{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T04:50:52Z","timestamp":1747198252861,"version":"3.40.5"},"reference-count":23,"publisher":"Walter de Gruyter GmbH","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,5,25]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2021-0725_abs_001\">\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Adrenal insufficiency (AI) is a life-threatening condition caused by an impaired secretion of the adrenal glucocorticoid and mineralocorticoid hormones. It comprises a heterogeneous group of primary, secondary and acquired disorders. Presentation differs according to the child\u2019s age, but it usually presents with nonspecific and insidious symptoms and signs. The main purpose of this study was to describe and compare patients with primary or secondary AI.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0725_abs_002\">\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Retrospective analysis of all patients with adrenal insufficiency followed at the Pediatric Endocrinology Unit in a tertiary care Portuguese hospital over the last 30 years. Data on family history, age at the first manifestation and at etiological diagnosis, and clinical presentation (symptoms, signs and laboratory evaluation) was gathered for all patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0725_abs_003\">\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Twenty-eight patients with AI were included; 67.9% were male, with a median (25th\u201375th percentile, P25\u2013P75) age of 1 (0.5\u201336) month at the first presentation. The principal diagnostic categories were panhypopituitarism (42.9%) and congenital adrenal hyperplasia (25%). The most frequent manifestations (75%) were vomiting and weight loss. They were followed for a median (P25\u2013P75) period of 3.5 (0.6\u201315.5) years. In respect to neurodevelopmental delay and learning difficulties, they were more common in the secondary AI group.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0725_abs_004\">\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Despite medical advances, the diagnosis and management of AI remains a challenge, particularly in the pediatric population, and clinicians must have a high index of suspicion. An early identification of AI can prevent a potential lethal outcome, which may result from severe cardiovascular and hemodynamic instability.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2021-0725","type":"journal-article","created":{"date-parts":[[2022,3,31]],"date-time":"2022-03-31T10:22:20Z","timestamp":1648722140000},"page":"631-638","source":"Crossref","is-referenced-by-count":1,"title":["Pediatric adrenal insufficiency: thirty years experience at a Portuguese hospital"],"prefix":"10.1515","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5474-7236","authenticated-orcid":false,"given":"Sara M. F. S.","family":"Mosca","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universit\u00e1rio do Porto (CMIN-CHUPorto) , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8729-2787","authenticated-orcid":false,"given":"Tiago S.","family":"Santos","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes and Metabolism , CHUPorto , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana C. B.","family":"Mendes","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universit\u00e1rio do Porto (CMIN-CHUPorto) , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2768-9819","authenticated-orcid":false,"given":"Lu\u00eds F. M.","family":"Ribeiro","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universit\u00e1rio do Porto (CMIN-CHUPorto) , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joana M. C.","family":"Freitas","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universit\u00e1rio do Porto (CMIN-CHUPorto) , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0661-8947","authenticated-orcid":false,"given":"Maria J. R.","family":"Oliveira","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universit\u00e1rio do Porto (CMIN-CHUPorto) , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla A.","family":"Rocha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Endocrinology Unit , Centro Materno-Infantil do Norte, Centro Hospitalar Universit\u00e1rio do Porto (CMIN-CHUPorto) , Porto , Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8501-6639","authenticated-orcid":false,"given":"Teresa M. 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