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61
I. ДИАГНОСТИКА И ЛЕЧЕНИЕ
МРНТИ 76.29.35
ABOUT THE АUTHORS
Medeubekov U.Sh. - Vice-chairman of the board JSC «National scientific center of surgery named after A.N. Syzganov», Doctor of medical sciences, Professor. e-mail: medeubek@mail.ru
Sagatov I.Y. - Chief of the management department of research work of JSC «National scientific center of surgery named after A.N. Syzganov», Doctor of medical sciences.
e-mail: inkar_sagatov@mail.ru
VyrovshchikovN.V., Bokova N.V., Ahmedov SH.I., Gvozdyrev K.E., Dzhafarov O.R., Dzhusupov S.M.
- interns, 7th year of education, specialty -surgery, Department of surgery with courses of anesthesiology and intensive care KRMU.
Keywords
bullous emphysema, pulmonary bullae, surgical reduction of lung volume, valvular bronchoblocation
АВТОРЛАР ТУРАЛЫ
У.Ш. Медеубеков - «А.Н.Сызганов атындаш Y-FXO» Басцармасы терагасыньщорынбасары, м.ьд., профессор. e-mail: medeubek@mail.ru .Е.Сататов - «А.Н.Сьзанов атындаш YFXO» -ГЗЖ менеджментi бел/мшщ бас-шысы, м.ьд. e-mail: inkar_sagatov@mail.ru Н.В. Выровщиков, Н.В. Бокова, Ш.И. Ахмедов, К.Е. Гвоздырев, О.Р. Джафаров, С.М. Джусупов -«хирургия» мамандь^ы бойынша 7 курс интерндерi, %РМУхирургия кафедрасы анестезиология жэне реаниматология курсымен б/рге.
Туй1н ceздep
екпенщ буллезд! эмфизема-сы, вкпе булласылары, вкпе мвлшершщ хирургиялык редукциясы, клапанды бронхо-
блокация.
THE PULMONARY BULLOUS EMPHYSEMA. LITERATURE REVIEW
Medeubekov U.Sh., Sagatov I.Y., Vyrovshchikov N.V., Bokova N.V., Akhmedov SH.I., Gvozdyrev K.E., Dzhafarov O.R., Dzhusupov S.M, Sapunov A.V.
JSC "National scientific center of surgery named after A.N. Syzganov", Kazakh-Russian medical university, Almaty, Kazakhstan
Abstract
At the moment, there are some differences in the papers about bullous emphysema of the lungs. Some authors prefer to divide this pathology into a separate nosological unit, despite the general tendency to consider bullous emphysema as one of the manifestations of emphysema of the lungs, which in most cases is a clinical form of COPD. But the dilemma, in turn, does not solve the problem of qualitative diagnosis and treatment of this pathology. Currently, there is a small amount of works related to epidemiology and structured data on the treatment of bullous emphysema, although this pathology has a significant influence on the population. This review article will show the etiology, pathogenesis, classification, signs and symptoms, diagnosis and treatment of bullous emphysema.
6KneHity буллeздi эмфизeмаcы. Эдeбиeт шолуы
Медеубеков ¥.Ш., Саратов I.E., Выровщиков Н.В., Бокова Н.В.,
Ахмедов Ш.И., Гвоздырев К.Е., Джафаров О.Р., Джусупов С.М., Сапунов А.В.
А.Н.Сыз?анов атында?ы Улттык, ?ылыми хирургия орталы^ы, Казакстан-Ресей медициналык университету Алматы, Казакстан
Ацдатпа
Казрп уакытта екпенщ буллезц эмфиземасына арнал€ан жумыстарда кейбр айырмашылыктар бар. Квп жа^цайда СОвА клиникалык нысаны болып табылатын буллезц эмфиземаны вкпе эмфиземасы KeptiicTepiHin 6ipi релнце карастыруцыц жалпы урДсне карамастан, кейб'р авторлар бул патологияны жеке нозологиялык б'рл'кке белуд жактайцы. Бipак бул мэселе, ез кезепнце, осы патологияны сапалы циагностикалау мен емцеу мэселеан шешпейД. Kазipгiуакытта эпидемиология мэселелерне жэне буллезД эмфиземаны емцеу туралы курылымцалран церектерге арналган азцаран ецбектер бар, цегенмен, кейбр церектер бойынша, бул патология популяция ара-сынца улкен мацызга ие. Бул шолу макалаца екпе^ц буллезц эмфиземасыныц этиологиясы, патогeнeзi, жiктeлуi, клиникалык керЫа, циагностикасы жэне емцеу мэceлeлepi карастырылацы.
