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https://doi.org/10.14300/mnnc.2019.14057

Clinical and functional peculiarities of bronche-obstructive syndrome in children with connective tissue dysplasia

[Pediatrics]
Angelina Kalmykova; Natalia Fedko; Natalya Zarytovskaya; Victoriya Kalmykova; Asiyat Dzhanibekova;

117 children aged from 5 to 15 years with diseases accompanied by recurrent broncho-obstructive syndrome (BOS) were examined. Clinical and functional features of BOS were assessed among patients who had at least 8 external stigmas of disembriogenesis as markers of Undifferented Connectivc Tissue Dysplasia (UCTD) (I group) and 60 children without external markers of connective tissue dysplasia (II group).
In patients with the BOS on the background of UCTD, the proximal nature of the violation of the patency of the bronchial tree was observed. Multiple phenotypic signs of UCTD, causing morphofunctional changes in the bronchopulmonary system, correlated with the earliest manifestation of age, more severe and prolonged manifestation of the BOS in children. Knowledge of these parameters is useful for the timely prevention of adverse prognosis and the appropriate correction of therapeutic measures.

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References:
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Keywords: children, undifferentiated connective tissue dysplasia syndrome, bronchial obstruction syndrome