Filipenko P S, Malookaia Iu S
Klin Med (Mosk). 2006;84(12):13-9.
Connective tissue (CT) is a multifunctional universal structure of great importance to the human organism. Constituting about 50% of the body mass, CT forms a frame (skeleton) and outer cover (skin), as well as the inner medium, through which all structural elements receive nutrients and extract metabolic products. The great number of links, constituting the CT system, each of which is controlled genetically and is liable to genetic lesions, creates conditions for heterogeneity of malformations and diseases involving CT. Non-differentiated CT dysplasia (NDCTD) is a genetically heterogenous group, presenting a basis for various chronic diseases. NDCTD may present the cause of dysplastic changes in the CT of different organs and systems. Thanks to modern diagnostic techniques, NDCTD is revealed frequently. NDCTD is underlied by molecular-, onto-, and pathogenetic mechanisms, leading to structural and functional changes in CT. This CT "weakness" is manifested by the peculiarities of the structure of various organs and systems. Mesenchimal heart dysplasias are the most widespread visceral markers of the given CT pathology. CT dysplasias of the heart are often combined with varied manifestations of system CT anomaly. Mitral valve prolapse (MVP) is the most wide-spread and well-studied minor heart anomalies. Primary MVP is a hereditary or congenital pathology and is not connected with a particular disease. It is a genetic pathology--CT dysplasia with autosomal dominant inheritance. Patients with MVP have an increased expression of Bw35 antigen of HLA system, which causes dysmetabolism of collagen in the mitral cusps. It has been revealed that tissue deficiency of magnesium is associated with antigen expression and correlates with clinical symptoms in MVP. Exogenic factors influencing MVP have been described.
结缔组织(CT)是一种对人体极为重要的多功能通用结构。结缔组织约占体重的50%,形成了一个框架(骨骼)和外层覆盖物(皮肤),以及内部介质,所有结构元素通过该介质获取营养并排出代谢产物。构成CT系统的大量连接,每个连接都受基因控制且易发生基因病变,这为涉及CT的畸形和疾病的异质性创造了条件。未分化CT发育异常(NDCTD)是一个基因异质性群体,是各种慢性疾病的基础。NDCTD可能是不同器官和系统CT发育异常变化的原因。由于现代诊断技术,NDCTD经常被发现。NDCTD的基础是分子、个体发生和致病机制,导致CT的结构和功能发生变化。这种CT“弱点”通过各种器官和系统结构的特殊性表现出来。间质性心脏发育异常是这种CT病理最常见的内脏标志物。心脏的CT发育异常常与系统性CT异常的各种表现相结合。二尖瓣脱垂(MVP)是最常见且研究充分的轻度心脏异常。原发性MVP是一种遗传性或先天性病理,与特定疾病无关。它是一种遗传病理——具有常染色体显性遗传的CT发育异常。MVP患者HLA系统的Bw35抗原表达增加,这导致二尖瓣叶中胶原蛋白代谢异常。研究发现,镁的组织缺乏与抗原表达相关,并与MVP的临床症状相关。已经描述了影响MVP的外源性因素。