ОБ АВТОРАХ
Медеубеков У.Ш. - заместитель Председателя правления АО «ННЦХ им. А.Н.
Сызганова», д.м.н., профессор. e-mail: medeubek@mail.ru Сагатов И.Е. - руководитель отдела менеджмента НИР АО «ННЦХ им. А.Н.
Сызганова», д.м.н. e-mail: inkar_sagatov@mail.ru Выровщиков Н.В., Бокова Н.В., Ахмедов Ш.И., Гвоздырев К.Е., Джафаров О.Р., Джусупов С.М. -интерны, 7 курс, специальность -хирургия, кафедра хирургии с курсами анестезиологии и реанимации КРМУ.
Ключевые слова
буллёзная эмфизема лёгких, лёгочные буллы, хирургическая редукция объёма легких, клапанная бронхоблокация
Буллёзная эмфизема лёгких. Обзор литературы
Медеубеков У.Ш., Сагатов И.Е., Выровщиков Н.В., Бокова Н.В.,
Ахмедов Ш.И., Гвоздырев К.Е., Джафаров О.Р., Джусупов С.М., Сапунов А.В.
Национальный научный центр хирургии им. А.Н. Сызганова, Казахско-Российский медицинский университет, Алматы, Казахстан
Аннотация
На текущий момент имеется некоторые различия в работах, посвященных буллёзной эмфиземе лёгких. Некоторые авторы предпочитают выделять данную патологию в отдельную нозологическую единицу, несмотря на общую тенденцию рассматривать буллёзную эмфизему как одно из проявлений эмфиземы лёгких, в большинстве случаев являющейся клинической формой ХОБЛ. Но этот вопрос, в свою очередь, не решает проблему качественной диагностики и лечения данной патологии. На текущий момент имеется малое количество работ, посвященных вопросам эпидемиологии и структурированных данных о лечении буллёзной эмфиземы, хотя по некоторым данным, эта патология несёт весомое значение среди популяции. В этой обзорной статье будут рассмотрены вопросы этиологии, патогенеза, классификации, клинической картины, диагностики и лечения буллёзной эмфиземы лёгких.
Introduction
Bullous emphysema (BE) of the lungs is considered as a variant of emphysema of the lungs and is characterized by the destruction, stretching or merging of the air spaces of the adjacent alveolar walls with the formation of cavities larger than 1 cm which are called bullae. The bullae is a limited thin-walled space consisting of compressed or stretched sections of the lung parenchyma, filled with air [1,2]. Bullae can have primary and secondary origin and occur in the context of different clinical situations, which implies some differences in terminology: bullous lung disease (BBL) is characterized by the formation and development of bullae in a relatively normal, unchanged pulmonary parenchyma of one or both lungs; BE is a consequence of chronic obstructive pulmonary disease (COPD) in which bullae are formed as a result of existing pathological emphysematous changes; in the vanishing lung syndrome normal parenchymal lung tissue is gradually replaced by numerous bullae, which is reflected in the dynamics of x-ray images; bullae can also occur in the last stages of pulmonary fibrosis in sarcoidosis or complicated pneumoconiosis [3,4,5].
There is may be a confusion between the definitions of BBL and BE, as some pathologists believe that BBL is a consequence of panacinar emphysema. This statement is not valid, since panacinar emphysema most often develops in the lower lobes, while BBL tends to develop in the upper lobes and there are a sufficient number of differences in pathophysiology between these diseases [6,7].
There are some differences between such pathomorphological structures as bull, bleb and cyst. The bleb is an accumulation of air between two layers of the visceral pleura - the outer and inner elastic layer, its origin is associated with pathological penetration of air from the pulmonary parenchyma into the visceral pleura [9]. Cysts are the cavities lined with a layer of epithelial cells; the x-ray picture of lung cysts can be very similar to bulls [10].
There is not so much epidemiological data on BE in the literature, but in general, it is known that EB affects more than 5% of the world population with a total prevalence of about 12% in adults over 30 years old in the structure of lung pathology. In
the United States, BE is on the 3rd place among diseases that lead to death, and kills more than 120,000 people per year [4].
