Establishment of in-hospital clinical network for patients with neurofibromatosis type 1 in Nagoya University Hospital
| Autor: | Norie Nonobe, Seiji Mizuno, Yoshihiro Nishida, Miki Kanbe, Kanako Ishizuka, Hiroyuki Kidokoro, Kunihiro Ikuta, Takuya Takeichi, Atsushi Natsume, Yukako Muramatsu, Norio Ozaki, Maki Morikawa, Naoko Ishihara, Shiro Imagama |
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| Rok vydání: | 2021 |
| Předmět: |
Adult
Male 0301 basic medicine Pediatrics medicine.medical_specialty Neurofibromatosis 1 Adolescent Science Scoliosis Disease 030105 genetics & heredity Article Hospitals University Young Adult 03 medical and health sciences 0302 clinical medicine Japan Forearm medicine Humans Neurofibromatosis Child Aged Preventive healthcare Preventive medicine Aged 80 and over Information Services Multidisciplinary business.industry Incidence (epidemiology) Medical record Infant Sarcoma Middle Aged medicine.disease medicine.anatomical_structure Child Preschool 030220 oncology & carcinogenesis Orthopedic surgery Hospital Information Systems Medicine Female business |
| Zdroj: | Scientific Reports Scientific Reports, Vol 11, Iss 1, Pp 1-7 (2021) |
| ISSN: | 2045-2322 |
| DOI: | 10.1038/s41598-021-91345-6 |
| Popis: | Background: Neurofibromatosis type 1 (NF1) is a genetic multisystem disorder. Clinicians must be aware of the diverse clinical features of this disorder in order to provide optimal care for it. We have set up an NF1 in-hospital medical care network of specialists regardless of patient age, launching a multidisciplinary approach to the disease for the first time in Japan.Results: From January 2014 to December 2020, 246 patients were enrolled in the NF1 patient list and medical records. Mean age was 26.0 years ranging from 3 months to 80 years. The age distribution at the first visit was wider as the age was lower. There were 107 males (41%) and 139 females. After 2011, the number of patients has increased since the year when the medical care network was started. Regarding orthopedic signs, scoliosis was present in 60 cases (26%), and bone abnormalities in the upper arm, forearm, and tibia in 8 cases (3.5%). Neurofibromas other than cutaneous neurofibromas were present in 90 cases (39%), and MPNST in 17 cases (7.4%).Conclusions: We launched a multidisciplinary NF1 clinic system for the first time in Japan. For patients with NF1, which is a hereditary and systemic disease associated with a high incidence of malignant tumors, it will be of great benefit when the number of such clinics in Japan and the rest of Asia is increased. |
| Databáze: | OpenAIRE |
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