{"created":"2023-05-15T16:30:21.801847+00:00","id":1460,"links":{},"metadata":{"_buckets":{"deposit":"ed11599a-7fd6-4d92-980a-e5ee3bf4fa89"},"_deposit":{"created_by":2,"id":"1460","owners":[2],"pid":{"revision_id":0,"type":"depid","value":"1460"},"status":"published"},"_oai":{"id":"oai:nagasaki-u.repo.nii.ac.jp:00001460","sets":["10:11"]},"author_link":["6942","6952","6954","6938","6951","6949","6958","6959","6944","6939","6947","6950","6953","6957","6940","6948","6956","6943","6946","6955","6960","6941","6945"],"item_2_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2018-11-20","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageStart":"257","bibliographicVolumeNumber":"20","bibliographic_titles":[{"bibliographic_title":"Arthritis Research & Therapy"}]}]},"item_2_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"BACKGROUND: We showed previously that Japanese individuals with familial Mediterranean fever (FMF) have a more atypical phenotype compared to endemic areas. The clinical differences between young-onset FMF (YOFMF), adult-onset FMF(AOFMF), and late-onset FMF(LOFMF)in Japan are unclear. METHODS: We enrolled 395 consecutive patients. We defined YOFMF, AOFMF, and LOFMF as the onset of FMF at<20, 20-39, and??40years of age, respectively. We compared clinical manifestations and MEFV mutations patterns among these groups. RESULTS: Median ages at onset were YOFMF 12.5 years (n?=?182), AOFMF 28 years (n?=?115), and LOFMF 51years (n?=?90). A family history, MEFV mutations in exon 10, and more than two MEFV mutations were significantly more frequent in the earlier-onset groups (p?