Etiology
At the moment, there is no well-defined etiolog-ical factor leading to the development of bullae in lung tissue. According to available data, pulmonary bullae are formed in the presence of the following factors and diseases:
- Bullae development is associated with long-term smoking. The formation of giant bullae is associated with marijuana smoking in some patients, but this claim can be challenged due to the simultaneous smoking of tobacco by the same patients [11].
- Cases of association of BBL with HIV infection are described, including in patients with drug dependence, in particular those who use injections of methadone, methylphenidate or talc-containing substances. When injected with methylphenidate, a similar pattern of pulmonary bullae development is observed, as in patients with alpha-1 antitrypsin deficiency [12,13].
- Bullae also develop in patients with such diseases as: Marfan syndrome, Ehlers-Danlos syndrome type IV, Aspergen syndrome, al-pha-1 antitrypsin deficiency, 1-antichymo-trypsin deficiency, polyangiitis with granulomatosis, sarcoidosis [14,15].
- Also, bullae can be formed as a result of emphysema of distal acinuses, as a result of long-term chronic inflammation and destructive changes in the terminal bronchioles and respiratory bronchioles of the first order; cases of bullae formation associated with inhalation of crushed glass fiber are also described [6,15].
Classification
The following will be a classification based on the etiology and pathomorphological characteristics of bullae:
Anatomically pulmonary bullae are classified into three main types:
- Type I bullae are characterized by the presence of a narrow neck that connects the bullae
Primary bullae Secondary bullae Lung fibrosis Hereditary disorders
Vanising lung syndrome; Single giant bullae; Bullous lung disease. Emphysema: - paraseptal; - panacinar (panlobular); - centriacinar (centri-lobular). Sarcoidosis; Idiopathic pulmonary fibrosis; Conglomerate silicosis; Cirrhotictuberculosis. Alpha-1 antitrypsin deficiency; Ehlers-Danlos Syndrome; Salla disease; Marfan syndrome; Fabry Disease; Cutis laxa.
Table 1.
Classification of pulmonary bulls [6,8].
with the pulmonary parenchyma. This type of bullae can be caused by volumetric hyperventilation or formed at the site of damaged lung tissue. The walls of type I bullae are thin, the contents are represented by an empty cavity. Type I bullae are usually located in the area of the apices of the lungs and along the edge of the tongue of the lower lobe of the left lung, as well as in the area of the middle lobe. They very often develop in association with para-septal emphysema [6,17].
- Type II bullae develop from subpleural parenchyma and are characterized by the presence of a neck of panacinar emphysematous pulmonary tissue. The bullae cavity can be filled with emphysematous pulmonary tissue, in which small vessels are preserved. Unlike type I bullae, the wall of these bullae is formed by a pleura covered with intact mesothelial cells. Type II bullae most often develop in the area of the upper lobes, the anterior surface of the middle lobes and in the area of the lower lobes of the lungs adjacent to the diaphragm [8,17].
- Type III bullae consists of a moderately hyperventilated portion of the lung connected to the rest of the lung by a broad base extending deep into the parenchyma. It is believed that this type of bullae occurs in atrophic forms of pulmonary emphysema [6,18].
A group of Russian researchers proposed to subdivide bullous emphysema into homogeneous (total lesion of lung tissue) and heterogeneous (with predominant lesion of segments) forms. It is also proposed to introduce a combined form of emphysema of the lungs, which implies the presence of signs of both bullous and diffuse (without bull) emphysema [19].
Pathogenesis
According to the present knowledge, given the lackof specific studies on BE, the genes involved in the molecular pathogenesis of BE are probably the same involved in the development of DE. However, a 4-bpdeletion in the Birt-Hogg-Dube gene (FLCN) hasrecently been shown to be strongly associated with dominantly inherited spontaneous pneumothorax, "(SP)"the main complication of subpleural bullae, in a large Finnish pedigree with a tendency to SP development [20].
A number of case reports showed an association between BE and lung cancer arising from scarred and contracted areas close to a bulla wall, although specific studies at the molecular level have not been conducted on this field [21].
