دورية أكاديمية
Pectus excavatum and heritable disorders of the connective tissue
العنوان: | Pectus excavatum and heritable disorders of the connective tissue |
---|---|
المؤلفون: | Francesca Tocchioni, Marco Ghionzoli, Antonio Messineo, Paolo Romagnoli |
المصدر: | Pediatric Reports, Vol 5, Iss 3, Pp e15-e15 (2013) |
بيانات النشر: | MDPI AG, 2013. |
سنة النشر: | 2013 |
المجموعة: | LCC:Medicine LCC:Pediatrics |
مصطلحات موضوعية: | pectus excavatum, Marfan syndrome, MASS, fibrillin, fibrillinopathies, Medicine, Pediatrics, RJ1-570 |
الوصف: | Pectus excavatum, the most frequent congenital chest wall deformity, may be rarely observed as a sole deformity or as a sign of an underlying connective tissue disorder. To date, only few studies have described correlations between this deformity and heritable connective tissue disorders such as Marfan, Ehlers-Danlos, Poland, MASS (Mitral valve prolapse, not progressive Aortic enlargement, Skeletal and Skin alterations) phenotype among others. When concurring with connective tissue disorder, cardiopulmonary and vascular involvement may be associated to the thoracic defect. Ruling out the concomitance of pectus excavatum and connective tissue disorders, therefore, may have a direct implication both on surgical outcome and long term prognosis. In this review we focused on biological bases of connective tissue disorders which may be relevant to the pathogenesis of pectus excavatum, portraying surgical and clinical implication of their concurrence. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2036-749X 2036-7503 |
Relation: | http://www.pagepress.org/journals/index.php/pr/article/view/4874; https://doaj.org/toc/2036-749X; https://doaj.org/toc/2036-7503 |
DOI: | 10.4081/pr.2013.e15 |
URL الوصول: | https://doaj.org/article/831bd9f1d6db4f2eb219c1e28dc9e3e3 |
رقم الأكسشن: | edsdoj.831bd9f1d6db4f2eb219c1e28dc9e3e3 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 2036749X 20367503 |
---|---|
DOI: | 10.4081/pr.2013.e15 |