In recent years, medical practitioners have increasingly encountered a condition such as bronchial obstruction. Obstruction translated from Latin means a barrier, an obstacle.
Various factors influence the onset and development of bronchial obstructive syndrome (BOS) in young children. So, a viral infection is of great importance. The viruses that most often cause obstructive syndrome include respiratory syncytial virus (about 50%), then parainfluenza virus, pneumonia mycoplasma, chlamydia, less commonly influenza viruses and adenovirus. In the development of bronchial obstruction in children, a certain role is played by age-related structural features of the bronchial tree, characteristic of children in the first years of life.
Factors such as longer sleep, frequent crying, and predominant stay on the back in the first months of life also have an undeniable effect on respiratory disorders in a young child.
Early childhood is characterized by the imperfection of many immunological mechanisms: the formation of interferon in the upper respiratory tract is significantly reduced, the level of serum immunoglobulin A (by the end of the first year of life is 28% of adult levels), as well as secretory immunoglobulin A (maximum values ​​are determined only by 10-11 years), the functional activity of the T-system of immunity is also reduced. The influence of premorbid background factors on the development of biofeedback is recognized by most researchers. This is perinatal pathology, aggravated allergic history, bronchial hyperreactivity, rickets, dystrophy, thymus hyperplasia, early artificial feeding, a respiratory disease at the age of 6-12 months.
Among environmental factors that can lead to the development of obstructive syndrome, passive smoking in the family is especially important. Under the influence of tobacco smoke, hypertrophy of the bronchial mucous glands occurs, mucociliary clearance is disrupted, the progress of mucus is slowed down. Passive smoking contributes to the destruction of the epithelium of the bronchi. Tobacco smoke is an inhibitor of chemotaxis of neutrophils. The number of alveolar macrophages under its influence increases, but their phagocytic activity decreases. With prolonged exposure to tobacco smoke affects the immune system: it reduces the activity of T-lymphocytes, inhibits the synthesis of antibodies of the main classes, stimulates the synthesis of immunoglobulins E, increases the activity of the vagus nerve. Children of the first year of life are considered especially vulnerable. Parental alcoholism also has a certain effect, namely, alcohol leads to the development of alcoholic fetopathy. It has been proven that alcohol reduces the excretion of pathogenic microbes, causes bronchial atony, and inhibits the development of a protective inflammatory reaction.
Another important adverse factor is environmental pollution. Thus, obstructive syndrome in young children is among the multifactorial diseases.
In the genesis of bronchial obstruction are various mechanisms – immune and non-immune: a) dystonia, b) hypertrophy of muscle tissue, c) hypercrinia d) dyskrinia, e) impaired mucociliary clearance (mucociliary insufficiency), e) edema, g) inflammatory infiltration, h) hyperplasia and metaplasia of the mucous membrane, and) compression, obstruction, deformation of the bronchi, j) defects in systemic and local immunity, defects in the macrophage system.
Dystonia (a violation of the muscle tone of the bronchi) can develop according to the hypertonic (bronchospasm) or hypotonic (bronchodilation) type. Bronchospasm occurs both in conditions of pathology and in the normal life of the child’s body. Under normal conditions, this is a protective reaction of the body aimed at the implementation of many physiological processes: evacuation of bronchial secretions, cough reflexes, mucus secretion from goblet cells, etc. Next to the concept of bronchospasm is the concept of bronchial hyperreactivity. The basis of hyperreactivity is an imbalance between the sensitivity of the receptor apparatus of the sympathetic and parasympathetic parts of the autonomic nervous system, shifted towards the prevalence of the latter (parasympathetic). A similar mechanism is observed in 94–97% of cases of hyperreactivity. Along with this, in some patients, the tendency to bronchospasm may be due to a high degree of sensitivity of histamine receptors. Hyperreactivity can be of primary (hereditary) or secondary (acquired) origin. Primary bronchial hyperreactivity is one of the background conditions in the development of bronchial obstruction in young children, however, it manifests itself only after repeated or prolonged exposure to various factors: passive smoking, viral infections, cold irritant, aeronautics, etc. Primary bronchial hyperreactivity can be assumed with the early development of a bronchospasm clinic (during the first half of life), and confirmed by conducting a bronchial provocation test with acetylcholine (a mediator of the parasympathetic nervous system) in parents. Viral infections, physical and chemical agents, hypothermia, physical stress and barometric fluctuations are among the factors that can cause the formation of nonspecific (secondary) hyperreactivity of the bronchial tract. It is based on numerous changes in ARVI, but the main role belongs to the destruction of bronchial epithelium with the subsequent development of increased sensitivity of irritative receptors.
However, it should be noted that the formation of hyperreactivity in acute respiratory viral infections does not occur in all patients with viral diseases, and therefore the purely hereditary nature of this phenomenon is assumed. The ability to develop bronchospasm can also be promoted by the altered reactivity of target cells (“mast cell membrane instability state”). This phenomenon can be formed under the influence of infectious agents, histaminoliber agents, a cold irritant, or hereditarily determined. As a result, biologically active substances are released without a previous immunological phase (pseudo-allergic reaction). Thus, the factors contributing to the development of bronchospasm include: infectious, allergic, psychogenic factors, changes in the concentration of physical and chemical substances in exhaled air, hyperventilation, physical activity, mechanical irritation of the respiratory tract, reflexes of internal organs. Therefore, there are a large number of reasons that in different ways can cause a similar reaction from the side of the bronchial tree – bronchospasm, which is clinically realized as an obstructive syndrome. Young children, despite the poor development of the smooth muscle system of the bronchi, can give a typical, clinically pronounced bronchospasm. In pathological conditions, hypertrophy of the muscle tissue of the bronchi, mucosal hyperplasia, which subsequently contribute to the development of recurrent bronchospasm, can occur. Hypotonic dystonia can occur as a result of a prolonged course of bronchitis or suppurative process, also with severe systemic hypotension.
Violation of bronchial secretion (dyskrinia and hypercrinia) may be one of the causes of bronchial obstruction. The activity of the mucous and serous glands is regulated by the parasympathetic nervous system, acetylcholine stimulates their activity. The mucus covering the epithelium of the respiratory tract, performs the function of protecting the bronchial tree from damaging agents. Mucus secretion is carried out by the glands of the trachea and bronchi. The composition of the secret includes a number of bactericidal substances: interferon, transferrin, surfactant, immunoglobulins. In addition, the secretion includes exudate and transudate plasma, products of degeneration of cells and microorganisms, proteins, lipids, carbohydrates, DNA.