Bullae develop after retraction and collapse of surrounding lung away from a region of weakness. The mean pressure inside the lung bullae is nega-
tive and shows a constant parallelism with the pleural pressure. The atelectasis of the surrounding areas, observed at times, is due to the elastic retraction of normal parenchyma and not to the compression by the bulla [21,22].BE complicating DE substantially contributes to the functional deterioration and causes significant con-founding effects on the functional assessment [20]. These findings can be explained by the contribution of lung bullae to the airways obstruction, because of their complete loss of elastic recoil. The static elastic recoil pressure of the emphysematous lung is, the refore, further decreased. Even if the bullae remain in free communication with the airway, they do not significantly participate in the ventilation [22]. Further more, the chest wall mechanics is altered because of the los-sof linkage with the nonbullous lung tissue, leading toincreased chest wall work and worsening of hyper-in-flationand sensation of dyspnea [21,23]. The confound ingfunctional effect of bullae depends on BE extent: relatively milder obstruction can be observed with severe BE, whereas moderate BE causes modest deterioration of diffusing capacity [23].
Clinical manifestations
One of the main clinical symptoms of bullous emphysema is shortness of breath, which can be asymptomatic in many patients [24]. The development of shortness of breath may be an indication for surgery, since Bulls can occupy more than 30% of hemithorax. Shortness of breath at the onset of the disease appears only with significant physical exertion, and initially patients often do not notice it. Shortness of breath in patients with bullous emphysema is dangerous because it does not manifest for many years, and progressing, it turns into a condition that threatens the patient's life. Tolerance to physical activity is reduced, since even at rest, the compensatory capabilities are at the limit [26]. Dyspnea usually has an expiratory character. Patients have a short, "sharp", "grabbing" breath and an elongated, sometimes step-shaped exhalation. They exhale with closed lips, puffing out their cheeks ("puffing"). At the same time, pressure in the bronchial tree rises, which reduces the expiratory collapse of small non-cartilaginous bronchi (due to a violation of the elastic properties of the lung tissue and an increase in intrathoracic pressure) and contributes to an increase in ventilation. As the disease progresses, patients may complain of shortness of breath with tension or wheezing [24,25].Patients often have a productive (wet) cough, often called a "smoker's cough," more pronounced usually in the morning. Cough is not a specific complaint of patients with bullous emphysema and is most often due to the presence of chronic bronchitis [25].
Body weight with bullous emphysema is reduced, which is associated with the intense work of the respiratory muscles, aimed at overcoming the high resistance of the terminal airway. Patients with pulmonary emphysema in the initial stages of the disease take a forced position on the abdomen with their head down and shoulder girdle, which brings them relief. In this situation, the patient achieves an increase in abdominal pressure, lifting up the diaphragm and improving its function. However, in severe emphysema with marked changes in the chest and fatigue of the respiratory muscles, a horizontal position causes intense diaphragm work, so patients are even forced to sleep in a sitting position [25,26]. Patients with bullous emphysema often occupy a sitting position with their torso slightly inclined forward, resting their hands on their knees or the edge of the bed, which allows the shoulder girdle to be fixed and additional muscle to be included in the act of breathing [27].
The color of the skin with emphysema is more pink than cyanotic. Slight cyanosis is caused by prolonged preservation of the gas composition of the blood, only in advanced cases does cyanosis appear, which is caused by the development of hy-percapnia [25,27]. There is a swelling of the cervical veins during exhalation due to increased intra-thoracic pressure.
Examination of the chest reveals a barrel-shaped chest. Kyphosis is sometimes observed. With percussion, a boxed sound is determined. There is an increase in the standing height of the tops, a shift of the lower borders of the lungs down and a sharp restriction of the mobility of the lower pulmonary margin. A decrease in cardiac and hepatic dullness due to increased airiness and an increase in lung tissue volume is characteristic [26,27]. Often there is a loud heart sound P2, which is a sign of pulmonary hypertension. In addition, a symptom of "drumsticks" may be present. Edema can be a sign of a decrease in the power of the right ventricle and pulmonary artery [24].
Diagnostics
Methods of laboratory diagnostics include studies of hematocrit, ABGs and PFTs, alpha-1-antitryp-sin. Methods of instrumental diagnosis of bullous emphysema are based on chest radiography in two projections, CT, spirometry.
Radiography. In the early stages of the pathological process, emphysema cannot be detected by traditional radiographic examination. Direct radiographic signs of emphysema are:
- thin-walled air cavities (usually large);
- extensive areas of pulmonary fields devoid of pulmonary pattern, usually in combination with the displacement or rupture of visible pulmonary vessels [28,29].
