{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T12:45:00Z","timestamp":1791895500243,"version":"4.3.4"},"reference-count":38,"publisher":"Walter de Gruyter GmbH","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,2,23]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2021-0545_abs_001\">\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Primary adrenal insufficiency (PAI) in children is an uncommon condition. Congenital adrenal hyperplasia (CAH) is the commonest cause followed by autoimmune disorders. Diagnosis and management are challenging especially in resource-limited settings. Studies from Africa are scanty and here we describe for the first time the clinical presentation, possible etiologies, and challenges in diagnosis and management of PAI in a large cohort of Sudanese children.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0545_abs_002\">\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>This was a descriptive hospital-based study where all patients diagnosed with PAI between 2006 and 2020 were reviewed. The diagnosis was based on clinical presentation, low morning cortisol\u00a0\u00b1\u00a0high adrenocorticotropic hormone (ACTH), or inadequate response of cortisol to synacthen stimulation. Challenges faced in diagnosis and management were identified.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0545_abs_003\">\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>From 422 PAI suspected patients, 309 (73.2%) had CAH, and 33 (7.8%) had PAI-like symptoms and were not furtherly discussed. Eighty patients (19%) had fulfilled the study criteria: 29 had Allgrove syndrome, nine auto-immune polyendocrinopathy syndrome, seven adrenoleukodystrophy, and one had an adrenal hemorrhage. Hyperpigmentation was the cardinal feature in 75 (93.8%) while the adrenal crisis was not uncommon. Lack of diagnostic facilities has obscured the etiology in 34 (42.5%) patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2021-0545_abs_004\">\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>PAI is not uncommon in Sudanese children where genetic causes outweigh the autoimmune ones. Many cases were missed due to nonspecific presentation, lack of awareness, and difficult access to tertiary health care facilities. In addition to the clinical findings, early morning cortisol\u00a0\u00b1\u00a0ACTH levels can be used in diagnosis where facilities are limited particularly synacthen stimulation test.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2021-0545","type":"journal-article","created":{"date-parts":[[2021,10,15]],"date-time":"2021-10-15T20:50:07Z","timestamp":1634331007000},"page":"231-237","source":"Crossref","is-referenced-by-count":8,"title":["Clinical profile, etiology, and diagnostic challenges of primary adrenal insufficiency in Sudanese children: 14-years\u2019 experience from a resource limited setting"],"prefix":"10.1515","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0868-0915","authenticated-orcid":false,"given":"Salwa A.","family":"Musa","sequence":"first","affiliation":[{"name":"Pediatric Endocrinology Unit , Gaafar Ibn Auf Children Hospital (GIA) , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9741-3383","authenticated-orcid":false,"given":"Samar S.","family":"Hassan","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology Unit , Gaafar Ibn Auf Children Hospital (GIA) , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amna I.","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology Unit , Gaafar Ibn Auf Children Hospital (GIA) , Khartoum , Sudan"},{"name":"Department of Pediatrics and Child Health, Faculty of Medicine , University of Khartoum , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Ngwiri","sequence":"additional","affiliation":[{"name":"Gertrude\u2019s Children Hospital , Nairobi , Kenya"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0823-1682","authenticated-orcid":false,"given":"Ghassan F.","family":"Fadlalbari","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology Unit , Gaafar Ibn Auf Children Hospital (GIA) , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Areej A.","family":"Ibrahim","sequence":"additional","affiliation":[{"name":"Sudan Childhood Diabetes Center , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omer O.","family":"Babiker","sequence":"additional","affiliation":[{"name":"Sudan Childhood Diabetes Center , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7121-8216","authenticated-orcid":false,"given":"Mohamed A.","family":"Abdullah","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology Unit , Gaafar Ibn Auf Children Hospital (GIA) , Khartoum , Sudan"},{"name":"Department of Pediatrics and Child Health, Faculty of Medicine , University of Khartoum , Khartoum , Sudan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2021,10,15]]},"reference":[{"key":"2022020803263082333_j_jpem-2021-0545_ref_001","unstructured":"Addison, T. 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