, Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence","family":"Guignat","sequence":"additional","affiliation":[{"name":"5Service d\u2019Endocrinologie, Centre de R\u00e9f\u00e9rence des Maladies Surr\u00e9naliennes Rares, H\u00f4pital Cochin, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe","family":"Vial","sequence":"additional","affiliation":[{"name":"6Service d\u2019Electroneuromyographie et Pathologies Neuromusculaires, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Morel","sequence":"additional","affiliation":[{"name":"1Laboratoire de Biochimie et Biologie Mol\u00e9culaire Grand Est, UM Pathologies Endocriniennes R\u00e9nales Musculaires et Mucoviscidose, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France"},{"name":"2Univ Lyon, Universit\u00e9 Claude Bernard Lyon 1, Lyon, France"},{"name":"3Centre de R\u00e9f\u00e9rence du D\u00e9veloppement G\u00e9nital: du F\u0153tus \u00e0 l\u2019Adulte, Fili\u00e8re Maladies Rares Endocriniennes, Bron, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Nicolino","sequence":"additional","affiliation":[{"name":"2Univ Lyon, Universit\u00e9 Claude Bernard Lyon 1, Lyon, France"},{"name":"3Centre de R\u00e9f\u00e9rence du D\u00e9veloppement G\u00e9nital: du F\u0153tus \u00e0 l\u2019Adulte, Fili\u00e8re Maladies Rares Endocriniennes, Bron, France"},{"name":"7Service de P\u00e9diatrie Endocrinologie, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald","family":"Raverot","sequence":"additional","affiliation":[{"name":"2Univ Lyon, Universit\u00e9 Claude Bernard Lyon 1, Lyon, France"},{"name":"4F\u00e9d\u00e9ration d\u2019Endocrinologie, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, 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