Bollero P, Arcuri L, Miranda M, Ottria L, Franco R, Barlattani A
Department of Systems Medicine, University of Rome "Tor Vergata", Rome, Italy.
Department of Clinical Sciences and Translational Medicine, University of Rome "Tor Vergata", Rome, Italy.
Oral Implantol (Rome). 2017 Sep 27;10(2):87-96. doi: 10.11138/orl/2017.10.2.087. eCollection 2017 Apr-Jun.
Marfan's Syndrome is a multisistemic pathology of connective tissues, a dominant autosomal transmission, first discovered by a French pediatrician, Antoine Bernard-Jean Marfan, who first found in some of his patients a disproportionate alteration of inferior infertility. This alteration was caused by the mutation of the FBN1 gene, located on the long arm of the chromosome 15, which encodes for an extracellular matrix protein, fibrin-1. Later it was discovered that the disease could occasionally be due also to the mutation of the TGFBR2 gene, which encodes for a TGF-beta receptor 1. The estimated incidence of the disease is 2-3 subjects affected every 10,000, in the absence of predilection ratial, ethnic, geographic and gender. It is believed that some 15,000 people in Italy suffer from Marfan Syndrome. The disease is characterized by a wide range of clinical manifestations that affect different organs. The study evaluates through a literature review the manifestations in the oral cavity of the marfan syndrome and the correct management of the patient during dental maneuvers.
马凡综合征是一种结缔组织的多系统病变，呈常染色体显性遗传，由法国儿科医生安托万·贝尔纳 - 让·马凡首次发现，他最初在一些患者中发现下半身不成比例的异常。这种异常是由位于15号染色体长臂上的FBN1基因突变引起的，该基因编码一种细胞外基质蛋白——原纤蛋白 - 1。后来发现，该疾病偶尔也可能归因于TGFBR2基因突变，该基因编码TGF - β受体1。在无种族、民族、地理和性别偏好的情况下，该疾病的估计发病率为每10000人中有2 - 3人患病。据信，意大利约有15000人患有马凡综合征。该疾病的特点是有广泛的临床表现，会影响不同器官。本研究通过文献综述评估马凡综合征在口腔中的表现以及在牙科操作过程中对患者的正确管理。
