title: [Clinical manifestations of the expiratory tracheal stenosis, and its effects on the progress of bronchopulmonary pathology].
abstract: 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.
authors.forename: P S
authors.initials: PS
authors.lastname: Filipenko
authors.forename: T B
authors.initials: TB
authors.lastname: Kuchmaeva
authors.forename: S P
authors.initials: SP
authors.lastname: Popov
mesh_terms: D000293:Adolescent; D000328:Adult; D018450:Disease Progression; D045853:Exhalation / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications* / Q000503:physiopathology; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014135:Tracheal Stenosis / Q000150:complications* / Q000503:physiopathology