Both these signs characterize bullous emphysema, in which large air cavities occur in the lung tissue. Intra-lobular emphysema can be detected by radiation examination only with the help of CT [30].
CT. Emphysema is a constant pathological increase in the air-containing spaces distal to the terminal bronchioles, accompanied by the destruction of their walls, in the absence of obvious fibrosis. Emphysema is usually classified into three main types depending on the predominant localization of destruction zones: centrilobular, panlobular and paraseptal. In the early stages of development, these forms of emphysema can be confidently distinguished in IPT. In the final stage of the disease, it is difficult or impossible to distinguish them not only with CT, but also with morphological examination.
Centrilobular emphysema is one of the most common forms and is usually the result of Smoking. It mainly affects the terminal bronchioles located in the Central part of the secondary pulmonary lobule. The changes are most pronounced in the upper lobes of the lungs, especially in the apical and posterior segments. In IPT and morphological examination, centrilobular emphysema in the initial stage of development is characterized by the presence of zones of reduced density, having a rounded shape and small dimensions (usually 2-5 mm). The walls of such air cavities are actually lung tissue [30,31].
Panlobular emphysema in typical cases is associated with a deficiency of Al-antitrypsin, but can also be observed in smokers, the elderly, with obliterating bronchiolitis. Panlobular emphysema is characterized by uniform destruction of pulmonary tissue within the secondary pulmonary lobule. The walls of such air cavities become invariable connective tissue partitions between the lobules. In areas of reduced density, preserved vessels may be visible. The most pronounced changes are usually observed in the lower lobes of the lungs. A common process leads to the formation of large areas of reduced density without visible walls and impoverishment of the vascular pattern. Bullae and cysts are usually absent.Such changes can be difficult to recognize in CT [30,32].
Paraseptal emphysema is characterized by involvement in the pathological process of the distal part of the secondary pulmonary lobule. Air cavities most often have subpleural localization. This form may be independent or be detected in combination with centrilobular emphysema. As a rule, paraseptal emphysema is asymptomatic functionally, but may be accompanied by the development of spontaneous pneumothorax. Often in such patients, bulls of different sizes and shapes are detected. Bull is
Table 2.
Pharmacotherapy of the clinical manifestations of bullous emphysema. This combination allows to reduce the pace of development of RB in patients with COPD [38].
defined as an air cavity with clear smooth thin walls with a diameter of more than 1 cm. Most bull subpleural localization regardless of size are manifestations of paraseptal emphysema.
Bullous emphysema is not an independent morphological concept, although it usually develops in connection with centrilobular and para-septal emphysema. Nevertheless, multiple large and giant bulls with visible walls are often described as a separate clinicorentgenological syndrome - "disappearing lung syndrome", "primary bullous lung disease", etc. Giant, increasing in size bulls can be found not only in middle-aged and elderly smokers with long Smoking experience, but also in relatively young people. VRCT is much better than radiography, reflects the prevalence of bullous changes and manifestations of paraseptal and centrilobular emphysema [31,32]
Spirometry. Pulmonary function testing is the standard for assessing emphysema. The ratio of forced expiratory volume per second (FEV1)/forced vital capacity (FVC) is usually <0.7. FVC decreases due to loss of elastic recoil of the lung. There will be an increase in total lung capacity, residual volume and functional residual capacity, as well as a decrease in vital capacity. A decrease in lung diffusion capacity for carbon monoxide (DLCO) will often be present due to the destruction of the lung interstitium. The basis of diagnostic search in patients with pulmonary bullous emphysema is radiation and endoscopic methods. Thoracoscopy should be a mandatory study in patients with BEL complicated by pneumothorax [28,31].
From laboratory methods, it may be useful to test for alpha-1-antitrypsin deficiency. A complete blood test may show increased hemoglobin and hematocrit due to reactive erythrocytosis from chronic hypoxia. Blood chemistry panels will show increased serum bicarbonate because of metabolic compensation for respiratory acidosis [29,33].
Treatment options, complications and prognosis
- Conservative treatment
To treat such a terrible manifestation of COPD, conservative treatment is used in a combination of drugs: beta-2 adrenomimetics with glucocortico-steroids in the form of inhalations. For example:
- Surgical treatment
For a long time until the middle of the last century there were no methods of surgical treatment of this pathology. Surgery was first proposed by O. Brantigan and E. Mueller in the mid-50s. At that time, the experience of specialists in performing operations in the chest cavity and anesthetic management of patients was insufficient, as a result, every fifth patient with resection of 20-30% of the lung. died. For a long time there was no significant progress in this direction, until in 1995 J. D. Cooper published a methodology and low mortality of patients in the postoperative period [37,41].
