1.2 Clinico-functional characteristic of bronho-pulmonary process at a mucoviscidosis and modern methods of functionaldiagnostics

Chronic lesion of lungs - the most serious clinical implication of a mucoviscidosis. Patients with a mucoviscidosis die of the respiratory disorders which have developed owing to infections and nejtrofilnogo inflammations [8, 51].

The microflora supports a chronic inflammation in respiratory tracts, promotes a destruction of a tissue of a lung, that in turn, and leads to development of respiratory insufficiency. Respiratory insufficiency is a mors principal cause at MV.

Defining in a clinical picture of a mucoviscidosis is chronic bronholegochnoj process which quite often proceeds with different degree of obstruction of respiratory tracts [15, 41, 60, 91, 124]. Changes from the party bronholegochnoj systems are taped already at a birth or in the first weeks of a life of the child in the form of a hypertrophy podslizistyh glands, obstruction of ducts and a hyperplasia of goblet cells of a trachea and large bronchuses. Infiltration by inflammatory cells concerns other early implications [41] acute and chronic peribronhioljarnaja. Result of these early changes is the obturation of peripheric respiratory tracts. The viscous bronchial secret representing very concentrated oversaturated solution, brakes movements of patholologically changed cilia of an epithelium of bronchuses even more, and secret components easily drop out in a deposit. The mechanism of self-cleaning of bronchuses is as a result broken. It promotes growth of pathogenic flora and development of an inflammation, bronchiolites and a bronchitis. Within the first year of a life or later, it is frequent after the virus infection reducing efficiency of local mechanisms of antimicrobic protection, in the bottom
Departments of a respiratory tract the considerable quantity of various pathogenic microbes [85] gets.

The big role in a pathogenesis of bronchial obstruction at MV is played by bronchiectasias. They are formed because istonchennye walls of bronchuses lose the elasticity - on an inspiration they are stretched, and on an exhalation are fallen down, that promotes obstruction intensifying [40, 41, 111, 118]. Also at these patients the peribronchial sclerosis and a focal pneumosclerosis [142] develops.

Believed, that at MV the inflammation is a consequence of a chronic infection. However in research Verhaeghe C. With co-authors from Belgium, published in 2007, authentic rising of level of proinflammatory fibers in lungs at foetuses with a mucoviscidosis that proves possibility of the inflammation previous an infection [205] has been taped.

The chronic inflammation inevitably conducts to damage of pulmonary structures. In reply to an inflammation of a wall of bronchuses react a thickening and number augmentation gladkomyshechnyh cells of bronchuses, and in larger degree it is expressed in peripheric respiratory tracts. The thickening of a wall of bronchuses at patients MV in a final stage of illness has been expressed more than at the asthmatics who have died in the asthmatic status [125].

Good diagnostic results at definition of degree of an inflammation of components of respiratory system are given by research of microelement structure of a secret by classical and new methods [78].

So, Postnikovoj I.V. is estimated efficiency of a crystallographic method in which character of growth of crystals depends on structure slozhnobelkovogo gel of bioliquids [82].

The method is based on a postulate that the pathological phenomena break metabolism self-control, simultaneously to disturbance of functions there is a structural reorganisation in biological mediums of an organism that causes crystallisation changes

Components of difficult systems. By the author it is spent complex, in a native kind and with addition kristalloobrazujushchih substances (NaCl, CuCl2), crystallographic research of bioliquids at 100 children is elderly 5­15 the years, suffering various diffusive obstructive bronholegochnymi diseases (DOBZ) in comparison with control group - 30 healthy children (group of health I) similar age. The crystallographic picture at an obstructive bronchitis (ABOUT), a mucoviscidosis (MB), a bronchial asthma (BA) has allowed to allocate characteristic structural signs in each subgroup. The taped differences between a crystallographic picture at healthy children and children sick ABOUT, MB, BA, allow, according to the author, to recommend complex crystallographic research as objective diagnostic and differentsialno-diagnostic procedure.

One of methods of a set of "classical" diagnostic standards is radial diagnostics. In Pridvizhkinoj T.S.'s work the estimation of efficiency of a computer tomography of the high permission in identification of pulmonary implications of a mucoviscidosis is given. For differential diagnostics use "a method of patterns of an order in diagnostics and the forecast" is offered, are shown high informativnost a method. Kt-research has been spent to 55 patients with the established diagnosis of a mucoviscidosis. Bronchiectasias which are taped at 47 (85 %) children, a thickening of a bronchial wall - at 42 (76 %), mosaic perfusion - at 39 (71 %), tsentrilobuljarnye nodules - at 24 (38 %), mucous stoppers - at 23 (42 %) have appeared the most frequent pulmonary implications of disease. The hyperadenosis in roots of lungs - at 12 (22 %) children, atelectases - at 15 (27 %), bullas at 4 (7 %), a pheumothorax - at 2 (3,6 %) [83] was less frequent pathological implications.

