دورية أكاديمية
أعرض في EDS

Pectus excavatum and heritable disorders of the connective tissue

التفاصيل البيبلوغرافية
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
الوصف
تدمد:2036749X
20367503
DOI:10.4081/pr.2013.e15