During the day, about 100 ml of mucus is secreted by a healthy person, which he swallows. With tracheobronchial secretion, inhaled particles, metabolic products, bacteria and viruses are released from the respiratory tract. All this provides protection of the respiratory tract from various damaging factors (bacteria, viruses, physical and chemical factors). In pathological conditions, significant quantitative and qualitative changes in the tracheobronchial secretion are observed. So, any adverse effect on the respiratory system, in most cases, causes an increase in the amount of secretion and an increase in its viscosity. Such a reaction is protective in nature and aims to protect the thin cover of the ciliary epithelium, to prevent access of the damaging agent to deeper parts of the bronchial tree and remove it from the body. Of particular importance is the exposure time of the damaging factor. So, if its effect was short-term, then hypersecretion can be reversible. If the exposure was long or the exposure to the damaging factor was of great strength, then the increased activity of the secreting system is fixed and in the future does not decrease to normal. The process of mucus formation is mainly associated with the function of goblet cells, the number and size of which increase significantly. In pathological conditions, transformation of some cells into goblet can occur, which also enhances mucus formation. At the same time, physiological methods of removing the tracheobronchial secretion become insufficient, which leads to airway obstruction by accumulating mucus and the development of the inflammatory process. The accumulation of viscous mucus can contribute to the development of the infectious process in the bronchial tree (purulent endobronchitis). The emergence of a large layer of secretion on top of epithelial cells greatly complicates the protective function of the respiratory tract, performed by the apparatus of ciliated cells (mucociliary clearance). The movement of mucus is carried out mainly due to the movement of the cilia of the epithelium of the bronchi and trachea. The ciliated epithelial cell has about 200 cilia, which produce up to 250 vibrations per minute. Optimal conditions for the functioning of the ciliated epithelium: Temperature from 18 to 37 degrees, pH = 7.5. Mucociliary transport is influenced by circadian rhythms, as well as mediators of the autonomic nervous system. Under the action of catecholamines, the activity of cilia increases, while under the action of atropine it decreases.
Primary immunity (immobility) of the ciliary apparatus can cause chronic rhinitis, sinusitis, otitis media, productive cough, as well as bronchitis. However, there is not always a parallelism between the severity of clinical manifestations and the degree of disruption of the ciliary apparatus. Negatively affects the function of ciliated epithelium, increasing the viscosity of mucus, inhalation of a number of chemicals (excess oxygen, ammonia, proteolytic enzymes, etc.). Damage to the ciliated epithelium can be caused by viruses, bacteria, fungi. Thus, infection with influenza viruses causes total desquamation of the bronchial epithelium, up to the basement membrane, which leads to the formation of “bald spots”, often significant in area. The breaks resulting from this in the escalator ladder contribute to the appearance of foci of mucostasis, “mucous plugs”.
Edema and hyperplasia of the mucous membrane of the respiratory tract are also one of the causes of obstruction of the respiratory tract. Developed lymphatic and circulatory systems of the respiratory tract of the child provide him with many physiological functions. However, under conditions of pathology, a swelling characteristic is the thickening of all layers of the bronchial wall — the submucous and mucous layers, the basement membrane, which leads to impaired bronchial patency. With recurrent bronchopulmonary diseases, the structure of the epithelium is disturbed, its hyperplasia and squamous metaplasia are noted.
In young children, compression, obstruction and deformation of the bronchi can often be observed. First of all, the anatomical and physiological features of the bronchial tree are predisposed to this. For example, a relatively narrow and long midlobar bronchus is easily compressed by the enlarged lymph nodes of the lung root. Obstruction of the bronchi by mucus, foreign bodies, etc.
is of some importance . Inflammatory infiltration is an important factor in mechanical obstruction. Inflammation can be caused by allergic, infectious, toxic, physical and neurogenic effects. Active participation in inflammatory reactions is played by eosinophils, macrophages, neutrophils, platelets that secrete biologically active substances (BAS), in particular platelet aggregation factor, inducing platelet aggregation, excretion of serotonin, histamine, which leads to bronchospasm, impaired respiratory microcirculation pathways, and from here to the exit of plasma, enhances the production of IgE, causes non-specific bronchial hyperreactivity. Inflammatory cell infiltration with eosinophils and neutrophils with subsequent involvement of mononuclear cells is often mediated by mast cell degranulation and occurs several hours after the release of biologically active substances. Undoubtedly, the participation of the nervous system in the development of inflammation. In case of damage to the bronchial epithelium, sensitive nerve endings become available, upon irritation of which the release of neuropeptides-mediators of the non-cholin- and non-adrenergic nervous systems, playing an important role in the development of neurogenic inflammation. Local inflammation in the bronchi is also regulated by the central nervous system. Monocytes secrete interleukin-1, which, when released at the site of inflammation, enters the brain. Inflammation is the main cause of the formation of bronchial hyperreactivity. Hyperresponsiveness of the bronchi is formed regardless of what cause caused inflammation of the bronchi. Inflammatory infiltration is a reversible process.
Defects of local and systemic immunity can underlie BF in children, although bronchial obstruction is not strictly specific for immunodeficiency states. In immunodeficiency states in the B-system of immunity, in violation of the phagocytic function of neutrophils and macrophages, in deficiency of secretory immunoglobulins, the genesis of bronchial obstruction is caused by endobronchitis, the development of dyskinesia and bronchial tree dyskrinia. For example, the protective effect of secretory immunoglobulin A is manifested by its ability to prevent the adhesion of bacteria to the mucous membrane of the bronchi, as well as agglutinate bacteria, neutralize toxins and viruses. With a defect in this line of defense, purulent endobronchitis develops, accompanied by inflammatory infiltration, dyskrinia, hyperplasia and epithelial metaplasia, dystonia.
With a tendency to increased production of immunoglobulin E, combined with a decrease in the number of T-suppressors, there is a fixation of immunoglobulin E molecules on the mast cell membrane, followed by their degranulation, release of biologically active substances and the development of a triad of mechanisms – spasm, hypersecretion, edema with prevailing bronchospasm.
Thus, the main mechanisms for the development of bronchial obstruction were considered, and the specific gravity of each of them depends on the cause of the pathological process.
Based on the literature and our own observations, we can distinguish the following groups of diseases accompanied by syndrome of bronchial obstruction:
1. Respiratory diseases:
1.1. Infectious and inflammatory diseases (acute respiratory infections, bronchitis, bronchiolitis, pneumonia)
1.2. Allergic diseases (asthmatic bronchitis, bronchial asthma)
1.3. Bronchopulmonary dysplasia
1.4. Malformations of the bronchopulmonary system
1.5. Tumors of the trachea and bronchi
2. Foreign bodies of the trachea, bronchi, esophagus
3. Diseases of the aspiration genesis (or aspiration obstructive bronchitis) – gastroesophageal reflux, tracheoesophageal fistula, malformations of the gastrointestinal tract, diaphragmatic hernia
4. Diseases of the cardiovascular system of the congenital and nature (CHD with hypertension of the pulmonary circulation, anomalies of large vessels, congenital non-rheumatic carditis, etc.)
