{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T03:18:43Z","timestamp":1764645523266},"reference-count":10,"publisher":"Walter de Gruyter GmbH","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,8,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2020-0036_abs_001\">\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>To present a case report of succesfully metyrapone treatment of a neonatal patient with McCune\u2013Albrigth syndrome (MAS), a rare disease caused by a genetically mosaic disorder and is characterized by variable hyperfunctional endocrinopathies, bone dysplasia, and caf\u00e9-au-lait spots.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2020-0036_abs_002\">\n                  <jats:title>Case presentation<\/jats:title>\n                  <jats:p>A preterm newborn was admitted to hospital and she presented difficulty controlling hypertension, caf\u00e9-au-lait spots, and failure to thrive. An abdominal ultrasound and a magnetic resonance showed a high volume of both suprarenal glands. Therefore, MAS was suspected. Laboratory data confirmed adrenocorticotropic hormone-independent Cushing\u2019s syndrome with hepatic dysfunction and metyrapone treatment was initiated. A progressive normalization of cortisol levels was achieved despite poor oral tolerance.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2020-0036_abs_003\">\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Our case shows that metyrapone is useful in the management of neonatal Cushing\u2019s syndrome due to McCune\u2013Albright syndrome.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2020-0036","type":"journal-article","created":{"date-parts":[[2020,7,28]],"date-time":"2020-07-28T10:21:33Z","timestamp":1595931693000},"page":"1093-1096","source":"Crossref","is-referenced-by-count":6,"title":["Metyrapone as treatment in the neonatal McCune\u2013Albright syndrome"],"prefix":"10.1515","volume":"33","author":[{"given":"Carmen","family":"de Mingo","sequence":"first","affiliation":[{"name":"Division of Pediatric Endocrinology , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda","family":"Brugada","sequence":"additional","affiliation":[{"name":"Neonatal Research Group , Health Research Institute La Fe, University and Polytechnic Hospital La Fe , Valencia , Spain"},{"name":"Division of Neonatology , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Le\u00f3n","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francisca","family":"Moreno","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nieves","family":"Vila","sequence":"additional","affiliation":[{"name":"Division of Pharmacy , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tom\u00e1s","family":"Palanques-Pastor","sequence":"additional","affiliation":[{"name":"Division of Pharmacy , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 Luis","family":"Poveda","sequence":"additional","affiliation":[{"name":"Division of Pharmacy , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos","family":"Orti","sequence":"additional","affiliation":[{"name":"Division of Neonatology , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana","family":"Garc\u00eda-Robles","sequence":"additional","affiliation":[{"name":"Neonatal Research Group , Health Research Institute La Fe, University and Polytechnic Hospital La Fe , Valencia , Spain"},{"name":"Division of Neonatology , University and Polytechnic Hospital La Fe , Valencia , Spain"},{"name":"Division of Pharmacy , University and Polytechnic Hospital La Fe , Valencia , Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"374","published-online":{"date-parts":[[2020,7,22]]},"reference":[{"key":"2021082202005793232_j_jpem-2020-0036_ref_001","doi-asserted-by":"crossref","unstructured":"Verbeeten, K, Hadjiyannakis, S, Cameron, M, McDonald, J. Rectal metyrapone for treatment of hypercortisolism in an infant with McCune-Albright syndrome. J Pediatr Pharmacol Ther 2017; 22: 233\u20136. https:\/\/doi.org\/10.5863\/1551-6776-22.3.233.","DOI":"10.5863\/1551-6776-22.3.233"},{"key":"2021082202005793232_j_jpem-2020-0036_ref_002","doi-asserted-by":"crossref","unstructured":"Louren\u00e7o, R, Dias, P, Gouveia, R, Sousa, AB, Oliveira, G. Neonatal McCune-Albright syndrome with systemic involvement: a case report. J Med Case Rep 2015; 9: 189. https:\/\/doi.org\/10.1186\/s13256-015-0689-2.","DOI":"10.1186\/s13256-015-0689-2"},{"key":"2021082202005793232_j_jpem-2020-0036_ref_003","doi-asserted-by":"crossref","unstructured":"Dumitrescu, CE, Collins, MT. McCune-Albright syndrome. Orphanet J Rare Dis 2008; 3: 12. https:\/\/doi.org\/10.1186\/1750-1172-3-12.","DOI":"10.1186\/1750-1172-3-12"},{"key":"2021082202005793232_j_jpem-2020-0036_ref_004","doi-asserted-by":"crossref","unstructured":"Greaves, RF. Hormone profiles in extremely preterm infants. Clin Biochem 2014; 47: 744\u20136. https:\/\/doi.org\/10.1016\/j.clinbiochem.2014.05.040.","DOI":"10.1016\/j.clinbiochem.2014.05.040"},{"key":"2021082202005793232_j_jpem-2020-0036_ref_005","doi-asserted-by":"crossref","unstructured":"Brown, RJ, Kelly, MH, Collins, MT. Cushing syndrome in the McCune-Albright syndrome. J Clin Endocrinol Metab 2010; 95: 1508\u201315. https:\/\/doi.org\/10.1210\/jc.2009-2321.","DOI":"10.1210\/jc.2009-2321"},{"key":"2021082202005793232_j_jpem-2020-0036_ref_006","doi-asserted-by":"crossref","unstructured":"Gillis, D, R\u00f6sler, A, Hannon, TS, Koplewitz, BZ, Hirsch, HJ. Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome. 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