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Introduction a problem Urgency

Mucoviscidosis (MV) - one of the most frequent monogennonasleduemyh diseases with the multiorgan manifestation, caused by a mutation of a gene of a transmembrane regulator of conductivity (CFTR). poliorgannost lesions at MV, low life expectancy sick (on the average 40 years), an early invalidism dictate necessity of constant carrying out of medical actions and a dispensary observation.

In our country MV is the important mediko-social problem that is bound with big moral, physical and material inputs of a family, organs of practical public health services and a society as a whole on diagnostics, treatment, aftertreatment and social adaptation of patients with MV. In the developed countries last years growth of number of patients MV of teenage, youthful age and adults that testifies to its gradual transformation from fatal disease of children's age in a chronic pathology of adults [36, 40, 41, 111, 122] becomes perceptible.

Now the number of patients MV in the USA makes more than 30000, in Europe more than 35000 persons. In the Russian Federation on the account 2000 children consist for today of 2600 patients, of them. However by calculations in the Russian Federation the number of patients MV should make nearby 12000. It is obvious, that many patients are not diagnosed in due time. Average life expectancy of these patients in the developed countries for last 30 years was enlarged with 14 till 32 years (35 - 40 years), in the Russian Federation with 16 to 24 (till 29 years) [40, 122].

From the moment of identification of gene MV the cases of illnesses similar with MV, but not approaching under the classical description began to be taped: bilateral absence of a deferent duct (CBAVD), disseminirovannye bronchiectasias, a chronic pancreatitis, diffusive panbronhiolit [124]. Working group the CART, the International association of a mucoviscidosis,
The European thematic network on mucoviscidosis problems has developed the project of its classification for МКБ-11 (2005):

1. Classical MV with pancreatic insufficiency (PI);

2. Classical MV with not broken function of a pancreas (PS);

3. Atypical MV;

4. Isolated obstructive azoospermija;

5. A chronic pancreatitis;

6. Allergic bronholegochnyj an aspergillosis (ABPA);

7. disseminirovannye bronchiectasias;

8. Diffusive panbronhiolit;

9. A sclerosing cholangitis;

10. Neonatal gipertripsinogenemija [8, 41, 42, 47, 102].

On quality and life expectancy of patients MV the greatest influence is rendered by disturbances from organs of breath which are characterised by the expressed immune answer with intensive migration of the neutrophils producing free radicals and proteolytic enzymes (elastase). In bronholegochnom a secret of such patients the raised maintenance of proinflammatory cytokines (FNOa, ИЛ1р, ИЛ6, ИЛ8) [85, 96] is found out.

Thus, the immune system of patients MV functions in the conditions of a superfluous antigenic load that it is possible to survey as the permanent stress demanding constant mobilisation of adaptic systems of an organism [136].

Efficiency of therapy and, hence, the disease forecast directly depends on early diagnostics and timely statement of patient MV on the account [40, 41, 45, 102]. At MV traditionally allocate 3 forms: pulmonary, intestinal and admixed (is pulmonary-intestinal).

Most
Pulmonary form MV in this connection studying of disturbance of ventilating function of lungs is actual at this disease [40, 102] as its condition often defines the disease forecast is widespread.

Research objective: to tap patterns of changes

Ventilating function of lungs at children sick of a mucoviscidosis, depending on age and severity level.

Research problems

1. To study clinico-functional features of a current of a mucoviscidosis at children at the age from 3 months till 18 years

2. To give the characteristic of function of external breath at various severity level of a mucoviscidosis at children

3. At children of younger age sick of a mucoviscidosis to define possibility of use of methods bronhofonografii and an impulsive oscillometry for research of bronchial passableness

4. To estimate influence of complex treatment of a mucoviscidosis on function of external breath at children

Scientific novelty

For the first time changes of ventilating function of lungs at children of the younger age, suffering are established by a mucoviscidosis.

Changes of bronchial passableness at sick of a mucoviscidosis of younger age (from 2 months till 7 years) are defined.

It is investigated FVD on the basis of studying of parametres of an impulsive oscillometry and bronhofonografii depending on gravity and the period of illness at children of younger age.

Diagnostics possibility bronhoobstruktivnogo a syndrome by a method bronhofonografii and an impulsive oscillometry is proved.

The clinico-functional characteristic of a current bronholegochnogo process at children with a mucoviscidosis depending on the form and gravity of disease, including at children of younger age is given.

Criteria of the forecast of disease on the basis klinikoyofunktsionalnyh researches in a catamnesis at children with a mucoviscidosis are developed.

The practical importance

Criteria of diagnostics bronhoobstruktivnogo a syndrome by data bronhofonografii and an impulsive oscillometry at children with MV are defined.

Features of changes characteristic for disturbances of bronchial passableness by data bronhofonografii and an impulsive oscillometry, at children of early age depending on the period and gravity MV are taped.

It is established, that the investigated methods of functional diagnostics can serve as criteria of efficiency of spent therapy at children of early age.

Necessity of use bronhofonografii and an impulsive oscillometry for diagnostics at children of the first years of a life with MV is proved.

The obtained data have allowed to improve diagnostics of ventilating function of lungs and to individualise treatment at children with a mucoviscidosis, including at children of younger age.

Introduction in practice

Results of the spent researches are used in work of unit of pulmonology and allergology of scientific research institute of pediatrics of a name of G.N.Speransky NTSZD the Russian Academy of Medical Science and in unit of clinical immunology and allergology GBU DGKB № 9 of G.N.Speransky

Work approbation

Dissertation substantive provisions are reported and discussed on IX National congress on a mucoviscidosis the Mucoviscidosis at children and adults - 2009; IV European congress of pediatrists (4th Europaediatrics, Moscow, 2009). Dissertation approbation is spent at joint session of the problem commissions of 10.03"Illnesses of organs of breath at children and 10.04"Allergic illnesses at children and teenagers scientific research institute of pediatrics FGBU Centre of science of health of children the Russian Academy of Medical Science.

Personal participation of the author of dissertation

The author immediately participated in the organisation and work carrying out: at a formulation of the purpose and research problems, working out of methodical approaches, a set of a clinical material, carrying out of functional methods of researches, statistical processing, the analysis and interpreting of the received data.

Publications

On a dissertation theme 6 printing works, including 4 articles in the magazines reviewed VAKom are published.

Volume and dissertation structure

The dissertation consists of introduction, 4 heads, the conclusion, conclusions, practical references, the literature list. Work is illustrated by 22 tables and 28 drawings, 2 clinical examples. The bibliographic index contains 211 sources, from them 109 domestic and 102 foreign authors.

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Scientific source TARASOVA OLGA VLADIMIROVNA. Ventilating function of lungs at children sick of a mucoviscidosis, at the present stage. The dissertation on competition of a scientific degree of the candidate of medical sciences. Moscow - 2014. 2014

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