Authors recognise, that KT the standard roentgenography in an estimation of prevalence, gravity and 18 is more exact method, than

Dynamics of pulmonary implications at patients with a mucoviscidosis [83, 165, 166].

Radial methods allow to investigate morphological changes in respiratory system at a mucoviscidosis [8, 165, 166].

For an estimation of disturbances of breath, undoubtedly, the functional researches allowing at earlier stages of development of disease to trace are informative and in due time to correct changes of functions of respiratory system [8, 23, 26, 97, 100].

Researches of function of external breath tap obstructive disturbances in a combination to augmentation of residual volume [64, 65]. Are possible also restriktivnye changes as result of the developed pneumosclerosis. Diffuzionnaja ability of lungs decreases at later stages of disease at the expense of a hypoventilation. On an electrocardiogram tachycardia signs, an overload of the right departments of heart on the Echo-kg [116] are visible.

In typical cases, at the baby sick MV, clinically note proof dry tussis, a fat fetid chair, a delay of physical development. Can prevail either respiratory symptoms, or the symptoms bound to a lesion of a gastroenteric tract [41].

As the characteristic complaint consider excruciating paroxysmal tussis with viscous, trudnootdeljaemoj a sputum, a dyspnea at moderate exercise stresses and in rest. Almost 60 % of patients complain of the chronic cold bound with polipozom and inflammatory process in adnexal sinuses of a nose [42, 103].

Progress in diagnostics and mucoviscidosis treatment has led to substantial growth of life expectancy at these patients. By 2001 average life expectancy of patients mukovitsidozom has made 25 years. For last decade a share of adult patients
It was enlarged by 30-40 % and has made about 30-50 % from the general number of patients [40, 111, 122].

In particular, dynamic research of indicators,

Characterising a curve a stream - volume, use as the criteria of modern correction defining life expectancy and necessity for transplantation of lungs [26, 41, 44, 111, 161, 168, 189]. Because the beginning and rate of advance of disease defines character of a current and a mortality, it is necessary to define functional changes of lungs as soon as possible. Also consider as the most sensitive indicators of a clinical condition: masso-rostovoe a parity and indicators FVD (FZHEL and ОФВ1) [8, 200]. Besides, there is an interrelation between CFTR - a genotype and a clinical, radiological and functional condition of lungs [37, 43, 89, 132]. The age in which the chronic infection joins, especially Pseudomonas aeruginosa, also is a specific risk factor for mucoviscidosis advance [41, 94, 102].

According to a number of authors, being based on functional parametres of lungs as on specific indicators of activity of the process, received as a result bodipletizmografii, it is possible to estimate advance of disease of lungs at a mucoviscidosis [18, 110, 164].

Measurement of residual pulmonary volume, rate of the forced exhalation and intrapulmonary distribution of gases were used for an estimation of type and dysfunction degree. Measurements have been executed with the help bodipletizmografii and a method of washing away of nitrogen at breath (multiple breath nitrogen washout - MBNW) [52, 53]. These techniques are referred on studying of the general capacity of lungs (OEL) and bronchial resistance that allows to diagnose as obstructive, and restriktivnye disturbances. Thanking their active introduction in clinical practice became
Possible research FVD at children after four summer age, that was not represented possible in connection with complexity of performance by children of traditional respiratory maneuvers [194] earlier. In spent scientific researches by the specific purpose definition FOE is with the help bodipletizmografii, to express a clearance of lungs (IKL) through intrapulmonary distribution of gases, to define obstructive disturbances (FOE, ОФВ1, МОС50), resistance of respiratory tracts [66, 91].

A number of researchers have proved, that ОФВ1 is the good prognostic factor for a life expectancy estimation [26, 110]. But the potential clinical importance of this indicator raises, as only on the basis of data ОФВ1 it will be possible to make the decision on transplantation of lungs [8, 64, 111, 189]. It is especially important for a category of young patients with a mucoviscidosis. Underlining complexity of data acquisition of functional measurements at the small children sick of a mucoviscidosis, Nixon and colleagues have proved, that mortality rising at infection P. Aeruginosa does not find reflexion in indicators ОФВ1 and FZHEL [47, 172]. The analysis of indicators ОФВ1 sick of a mucoviscidosis has taped, that till 13 years at patients clinical implications of obstruction are not strongly pronounced. In Wisconsin the program of neonatal screening of a mucoviscidosis in which frameworks behind group of 64 patients observation within 16 years was conducted has been framed. After the analysis of the received data V. With colleagues have found out, that the bronchial obstruction as a symptom of a lesion of lungs develops rather late [131].

