Filipenko P S, Kuchmaeva T B, Popov S P
Klin Med (Mosk). 2006;84(9):22-4.
Non-differentiated connective tissue dysplasia (NDCTD) presents a genetic-related anomaly of the mesenchimal matrix leading to the dysfunction of various organs and systems, the degree of which determine the severity of the clinical picture as well as individual prognosis. Despite high prevalence, NDCTD is often neglected by practitioners because the clinical manifestations of this pathology are very polymorphic due to the fact that connective tissue (CT) is spread throughout the organism. Various changes in the skin, locomotor apparatus, and, especially, the internal organs determine the individual prognosis and cannot but influence the course of concomitant diseases. Among visceral NDCTD markers the most well-known ones are mesenchimal cardial dysplasias, such as valvular prolapses, false chords, interventricalar septum and Valsalva sinus aneurisms etc. However, the generalized CT defect in NDCTD implies the presence of polyorganic symptoms thus stimulating search for new visceral markers of CD dysplasia. Various internal manifestations of NDCTD, such as expiratory tracheal stenosis, have their own pathogenetic features, as well as specific clinical and functional manifestations.
未分化结缔组织发育异常（NDCTD）呈现出一种与遗传相关的间充质基质异常，导致各个器官和系统功能障碍，其程度决定了临床表现的严重程度以及个体预后。尽管患病率很高，但NDCTD常常被从业者忽视，因为这种病理的临床表现非常多样，原因是结缔组织（CT）遍布全身。皮肤、运动器官尤其是内脏器官的各种变化决定了个体预后，并且必然会影响伴发疾病的病程。在内脏NDCTD标志物中，最广为人知的是间充质心脏发育异常，如瓣膜脱垂、假腱索、室间隔和主动脉窦瘤等。然而，NDCTD中普遍存在的CT缺陷意味着存在多器官症状，从而促使人们寻找新的结缔组织发育异常的内脏标志物。NDCTD的各种内部表现，如呼气性气管狭窄，都有其自身的发病机制特点以及特定的临床和功能表现。