5. Diseases of the central and peripheral nervous system:
5.1. Cranio-spinal injury
5.2. Cerebral palsy
5.3. Myopathies
5.4. Neuroinfections (poliomyelitis, etc.)
5.5. Hysteria, epilepsy, etc.
6. Hereditary metabolic abnormalities:
6.1. Cystic fibrosis
6.2. Malabsorption Syndrome
6.3. Rickets-like diseases
6.4. Mucopolysaccharidoses
6.5. Alpha-1-antitrypsin deficiency, etc.
7. Congenital and acquired immunodeficiency conditions
8. Rare diseases: Piper syndrome, Lawrence-Moon-Barde-Beadle syndrome, Cartagener syndrome, etc.
9. Other conditions:
9.1. Injuries and burns
9.2. Poisoning
9.3. Exposure to various physical and chemical environmental factors
9.4. Compression of the trachea and bronchi of extrapulmonary origin

In the course of bronchial obstructive syndrome (BOS) can be acute, protracted, recurring and continuously recurring (in the case of bronchopulmonary dysplasia, obliterating bronchiolitis, etc.). The severity of obstruction can be distinguished: mild obstruction (1 degree), moderate (2 degree), severe (3 degree).
Despite the variety of reasons, bronchial obstruction syndrome is characterized by the following most typical clinical symptoms: prolonged expiration (in severe cases and increased respiratory rate), the appearance of expiratory noise (wheezing, noisy, sawing, wheezing), in most young children – participation in the act respiratory support muscles. It must be remembered that biofeedback is not an independent diagnosis, but is a syndrome of a disease, the nosological form of which in all cases should be clarified with the help of clinical and medical data, laboratory and instrumental examination methods (examination of peripheral blood, immunological status, allergological tests, bronchoscopy , bronchography, radiography of the respiratory system, examination of the function of external respiration, scintigraphy, angiopulmonography, computed tomography, etc.)
Severe cases of bronchial obstruction, as well as repeated cases of the diseases occurring with this syndrome, require clarification of the genesis of biofeedback, adequate treatment, prevention and predicting the further course of the disease.
BRONCH OBSTRUCTIVE SYNDROME OF INFECTIOUS GENESIS
Acute bronchial obstructive syndrome often occurs in young children with viral and viral-bacterial infections of the respiratory tract (ARI). In the genesis of bronchial obstruction in acute respiratory infections, mucosal edema, inflammatory infiltration, and hypersecretion are of primary importance. The mechanism of bronchospasm is less pronounced , which is due to either an increased sensitivity of the interoreceptors of the cholinergic link of the ANS (primary or secondary hyperreactivity), or a blockade of β2-adrenergic receptors. A distinct effect of a number of viruses on increasing the level of IgE and IgG, inhibition of T-suppressor function of lymphocytes was noted. In world literature, the separation of acute obstructive bronchitis and bronchiolitis is not recognized by all pulmonologists. However, in our country, in the classification of 1981, acute obstructive bronchitis and bronchiolitis are distinguished into independent nosological forms. Acute obstructive bronchitis can cause any viruses, as well as mycoplasma pneumonia, chlamydia. The clinical picture of biofeedback does not depend on the type of virus, however, with the introduction of adenovirus, mycoplasma pneumonia, chlamydia, as well as with the layering of a bacterial infection, a prolonged course of bronchial obstruction is possible. In the genesis of obstruction, edema, hypersecretion leads, with bacterial infection, the development of purulent endobronchitis is characteristic. The phenomena of bronchospasm against the background of acute respiratory infections are expressed minimally and have no effect on the clinical picture. Acute obstructive bronchitis often develops in young children, but may be in children of preschool and school age. However, the older the child, the more often there is a need for a differentiated approach in assessing BF. In older children, the first episode of biofeedback may be based on an allergic nature. In most cases, acute obstructive bronchitis is characterized by an acute onset, fever up to febrile numbers, mucous rhinitis, a short dry cough with a rapid transition to wet, the presence of intoxication phenomena – refusal of the chest, loss of appetite, poor sleep, the child becomes lethargic, capricious . On days 2-4, already against the background of pronounced catarrhal phenomena and an increase in body temperature, bronchial obstructive syndrome develops: expiratory dyspnea without pronounced tachypnea (40-60 breaths per minute), oral crepitus, sometimes distance rales in the form of noisy, wheezing breathing, box-like shade sound, with auscultation, an extended exhalation, dry, buzzing rales, wet rales of various sizes on both sides. Bronchial obstructive syndrome lasts for 3-7-9 or more days depending on the nature of the infection and gradually disappears in parallel with the subsidence of inflammatory changes in the bronchi.
The basis of treatment is therapy aimed at improving the drainage function of the bronchi, which includes the following: active oral rehydration, inhalation with mucolytics (2% soda solution, 1% salt solution, physiological solution, mineral alkaline water), distracting procedures (hot manual and foot baths with a gradual increase in temperature from 37 to 41 degrees for 10-15 minutes, hot wraps according to Zalmanov, zone can massage, thermal procedures with home remedies for the chest (mustard, fir oil and other remedies with a pungent odor are excluded), expectorants (bromhexine and its analogues, thermopsis, terpinghydrate, potassium iodide from 3-4 days of illness, herbs and other drugs with a mucolytic effect; antitussive drugs, for example, glaucine, glauvent, libexin, stoptussin, as well as broncholithin and solutan are excluded), massage, postural drainage, cough gymnastics, electrical procedures (in the absence of the effect of the above therapy), anti-inflammatory drugs can be differentially applied. The use of antispasmodics (aminophylline and its analogues, no-spa, adrenergic agonists) is either ineffective (in cases where hypersecretion is leading in the genesis of biofeedback), or gives a small effect (if edema prevails in the genesis). With obstructive bronchitis in children with rickets, it is necessary to simultaneously treat rickets. With severe respiratory failure, oxygen therapy. Antibiotic therapy is not mandatory. The approach to the appointment of these funds should be strictly individual.
Indications for the use of antibacterial drugs can be the following signs: prolonged hyperthermia, lack of effect from the therapy, the presence of areas of persistent hypoventilation in the lungs and / or asymmetry of physical data, increased toxicosis, signs of brain hypoxia, the appearance of purulent sputum, uneven increase in the pulmonary pattern on the radiograph, and in blood tests, leukocytosis, neutrophilia, an increase in ESR, sensitization by previous frequent acute respiratory infections or a disease transferred shortly before the present episode. In such cases, one should refrain from using penicillin, such as an antibiotic, which has a high degree of sensitizing activity. Frequent repeated acute respiratory infections or severe once-transmitted infection (pertussis, mycoplasma, adenovirus infection) lead to pathological dyskrinia, hypercrinia, epithelial metaplasia, purulent endobronchitis, which contributes to the persistence of the bacterial flora and the maintenance of prolonged catarrhal and obstructive pulmonary syndrome. In this case, inpatient treatment and examination with sampling of material from the bronchial tree is necessary to assess the nature of the cytogram, as well as to identify the causative bacterial flora with a determination of its sensitivity to antibiotics.
