Abstract
A systematic analysis of the data available in modern literature concerning the problem of connective tissue dysplasia in children and adolescents, and the results of studying the course of bronchial obstruction syndrome in patients with this pathology, was carried out. Mechanisms of connective tissue dysplasia is imperfect, elucidation of the genetic causes of hereditary connective tissue disorders has not yet led to cardinal improvements in diagnosis, prevention and treatment. Connective tissue dysplasia is characterized by the presence of changes in the bronchopulmonary system: tracheobronchial dyskinesia (expiratory collapse of the trachea and large bronchi), tracheobronchomalacia and tracheobronchomegaly, apical bullae, confirmed by radiographic and spontaneous pneumothorax (more common in young men), the presence of thoracodiaphragmatic syndrome, which includes an asthenic form of the chest, deformities chest and / or spine, change in standing height and excursions of the diaphragm. The etiological and pathogenetic factors in the development of bronchiectasis remain ambiguous. Patients with connective tissue dysplasia require special attention and deeper study. The presence of thoracodiaphragmatic and bronchopulmonary syndrome in children contributes to a longer course of broncho-obstructive syndrome, a brighter, vegetative color of seizures is described. It is noted that these patients need more aggressive and long-term therapy.
Published Version
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