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https://doi.org/10.14300/mnnc.2016.11128

Connective tissue dysplasia syndrome as a risk factor of severe course of bronchial obstruction syndrome in children

[Pediatrics]
Natalia Fedko; Angelina Kalmykova; Natalia Lagodina; Valentina Muravyeva; Asiyat Dzhanibekova; Olga Galimova; Elena Voronkina; Olga Rubacheva;

The peculiarities of external respiration and clinical course of some diseases of the respiratory system that occur with obstructive syndrome in children with phenotypic characteristics of undifferentiated connective tissue dysplasia (UDCT) were studied. 117 children were surveyed, aged 4 to 15 years with chronic and recurrent bronchopulmonary diseases, who
had at least 8 external stigmas of dysembryogenesis as markers of UDST (group I) and 60 with similar pathology, but without external markers of connective tissue dysplasia (group II – control). The study was conducted in several stages. Patients with the syndrome of bronchial obstruction on the background of NDCTD showed manifestation of bronchial obstruction the earliest by age and different in severity and duration, mainly dominated by the proximal nature of impaired patency of the bronchial tree, severe course of the disease was more frequent; such patients are most resistant to bronchodilator therapy. These results are important for the development of individual approaches to the diagnosis, treatment, rehabilitation and prevention of possible complications at USTD in children.

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Keywords: children, undifferentiated dysplasia of connective tissue, bronchoobstructive syndrome