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Bronchoectatic disease — chronic acquired, and in some cases the inborn disease which is characterized by local suppurative process in is irreversible changed (expanded, deformed) and functionally defective bronchial tubes, mainly lower parts of lungs.
Along with a bronchoectatic disease as independent nosological unit which pathomorphologic substrate is primary bronchietasia (bronchiectasias) allocate a secondary bronchietasia (bronchiectasias) which is complication or display of other disease. Most often secondary bronchiectasias arise as result of the postponed clumsy pneumonia, whooping cough, form at abscess of lungs, tuberculosis, etc. At secondary bronchiectasias usually there are pathological changes in respiratory department of lungs that distinguishes secondary bronchiectasias from a bronchoectatic disease.
It is difficult to estimate true prevalence of a bronchoectatic disease since verification of a disease demands special invasive (bronchography) or expensive (a computer tomography) researches. According to various authors prevalence of bronchiectasias has considerable variability – from 1,2 to 30 for 1000 of the population. The greatest prevalence of bronchiectasias is revealed in ecologically adverse regions of residence (the region of the Far North, Primorye), and t.zh. at persons with addictions (tobacco smoking). At men bronchiectasias occur more often than at women, in the ratio 3:1.
Bronchiectasias most often develop in segmental and subsegmental bronchi. At histologic research find an inflammation of a bronchial wall and surrounding fabrics, an ulceration of a bronchial wall, destruction and substitution of its main components (a cartilage, muscle and elastic fibers) connecting fabric and a hyperplasia of mucous glands. Expanded sites of bronchial tubes are filled with a dense purulent phlegm, and distal small bronchial tubes are corked with slime, or sclerosed and obliterated. Departments of the lungs supplied by the affected bronchial tubes reveal fibrosis, emphysema, the bronchial pneumonia centers, atelectases. Bronchial arteries and their anastomosis with pulmonary arteries are expanded, walls of vessels are hypertrophied.
According to Read's classification distinguish three types of bronchiectasias: cylindrical (uniform expansion of a bronchial tube up to the place of obstruction by slime of distal small bronchial tubes), varicose (in the form of beads or a beads, the reminding varicose veins) and meshotchaty (in the form of the cul-de-sac terminating almost under a pleura; distal bronchial tubes at the same time are not defined).
Etiology and pathogeny
Bronchiectasias are a result of destruction of a bronchial wall owing to various reasons (deficit of inhibitors of proteases, an inflammation). The inflammation as a rule has secondary character and is most often caused by an infection. Damage of an epithelium of bronchial tubes by bacterial toxins, for example pigments and Pseudomonas aeruginosa and Haemophilus influenzae, a proteases then mediators of an inflammation which are released from neutrophils leads to disturbance of physiological protective mechanisms, the slime current mainly ascending. As a result in bronchial tubes favorable conditions for growth of bacteria are created. There is a vicious circle: an inflammation - damage of an epithelium - disturbance of the ascending slime current - infection - an inflammation.
Bronchiectasias can be a consequence of a large number of infectious diseases. Before introduction of mass vaccination whooping cough and clumsy pneumonia were the frequent reasons. Now among viral diseases the adenoviral infection and flu which was especially complicated by pneumonia are in the lead. Not the treated or started bacterial pneumonia, especially necrotic can be complicated by bronchiectasias (for example, the caused Staphylococcus aureus, Klebsiella spp. or anaerobe bacterias). Recurrent bacterial infections caused (at least, partly) bronchiectasias at HIV-positive people. At tuberculosis bronchiectasias form or as a result of a necrosis of a pulmonary parenchyma and a bronchial wall, or as a result of obstruction of bronchial tubes (a stricture, a prelum the increased lymph nodes). The infections caused by atypical microorganisms can also promote development of bronchiectasias, but is much more rare. Disturbances of bronchopulmonary protective mechanisms, both local, and generalized contribute to recurrent infections.
