{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:37:26Z","timestamp":1787020646074,"version":"build-2736575974"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T00:00:00Z","timestamp":1594425600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T00:00:00Z","timestamp":1594425600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>We describe two patients of 11 and 9\u2009years of age with hyperferritinemic syndromes, one with impending macrophage activation syndrome (MAS) and one with overt MAS, who were refractory or intolerant to conventional therapies, but improved dramatically with canakinumab.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our report indicates that canakinumab may be efficacious in the management of hyperferritinemic syndromes, including MAS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s12969-020-00450-9","type":"journal-article","created":{"date-parts":[[2020,7,11]],"date-time":"2020-07-11T01:02:48Z","timestamp":1594429368000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Successful treatment of refractory hyperferritinemic syndromes with canakinumab: a report of two cases"],"prefix":"10.1186","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9626-5470","authenticated-orcid":false,"given":"Riccardo","family":"Papa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Natoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Caorsi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Minoia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Gattorno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelo","family":"Ravelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,7,11]]},"reference":[{"key":"450_CR1","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1093\/intimm\/dxy012","volume":"30","author":"GS Schulert","year":"2018","unstructured":"Schulert GS, Canna SW. Convergent pathways of the hyperferritinemic syndromes. Int Immunol. 2018;30:195\u2013203.","journal-title":"Int Immunol"},{"key":"450_CR2","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1146\/annurev-pathol-020117-043625","volume":"13","author":"H Al-Samkari","year":"2018","unstructured":"Al-Samkari H, Berliner N. Hemophagocytic Lymphohistiocytosis. Annu Rev Pathol. 2018;13:27\u201349.","journal-title":"Annu Rev Pathol"},{"key":"450_CR3","doi-asserted-by":"publisher","first-page":"927","DOI":"10.1016\/j.hoc.2015.06.010","volume":"29","author":"A Ravelli","year":"2015","unstructured":"Ravelli A, Dav\u00ec S, Minoia F, Martini A, Cron RQ. Macrophage activation syndrome. Hematol Oncol Clin North Am. 2015;29:927\u201341.","journal-title":"Hematol Oncol Clin North Am"},{"key":"450_CR4","doi-asserted-by":"publisher","first-page":"119","DOI":"10.3389\/fimmu.2019.00119","volume":"10","author":"CB Crayne","year":"2019","unstructured":"Crayne CB, Albeituni S, Nichols KE, Cron RQ. 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AR has received grant support and\/or speaking or consultant fees from AbbVie, Bristol-Myers Squibb, Novartis, Pfizer, Roche and Johnson & Johnson. The other authors do not have conflict of interests to declare.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"56"}}