With repeated episodes of biofeedback on the background of acute respiratory infections, a differentiated approach to assessing the causes of recurrence of bronchial obstruction. In some children, after a single biofeedback, subsequent acute respiratory infections occur with symptoms of bronchial obstruction, that is, biofeedback takes a relapsing course. Contributes to this, as a rule, to some extent aggravated premorbid background: prematurity, alcoholic fetopathy, rickets, dystrophy such as malnutrition, perinatal encephalopathy. In these situations, another mechanism, the dystonia of the bronchial tree of the hypotonic type, is attached to the already known obstruction mechanisms.
In cases of alcoholic fetopathy, dyskrinia and dyskinesia are congenital. Only a detailed correction of disorders arising from these diseases can give a positive effect in the future and contribute to the cessation of relapse. In some patients, repeated episodes of biofeedback can occur not only with a viral infection, but also when exposed to certain non-specific factors, such as cold, physical exertion, hyperventilation, pungent odors, and other irritant factors. In this case, the biofeedback is caused by bronchospasm of the tonic type, which is associated with the development of nonspecific bronchial hyperreactivity. In the literature indicate various periods of the duration of this phenomenon, from 7 days to 3-8 months. In this case, treatment should be aimed at eliminating the imbalance of the ANS.
Acute bronchiolitis is observed mainly in children of the first half of life, but can occur up to 2 years. More often due to respiratory syncytial infection. With bronchiolitis, small bronchi, bronchioles, and alveolar passages are affected. The narrowing of the lumen of the bronchi and bronchioles, due to edema and cellular infiltration of the mucous membrane, leads to the development of severe respiratory failure.
Bronchospasm with bronchiolitis is not of great importance, which is confirmed by the lack of effect from the use of bronchospasmolytic drugs. The clinical picture is determined by severe respiratory failure: perioral cyanosis, acrocyanosis, tachypnea up to 60-80-100 breaths per minute, with the prevalence of the expiratory component, “oral” crepitus, retraction of compliant places of the chest. Percussion over the lungs is determined by the boxed shade of the percussion type; during auscultation – a lot of small moist and crepitious wheezing in all fields of the lungs during inhalation and exhalation, exhalation is elongated and difficult, with shallow breathing, exhalation can have a normal duration with a sharply reduced tidal volume. This clinical picture of the disease develops gradually, over several days, less often acutely, against the background of acute respiratory infections and is accompanied by a sharp deterioration in the condition. In this case, a cough of a paroxysmal nature occurs, appetite decreases, anxiety appears. The temperature is often febrile, sometimes subfebrile or normal. An X-ray examination of the lungs reveals bloating of the lungs, a sharp increase in bronchial pattern with a high prevalence of these changes, high standing of the dome of the diaphragm, horizontal location of the ribs. The cyclical nature (10-14 days) and the absence of repeated episodes are characteristic. However, during follow-up observations it was found that a significant part of children (according to S.V. Rachinsky et al. More than 50%) subsequently go on to bronchial asthma. Treatment of acute bronchiolitis includes a number of measures aimed at combating respiratory failure. A mandatory method of therapy is oxygen therapy in an oxygen tent.
To replenish water loss during hyperventilation, it is desirable to use the method of oral rehydration, which is considered less invasive compared to intravenous drip infusion and makes it possible to conduct continuous oxygen therapy. The best drug used for this purpose is rehydron. To combat viral infection, the use of interferon and its analogues is indicated. Most modern authors, despite the viral nature of bronchiolitis, consider it necessary to prescribe antibiotic therapy in the presence of symptoms of severe respiratory failure and difficulties in eliminating secondary bacterial infection. Indications for antibiotic therapy are the same as for obstructive bronchitis. It is better not to use antihistamines with bronchiolitis taking into account their atropine-like effect. Perhaps the use in severe cases of hormonal therapy (the first 3-5 days of illness).
Obliterating bronchiolitis is characterized by a frequent transition to a chronic disease. A widespread lesion of the epithelium of bronchioles occurs with the subsequent organization of exudate and granulomatous reaction, and then obliteration of their lumen. The etiology of obliterating bronchiolitis is predominantly viral (adenovirus, pertussis virus, measles, etc.). Mostly children of the first years of life suffer. The mucous membrane in the bronchioles and small bronchi is completely destroyed, their lumen is filled with vascularized fibrous tissue. Obliteration usually captures terminal bronchioles, the lumen of the bronchioles below the site of occlusion is often extended, endarteritis develops with a pronounced narrowing of the branches of the pulmonary artery. With widespread obliteration, alveolar tissue is in a state of atelectatic sclerosis. Sometimes the airiness of the alveolar tissue is maintained. The genesis of pulmonary changes in these cases is associated with a secondary violation of capillary blood flow.
The clinical picture of obliterating bronchiolitis proceeds cyclically. In the first (acute) period, clinical signs are observed that are characteristic for the course of acute broncholitis, but with more pronounced disorders. In addition, respiratory failure in these cases persists for a long time and even rises within 2 weeks, the temperature keeps on febrile numbers. On radiographs, “cotton” shadows are determined. Pronounced obstructive phenomena occur after normalization of temperature. In the second period, the child’s well-being improves, but the phenomena of obstruction remain pronounced, moist rales of various sizes, wheezing on exhalation are heard in the lungs. Obstruction may periodically worsen, sometimes resembling an asthmatic attack. After 6-8 weeks, the phenomenon of “translucent lung” is formed in some children. A diffuse lung lesion with a progressive course is possible. Treatment of obliterating bronchiolitis is very difficult due to the lack of etiotropic drugs. Antibiotics in the acute period do not prevent persistent obliteration of bronchioles. Corticosteroid drugs are already ineffective in the current sclerotic process, therefore, their use in the early stages with a particularly severe course of acute bronchiolitis (prednisone at the rate of 2-3 mg / kg) can be recommended. In the second period, the dose of prednisone is gradually reduced, in the period of increased obstruction, aminophylline and sympathomimetics are prescribed, postural drainage and vibration massage are used.
BOS in acute pneumonia is rare and is due to the development of bronchitis, according to our data, only in 2.6% of cases. Moreover, the picture of bronchial obstruction is typical and is combined with the clinical and radiological picture of bronchopneumonia. According to Tatochenko V.K. et al. (1981) the presence of obstructive syndrome in the early stages of acute respiratory viral infections allows to exclude pneumonia with a probability exceeding 95%.
BRONCH-OBSTRUCTIVE SYNDROME OF ALLERGIC GENESIS.
