{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T13:53:39Z","timestamp":1753883619054,"version":"3.41.2"},"reference-count":15,"publisher":"The Endocrine Society","issue":"12","license":[{"start":{"date-parts":[[2021,9,23]],"date-time":"2021-09-23T00:00:00Z","timestamp":1632355200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Type 1 autoimmune polyglandular syndrome (APS1) is a rare hereditary disease affecting nearly 600 patients worldwide. The first of its cardinal manifestations, chronic mucocutaneous candidiasis, hypoparathyroidism, or Addison\u2019s disease, presents in childhood. Additional nonclassical landmarks of APS1 continue to develop as late as the fifth decade of life. Two thirds of patients develop the full triad before 25 years of age. Only 20% of patients develop the entire triad simultaneously. Addison\u2019s disease is rarely reported as the first manifestation. According to APS1 classifications, restricted criteria for a single cardinal component, although elements of suspicion are not sufficient to diagnose APS1. This case report is peculiar as hypoadrenalism was the first and only manifestation of APS1 for nearly 3 decades since its diagnosis. Theoretically, exceptions from the protocol of APS1 diagnostic criteria would be recognized as acceptable for diagnosis in the future, when similar case reports of only 1 component of APS1 appear.<\/jats:p>","DOI":"10.1210\/jendso\/bvab151","type":"journal-article","created":{"date-parts":[[2021,9,23]],"date-time":"2021-09-23T17:25:39Z","timestamp":1632417939000},"source":"Crossref","is-referenced-by-count":0,"title":["Hypoadrenalism as the Single Presentation of Autoimmune Polyglandular Syndrome Type 1"],"prefix":"10.1210","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2249-8543","authenticated-orcid":false,"given":"Nadim H","family":"Nasser","sequence":"first","affiliation":[{"name":"Clalit Health Organization, Haifa, Israel"},{"name":"Bar-Ilan University Faculty of Medicine,\u202fSafed, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2593-8244","authenticated-orcid":false,"given":"Nadra G","family":"Samra","sequence":"additional","affiliation":[{"name":"Genetic Unit. Ziv Health Centre, Safed, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8016-320X","authenticated-orcid":false,"given":"Deeb D","family":"Naccache","sequence":"additional","affiliation":[{"name":"Institute of Endocrinology and Centre for Excellence in Diabetes and Obesity, Rambam Health Care Campus, Haifa, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2021,9,23]]},"reference":[{"key":"2021120512260000900_CIT0001","first-page":"212","article-title":"Eine biglandulare Erkrankung (Nebennieren und Schilddruse bei Morbus Addisonni)","volume":"21","author":"Schmidt","year":"1926","journal-title":"Dtsch Pathol Ges"},{"key":"2021120512260000900_CIT0002","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1210\/jcem-6-7-493","article-title":"Chronic idiopathic hypoparathyroidism with superimposed Addison\u2019s disease in a child","volume":"6","author":"Leonard","year":"1946","journal-title":"J Clin Endocrinol Metab."},{"issue":"5","key":"2021120512260000900_CIT0003","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1097\/00005792-198109000-00003","article-title":"Two types of autoimmune Addison\u02bcs disease associated with different polyglandular autoimmune (PGA) syndromes","volume":"60","author":"Neufeld","year":"1981","journal-title":"Medicine."},{"key":"2021120512260000900_CIT0004","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.3389\/fimmu.2018.01835","article-title":"A novel homozygous mutation of the AIRE gene in an APECED patient from pakistan: case report and review of the literature","volume":"9","author":"Pellegrino","year":"2018","journal-title":"Front Immunol."},{"issue":"10","key":"2021120512260000900_CIT0005","doi-asserted-by":"crossref","first-page":"1374","DOI":"10.1210\/jcem-16-10-1374","article-title":"The syndrome of familial juvenile hypoadrenocorticism, hypoparathyroidism and superficial moniliasis","volume":"16","author":"Whitaker","year":"1956","journal-title":"J Clin Endocrinol Metab."