Clinical, Immunological, and Microbiological Aspects of Cystic Fibrosis in Children: Optimization of Management Tactics

Abstract

Cystic fibrosis (CF) remains one of the most significant hereditary diseases, characterized by a severe progressive course, multisystemic involvement, and high medico-social significance. This study aimed to comprehensively analyze the clinical, immunological, and microbiological aspects of CF in children to develop and substantiate differentiated therapeutic approaches. A total of 28,449 medical histories were retrospectively analyzed, identifying 307 children with CF (1.04%). A prospective study included 106 children with a mixed form of CF (aged 1 month to 14 years), 35 children with recurrent bronchitis with broncho-obstructive syndrome (RB+BOS), and 20 healthy controls. The study employed clinical, functional (spirometry), immunological (cellular and humoral immunity, cytokine profile), microbiological (sputum and stool cultures), and biochemical (vitamin D) methods. The investigation revealed a high prevalence of nutritional deficiency (88.2%), predominant combined ventilatory disorders (62.5%), and significant immunological imbalance characterized by T-lymphocyte depletion, elevated pro-inflammatory cytokines (IL-8, TNF-α), and vitamin D deficiency (86.8%). Microbiological analysis showed a predominance of P. aeruginosa (45.3%) and S. aureus (30.2%) in the respiratory tract, with a high frequency of intestinal dysbiosis (86.8%). Differentiated therapy, including an immunomodulator (IRS-19), a probiotic (Saccharomyces boulardii), and nutritional support, demonstrated the highest efficacy in Group III, leading to a 29.0% increase in FEV1, normalization of nutritional status, a 2.3-fold reduction in exacerbation frequency, and eradication of P. aeruginosa in 64.0% of patients. The developed management algorithm, based on a validated risk stratification scale (sensitivity 82%, specificity 76%), allows for personalized therapy and significantly improves the prognosis and quality of life for children with CF.