The data of scientific researches cited above confirm that measurement of functions of external breath is the important part of clinical researches and the control of degree of development of a mucoviscidosis. However necessity of performance of the intelligent forced respiratory movements is essential restriction for application of such methods for small children.

Lungs at a mucoviscidosis are amazed at the earliest stages of a life. Changes of structure and functions of lungs at children proceed at first asymptomatically [16, 19, 91]. To register structural changes of lungs and anomaly of a current of a mucoviscidosis at children of preschool age use a computer tomography of the high permission (high-resolution computed tomography - HRCT) [83]. Measurement of respiratory function of lungs - the integral component of the clinical control over a current of a mucoviscidosis at children of advanced age and at adults. A spirometry most often use for the control of efficiency of spent therapy and the forecast of exacerbations at children of advanced age, but the similar method is important and for children of preschool age. In some foreign works used RTS-method when by means of a waistcoat round a thorax of the child air in the exhalation beginning is quickly forced, than the maximum expiratory effort is reached. Updatings of this method for the purpose of measurement of volume of the forced exhalation during certain time (0,5) have been offered. However these methods have not found applications in wide practice [110, 142].

Last years all over the world for an estimation of mechanical properties of lungs use a method of the forced oscillations (MFO). The method is attractive that it is non-invasive, does not depend on efforts of the patient and demands the minimum cooperation. MFO, studying behaviour of the apparatus of ventilation in a range of certain frequencies of external fluctuations of air from any source, considerably exceeding usual frequency of breath, allows to estimate the general resistance of respiratory tracts to a stream of air and its various kinds (not elastic resistance of a friction, elastic resistance, inertial resistance), that is obvious advantage of the given updating [106, 149]. At the same time research possibility at spontaneous breath, not demanding a special strain from the examinee, constantly stimulated researchers to
To perfection of a technique and working out of new devices. Brennan with co-authors has found out in children of preschool age, patients MV, disturbance of function of fine respiratory tracts which correlated with gravity of inflammatory process. Similar disturbances have been taped and at adult patients with MV [125, 200].

Impulnaja an oscillometry (IOS) - one of updatings MOF [64]. IOS is a non-invasive method for definition of the general respiratory resistance - a respiratory impedance, at quiet breath [66]. The respiratory impedance - Z consists from resistive - R and reactive - H components. Use oscillations with a frequency range from 5 to 35 Hz. For each frequency count R - the valid part of an impedance representing frictional resistance and H - an imaginary part which represents the sum of elastic and inertial resistance of the apparatus of ventilation [108]. In a studied frequency range (5-35 Hz) elastic part H is presented by negative value, and an inertial part - positive [110]. Method IOS allows not only to distinguish obstructive disturbances, but also to differentiate them on central and peripheric, i.e. to study function of fine respiratory tracts [108].

Simplicity of technics IO which is not demanding performance of maneuver FZHEL as quiet, spontaneous breath is investigated, a procedure brevity (some seconds), allow to use a method in pediatric practice at children of early age [2, 4, 5, 51, 64, 66, 97, 108].

Possibilities of a method of an impulsive oscillometry for diagnostics of early disturbances of function of external breath at teenagers are estimated as positive by a number of authors [4, 5, 51, 55]. In particular it is noticed, that the impulsive oscillometry allows to define degree of expression and
Level of obstruction of respiratory tracts at teenagers with BA at normal functional indicators by results of a classical spirometry [4].

A.V.Chernjak, E.L.Amelin have spent an estimation of a respiratory impedance and its making definition of interrelation of these parametres with the parametres traditionally used for an estimation of respiratory function, at sick of a mucoviscidosis [110]. Authors use following functional methods research: a spirometry, the general bodipletizmografija and IOS at 66 sick of a mucoviscidosis, observed in the centre of a mucoviscidosis of adults on the basis of pulmonology scientific research institute, Moscow. At sick the mucoviscidosis had been taped obstructive disturbances (ОФВ1 - 57 %долж) and a hyperinflation of lungs (OOL - 253 %долж, FOE - 158 %долж, OOL / OEL - 52 %). At carrying out IOS augmentation R which had frequency dependence (R5 - 173 %долж was revealed, R20 - 140 %долж) and depression of reactive component Х5 (-0,23 kPa / л-1/), that led to shift of resonant frequency in higher frequency range (FR - 18 Hz, AH - 1,38 kPa / l - 1). There is an authentic correlation dependence between parametres IOS (FR, X20, R5, R20, AH) and the main indicator of gravity of obstructive disturbances (with ОФВ1 - 0,74; 0,78;-0,42;-0,31 and-0,76 accordingly). The obtained data, according to authors, have shown, that measurement of a respiratory impedance allows to estimate degree of bronchial obstruction. IOS does not depend on efforts of the patient and can be used in clinical practice, including at patients with serious respiratory disturbances [110].

