دورية أكاديمية

Progress in the Research of the Spinal Deformity Related to Marfan Syndrome

التفاصيل البيبلوغرافية
العنوان: Progress in the Research of the Spinal Deformity Related to Marfan Syndrome
المؤلفون: LIN Guanfeng, WU Nan, WANG Shengru, YANG Yang, ZHANG Jianguo
المصدر: 罕见病研究, Vol 2, Iss 4, Pp 476-482 (2023)
بيانات النشر: Editorial Office of Journal of Rare Diseases, 2023.
سنة النشر: 2023
المجموعة: LCC:Medicine
مصطلحات موضوعية: marfan syndrome, spinal deformity, skeletal abnormalities, correction surgery, Medicine
الوصف: Marfan syndrome(MFS) is an autosomal dominant systemic connective tissue disease. The incidence rate of MFS is about 2-3 per 10 000. Main cause of MFS is FBN1 gene mutation. About 2/3 of MFS patients have spinal deformities, showing symptoms of scoliosis, thoracic lordosis and lumbar kyphosis, severe spondylolisthesis, dural dilatation and pedicle dystrophy. MFS scoliosis develops with age and may continue even after bone maturation. Conservative treatments such as brace are usually ineffective. Surgical treatment of main curve > 40°-45 °is recommended, but due to the special anatomical structure of MFS patients, such surgical complications as dural leakage, failure of internal fixation and revision surgery are not uncommon.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: Chinese
تدمد: 2097-0501
Relation: https://doaj.org/toc/2097-0501
DOI: 10.12376/j.issn.2097-0501.2023.04.003
URL الوصول: https://doaj.org/article/d7d1d8b3dbaf4f1bb6aa17e19726f875
رقم الأكسشن: edsdoj.7d1d8b3dbaf4f1bb6aa17e19726f875
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:20970501
DOI:10.12376/j.issn.2097-0501.2023.04.003