フルテキストファイル
著者
Ehara, Yuko Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine,Tottori University
Yamamoto, Osamu Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University 研究者総覧 KAKEN
Kosaki, Kenjiro Center for Medical Genetics, Keio University School of Medicine
Yoshida, Yuichi Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University 研究者総覧 KAKEN
キーワード
clinical severity
diffuse plexiform neurofibromas
DNB classification
neurofibromatosis 1
quality of life
抄録
Neurofibromatosis 1 (NF1) is a genetic disease characterized by cutaneous, neurological and osseous complications. Although clinical manifestations of NF1 are variable, there has been no report on evaluation of severity in patients with NF1. To elucidate the grade of severity of NF1, a retrospective study was conducted in 124 NF1 patients at the Department of Dermatology of Tottori University Hospital in 2007–2016. The DNB classification (dermatological, neurological and bone manifestations) in Japan was used for assessment. Based on our current epidemiological data, there were 55 patients (44.3%) in stage 1, 23 (18.6%) in stage 2, three (2.4%) in stage 3, one (0.8%) in stage 4 and 42 (33.9%) in stage 5. The grade of severity in patients with NF1 tended to be higher with aging. Remarkably, 61.8% of the patients in stage 5 had diffuse plexiform neurofibromas with functional disability. We should pay attention to diffuse plexiform neurofibromas that greatly affect quality of life in patients with NF1.
出版者
WILEY
資料タイプ
学術雑誌論文
外部リンク
ISSN
03852407
EISSN
13468138
書誌ID
AA00696658
掲載誌名
JOURNAL OF DERMATOLOGY
最新掲載誌名
JOURNAL OF DERMATOLOGY
44
11
開始ページ
1262
終了ページ
1267
発行日
2017-11
出版者DOI
著者版フラグ
著者版
著作権表記
(C) 2017 Japanese Dermatological Association
掲載情報
Ehara Yuko, Yamamoto Osamu, Kosaki Kenjiro, et al. Clinical severity in Japanese patients with neurofibromatosis 1 based on DNB classification. JOURNAL OF DERMATOLOGY. 2017. 44(11). 1262-1267. doi:10.1111/1346-8138.13902
部局名
医学部・医学系研究科・医学部附属病院
言語
英語
Web of Science Key ut
WOS:000414362100028