THE DISSERTATION MAINTENANCE

Materials and research methods

According to objects in view and research problems complex inspection 158 sick (88 boys and 70 girls) with verified diagnosis MV at the age from 6 months till 18 years (middle age 6,7±1,3) which have made the basic group is spent.

As control group it has been surveyed conditionally healthy 68 children at the age from 3 till 17 years by which the identical set of immunologic inspection has been executed. Blood sampling was spent according to the current version of the Helsinki Declaration, under condition of absence of necessity of additional blood samplings or out-patient visitings of clinic.

The control group for carrying out of genetic researches is made of 91 healthy Russian inhabitants of of Tomsk. From all individuals the informed consent to participation in mediko-epidemiological research has been received.

According to design of the research, sick have been grouped taking into account disease severity level. The first group have made 90 (57,0 %) children with the serious form of the disease, the second - 59 (37,4 %) patients with srednetjazhelym a current, third – 9 (5,7 %) patients with easy degree of an illness.

Considering, that character of a microbic infection in a bronchial tree at sick of a mucoviscidosis in many respects causes specificity of inflammatory process, and feature of a current of a basic disease we have parted patients on groups taking into account the microbiological status. The first group was made by patients at whom the dominating microbic agent in a bronchial tree was Pseudomonas aeruginosa - 72 children (45,6 %). At patients of the second group colonisation of respiratory tract Burkholderia cepacia - 39 children (24,7 %) is taped. The third group has been presented by children with MV at which infectious process was induced S.аureus (34 children – 21,5 %). In the fourth group (13 children (8,2 %)) diagnostically significant growth are not defined.

Researches were spent on the basis of own observations and data of the medical documentation (a clinical case history, the conclusion of experts in paraclinic methods of inspection). To a forage of that, we had been developed the detailed questionnaire-questionnaire which reflected a state of health of the child from the moment of a birth, development and a disease current.

All children of the basic group are surveyed according to standards of hi-tech medical aid Minzdravsotsrazvitija of Russia for sick of a mucoviscidosis.

Besides, we used the additional immunologic inspection including definition the maintenance of components of a complement (С3, С4 and С5а, the factor), ИЛ-1 β, ИЛ-4, ИЛ-6, ИЛ-8, ИЛ-10, indicators interferonovogo the status (IFN - γ and IFN - α) in blood serum and in a sputum. Research was spent a method tverdofaznogo an enzyme immunoassay by means of commercial immunoenzymatic sets of Open Company "Cytokine" (S-peterburg) and «VSM Diagnostics» (USA). Lizotsimnaja activity of blood serum and a saliva, bactericidal activity of blood serum defined nefelometricheskim a method.

Genetic inspection included genotipirovanie polymorphic markers of gene CFTR and genes-modifiers of the immune answer: IFN - γ, ИЛ-4, ИЛ-4Rа. The analysis of genetic polymorphisms carried out with the help polimeraznoj chain reaction (PTSR), using structure prajmerov and parametres of temperature cycles, with the subsequent analysis of polymorphism of lengths restriktsionnyh fragments (PDRF).

Genetic inspection was spent on the basis of scientific research institute of medical genetics TNTS FROM the Russian Academy of Medical Science, by of Tomsk together with the professor, d.m.n. Kondratevoj E.V. and the senior lecturer Jankinoj G. N.

Statistical processing of the received data spent with use of parametrical and nonparametric statistics depending on scales and character of distribution of variables. Calculation of criteria was spent with package use «Statistica 6.0».