Currently, doctors are increasingly faced with bronchial obstruction of allergic genesis, that is, with such nosological units as bronchial asthma and asthmatic bronchitis. These forms of respiratory allergosis can be diagnosed in the first year of life. Obstruction in these diseases is due to two underlying mechanisms: hyperreactivity of the bronchial tree and inflammation of the mucous membrane. Bronchospasm, which gives clinical symptoms of the disease, is a consequence of these two processes, as well as edema, dyskrinia, hypercrinia, which are less pronounced.
Currently, most researchers recognize the heterogeneity of bronchial asthma and asthmatic bronchitis. The etiological factors can be various allergens: house dust, pollen of plants and trees, dandruff and animal dander, medicines, food, dry food for aquarium fish, etc. Non-specific factors, such as physical activity, cooling, sudden changes in weather conditions, smells, chemical agents, and mental stress can also provoke attacks. The effect of these agents is associated with hyperreactivity of the receptor apparatus, due to the imbalance between the adrenergic and cholinergic links of the ANS.
In young children, hyperreactivity is more often primary. A number of patients showed a clear infectious dependence of exacerbations (seizures) against the background of respiratory viral infections, which complicates the diagnosis of the diseases under discussion.
Significant difficulties are presented by the differential diagnosis between asthmatic bronchitis and obstructive bronchitis of an infectious genesis. In favor of asthmatic bronchitis there is aggravated heredity, aggravated by the allergic history itself (skin manifestations of allergy, “small” forms of respiratory allergosis – allergic rhinitis, laryngitis, tracheitis, bronchitis the presence of a connection between the occurrence of the disease and a causative allergen, the absence of such a connection with the infection, the positive effect of elimination, recurrence of seizures, and their uniformity.The following symptoms are characteristic of the clinical picture: the absence of intoxication phenomena, distant wheezing or sawing breathing, expiratory dyspnea involving auxiliary мускулатуры, в легких выслушиваются сухие свистящие хрипы и немногочисленные влажные, количество которых увеличивается после купирования бронхоспазма. Приступ возникает, как правило, в первый день заболевания и ликвидируется в короткие сроки, в течение 1-3 дней. В пользу астматического бронхита также свидетельствует положителльный эффект на введение бронхоспазмолитиков (ксантины, адреномиметики и др.). Бронхиальная астма должна рассматриваться как хроническое воспалительное заболевание аллергического генеза. Характерным признаком бронхиальной астмы является приступ удушья, который у детей раннего возраста проявляется резко выраженным беспокойством ребенка («мечется», «не находит себе места»), вздутием грудной клетки, фиксацией плечевого пояса в фазе вдоха, тахипноэ с незначительным преобладанием экспираторного компонента, нарушение проведения дыхания в базальных отделах легких, выраженным периоральным цианозом.
В терапии бронхиальной астмы и астматического бронхита применяются два вида медицинских препаратов: бронходиляторы и противовоспалительные средства. Теофиллины, в2-агонисты обеспечивают только снятие бронхоспазма (прекращают спастическое сокращение мышц бронхиальных стенок) и не влияют на патологический процесс, лежащий в основе заболевания—это препараты острого периода. Главенствующим в терапии должно быть лечение воспалительного процесса на ранней стадии заболевания. Противовоспалительные препараты применяются как базисные, профилактические средства, прерывающие развитие бронхиальных воспалительных процессов. К этим препаратам относятся—интал, дитек, задитен, тайлед, ингаляционные кортикостероиды. Терапия проводится длительно.
БРОНХОЛЕГОЧНАЯ ДИСПЛАЗИЯ (БЛД).
Бронхолегочная дисплазия — это форма хронического заболевания легких у новорожденных и грудных детей. Она отчасти является ятрогенным заболеванием. В этиологии БЛД участвуют многие факторы: воздействие кислорода в высоких концентрациях, ИВЛ, отек легких, шунт слева направо при открытом артериальном протоке, повторные бактериальные пневмонии.
Высокая концентрация вдыхаемого кислорода приводит к гипертрофии слизистой оболочки, слизисто-реснитчатой дисфункции, задержке секрета, что приводит к обструкции воздухоносных путей с возможным выходом в последующем на ателектазы, кистозную эмфизему.
Положительное давление в воздухоносных путях способствует разрыву дистальных воздухоносных путей, приводящему к обструкции бронхов, а в дальнейшем и к перибронхиальному фиброзу.
Таким образом, у детей с БЛД трахеобронхиальные изменения следует рассматривать как причину персистирующих нарушений паренхимы легких.
У многих детей с БЛД отмечаются свистящие хрипы и цианоз в сочетании со вздутием грудной клетки, видимом на рентгенограмме; признаки легочной гипертензии, как результат увеличения легочного сосудистого сопротивления в следствие хронической гипоксии, низкого объема легких, облитерации легочного сосудистого русла; отставание в росте и массе; неврологические нарушения разной степени выраженности.
Смертность детей, страдающих БЛД, в первые годы жизни колеблется от 30 до 50%. Причиной смерти является прогрессирующая гипоксемия, пневмония, пневмоторакс и сердечная недостаточность. Среди отдаленных последствий БЛД могут наблюдаться обструкция и гиперреактивность дыхательных путей.
БРОНХООБСТРУКТИВНЫЙ СИНДРОМ ПРИ ПОРОКАХ РАЗВИТИЯ БРОНХОЛЕГОЧНОЙ СИСТЕМЫ.
Частота пороков развития у больных с хроническими заболеваниями легких колеблется от 1,4-10 до 20-50% по данным различных авторов. БОС при пороках развития нередко выявляется уже на первом году жизни на фоне впервые возникшего инфекционного процесса в дыхательных путях. У части детей обструктивный синдром может выявляться и без инфекционного поражения органов дыхания. Необходимо отметить, что пороки бронхолегочной системы характеризуются большим разнообразием, поэтому остановимся только на некоторых из них.
Аномалии ветвления бронхов могут способствовать изменению аэродинамических характеристик воздушного потока и трансформировать течение заболеваний органов дыхания.
Стенозы трахеи могут быть связаны как с врожденными дефектами ее стенки, так и со сдавлением извне. Сдавление трахеи могут вызвать аномалии развития аорты и ее ветвей, аномалии легочной артерии, увеличенная вилочковая железа, врожденные кисты и опухоли средостения. При аномалии развития аорты или ее ветвей, сосуды, охватывая трахею и пищевод, вызывают сужение их просвета.
В случае значительного сужения трахеи сосудистым кольцом, дети рано начинают болеть пневмонией, которая у них принимает затяжное течение и сопровождается бронхообструктивным синдромом. Характерна поза ребенка— запрокинутая голова, что уменьшает давление на трахею. Для этих больных характерно сочетание бронхиальной обструкции и дисфагии.