IOS allows to study objective characteristics of mechanical properties of the apparatus of ventilation. Definition of absolute sizes of components of an impedance in diagnostically significant frequency range gives the chance to estimate a role of various kinds of resistance of the apparatus of ventilation [64, 108, 110, 125, 127].

Now the appreciable role is taken away to creation of objective acoustic methods of an estimation of function of external breath (FVD), allowing essentially to lower level of the diagnostic mistakes inherent in traditional methods of research of lungs [144]. neinvazivnost acoustic methods of diagnostics of a pathology of respiratory system are drawn by the big attention of doctors and experts in the field of acoustics [1, 21, 24, 26, 70, 73, 74, 76, 117, 128, 135].

Bronhofonografichesky research of lungs is a method of an estimation of a pattern of the breath, based on the analysis of peak-frequency characteristics of a spectrum of respiratory hums [70, 74, 76, 100]. Simplicity and neinvazivnost bronhofonograficheskogo researches of lungs, inspection carrying out at quiet breath of the child allows to apply the given technique at children from first days of a life [97, 99, 100]. The method does not have contraindications and by-effects [70, 75, 76, 87]. The computer analysis of respiratory hums excludes subjectivity of an estimation, gives the chance to survey even the small children, since. Does not demand performance of active actions [97, 99, 100].

In Russia the group of authors had been offered computer system - Pattern, allowing to register respiratory hums [20, 70].

The given method allows to investigate dynamics of various diseases bronholegochnoj systems, to carry out the level-by-level analysis bronhofonogramm and to tap the necessary part of an acoustic pattern depending on gravity of obstructive process [23, 25, 55, 56, 58, 71, 72, 74, 76, 87, 99]. The analysis repiratornyh hums is the initial stage in diagnostics of diseases of lungs. Their competent estimation allows to focus correctly the further diagnostic search [70, 75, 76, 87, 99, 100, 138]. Many authors underline, that data of the analysis of respiratory hums well correlate data such hi-tech and expensive

Researches, as a computer tomography of the high permission. Simplicity and availability akustichekih research methods allows to estimate dynamics of treatment and to correct medical process. A combination of the data received by method BFG, to high-speed and time indicators FVD give fuller picture of ventilating disturbances. Expression of obstruction can be estimated, using the data received by a method bronhofonografii with simultaneous carrying out

Functional tests with bronhodilatatorami [72, 74].

Thus, bronhofonograficheskoe research of lungs is simple in application, a non-invasive and highly effective method in diagnostics of illnesses of organs of breath. It can be applied at children from first days of a life since does not demand forced ventilation maneuver. The computer analysis of respiratory hums excludes subjectivity of an estimation of indicators [138].

Inhalation tests with bronhodilatatorami allow to solve the questions bound to convertibility of bronchial obstruction [28, 31, 32, 35, 53, 61]. At present the question on necessity of carrying out of these tests remains opened, but a number of authors underline necessity of carrying out of similar tests because patients can have a blockade v-adrenoretseptorov bronchuses and sensitivity depression to a preparation [142, 143].

The estimation bronhodilatatsionnogo the test is spent on pure gain ОФВ1, more often. It is necessary to pay attention to reproducibility of indicators. The difference between the maximum and minimum value of indicators ОФВ1 and FZHEL should not exceed 5 %. The confidential interval at children and teenagers of 95 % that makes 190 ml, therefore a gain of indicator ОФВ1 on 190 ml and more should be estimated as the positive answer to inhalation bronhodilatatora [68, 69, 66].

Illness advance is accompanied by obstructive changes in lungs which accrue eventually. Gravity increase bronholegochnogo process leads to gas exchange disturbance, is accompanied by an anoxemia and a hypercapnia. All it as a result leads to complications from other organs and systems and, first of all, from cardiovascular system up to development of pulmonary heart. The fatal outcome of the disease occurs usually from a pulmonary heart [40, 50, 91, 111, 162]. That is why timely estimation FVD has important both diagnostic, and prognostic value, is an integral part of monitoring of a condition of the patient and is necessary for an estimation of efficiency of spent therapy [146].

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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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