The basic reason of local disturbances — obstruction of a bronchial tube therefore the infected slime cannot be removed, develops a chronic inflammation. Slowly growing endophytic tumors, for example bronchial tube carcinoid are complicated by bronchiectasias. Other important reason of obstruction, especially at children — foreign bodys of respiratory tracts. The stricture, obstruction of bronchial tubes viscous slime or a phlegm and a prelum the increased lymph nodes belong to more rare reasons.
Generalized disturbances of protective mechanisms are observed at hypogammaglobulinemias, primary tsiliarny dyskinesia, a mucoviscidosis.
Widespread defeat is characteristic of bronchiectasias which arise at these diseases. From hypogammaglobulinemias most often leads deficit of immunoglobulins of all classes to bronchiectasias. The combination of bronchiectasias to sinusitis and skin infections is characteristic of it. Less often bronchiectasias arise at the isolated deficit of IgA or the combined deficit of IgA and separate subclasses of IgG (usually IgG2 or IgG4).
Primary tsiliarny dyskinesia is a dysfunction of cilia of a ciliary epithelium owing to structural anomalies. The movements of cilia become in-coordinate, the ascending current of slime and clarification of respiratory tracts from bacteria are as a result broken. The disease is shown by bronchiectasias, recurrent sinusitis and average otitis. Men usually have infertility as mobility of spermatozoa depends on function of flagellums which at primary tsiliarny dyskinesia is also broken. Approximately at a half of patients Kartagener's syndrome — bronchiectasias, sinusitis and situs inversus (the return arrangement of internals) is observed. Believe that normal function of cilia is necessary for the correct rotation of internals in the course of an embryogenesis.
At a mucoviscidosis the ascending current of slime is broken because of sharp increase of its viscosity. It leads to planting of bronchial tubes bacteria and to recurrent infections. The most frequent activators — muciparous strains of Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae, Escherichia coli and Burkholderia cepacia.
In addition to infectious factors of destruction of a bronchial wall with development of bronchiectasias inhalation of toxic gases (for example ammonia), the aspiratsiiya of gastric contents, allergic and autoimmune diseases promote. It is considered, for example, that in development of bronchiectasias at an allergic bronchopulmonary aspergillosis, in addition to obstruction of bronchial tubes a viscous phlegm, an important role is played by allergic reactions to Aspergillus spp. Rare localization of bronchiectasias — in large bronchial tubes is characteristic of this disease.
Bronchiectasias are occasionally observed at nonspecific ulcer colitis, a pseudorheumatism and Shegren's syndrome, however the role of immune mechanisms in damage of bronchial tubes at these diseases is still unknown. Sometimes insufficiency an alpha of 1 antitrypsin is complicated by bronchiectasias, however early pan-acinar emphysema of lungs is more characteristic of it.
Classification of a bronchoectatic disease
- in a bronchiectasia form (by results of a bronchography): cylindrical, meshotchaty, varicose, mixed.
- On prevalence: one - bilateral, with the indication of exact localization of changes on lung segments.
- On degree of manifestation of clinical manifestations (intoxication, etc.) allocate an easy, medium-weight, severe form of an illness.
- On a disease phase at the time of inspection: aggravation, remission.
At an easy form 1-2 aggravations within a year, remissions long are observed, during remission patients feel almost healthy and quite efficient.
At moderately severe aggravation diseases more frequent and long, about 50-100 ml of a phlegm are allocated per day. In a remission phase cough proceeds, separates constantly a phlegm. Moderate disturbances of respiratory function are characteristic, tolerance to loadings and working capacity decrease.
The severe form is characterized by the frequent and long aggravations which are followed by fervescence, allocation more than 200 ml of a phlegm, is frequent with a fetid smell, patients lose working capacity. Remissions are short-term, are observed only after prolonged treatment. Patients remain disabled and during remissions. At the complicated bronchoectatic disease form to the signs characteristic of a severe form, various complications join: pulmonary heart, pulmonary heart, amyloidosis of kidneys, myocardial dystrophy, pneumorrhagia, etc.
