{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T19:29:00Z","timestamp":1790882940792,"version":"4.1.0"},"reference-count":11,"publisher":"Walter de Gruyter GmbH","issue":"7","funder":[{"name":"Nagasaki Pediatric Alumni Association"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,7,28]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec id=\"j_jpem-2019-0581_abs_001_w2aab3b7c41b1b6b1aab1c16b2Aa\">\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Denosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand, which strongly suppresses osteoclasts. Cherubism is a rare autosomal dominant disorder characterized by symmetrical swelling of the jaws, in which the bone is replaced by a fibrous granuloma containing osteoclast-like giant cells.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2019-0581_abs_002_w2aab3b7c41b1b6b1aab1c16b3Aa\">\n                  <jats:title>Case presentation<\/jats:title>\n                  <jats:p>We report the efficacy and safety of denosumab treatment in a prepubertal boy with progressive cherubism. The treatment consisting of eight subcutaneous denosumab injections (120 mg\/dose) in 6 months not only suppressed the expansion of the osteolytic lesions but also dramatically ossified them. However, a transiently decreased growth rate and rebounded asymptomatic hypercalcemia were associated with the treatment.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec id=\"j_jpem-2019-0581_abs_003_w2aab3b7c41b1b6b1aab1c16b4Aa\">\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>The present case demonstrated the therapeutic potential of denosumab for treatment of cherubism, although adverse effects, especially those on childhood growth, remain obscure. Further studies are needed to establish a safe and effective protocol for denosumab treatment of children.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1515\/jpem-2019-0581","type":"journal-article","created":{"date-parts":[[2020,7,13]],"date-time":"2020-07-13T11:48:58Z","timestamp":1594640938000},"page":"963-966","source":"Crossref","is-referenced-by-count":20,"title":["Efficacy and safety of denosumab treatment in a prepubertal patient with cherubism"],"prefix":"10.1515","volume":"33","author":[{"given":"Haruka","family":"Kawamura","sequence":"first","affiliation":[{"name":"Department of Pediatrics , Nagasaki University Hospital , Nagasaki , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1878-1717","authenticated-orcid":false,"given":"Satoshi","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics , Nagasaki University Hospital , Nagasaki , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"I","sequence":"additional","affiliation":[{"name":"Department of Oral and Maxillofacial Surgery , Nagasaki University Hospital , Nagasaki , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Izumi","family":"Asahina","sequence":"additional","affiliation":[{"name":"Department of Oral and Maxillofacial Surgery , Nagasaki University Hospital , Nagasaki , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Moriuchi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics , Nagasaki University Hospital , Nagasaki , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sumito","family":"Dateki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics , Nagasaki University Hospital , Nagasaki , Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2020,7,9]]},"reference":[{"key":"2023040101122166915_j_jpem-2019-0581_ref_001_w2aab3b7c41b1b6b1ab2b2b1Aa","doi-asserted-by":"crossref","unstructured":"Baron, R, Ferrari, S, Russell, RG. Denosumab and bisphosphonates: different mechanisms of action and effects. Bone 2011; 48: 677\u201392. https:\/\/doi.org\/10.1016\/j.bone.2010.11.020.","DOI":"10.1016\/j.bone.2010.11.020"},{"key":"2023040101122166915_j_jpem-2019-0581_ref_002_w2aab3b7c41b1b6b1ab2b2b2Aa","doi-asserted-by":"crossref","unstructured":"Ueki, Y, Lin, CY, Senoo, M, Ebihara, T, Agata, N, Onji, M, et al. Increased myeloid cell responses to M-CSF and RANKL cause bone loss and inflammation in SH3BP2 \u201ccherubism\u201d mice. Cell 2007; 128: 71\u201383. https:\/\/doi.org\/10.1016\/j.cell.2006.10.047.","DOI":"10.1016\/j.cell.2006.10.047"},{"key":"2023040101122166915_j_jpem-2019-0581_ref_003_w2aab3b7c41b1b6b1ab2b2b3Aa","doi-asserted-by":"crossref","unstructured":"Kittaka, M, Mayahara, K, Mukai, T, Yoshimoto, T, Yoshitaka, T, Gorski, JP, et al. Cherubism mice also deficient in c-Fos exhibit inflammatory bone destruction executed by macrophages that express MMP14 despite the absence of TRAP+ osteoclasts. 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