},{"issue":"7","key":"2021120512260000900_CIT0006","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3390\/ijms17071106","article-title":"Type 1 diabetes in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED): a \u201crare\u201d manifestation in a \u201crare\u201d disease","volume":"17","author":"Fierabracci","year":"2016","journal-title":"Int J Mol Sci."},{"issue":"12","key":"2021120512260000900_CIT0007","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1056\/NEJMra1713301","article-title":"Autoimmune polyendocrine syndromes","volume":"378","author":"Husebye","year":"2018","journal-title":"N Engl J Med."},{"issue":"3","key":"2021120512260000900_CIT0008","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1080\/20009666.2019.1616523","article-title":"Autoimmune polyglandular syndrome type 1: a case report and brief review","volume":"9","author":"Nwosu","year":"2019","journal-title":"J Community Hosp Intern Med Perspect."},{"issue":"8","key":"2021120512260000900_CIT0009","doi-asserted-by":"crossref","first-page":"2975","DOI":"10.1210\/jc.2016-1821","article-title":"A longitudinal follow-up of autoimmune polyendocrine syndrome type 1","volume":"101","author":"Bruserud","year":"2016","journal-title":"J Clin Endocrinol Metab."},{"issue":"13","key":"2021120512260000900_CIT0010","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1172\/jci.insight.88782","article-title":"Redefined clinical features and diagnostic criteria in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy","volume":"1","author":"Ferre","year":"2016","journal-title":"JCI Insight."},{"issue":"2","key":"2021120512260000900_CIT0011","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1210\/jc.2015-1710","article-title":"Diagnosis and treatment of primary adrenal insufficiency: an endocrine society clinical practice guideline","volume":"101","author":"Bornstein","year":"2016","journal-title":"J Clin Endocrinol Metab."},{"issue":"6","key":"2021120512260000900_CIT0012","doi-asserted-by":"crossref","first-page":"915","DOI":"10.1016\/0002-9343(79)90445-5","article-title":"Renin and renin substrate in primary adrenal insufficiency: contrasting effects of glucocorticoid and mineralocorticoid deficiency","volume":"66","author":"Stockigt","year":"1979","journal-title":"Am J Med."},{"key":"2021120512260000900_CIT0013","doi-asserted-by":"crossref","first-page":"606860","DOI":"10.3389\/fimmu.2021.606860","article-title":"Autoimmune Addison\u2019s disease as part of the autoimmune polyglandular syndrome type 1: historical overview and current evidence","volume":"12","author":"Perniola","year":"2021","journal-title":"Front Immunol."},{"issue":"4","key":"2021120512260000900_CIT0014","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1210\/jcem.83.4.4682","article-title":"Clinical review 93: autoimmune polyglandular syndrome type 1","volume":"83","author":"Betterle","year":"1998","journal-title":"J Clin Endocrinol Metab."},{"issue":"3","key":"2021120512260000900_CIT0015","doi-asserted-by":"crossref","first-page":"536","DOI":"10.5114\/aoms.2011.23427","article-title":"Hepatitis and the polyglandular autoimmune syndrome, type 1","volume":"7","author":"Bia\u0142kowska","year":"2011","journal-title":"Arch Med Sci."}],"container-title":["Journal of the Endocrine Society"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/jes\/advance-article-pdf\/doi\/10.1210\/jendso\/bvab151\/40433555\/bvab151.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jes\/article-pdf\/5\/12\/bvab151\/41533844\/bvab151.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jes\/article-pdf\/5\/12\/bvab151\/41533844\/bvab151.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,5]],"date-time":"2021-12-05T12:26:20Z","timestamp":1638707180000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jes\/article\/doi\/10.1210\/jendso\/bvab151\/6374539"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9,23]]},"references-count":15,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2021,12,1]]}},"URL":"https:\/\/doi.org\/10.1210\/jendso\/bvab151","relation":{},"ISSN":["2472-1972"],"issn-type":[{"type":"electronic","value":"2472-1972"}],"subject":[],"published-other":{"date-parts":[[2021,12,1]]},"published":{"date-parts":[[2021,9,23]]},"article-number":"bvab151"}}