Examples of the formulation of the diagnosis: a bronchoectatic disease - cylindrical bronchiectasias in an average share of the right lung, a heavy current, an aggravation phase.
The bronchoectatic disease comes to light aged from 5 till 25 flyings more often, is more rare — later. As a rule, the illness begins to be shown in the first years or even months of life. Parents of sick children connect usually an onset of the illness with the postponed pneumonia or virus respiratory diseases.
Cough with department of a purulent phlegm with an unpleasant putrefactive smell. The phlegm clears the throat rather easily, "a full mouth". The greatest number of a phlegm departs in the mornings, it is usually best of all in a certain situation ("a position drainage"). At cylindrical bronchiectasias the phlegm departs easier and in bigger quantity. At saccular and spindle-shaped bronchiectasias the phlegm often separates hardly. The daily quantity of a phlegm makes from 20 to 200 ml and more. During remission the quantity of the separated phlegm is much less in comparison with an aggravation phase. At some patients during remission the phlegm can not be allocated.
The pneumorrhagia is observed in 50 — 70% of cases as result of bleeding inflamed mucous bronchial tubes. At injury of expanded bronchial arteries there is pulmonary bleeding. Usually the pneumorrhagia appears or becomes more expressed in the period of an exacerbation of a disease and during intensive exercise stresses. Cases of emergence of a pneumorrhagia in women are known during periods.
Lack of complaints or dry cough and pneumorrhagia are observed at bronchiectasias of an upper share — so-called dry bronchiectasias. At such form of a disease in expanded bronchial tubes there is no suppurative process.
Asthma is characteristic manifestation of a bronchoectatic disease. It is observed at 30-35% of patients mainly at an exercise stress and is caused by the accompanying chronic obstructive bronchitis and development of emphysema. An asthma disturbs patients a little at the beginning of a disease and becomes much more expressed in process of its progressing, also during an aggravation.
Thorax pains - are neither an obligatory, nor a natural sign of a bronchoectatic disease, but quite often disturb patients. They are caused by involvement of a pleura in pathological process and appear more often in the aggravation period. Strengthening of pain is characteristic during a breath.
Aggravations are shown by fever, increase in quantity of a phlegm and impurity in it of pus and blood. At the same time the inflammation usually does not go beyond bronchial tubes, but sometimes extends to a surrounding pulmonary parenchyma with development of bronchial pneumonia.
At external survey the following characteristic symptoms of a disease bolnykhvyyavlyatsya:
lag in physical development, more characteristic of the patients who got sick at children's age and having a severe form of a disease;
hypotrophy of muscles and decrease in an animal force, weight loss;
at a long current of a bronchoectatic disease change of trailer phalanxes of fingers of hands (more rare than legs) in the form of drum sticks, nails - in the form of hour glasses is possible;
cyanosis - at development pulmonary or a pulmonary heart at heavy patients;
lag of a thorax at breath on the party of defeat, and at development of emphysema of lungs — a "barrel-shaped" type of a thorax.
Percussion data are variable, are not characteristic, depend on existence of the accompanying processes: emphysemas of lungs, a pneumosclerosis, and also possible complications from a pleura.
At auscultation of lungs the center with firmness of the keeping low-frequency mixed (wet) rattles listened against rigid breath comes to light. In a remission phase rattles can disappear. At development of a bronkhoobstruktivny syndrome (secondary obstructive bronchitis) the exhalation becomes extended, the set of dry low-tone and high-tone rattles is listened. These symptoms are followed by the accruing asthma, decrease in tolerance to an exercise stress.
The most characteristic complications of a bronchoectatic disease are HOBL with the subsequent development of respiratory insufficiency recuring bronchial pneumonia, a chronic pulmonary heart, pulmonary bleeding, an amyloidosis of kidneys development of abscess of slight or metastatic abscesses in a brain, pleura empyemas is possible.
The general blood test — at an exacerbation of a disease the leukocytosis, shift of a leukocytic formula, increase in SOE are observed. It is necessary to emphasize that the specified changes can be caused by development of perifocal pneumonia. At a long current of a bronchoectatic disease hypochromia or normokhromny anemia is noted.
