From the phenotype of connective tissue dysplasia to the phenotype of bronchial asthma in children


DOI: https://dx.doi.org/10.18565/therapy.2020.6.59-63

Nesterenko Z.V.

St. Petersburg State Pediatric Medical University of the Ministry of Healthcare of Russia
The aim: to study the clinical features of the phenotypes of bronchial asthma (BA) in children with various phenotypes of connective tissue dysplasia (CTD).
Material and methods. The results of a survey of 55 children with BA and different CTD phenotypes are presented: in 69,2% of patients revealed an unclassified CTD phenotype (UP); 26,9% have Ehlers–Danlos-like CTD phenotype (EDLP); 3,8% have Marfan-like CTD phenotype (MLP); 5,4% of patients had increased CTD stigmatization (CTDS).
Results. Moderate asthma with the development of complications (pulmonary hypertension and fibrosis) was noted significantly more often in children with MLP and EDLP compared with patients with UP and CTDP. Atopic asthma was in 47,3% of children (Th2 phenotype) with high sensitivity to steroid therapy. In 41,8% of patients (100% with MLP, 64,3% with EDLP; 33,3% with UP), community-acquired pneumonia was diagnosed in the absence of atopy, eosinophilia and the presence of neutrophilia (non-Th2 phenotype). In this group, a longer episode of bronchial obstruction, low steroid effectiveness and the need for antibiotic therapy was noted. 10.9% of children had neutrophilia, signs of community-acquired pneumonia and manifestations of atopy and eosinophilia (21.1% with EDLP; 8.3% with UP), which corresponds to a mixed form of asthma, which also required antibiotic treatment.
Conclusion. The signs of MLP, EDLP in children with asthma and community-acquired pneumonia can be used in the diagnosis of the non-Th2 asthma phenotype in children, predicting poor treatment response to corticosteroids and the need for antibiotic treatment.
Keywords: phenotypes of connective tissue dysplasia, pheno-endotypes of asthma, genetic factors, allergy, children
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About the Autors

Zoya V. Nesterneko, MD, professor, professor of the Department of propedeutics of childhood diseases with a course of general child care of St. Petersburg State Pediatric Medical University of the Ministry of Healthcare of Russia. Address: 194100, Saint Petersburg, 2 Litovskaya Str. Tel.: +7 (965) 010-71-47. E-mail: zovas@mail.ru


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