{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T23:04:14Z","timestamp":1790377454734,"version":"4.1.0"},"reference-count":27,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100012306","name":"Universit\u00e0 degli Studi di Trieste","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100012306","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Endocrine"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>The aim of this study was to evaluate current real-world practices for the prevention and management of adrenal insufficiency (AI) among Italian physicians caring for children and adolescents with Duchenne muscular dystrophy (DMD) receiving chronic glucocorticoid (GC) therapy, a treatment known to improve motor and cardiopulmonary outcomes but to suppress the hypothalamic\u2013pituitary\u2013adrenal axis and increase the risk of adrenal crisis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A cross-sectional online survey was distributed via national networks to Italian pediatric endocrinologists and neuromuscular specialists. The questionnaire explored clinician characteristics, patient caseloads, glucocorticoid regimens, stress-dose recommendations for mild, moderate, and severe illness, and proactive measures such as family education, emergency kits, written plans, medical alert identification, and electronic hospital alerts. Participation was anonymous.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Thirty-five clinicians responded (57% pediatric endocrinologists, 43% neuromuscular specialists), most following 11\u201350 patients. Deflazacort was the predominant treatment (86%). For mild stress, 80% advised no additional GC. For moderate stress, 43% recommended no change to management, 31% provided oral hydrocortisone (HC), 17% advised extra daily GC, and 9% prescribed intramuscular HC. For severe stress, although 77% prescribed parenteral HC, 14% advised no change. Proactive measures were inconsistently implemented: 82% provided family education, 43% gave intramuscular HC prescriptions and training, 71% had written emergency plans, and 31% recommended medical alert identification.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This national survey reveals substantial variability and gaps in AI prevention and management for children and adolescents with DMD on chronic GC therapy in Italy. Harmonized national protocols, multidisciplinary coordination, and strengthened family education are needed to improve adrenal crisis prevention.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s12020-026-04596-6","type":"journal-article","created":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T13:04:20Z","timestamp":1775048660000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Survey of Adrenal Insufficiency Management for Duchenne muscular dystrophy in Italy"],"prefix":"10.1007","volume":"91","author":[{"given":"Gianluca","family":"Tornese","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sze Choong","family":"Wong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne Marie","family":"Sbrocchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernanda","family":"De Angelis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Zito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R.","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tommaso","family":"Aversa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,1]]},"reference":[{"issue":"3","key":"4596_CR1","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1016\/S1474-4422(18)30024-3","volume":"17","author":"DJ Birnkrant","year":"2018","unstructured":"D.J. 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GT has received speaker honoraria from Novo Nordisk, and Pfizer. DRW has been a consultant for PTC therapeutics, Catalyst, and Santhera. SCW has been a consultant for Santhera, Roche, and Novartis with funding directed to the institution, and has received speaker honoraria from Nutricia, Sandoz, Novo Nordisk, and Roche with funding directed to the institution.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}],"article-number":"125"}}