При сдавлении пищевода нарушается глотание и при приеме пищи нарастает бронхиальная обструкция. В клинике стеноза трахеи на первый план выступает экспираторный стридор, иногда смешанный, участие вспомогательной мускулатуры в акте дыхания, приступы цианоза и асфиксии. Стридор может усиливаться при физической нагрузке, беспокойстве, приеме пищи и особенно при ОРЗ. Наблюдающееся шумное дыхание может иметь различный характер: «хрипящее», «трещащее», «пилящее». В некоторых случаях наблюдается упорный «спастический» бронхит. Бронхолегочный процесс носит рецидивирующий или непрерывно-рецидивирующий характер. У части детей клиническую картину стеноза в последующем затушевывает хронический бронхолегочный процесс. У новорожденных клиника неотчетлива и может проявляться симптомами недостаточности кровообращения или раздражения центральной нервной системы. В дальнейшем симптомы врожденного стеноза впервые могут проявиться на фоне респираторного заболевания или аллергического набухания слизистой оболочки. Диагностика стеноза трахеи базируется на клинико- рентгенологических и эндоскопических данных. Из рентгенологических методов используются томография, контрастная трахеобронхография, обязательным является контрастное исседование пищевода, трахеобронхоскопия, в случае аномалии аорты—аортография.
Синдром Вильямса-Кембелла (СВК) проявляется генерализованными бронхоэктазами, обусловленными дефектом бронхиальных хрящей на уровне от 2 до 6-8 генераций. При СВК выявляют облитерирующий бронхиолит, являющийся следствием инфекции, наслоившейся на врожденную основу. Клиническая картина СВК характеризуется наличием бронхиальной обструкции и бронхолегочной инфекции. Клиническая симптоматикка проявляется чаще всего на первом году жизни. Заболеванию обычно предшествует респираторная инфекция. Начало заболевания чаще всего острое и сопровождается тяжелой дыхательной недостаточностью. Характерными клиническими признаками являются: постоянная одышка, усиливающаяся на физическую нагрузку, дистационные свистящие хрипы, кашель приступообразный с отхождением мокроты, центрально расположенная горбовидная деформация грудной клетки, «барабанные палочки», «часовые стекла», отставание в физическом развитии; перкуторно—коробочный звук; аускультативно—ослабленное повсеместно дыхание, сухие свистящие, жужжащие, разнокалиберные влажные хрипы. Рентгенологически—вздутие грудной клетки. Диагностика основана на бронхологическом исследовании. При бронхоскопии отмечается смыкание хрящевой и мембранной стенок крупных бронхов. При бронхографии находят распространенные расширения бронхов, начиная с субсегментарных. СВК необходимо дифференцировать с бронхиальной астмой. Прогноз при СВК неблагоприятный. Больные обычно погибают от прогрессирующей легочно-сердечной недостаточности. Лечение консервативное. При обострении—антибактериальные средства и средства, способствующие улучшению дренажной функции легких (ЛФК, постуральный дренаж, массаж, ингаляции с бронхолитическими и муколитическими средствами).
Врожденная лобарная эмфизема (ВЛЭ)—порок развития, в основе которого лежит сужение бронха с вентильным механизмом и развитием в результате этого эмфиземы соответствующего участка легкого. Нарушение бронхиальной проходимости обусловлено различными причинами: врожденный дефект бронхиальных хрящей, гипотрофия слизистой оболочки бронха с образованием складок и слизистых пробок, сдавление бронха извне бронхогенными кистами, опухолью, аномально расположенными сосудами и т.д. Однако одной из причин может быть врожденная патология самой легочной паренхимы, заключающаяся в гипоплазии эластических волокон. В результате этих изменений из доли легкого при выдохе удаляется меньше воздуха, чем поступает при вдохе, повышается внутрилегочное давление и паренхима пораженной доли перерастягивается. Возникает типичная обструкция. Основным клиническим проявлением заболевания является дыхательная недостаточность. При декомпенсированной форме наблюдаются приступы удушья с цианозом, одышкой, тяжелой легочно- сердечной недостаточностью. Над патологически измененной долей легкого перкуторный звук—коробочный, дыхание ослаблено или не прослушивается. Средостение смещено в здоровую сторону. В диагностике решающее значение имеет рентгенологическое обследование, ангиопульмография.
Бронхолегочное обследование при ВЛЭ не имеет большой информативности. Лечение—хирургическое—удаление пораженной доли—в основном при «синдроме напряжения». В компенсированных случаях лечение консервативное, «выжидательная тактика».
БРОНХООБСТРУКТИВНЫЙ СИНДРОМ ПРИ АСПИРАЦИИ ИНОРОДНЫХ ТЕЛ.
Аспирация инородных тел в детском возрасте не редкое явление. Наибольшее число аспираций отмечается в возрасте от 1 года до 3 лет (54%). Распространение инородных тел в трахеобронхиальном дереве зависит от величины, формы инородного тела, характера его поверхности и возможности перемещения по трахеобронхиальному дереву. По данным литературы инородные тела чаще локализованы в правом легком (от 54 до 70%). Осложнения, возникающие после аспирации инородных тел, могут появляться в различные сроки и иметь разный характер. Так, пневмония регистрируется в 23-25% случаев, трахеобронхит—в 14%, ателектаз—в 11%, бронхоэктатическая болезнь с ателектазами—в 3,2% и т.д. Успех в лечении этих осложнений зависит от своевременности эндоскопического удаления инородных тел.
Симптоматика инородных тел дыхательных путей в детском возрасте разнообразна и зависит от ряда факторов: общей реактивности организма и возраста больного, локализации и характера инородного тела, длительности пребывания аспирированного тела и инфицированности. Несмотря на разнообразие клинических симптомов, из них можно выделить наиболее характерные для определенной локализации инородного тела в дыхательных путях. Основными симптомами инородного тела в области гортани являются инспираторная одышка, осиплость голоса или афония, развитие удушья. Помогает диагностике, помимо ларингоскопии, трахеоскопии, указание в анамнезе на внезапное развитие клиники заболевания на фоне полного здоровья. Дети с измененной функцией гортани (дыхательной, голосообразующей) должны быть немедленно госпитализированы. Несколько чаще встречаются инородные тела трахеи—от 43 до66% (против 2,9-18% в случаях локализации в гортани). В этих случаях инородное тело может перемещаться между подскладочным пространством и бифуркацией трахеи. В момент аспирации возможен приступ удушья, отмечается приступообразный кашель. При локализации инородного тела в бронхах происходит рефлекторный спазм бронхиол, что клинически выражается внезапным появлением бронхиальной обструкции. Перкуторные и аускультативные данные, в отличие от бронхиальной обструкции другого генеза, носят четкий ассиметричный характер—ослабление дыхания соответствует зоне, в которой инородное тело вызвало гиповентиляцию. Рентгенологически можно определить тень аспирированного предмета, ателектаз, смещение средостения. Если инородное тело небольшое, проникло через голосовую щель и фиксировалось в одном из бронхов, то дыхание становится свободным, ребенок успокаивается после приступа кашля. Развитие БОС в этом случае может быть постепенным—локальный бронхит трансформируется в диффузный, что осложняет диагностику. При полной обтурации бронха развивается ателектаз. Постановке диагноза помогает тщательно собранный анамнез. В этой связи особо необходимо отметить клинические признаки, характерные для инородных тел у детей раннего возраста:
1. Недоразвитие рефлексогенных зон гортани, ее высокое стояние, воронкообразная форма способствуют бессимптомному проникновению инородного тела в дыхательные пути;
2. Одним из ярких симптомов является рвота, нередко многократная, что может симулировать проглатывание инородного тела, а не его аспирацию;
3. Самостоятельный выход инородных тел крайне редок;
4. Характерно быстрое развитие бактериальных осложнений (от нескольких часов до 1-2 суток, особенно в случае аспирации инородных тел органического характера), которые протекают с выраженным гнойным эндобронхитом на стороне поражения и последующим развитием пневмонии, принимающей затяжное течение;
5. Частое развитие бронхообструктивного синдрома.
