File
Authors
Yoshida, Yuichi Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University Researchers DB KAKEN
Ehara, Yuko Division of Dermatology, Department of Medicine of Sensory and Motor Organs, School of Medicine, Tottori University Faculty of Medicine
Koga, Monji Department of Dermatology, Fukuoka University School of Medicine
Imafuku, Shinichi Department of Dermatology, Fukuoka University School of Medicine
Yamamoto, Osamu Division of Dermatology, Department of Medicine of Sensory and Motor Organs, Faculty of Medicine, Tottori University Researchers DB KAKEN
Keywords
neurofibromatosis 1
epidemiology
treatment
survey
Abstract
Neurofibromatosis 1 has various complications. To elucidate the frequency of neurofibromatosis 1-related major complications requiring medical intervention, a nationwide retrospective study was conducted of 3,530 patients with neurofibromatosis 1 registered from 2001 to 2014 in Japan. The ratio of certified patients requiring medical intervention (>stage 3) was 82%. Patients classified in the most severe grade experienced dermatological complications (71.8% of patients), neurological complications (38.1%) and bone complications (33.3%). In patients with dermatological manifestations, medical treatment was needed for cutaneous neurofibromas (58%), diffuse plexiform neurofibromas (31%) and malignant peripheral nerve sheath tumours (10%). Patients with neurological manifestations needed medical treatment mainly for brain tumours (53%) and intellectual disability (26%). Patients with bone manifestations needed medical treatment for pseudoarthrosis (9%), scoliosis (55%) and bone defects (16%). It is necessary for physicians to be aware of neurofibromatosis 1-related complications requiring medical intervention in order to provide appropriate care for patients with neurofibromatosis 1.
Publisher
ACTA DERMATO-VENEREOLOGICA
Content Type
Journal Article
Link
ISSN
00015555
EISSN
16512057
NCID
AA00507245
Journal Title
ACTA DERMATO-VENEREOLOGICA
Current Journal Title
ACTA DERMATO-VENEREOLOGICA
Volume
98
Issue
8
Start Page
753
End Page
756
Published Date
2018-09-15
Publisher-DOI
Text Version
Publisher
Rights
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation (C) 2018 Acta Dermato-Venereologica.
Citation
Yoshida Yuichi, Ehara Yuko, Koga Monji, et al. Epidemiological Analysis of Major Complications Requiring Medical Intervention in Patients with Neurofibromatosis 1. ACTA DERMATO-VENEREOLOGICA. 2018. 98(8). 753-756. doi:10.2340/00015555-2975
Department
Faculty of Medicine/Graduate School of Medical Sciences/University Hospital
Language
English
Web of Science Key ut
WOS:000442511400006