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AIM: Study features of persistence of Burkholderia cepacia in mucoviscidosis patients. MATERIALS AND METHODS: In the period from 2008 to 2009, 56 B. cepacia strains isolated from children with mucoviscidosis were obtained. 114 medical histories of children with mucoviscidosis from various age groups were analyzed. The developed algorithm for identification and typing including phenotype and molecular biology methods was used to identify B. cepacia bacteria. Strain genotyping was carried out by RAPD-PCR with random oligonucleotide primer as well as pulse-electrophoresis. RESULTS: Persistence of associations ofmicroogranisms in 59.4% of cases was established to be the feature of persistent infection in mucoviscidosis. The feature of persistence of B. cepacia strains in patients with diagnosis ofmuco-viscidosis mixed form, severe course is persistence in association with Pseudomonas aeruginosa. B. cepacia bacteria that can persist in mucoviscidosis patients are characterized by resistance to many antibiotics. A prolonged (up to 1 year and 5 months) persistence of B. cepacia strains isolated from 1 patient was proven by using microflora monitoring of lower respiratory tract. CONCLUSION: B. cepacia bacteria may colonize lower respiratory tract of mucoviscidosis patients, persist for a long time and be transmitted between patients.

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AIM: To study microflora of lower respiratory tract of children from different age groups with cystic fibrosis during follow-up for determination of its variability and possible sources of infectious complications. MATERIALS AND METHODS: One hundred forty-one medical histories of patients from different age groups with cystic fibrosis living in various regions of Russian Federation were analyzed. Eighty-four children with cystic fibrosis living in Moscow and Moscow region treated as outpatients and inpatients were prospectively followed. For identification and characterization of microorganisms, microbiological, molecular biological, and statistical methods were used. RESULTS: It was demonstrated that chronic pseudomonas, staphylococcal or mixed infection was already diagnosed in 25% of children aged 1-4 years, and identified in 80% of patients to the age of 18 years. In two-thirds of cases association of microorganisms was identified, and in hospitalized patients these associations were comprised by 3-5 microorganisms in 60% of cases. Aside from main agents in associations (Pseudomonas aeruginosa and Staphylococcus aureus), representatives of Gram-negative nonfermentative microorganisms (Burkholderia cepacia, Stenotrophomonas maltophilia, Acinetobacter baumanii) were often identified that possibly determined by tropism of these species to lung tissue. CONCLUSION: Chronic mixed infection is characteristic for patients with cystic fibrosis. Identification of possible mechanisms of lung infection in patients with cystic fibrosis will allow to develop evidence-based system of prevention of infectious complications in these patients.

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The results of the studies show that patients with mucoviscidosis diagnosed for the first time who do not receive any adequate replacement pancreatic therapy should not be prescribed high doses of pancreatic enzymes along with antibacterial therapy when they are hospitalized on account of exacerbation of the bronchopulmonary process. Sharply increased lipid absorption can cause an aggravation of oxidative stress and impair the patient's state instead of improving it. In our opinion, such patients should be prescribed delayed adequate pancreatic therapy with immediate introduction of high doses of antioxidants (vitamins E, A, C, beta-carotene, etc.). There is no doubt that it is necessary to conduct further research into the problem for a greater number of patients.

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It is postulated that P. aeruginosa in monoculture or in association with Staphylococcus aureus keeps its leading position in chronic bacterial inflammatory broncho-pulmonary processes in children with cystic fibrosis. Antibiotic resistant strains of Burkholderia cepacia, Stenotrophomonas maltophila, Alcaligenes xylosoxidans were revealed (7.1% of the strains). P. aeruginosa strains were susceptible to aminoglycosides, ciprofloxacin, and polymixin B. Susceptibility of smooth and mucoid forms of P. aeruginosa to ceftazidime stayed at the level of 49.6-57.1%. Such microbial associations as P. aeruginosa sm. + S. aureus, P. aeruginosa sm. + P. aeruginosa muc. + S. aureus were mainly susceptible to ciprofloxacin, aminoglycosides and resistant to ceftasidime. Meropenem, cefepim and ciprofloxacin are highly effective antibiotics for the treatment of broncho-pulmonary processes exacerbations at children with chronic P. aeruginosa cystic fibrosis. Intravenous use of antibiotics out of hospital for the treatment of the children with cystic fibrosis is clinically effective, and is economically and psychologically reasonable. It should be used more widely in medical practice.