Biochemical research — in the period of an exacerbation of an illness is noted increase in content of sialic acids, fibrin, seromucoid, a gaptoglobin, an alpha - 2 and gamma-globulins (nonspecific signs of inflammatory process). At development of an amyloidosis of kidneys and a chronic renal failure the level of urea and creatinine increases.
The general analysis of urine — without characteristic changes, at development of an amyloidosis of kidneys the proteinuria and a cylindruria are characteristic.
Research of a phlegm includes microscopy of smears and crops. At bronchiectasias the phlegm contains a large number of neutrophils, plentiful and various microflora. Most often find Haemophilus influenzae, Streptococcus pneumoniae and Pseudomonas aeraginosa, is more rare — Staphylococcus aureus, anaerobe bacterias, atypical bacteria and other microorganisms. Presence of Pseudomonas aeruginosa is so characteristic that sometimes it for the first time forces to suspect bronchiectasias.
X-ray analysis of lungs. The X-ray analysis of a thorax is carried out surely, but its results are not specific. At easily proceeding bronchiectasias radiological changes can be absent in general. On the contrary, saccular bronchiectasias are well visible on the roentgenogram in the form of cavities, is frequent with the horizontal level of liquid. However it is hard to distinguish them from the cellular lung forming in the outcome of intersticial diseases of lungs, and violent emphysema. Expanded bronchial tubes with a reinforced wall look as double linear shadows (a symptom of "tram rails"), and those which are visible in cross-section — as ring-shaped shadows. If bronchial tubes are muciferous or a phlegm, they are visible as the wide polosovidny or branching shadows. Because of the lowered lightness of adjacent pulmonary fabric and atelectases the affected bronchial tubes often are located in parallel and closely to each other.
Bronchography. Best of all bronchiectasias are visible at a bronchography. This research is conducted after introduction to bronchial tubes through a catheter or the bronchoscope of iodinated fat-soluble radiopaque substances.
Kompyuterny tomography. Seychasbronkhografiya was almost completely forced out by KT Emergence of KT with high resolution increased sensitivity of noninvasive diagnosis of bronchiectasias as this method allows to receive cuts 1,0 — 1,5 mm thick.
Further inspection is sent to search for the reasons of bronchiectasias.
At limited defeat carry out a bronkhoskopiya by means of which it is easy to exclude obstruction of a bronchial tube. In certain cases defeat localization matters, for example, bronchiectasias of an upper share are characteristic of tuberculosis and an allergic bronchopulmonary aspergillosis.
At suspicion on an allergic bronchopulmonary aspergillosis (a combination of bronchial asthma and bronchiectasias of large bronchial tubes) skin tests, serological researches and crops of a phlegm on Aspergillus spp are shown.
Research of function of external respiration allows to reveal type of ventilating disturbances which appear at widespread process or at the accompanying obstructive pulmonary disease. Quite often at patients with bronchiectasias reveal reversible obstruction of bronchial tubes.
At widespread defeat measure chlorine level in sweat (for a mucoviscidosis exception), carry out quantitative definition of immunoglobulins, investigate structure and mobility of spermatozoa and nasal or bronchial cilia for an exception of primary tsiliarny dyskinesia.
Directed by the diagnosis of a bronchoectatic disease the following signs are important:
• instructions in the anamnesis on long (usually from early children's age) constant cough with department of a purulent phlegm in a large number;
• communication of the beginning of a disease with the postponed pneumonia or an acute respiratory infection;
• frequent flashes of inflammatory process (pneumonia) of the same localization;
• with firmness the remaining center of wet rattles (or several centers) during remission of a disease;
• existence of a thickening of trailer phalanxes of fingers of brushes in the form of "drum sticks" and nails in the form of "hour glasses";
• rough deformation of pulmonary drawing most often in the field of the lower segments or an average share of the right lung (at a X-ray analysis of lungs);
• identification at a bronchiectasia bronchography in the struck department.