Abstract
Cystic fibrosis is one of the severe childhood disorders with early manifestation of multiple organ involvement. The respiratory and digestive tracts are suffered the most, the prognosis of disease course depends on the degree of the impairment of these systems. The results of the analysis of cystic fibrosis prevalence in Primorsky Krai according to the neonatal immunoreactive trypsin screening in 102 251 newborn are shown in the article. The age, sex, clinical and severity structure of cystic fibrosis was studied in 71 children. The prevalence of 13 mutations of transmembrane regulator conductance gene in 41 patients was determined. Correlations between time of pulmonary complications manifestation, P. Aeruginosa infection development, chronic hypoxemia degree, malnutrition, genetic status and severity of the disease were found.
Highlights
Cystic fibrosis is one of the severe childhood disorders with early manifestation of multiple organ involvement
The respiratory and digestive tracts are suffered the most, the prognosis of disease course depends on the degree of the impairment of these systems
The results of the analysis of cystic fibrosis prevalence in Primorsky Krai according to the neonatal immunoreactive trypsin screening in 102 251 newborn are shown in the article
Summary
Cystic fibrosis is one of the severe childhood disorders with early manifestation of multiple organ involvement. Certain aspects of respiratory and digestive tract involvement in cystic fibrosis in children
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