Objective. To study the clinical efficacy of hydrolyzed milk protein formulas “Peptamen Junior” and “Peptamen” (Nestlé, Switzerland) in nutrition therapy for children with cystic fibrosis. Patients and methods. Forty patients with cystic fibrosis aged from 1 year to 17 years 11 months who underwent treatment and rehabilitation at the pulmonology department of the National Medical Research Center for Children’s Health from October 2020 to August 2021 were examined. Patients were divided into two equal groups, each receiving one product. During dynamic observation, the patients’ general condition, nutritional status, including anthropometry, caliperometry, and bioimpedanceometry in older children, as well as biochemical parameters (albumin, prealbumin, transferrin, total protein, glucose), the biological and nutritional value of the diet were evaluated. Results. There was a significant improvement in the physical development of children, in some of whom the reduction of malnutrition was observed. This was evidenced by the positive dynamics of weight-for-length and BMI-for-age Z-scores, the complete elimination of malnutrition in 3 (3.8%) patients and reduction of malnutrition severity in 5 (6.3%) children, a significant increase in the average upper arm muscle circumference from 14.96 ± 0.93 to 15.9 ± 0.8 mm (p < 0.05) in children aged between 1 and 10 years, an increase in the number of children with normal lean body mass Z-score from 25 to 33%. Biochemical markers remained within reference values. The use of specialized formulas in the diet of children with cystic fibrosis enriched therapeutic nutrition with protein by 26-28% and energy by 22-24%. Conclusion. The inclusion of hydrolyzed milk protein formulas “Peptamen Junior” and “Peptamen” (Nestlé, Switzerland) in nutrition therapy for children with cystic fibrosis can maintain their nutritional status at a satisfactory level, provide a positive prognosis of the disease, and improve the quality of life of patients and their families. Key words: cystic fibrosis, children, nutritional status, hydrolyzed milk protein formulas

Objective. To assess the efficacy of nocturnal hyperalimentation via a low-profile gastrostomy tube for in the improvement of nutritional status and lung function in children with severe cystic fibrosis (CF). Patients and methods. We used nocturnal hyperalimentation with a nutrient solution via a low-profile gastrostomy tube installed under endoscopic control to improve nutritional status of 16 CF children with severe protein-calorie malnutrition aged between 5.5 and 17.5 years. The mean duration of nocturnal hyperalimentation was 1.3 years (range: 7 months–54 months). The nutritional status was evaluated using the WHOAntho Plus software; pulmonary function was evaluated by assessing forced expiratory volume in 1 minute (FEV1) and the number of bronchopulmonary exacerbations per year. Results. All children demonstrated an improvement in their weight and height. After the first 12 months, the mean body weight gain was 5 kg; during the second and third years, children gained 4 kg and nearly 2 kg, respectively. The mean increase in height was 7 cm in the first year, 10 cm in the second year, and 4 cm in the third year. All patients except one had an increase in FEV1 and fewer exacerbations. Complications after gastrostomy were rare and easily addressed. Conclusion. Nocturnal hyperalimentation via a percutaneous endoscopic gastrostomy tube is an effective and safe method of improving nutritional status in children with CF, which delays the progression of bronchopulmonary lesions and lung function reduction, improves the prognosis and quality of life. Key words: cystic fibrosis, children, nutritional status, lung function, enteral nutrition, low-profile gastrostomy tube, nocturnal hyperalimentation

