{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:16:12Z","timestamp":1784556972182,"version":"3.55.0"},"reference-count":0,"publisher":"Bioscientifica","issue":"8","license":[{"start":{"date-parts":[[2017,11,1]],"date-time":"2017-11-01T00:00:00Z","timestamp":1509494400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["ec.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>To characterize the clinical presentation, diagnostic workup, treatment and follow\u2010up of Portuguese patients with confirmed PAI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This multicentre retrospective study examined PAI patients in 12 Portuguese hospitals.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6\u2009\u00b1\u200919.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3\u2009\u00b1\u20098.3\u2009mg\/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100\u2009\u00b5g\/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment, 17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1530\/ec-17-0295","type":"journal-article","created":{"date-parts":[[2017,10,31]],"date-time":"2017-10-31T20:25:37Z","timestamp":1509481537000},"page":"935-942","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-10","source":"Crossref","is-referenced-by-count":6,"title":["Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group"],"prefix":"10.1530","volume":"6","author":[{"given":"Lia","family":"Ferreira","sequence":"first","affiliation":[{"name":"1Department of Endocrinology, Centro Hospitalar do Porto, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o","family":"Silva","sequence":"additional","affiliation":[{"name":"2Department of Endocrinology, Hospital das For\u00e7as Armadas, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana","family":"Garrido","sequence":"additional","affiliation":[{"name":"3Department of Endocrinology, Centro Hospitalar T\u00e2mega e Sousa, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Bello","sequence":"additional","affiliation":[{"name":"4Department of Endocrinology, Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Oliveira","sequence":"additional","affiliation":[{"name":"5Department of Endocrinology, Centro Hospitalar e Universit\u00e1rio de Coimbra, Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9lder","family":"Sim\u00f5es","sequence":"additional","affiliation":[{"name":"6Department of Endocrinology, Instituto Portugu\u00eas de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabel","family":"Paiva","sequence":"additional","affiliation":[{"name":"5Department of Endocrinology, Centro Hospitalar e Universit\u00e1rio de Coimbra, Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joana","family":"Guimar\u00e3es","sequence":"additional","affiliation":[{"name":"7Department of Endocrinology, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Ferreira","sequence":"additional","affiliation":[{"name":"8Department of Endocrinology, Centro Hospitalar de Leiria, Leiria, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Pereira","sequence":"additional","affiliation":[{"name":"8Department of Endocrinology, Centro Hospitalar de Leiria, Leiria, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Bettencourt-Silva","sequence":"additional","affiliation":[{"name":"9Department of Endocrinology, Centro Hospitalar de S\u00e3o Jo\u00e3o, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Filipa","family":"Martins","sequence":"additional","affiliation":[{"name":"10Department of Endocrinology, Centro Hospitalar Lisboa Norte, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiago","family":"Silva","sequence":"additional","affiliation":[{"name":"11Department of Endocrinology, Hospital Garcia da Orta, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vera","family":"Fernandes","sequence":"additional","affiliation":[{"name":"12Department of Endocrinology, Hospital de Braga, Braga, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Lopes","family":"Pereira","sequence":"additional","affiliation":[{"name":"12Department of Endocrinology, Hospital de Braga, Braga, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"_","family":"_","sequence":"additional","affiliation":[{"name":"1Department of Endocrinology, Centro Hospitalar do Porto, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","container-title":["Endocrine Connections"],"original-title":[],"link":[{"URL":"https:\/\/ec.bioscientifica.com\/view\/journals\/ec\/6\/8\/EC-17-0295.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/ec.bioscientifica.com\/downloadpdf\/journals\/ec\/6\/8\/EC-17-0295.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:42:32Z","timestamp":1784554952000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/ec\/article\/6\/8\/935-942\/35323"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,11]]},"references-count":0,"journal-issue":{"issue":"8"},"URL":"https:\/\/doi.org\/10.1530\/ec-17-0295","relation":{},"ISSN":["2049-3614"],"issn-type":[{"value":"2049-3614","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,11]]}}}