{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T11:49:03Z","timestamp":1778932143762,"version":"3.51.4"},"reference-count":28,"publisher":"Walter de Gruyter GmbH","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,7,26]]},"abstract":"<jats:title>Abstract<\/jats:title>\n<jats:sec id=\"j_jpem-2019-0030_s_999\">\n<jats:title id=\"d3356547e53128\">Background<\/jats:title>\n<jats:p>Adrenal insufficiency (AI) is a life-threatening disease characterized by deficient production of glucocorticoids and\/or mineralocorticoids. It is caused by primary or secondary\/tertiary adrenal failure. Prompt diagnosis and management are essential and may even be life-saving.<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2019-0030_s_998\">\n<jats:title id=\"d3356547e53136\">Methods<\/jats:title>\n<jats:p>We retrospectively collected clinical, laboratory and radiological data from AI patients observed over 34 years (1984\u20132017) in a pediatric endocrinology department of a tertiary care hospital.<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2019-0030_s_997\">\n<jats:title id=\"d3356547e53144\">Results<\/jats:title>\n<jats:p>Seventy AI patients were identified: 59% with primary adrenal insufficiency (PAI) and 41% with central adrenal insufficiency (CAI). PAI patients were diagnosed at 1.5\u2009\u00b1\u20094.4 years and followed for 11.6\u2009\u00b1\u20096.2 years; 85% had classical congenital adrenal hyperplasia (CAH) and 7% had autoimmune PAI. At presentation, 73% had hyponatremia and more than half had mucocutaneous hyperpigmentation, asthenia, anorexia, weight loss, nausea and vomiting. All the patients were treated with hydrocortisone and 90% were also on fludrocortisone. Regarding CAI patients, they were diagnosed at 5.4\u2009\u00b1\u20095.0 years and they were followed for 9.6\u2009\u00b1\u20096.4 years; craniopharyngioma was present in 31% of the cases and 14% had pituitary hypoplasia. Besides corticotropin, thyrotropin (93%), growth hormone (63%) and antidiuretic hormone (52%) were the most common hormone insufficiencies. The most frequent manifestations were hypoglycemia (34.5%), nausea\/vomiting (27.6%) and infectious diseases (27.6%); all the patients were treated with hydrocortisone.<\/jats:p><\/jats:sec>\n<jats:sec id=\"j_jpem-2019-0030_s_996\">\n<jats:title id=\"d3356547e53152\">Conclusions<\/jats:title>\n<jats:p>Despite medical advances, the diagnosis and management of AI remains a challenge, particularly in the pediatric population. Raising awareness and knowledge in medical teams and population about the disease is of crucial importance to improve clinical outcomes and to reduce disease morbidity\/mortality.<\/jats:p><\/jats:sec>","DOI":"10.1515\/jpem-2019-0030","type":"journal-article","created":{"date-parts":[[2019,6,13]],"date-time":"2019-06-13T09:09:32Z","timestamp":1560416972000},"page":"721-726","source":"Crossref","is-referenced-by-count":19,"title":["The spectrum of pediatric adrenal insufficiency: insights from 34 years of experience"],"prefix":"10.1515","volume":"32","author":[{"given":"Mara","family":"Ventura","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joana","family":"Serra-Caetano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita","family":"Cardoso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabel","family":"Dinis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miguel","family":"Melo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francisco","family":"Carrilho","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alice","family":"Mirante","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","reference":[{"key":"ref131","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1016\/j.jped.2013.09.007","article-title":"A 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