Respiratory tract infection is a major cause of complications and death in patients with cystic fibrosis (CF). Transmissible strains of Gram-negative non-fermenting Burkholderiales bacteria: Burkholderia cepacia complex, Achromobacter spp, Pandoraea spp. are quite alarming. In the paperfirstly there is presented a clinical case with mixed microbial infection of the CF patient with involvement of Pandoraea pnomenusa. There are reportedfeatures of diagnosis and treatment of a patient throughout 20 years, described the data of the examination of the patient and his airway microbiota. Timely identification of P. pnomenusa with the use of mass spectrometry MALDI-TOF and the molecular genetic techniques was shown to contribute to the isolation of the patient in the hospital, his transfer to the inpatient substitution therapy, that prevented the cross-infection of other CF patients. Permanent microbiological control revealed an increase of the antibiotic resistance of P. pnomenusa. The congruence of the antibiotic therapy with resistome of all identified microorganisms has allowed to develop an effective treatment schedule, which promoted to the elimination of P. aeruginosa and P. pnomenusa in the sputum of CF patients to minor amounts and preservation of this balance of microorganisms in the next six months.

The article presents the results of clinical observation of cystic fibrosis (CF) patients received the enteral nutrition product Nutrient Standard (Russia) as nutritional support. The high efficiency of this product has been shown for the correction of malnutrition in СF children.

Chronic Pseudomonas aeruginosa infection is the most common cause of the failure in treatment and of adverse outcomes among children with congenital malformation of the lung (CML) and cystic fibrosis (CF). Purpose: To evaluatae the safety of anti Pseudomonas aeruginosa vaccine in children with congenital malformation of the lung and cystic fibrosis. Material and methods: 81 hospitalized children aged 5 to 17 with congenital malformation of the lung and cystic fibrosis participated in the research. 47 patients not experiencing exacerbation during basic therapy were vaccinated with anti Pseudomonas aeruginosa vaccine Pseudovac that consists of structural and extra-cellular antigens of the 8 immunotypes of Pseudomonas aeruginosa. The control group included 34 children who were not vaccinated, and had P. aeruginosa acquisition or infection. All the patients were under observation for the period of one year. Results: Pain in the injection site was registered in 74.5% and 76.6% of cases after the first and the second administration respectively. Further injections (3rd, 4th and 5th) of the vaccine caused much less local pain. In the majority of cases pain was mild and disappeared without medication within 24 hours. Redness in the injection site was registered in 29.8% of cases after the second administration of the vaccine. After further injections it was significantly less common. Local swelling in the injection site of not more than 25 mm after the first injection of the vaccine was registered in 57% of the cases, and in 100% of the cases after the second injection. It lasted less than 24 hours. Among vaccinated patients fever up to 37.5 degrees Celsius was registered in 23,4% of the cases. And up to 38.5 degrees in 4,3% of the cases. The reaction was observed within two hours after the injection and appeared regardless of the number of administrations lasting no longer than 24 hours. There appeared other single general reactions such as headache, dizziness or dry cough lasting up to 6 hours.

A difficult problem of Pseudomonas infection antibacterial treatment in patients with cystic fibrosis and its historical aspects are discussed in this article. Different medicinal forms of antibiotics are compared; the dosages for treatment in cystic fibrosis are shown. Efficacy and safety of inhalation form of tobramycin solution in patients with cystic fibrosis and chronic Pseudomonas infection are considered. Appropriateness of its administration in order to eradicate Pseudomonas aeruginosa as soon as it is found in the sputum, as well as in chronic persistence of this pathogen to prevent relapses of bronchopulmonary process and improvement of pulmonary function and microbiological stability is shown. The authors demonstrate their own data on usage of this drug, confirming its high tolerability and clinical effect.