The essence of the lung volume reduction surgery (LVRS) is to remove the altered pulmonary parenchyma, which is dominated by the bulls, which compress the alveoli of the neighboring departments of the lung, as a result, gas exchange is reduced not only in the altered segment, but also in the healthy department. In addition to straightening the healthy parts of the lung, the load on the diaphragm decreases, the lung volume increases, which reduces the residual volumes and increases the volume of inhalation and exhalation [34,37,41].
At the moment, LVRS is performed both by open and thoracoscopic method. For open resection with bilateral lesions perform median sternotomy, and with unilateral-lateral thoracotomy [34,41].
Indications for LVRS [34,41]:
- FEV1 20-40% of the proper;
- residual volume >200% of due;
- total lung capacity >125% of due;
- upper lobe localization of emphysema;
- heterogeneity of emphysema (presence of unchanged lung tissue according to CT);
- the body mass index is 17-32 kg/m2;
- low physical endurance;
- motivation of the patient;
- patient's readiness for rehabilitation measures after surgery.
Contraindications to LVRS [34,41]:
- age >75 years;
- continuation of Smoking;
- bronchiectasis, acute respiratory infections;
- prior surgery on the chest;
- lower lobe localization of emphysema;
- "disappearing" lungs according to CT;
Formoterol/Budesonide (В) 4.5/160, 9/320
Salmeterol/Fluticasone (В) 50/100, 250, 500
Vilanterol/Fluticasone furoate (В) 25/100 (22/92)
Results Tlsesociety of thoracic surgeons U.S. NRiosal sRuTy re Ehe treatment of emphysema USA
% R
30 -dayncrtelity 5,6% 2,2%
Repeatedly needed help in the intensive care unit [8% 11,7%
Sepsix 56% 2,5%
Arrhythmia 16,2% 18,6%
Vethilotion> 48 hours 4,6% E3,6%
Retintobatiot 15,6% 21,8%
1,90%
- partial voltage C02 in arterial blood (C02) >55 mmHg.st.;
- meanpulmonapr mrtety pressure >35 mmHg. p.;
- left ventrieular ejection ftmctien <410%;
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A valve bronchomalacia carries more interest doe dlit rtoviTot of cert to ecCcsSi h-s n iht wide ceen^eece t)f Cmme at sufficipn0 cHaunosis o- bull iq Cde iertlnl CtoitP; the mnScirf ^ ttp rcer|mtrutilt reletest [lee e
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Table 3.
Complications from LVRS in the United States in 2014.[40]
85
■ 30 - day mortality
■ Repeatedly nee ded help in the mtensrre caee unit
■ Sepnis
n Arrhythmim
■ Aentilation> 48 hours
■ Re-intubotion
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ft Good claical effect (stopping air leakage thromdh JotrainSt straightening the lung)
if Weak positive dgncmics (reduction oC air leaktgk ^etmicccmd;iil -ao no staarghteneg oC rhe lung)
■ No effect (persisrent aidf discmenrge iSough Crises. no strtiehtenieg ecC the lung)
Complications from LVRS. [40]
Resultsofendobronchial valveinstallation.[41]
esthesia to install or remove the blocker. The phenomena of endobronchitis, arising from prolonged bronchial occlusion, are stopped independently without the formation of its stenosis [36,41].
The data obtained indicate the need for greater use of this method in patients with pulmonary bul-lous emphysema in the practice of thoracic departments [8].
Conclusion
Given the available epidemiological data, bullous emphysema is a relatively common lung pathology. It is determined that the development of bullae in the lung tissue, in general, can be associated with emphysema of the lungs (most often), as well as have a primary genesis. The pathophysiology of bul-lous emphysema is closely associated with chronic inflammation of the distal portions of the bronchial tree, as well as hereditary diseases associated with impaired synthesis of structural components of the lung parenchyma and some fermentopathy, which leads to destruction of walls of alveoli or change their properties, further contributing to the permanent increase in the size of the newly formed air spaces. Clinical manifestations of bullous emphysema are nonspecific, and are usually accompanied by re-
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