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AIM: To study clinical effectiveness and safety of a new mucolytic drug pulmozim (Switzerland) adjuvant to basic therapy in mucoviscidosis patients of different age in Russia. MATERIAL AND METHODS: 15 patients with mucoviscidosis aged 5-36 years, functional lung capacity and FEV-1 at least 40% received pulmozim for 30 days. The drug was given in a single daily dose 2.5 mg in inhalations. The study included three stages: initial 14 days--discontinuation of all mucolytic drugs, 30-day course of pulmozim, 14-day follow-up without pulmozim and other mucolytics. RESULTS: Pulmozim in the above regimen was effective in patients with mixed and moderate mucoviscidosis. The drug facilitates respiration, improves sputum rheology, normalizes general condition of the patient, is simple for use, has good organoleptic properties. CONCLUSION: Pulmozim is an effective mucolytic drug in combined treatment of mucoviscidosis.

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Cystic fibrosis (CF) is a common, serious, and frequently fatal autosomal recessive genetic disorder associated with the poor function of chloride channels. Chronic endobronchial inflammation and bacterial infection are main causes of morbidity and mortality due to CF. The study dealt with a relationship between progression and inflammation markers. Twenty one CF children with acute pulmonary exacerbation were examined. The signs of peripheral blood inflammation (responses of lymphocytes to PHA and their sensitivity to the antiproliferative effect of glucocorticoids) and in situ inflammation markers (sputum elastase activity, IL-8 and TNF-alpha, and protein concentrations in the same sputum specimens). These laboratory findings were used to calculate a "laboratory index" (LI). The clinical status of each patient was evaluated with a "clinical index" (CI), a parameter that includes respiratory secretion cultures, pulmonary function test results, nutritional status, and the presence of disease-related complications. There was a positive linear correlation between LI and CI. The presence of P. aeruginosa was strongly associated with the changes of inflammatory markers. CF patients with prolonged P. aeruginosa infection demonstrated extremely enhanced elastase activity and elevated amounts of sputum IL-8 and TNF-alpha as compared to uninfected subjects. The lung elastase activities, sputum protein contents, and TNF-alpha levels in individuals with short-term colonization were at or below those without P. aeruginosa infection. In patients with or without short-term colonization, the normalization of laboratory parameters was strongly related to evident clinical improvement. At the same time, antibiotic treatment failed to suppress an excessive inflammatory response in the lungs of patients with prolonged P. aeruginosa infection. The importance of individual inflammation markers is discussed in the paper.

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The efficacy of ciprofloxacin was estimated for the last 3 years in the treatment of 139 children with mucoviscidosis. The drug was mainly used under hospital conditions. In 119 cases ciprofloxacin was administered orally in a daily dose of 25 to 50 mg/kg divided into 2 portions. The treatment course was 14-42 days. In 20 cases ciprofloxacin was used intravenously in a daily dose of 10 to 23 mg/kg injected twice a day with an interval of 12 hours. The treatment course was 14-18 days. Ciprofloxacin was used either alone (exclusively in outpatients) or in combination with the 2nd or 3rd generation cephalosporins or aminoglycosides. The positive effect was practically stated in all the patients in 10-14 days from the therapy start. The clinical remission at the background of the ciprofloxacin therapy in the inpatients was observed 3 days earlier than that in the inpatients treated without the use of ciprofloxacin. The remission period in the outpatients averaged 62 days while in the cases treated with gentamicin it was 35 days and in the cases treated with azlocillin it was 30 days. The adverse reactions were recorded in 8 per cent of the patients.

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Mucoviscidosis is serious inherited disease with lesions of almost all secretory cells of mucosa and exocrine glands, following by disturbances of metabolism, digestion and nutritional status. Using of modern high effective pancreatic enzymes does not allow to reach a normalization of protein metabolism and digestion especially of fats. For success treatment of this disease the effective dietetic methods should be used including dietary intake of special food products which can supply enough energy and nutrients in small volume. The nutrient contents of a few foreign substitution of breast milk and food product for older children are given. The results of treatment of 86 children suffering from mucoviscidosis are presented. Milk proteins (casein, whey proteins), hydrolysates of milk protein and soy protein isolates are main resources of protein component in special food. Fat components are produced from middle-chain triglycerides (60-70%) and vegetable oils. Carbohydrates in special food are made from glucose polymers, glucose, fructose and sucrose. Positive results of clinical trials of foreign special food have been an example for creation of two home-produced food for treatment of children with mucoviscidosis: 1) adapted version "A" for children in age under 1 year, and 2) version "B" for older children. The products were created on basis of native milk protein with ratio casein to whey protein as 60:40. Middle-chain triglycerides compose 70-75% of fats. Carbohydrates are presented by dextrin-maltose (85%), glucose and sucrose. In present time these special products are under clinical trial in scientific medico-genetic center of Russia' Academy of Medical Science.

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