Background : A long-term plating of Pseudomonas aeruginosa in patients with cystic fibrosis is a sign of damage of the lung tissues with rapid progression of the disease and decrease in a respiratory function. The early pathogen detection is necessary for the timely prescription of an antibiotic for the purpose of a complete eradication of P. aeruginosa. Objective : Our aim was to determine the efficiency and safety of an inhalation form of the colistimethate sodium antibiotic in children with cystic fibrosis during the initial detection of P. aeruginosa. Methods : In a retrospective continuous study it was analyzed the results of inhalation use of the colistimethate sodium in a dose of 2 million IU/day in children with moderate cystic fibrosis with newly diagnosed P. aeruginosa. Results : The analysis included data of 25 children at the age of 2–10 years, 17 of them were treated with colistimethate sodium for 6 months, 8 — for 12 months. P. aeruginosa eradication was detected in 22 (88%) children. Children, who received antibiotic therapy for 6 months, at the end of the treatment showed an increase in forced expiratory volume for the 1st second (FEV-1) from 67.1 ± 2.2 to 80.4 ± 1.9% (р = 0.012), but in 3 months without inhalations there was a decrease in indicator values (to 75.9 ± 5.7%; p = 0.069). With the duration of inhalations of 12 months, the value FEV-1 indicator also increased: from 65.9 ± 3.8 to 81.5 ± 3.1% (р = 0.011). However, in the following 3 months without therapy these children did not have any significant decrease in FEV-1 (80.6 ± 3.4%; р = 0.073). There were no allergic reactions during the treatment; bronchospasm was observed in one child. For the entire period of management any P. aeruginosa strain, resistant to the colistimethate sodium, was not revealed. Conclusion : During the initial detection of P. aeruginosa in children with cystic fibrosis, a long-term therapy, including inhalations with colistimethate sodium, leads to a bacterial eradication and restoration of a respiratory function. Key words: children; cystic fibrosis; chronic Pseudomonas aeruginosa infection; treatment; colistimethate sodium; inhalations.

Background: A long-term plating of Pseudomonas aeruginosa in patients with cystic fibrosis is a sign of damage of the lung tissues with rapid progression of the disease and decrease in a respiratory function. The early pathogen detection is necessary for the timely prescription of an antibiotic for the purpose of a complete eradication of P. aeruginosa. Objective: Our aim was to determine the efficiency and safety of an inhalation form of the colistimethate sodium antibiotic in children with cystic fibrosis during the initial detection of P. aeruginosa. Methods: In a retrospective continuous study it was analyzed the results of inhalation use of the colistimethate sodium in a dose of 2 million IU/day in children with moderate cystic fibrosis with newly diagnosed P. aeruginosa. Results: The analysis included data of 25 children at the age of 2–10 years, 17 of them were treated with colistimethate sodium for 6 months, 8 — for 12 months. P. aeruginosa eradication was detected in 22 (88%) children. Children, who received antibiotic therapy for 6 months, at the end of the treatment showed an increase in forced expiratory volume for the 1st second (FEV-1) from 67.1 ± 2.2 to 80.4 ± 1.9% (р = 0.012), but in 3 months without inhalations there was a decrease in indicator values (to 75.9 ± 5.7%; p = 0.069). With the duration of inhalations of 12 months, the value FEV-1 indicator also increased: from 65.9 ± 3.8 to 81.5 ± 3.1% (р = 0.011). However, in the following 3 months without therapy these children did not have any significant decrease in FEV-1 (80.6 ± 3.4%; р = 0.073). There were no allergic reactions during the treatment; bronchospasm was observed in one child. For the entire period of management any P. aeruginosa strain, resistant to the colistimethate sodium, was not revealed. Conclusion: During the initial detection of P. aeruginosa in children with cystic fibrosis, a long-term therapy, including inhalations with colistimethate sodium, leads to a bacterial eradication and restoration of a respiratory function. Key words: children; cystic fibrosis; chronic Pseudomonas aeruginosa infection; treatment; colistimethate sodium; inhalations.

The authors show the data on current microbiological pattern in children with acute and chronic respiratory disorders and dynamics of microflora susceptibility to the most frequently used antibiotics over recent years. The principles of antibiotic choice and control for their efficiency, peculiarities of their usage in children and the most common side effects are given. New aspects of the usage of combined antibacterial agents - ecoantibiotics – are discussed. Including of the Lactulose Anhydro into their composition allows to achieve high microbiological efficacy and does not cause antibiotic-induced diarrhea, which has a great importance in pediatric practice.