БРОНХООБСТРУКТИВНЫЙ СИНДРОМ АСПИРАЦИОННОГО ГЕНЕЗА.
В основе бронхообструктивного синдрома аспирационного генеза могут лежать различные заболевания и состояния: гастроэзофагальный рефлюкс, трахеопищеводный свищ, пороки развития желудочно-кишечного тракта, диафрагмальная грыжа и др. В последнее время изучается синдром гастроэзофагального рефлюкса (ГЭР).
GER develops as a result of persistent and frequent entry of stomach contents into the esophagus or as a result of aspiration of small amounts of gastric contents into the respiratory tract (chronic microaspiration) mainly during sleep. The main cause of GER is considered to be a decrease in tone and periodic relaxation of the lower esophageal sphincter. An important role in the development of GER is played by autonomic disorders of the sphincter, including as a result of traumatic brain injury. GER can be a manifestation of functional and organic lesions of the central nervous system. The manifestations of GER include: 1) frequent regurgitation in infancy; 2) periodically occurring belching, nausea, discomfort behind the sternum, vomiting after coughing; 3) the occurrence of cough or suffocation mainly at night; 4) increased or the appearance of cough in a prone position; 5) prolonged coughing after an attack. For the diagnosis of GER, an X-ray contrast study of the esophagus, intra-esophageal pH metry, fibroesophagogastroduodenoscopy, bronchoscopy are used.
The basis of the treatment of bronchial obstructive syndrome with GER is antireflux therapy. The effect occurs on 2-3 days from the start of treatment. Antireflux therapy includes the following points. Diet – the last meal should be 3 hours before bedtime. GER-enhancing foods are excluded: tomatoes, coffee, cocoa, chocolate, fresh cucumbers, citrus fruits, sweets, fatty and spicy dishes. During sleep, an elevated position at an angle of at least 40 degrees is recommended, sleep on the stomach or right side. Alkaline drink is also prescribed (1% soda solution, mineral water), with increased acidity of gastric juice — antacids, cerucal at the rate of 0.7-0.8 mg / kg in 4 divided doses after meals for 10-14 days, enzymes.
Tracheo- and bronchoesophageal fistulas often appear already at the first feeding of a child with attacks of suffocation, cough, cyanosis. This is observed in cases of wide communication of the esophagus with the respiratory tract. In the future, either aspiration bronchitis or pneumonia rapidly develops. Narrow fistulas may go unnoticed for a long time, even before preschool age. The persistence of physical changes is characteristic of aspiration bronchitis, the diffuse nature of the process, the frequent development of biofeedback, as well as the discharge of a large amount of mucus. Aspiration bronchitis can also be based on dysphagia resulting from paresis of the swallowing muscles and impaired coordination of the swallowing act, which may be due to birth injury or systemic diseases of the neuromuscular system. One of the causes of aspiration bronchitis may be paresis of the soft palate. Aspiration here has a different mechanism: food enters the nasopharynx, and then with a stream of air enters the respiratory tract. Clinically, in such children, milk flows from the nose when feeding. Impaired coordination of swallowing is often observed in children with congenital stridor.
BRONCH OBSTRUCTIVE SYNDROME IN DISEASES OF THE CARDIOVASCULAR SYSTEM OF CONGENITAL AND ACQUIRED CHARACTER.
The genesis of bronchial obstructive syndrome in congenital heart defects can be different. More often, biofeedback is observed in heart defects with enrichment of the pulmonary circulation and is caused by hemodynamic disturbances. The most common complications of defects accompanied by hypervolemia and pulmonary hypertension include repeated respiratory infections and pneumonia, atelectasis, chronic bronchopulmonary pathology. The severity of BF in congenital heart disease may be different, but changes in the cardiovascular system come to the fore in the clinical picture of the disease , which facilitates the interpretation of the mechanism of BF.
Congenital non-rheumatic carditis. The most constant sign of this pathology should be considered cardiovascular insufficiency with a predominance of left ventricular failure, which appears in the first half of life. At the same time, shortness of breath in 1/4 of the patients in the lungs listens to different-sized moist and dry wheezing, often interpreted as “obstructive syndrome”.
Acquired carditis (acute carditis). As a rule, the first signs of the disease appear on the background of acute respiratory viral infections or 1-2 weeks after it. At the very beginning, acute carditis is manifested by signs of left ventricular failure: shortness of breath, sometimes noisy wheezing, which is why it is often diagnosed with pneumonia with obstructive syndrome, asthmatic bronchitis, or congenital stridor. Along with shortness of breath, cardiac arrhythmias are detected: tachycardia, brady or tachyarrhythmia, cardiomegaly.
Malformations of the pulmonary vessels. Among these defects, agenesis or hypoplasia of the pulmonary artery and its branches is most common, less commonly abnormal pulmonary vein infiltration, arteriovenous aneurysms. Agenesis and pulmonary hypoplasia are often combined with heart damage. There are no specific clinical signs of this defect. Frequent acute respiratory infections, mainly with bronchial obstructive syndrome, are characteristic. On examination, flattening of the chest on the side of the lesion is observed, in the same place – weakened breathing with inconsistent wheezing. Radiographically, on the side of the lesion, a narrowing of the pulmonary field, depletion of the vascular pattern are noted, as a result, an impression of super transparency is created. Bronchography — without pathology; on scintigrams, either a complete absence of pulmonary blood flow, or a gross violation of it. Important for the diagnosis of this defect is angiopulmonography.
DISEASES OF THE CENTRAL AND PERIPHERAL NERVOUS SYSTEM.
In children with birth trauma, central nervous system injuries, hypertensive hydrocephalic syndrome, with severe brain development defects, the coordination of the act of swallowing and sucking may be impaired, as a result of which aspiration of food, mainly liquid with the development of biofeedback, is possible. The sucking and swallowing reflex often more fully recovers during the first weeks or months of life, but sometimes these disorders persist for years. With myopathies (Werdnig-Hoffmann amyotrophy, Oppenheim disease), dysphagia develops, associated with paresis of the swallowing muscles, followed by the development of aspiration bronchitis. In these children over the age of one year, the pharyngeal reflex is often reduced, which helps in the differential diagnosis of dysphagia. Violation of swallowing in diseases of the neuromuscular apparatus is wave-like in nature: periods of improvement are followed by increased aspiration processes mainly against the background of acute respiratory infections. Increased breathing and the direct effect of viral infection on the neuromuscular system can exacerbate swallowing disorders. The development of biofeedback in congenital myopathies, in case of neuroinfections (poliomyelitis), in sluggish forms of cerebral palsy, in premature babies, and in alcoholic fetopathy can also be associated with bronchial tree dyskinesia. In children with perinatal encephalopathy (various syndromes), alcoholic fetopathy, biofeedback can often be observed due to severe dyskrinia, which is constantly observed and significantly increases against the background of acute respiratory infections.
BRONCH-OBSTRUCTIVE SYNDROME WITH EXCHANGE ANOMALIES.
Bronchial obstructive syndrome often occurs with hereditary metabolic abnormalities that occur with damage to the bronchopulmonary system. The genesis of obstruction is complex and is due to impaired cell function and pathological changes arising in connection with this, both on the part of the respiratory system and other organs and systems. Most often, bronchial obstructive syndrome occurs with cystic fibrosis, malabsorption syndrome, rickets-like diseases, less often with alpha-1 antitrypsin deficiency, mucopolysaccharidosis.
Cystic fibrosis is the most common monogenic disease with an early manifestation, severe course and a serious prognosis. Cystic fibrosis is transmitted by an autosomal recessive type, the risk of birth in a patient’s family is 25% for each new pregnancy. The frequency of heterozygous carriers of the cystic fibrosis gene is 5%, in Russia there are more than 8 million.
Cystic fibrosis is known to be caused by mutations in the gene (located in the middle of the long arm of the 7th chromosome), which is responsible for the molecular structure of the protein, which is located in the membrane of glandular cells lining the excretory ducts pancreas, intestines, bronchopulmonary system, urogenital tract and regulates electrolyte (mainly chloride) transport between these cells and intercellular fluid. A defective protein is destroyed in the cell, which leads to the dehydration of secrets, that is, the secretion of increased viscosity and the development of clinical symptoms and syndromes from the above organs and systems. Currently, the total number of mutations exceeds 500. The most common mutation is F-508, in Russia, in 58% of cases.
Mixed pulmonary intestinal form is distinguished — in 76.5%, mainly pulmonary — in 21% and mainly intestinal — in 2.5% of patients. Bronchopulmonary changes dominate the clinical picture, determine its course and prognosis in 90-95% of patients with cystic fibrosis.
Respiratory syndrome most often begins to appear at the age of 2 months to 1 year, either independently of the intestinal syndrome, or in parallel with it. Pulmonary syndrome can begin with pneumonia, or with bronchial obstructive syndrome, or with a combination of both.
The disease begins with a cough, which is unproductive, whooping cough, painful. Sputum, saliva, mucus in the nose in sick children is viscous, sticky, thick. The genesis of bronchial obstruction in cystic fibrosis is due to impaired mucociliary clearance due to the phenomena of dyskrinia, dyskinesia, edema, and hyperplastic processes.
Bronchial obstructive syndrome immediately becomes protracted or recurrent. Enhances obstruction purulent endobronchitis, developing as a result of layering of the infection. Small bronchi and bronchioles are involved in the process. As a result of persistent airway obstruction, sick children develop bloating, which is an early and constant sign of obstruction. As a result of the long course of the bronchopulmonary process, bronchiectasis and pneumosclerosis are formed. With cystic fibrosis, atelectasis often occurs, which can be multiple, “migrating” (subsegmental, segmental, lobar), not long straightened out. Most children with cystic fibrosis lag behind in physical development. The lag in physical development is caused not so much by intestinal syndrome, which is well compensated by enzyme preparations, as by the presence of chronic hypoxia and purulent intoxication due to bronchopulmonary changes. On examination, deformity of the chest often attracts attention in the form of a barrel-shaped form (due to swelling), less often, due to keeled deformation. Deformation of the fingers and toes appears in the form of “drumsticks”, nails in the form of “watch glasses”. With percussion of the lungs, the “variegation” of pulmonary sound is determined, namely the alternation of sections of shortening of pulmonary sound with sections of box sound. Typical for cystic fibrosis is the presence of constantly listening moist rales of various sizes, however, in some children, especially during the period of exacerbation of the process, wheezing may not be heard, but significant weakening of breathing is determined mainly in the basal parts of the lung due to the accumulation of a large amount of viscous sputum. In patients with cystic fibrosis, exacerbations of the bronchopulmonary process of a bronchitis or pneumonic type are often noted. With an exacerbation of the bronchopulmonary process, obstructive syndrome occurs or intensifies, shortness of breath appears at rest, cyanosis (perioral, acrocyanosis) tachycardia, wheezing either disappears or their number increases. In some cases, an increase in obstructive syndrome occurs due to the development of severe bronchospasm, which is most likely due to pathoreceptor disorders and to a lesser extent associated with allergies (usually drug). With exacerbation of the pneumonic type, a protracted or recurrent course of pneumonia is characteristic with abscess formation, early development of bronchiectasis. The presence of massive upper lobar pneumonia in infants is more characteristic of cystic fibrosis. Radiologically, one of the most constant signs of cystic fibrosis is bloating, diffuse pulmonary changes — a pronounced thickening of the walls of the bronchi, smearing of small elements of the bronchovascular pattern, general turbidity of the background, widening of the shadow of the roots of the lungs to the peripheral parts, and their deformation. As the disease progresses, there is a consistent change in the pathogens of the infectious process. Patients with cystic fibrosis with chronic Pseudomonas infection are especially distinguished. The syndrome of bronchial obstruction in these patients is pronounced significantly and poorly amenable to therapy, which is associated with the peculiarity of Pseudomonas aeruginosa that secrete proteases that increase the viscosity of sputum and enhance a gene defect.
Along with bronchopulmonary pathology, patients with cystic fibrosis often have gastrointestinal disturbances, which in some cases precede respiratory disorders. Intestinal disorders are manifested in the form of signs of pancreatic insufficiency, dystrophy, dyspeptic disorders (stools are plentiful, mushy, quickened, oily in appearance, with a fetid odor, steatorrhea), against the background of increased appetite.
Diagnosis of cystic fibrosis based on clinical signs is difficult due to the large polymorphism of symptoms not only from the bronchopulmonary system, but also from the gastrointestinal tract .