Tuesday, April 2, 2013

Chronic Bronchopulmonary Disease In Children.

Chronic bronchopulmonary pathology - is an inflammatory process of infectious origin (caused by the microflora), which is the recurring, usually bronchogenic spread and is based on functional and organic lesions of bronchi, vessels, parenchyma and interstitial tissue of the lungs, pleura and the subsequent development of sclerosis.

          Actuallity of the problem of chronic bronchopulmonary pathology.
• a considerable incidence - 0.2-1% among children (according to the pathologists,
        in adults it occurs in 1.5-12% dead)
• severe                                
• irreversible complications (sclerosis, bronchiectasis, cardiovascular
     failure, brain abscess, abscess lung formation, amyloidosis)
• disability of children and later adults, possible adverse health effects

                  The problem of chronic bronchopulmonary pathology always aroused the attention of pulmonologists. Perhaps no disease of bronchopulmonary system has  caused so much discussion and debate among scientists as chronic bronchopulmonary pathology. The term "chronic bronchopulmonary disease" has been repeatedly revised, but attempts to replace it by bronchiectasis, pneumosclerosis or chronic bronchitis have been in vain. After all, none of them could not reflect the depth of pathomorphologic changes, but was only evidence of adverse trends (bronchiectasis, pneumosclerosis) or a component of the pathological process (chronic bronchitis). The term "chronic bronchopulmonary disease" encompasses a range of morphological changes in all anatomical structures of the lung.
       Now scientists abroad again reject the term "chronic bronchopulmonary disease", finding him an alternative - chronic bronchitis.
       Statistics say that over the past years there was no increase in chronic bronchopulmonary diseases among children. But this is not indicative of its absence. Timely diagnosis and treatment of acute pneumonia, the effectiveness of antibacterial new generation medicines significantly reduced risk of chronic inflammatory process.
        At the same time, the effects of environmental catastrophes, increasing the number of sick children was continued, and especially during the first 3-5 years of life. Reduced immunological resistance of the organism, the low sensitivity of the respiratory tract flora to antibiotics and the increasing allergy to them create the complete objective prerequisites for the emergence of chronic bronchopulmonary pathology. Clinical experience proves that, the more so that the low social and cultural level of the population today leads to an increase in incompletely treated patients with acute pneumonia.

                             Etiology, pathogenesis and pathomorphology

               The etiology, pathogenesis and morbid anatomy are complex and many aspects were studied. The factors that cause predisposition to the development of chronic respiratory pathology, are:
·        prematurity
·        anomalies of the constitution, especially exudative-catarrhal
·        malnutrition 
·        rickets
·        artificial feeding
·        pollution of the atmosphere
·        bad living conditions.
               Promotion factors:
• acute pneumonia (80%)
• whooping cough, measles
• frequent ARI
• foreign body of airway
• congenital and acquired malformations of the bronchopulmonary system
• hereditary diseases (cystic fibrosis).
                This factors contribute:
·        reducing overall reactivity (resistance)
·        violation of local immunity
·        disorders of drainage (evacuation) and the barrier function of bronchi.

             The reason supporting a chronic inflammatory process is the microflora (inflyuenza coli, streptococci, staphylococci, pneumococci, Gram-negative bacteria) or its α-forms which are in bronchopulmonary lymph nodes, in foci of chronic infection (tonsillitis, adenoids, sinusitis, carious teeth, cholecystitis). In children, the main way of spreading of infection is bronchogenic.
             The inflammatory process is consistent and progressive in nature: single-layered ciliated epithelium is replaced by a cubic (violation of bronchial drainage function) → damage nerve endings bronchi (disturbed peristalsis), connective tissue is developing, changing the size, form and elasticity of the bronchi → development of panbronchitis facilitates the induction of inflammation in the lung interstitium in intraalveolar septums, directly into the alveoli. Thus, in chronic respiratory pathology all structures of the bronchopulmonary system are affected (bronchi, interstitial tissue, parenchyma, vessels, nerves, pleura), but according to the clinic we may only speak about the prevalence of changes in the bronchi, interstitium or parenchyma of the lung.

            This is confirmed by pathological data, when according to the severity of the pathological process such consecutive changes are observed: 1) chronic bronchitis with bronchiectasis and emphysema, 2) chronic bronchitis with bronchiectasis and atelectasis, and 3) chronic deforming bronchitis.

              Pathoanatomic examination provides a diverse macroscopic picture in conjunction with the histological heterogeneity of lesions in chronic bronchopulmonary disease. In the same case different changes are revealed: chronic inflammation of the interstitium, carnification, sclerosis, deforming bronchitis, bronchiectasis, emphysema, atelectasis, worsening the inflammatory process. There are lymphangitis, abscesses, etc.
            The main among the histological changes are: organization of exudate in the alveoli, chronic inflammation of the intermediate tissue, chronic bronchitis with bronchiectasis (or deforming bronchitis), foci of acute inflammation.

Chronic bronchitis. Bronchoectasis.
Lung emphysema. Pneumosclerosis.

Micropreparation of  lung in diffuse interstitial pneumosclerosis: intraalveolar septums are greatly thickened and sclerotic; coloured by Van Gieson; × 24.
Micropreparation of lung at local pneumosclerosis: substitution of the alveoli, bronchioles, alveolar ducts by proliferation of connective tissue; coloured by Van Gieson; × 24.
                 Morphological studies indicate that at the presence of purulent bronchitis lymph capillaries and efferent lymphatic vessels are constantly involved in the inflammation process. This is explained by the fact that the resorption of purulent exudate and the output is mainly done by lymphatic pathways. Later purulent lymphangitis is changed by lymphangiosclerosis, obliteration of the lumen, causing resorption and mechanical failure. At the same time, disruption of lymphatic drainage of the bronchial wall is of particular importance, since sharply reduced ability to clean itself, and therefore, there is stagnation of secretions, especially in the bronchioles, activation of the microflora and the development of peribronchial inflammation.
               Arround the lymphatic capillaries there is observed infiltration by lymphocytes and plasmocytes with the formation of lymphomas. In addition to induction of cellular elements, lymphostasis sharply violates vascular tissue ratio, tissue metabolism, which lead to degeneration of the structural components of the bronchi with subsequent development of cylindrical bronchiectasis and reticular pneumosclerosis. Thus, there is convincing evidence: bronchitis is only the initial phase, followed by involvement in the process lung parenchyma, which is more appropriate to designate as a chronic inflammation of the structural components of the lungs (chronic bronchopulmonary disease). In contrast to acute pneumonia, respiratory failure (ventilation, diffusion, distribution) develops gradually, slowly.
               Circulatory hypoxia also slowly joins respiratory one with the development of hypertension in the pulmonary circulation and pulmonary heart. Hypoxia is defined already in the preterminal stage of disease. Prolonged hypoxemia leads to disruption of all metabolic functions of organs and systems, and in severe cases - to morphological changes. This manifests itself in reducing the child's memory, his irritability, aggression (CNS), liver enlargement and functional disability decrease enzymatic function of the stomach and intestines (digestion tract), dryness of the skin, mucous membranes, papillae atrophy of tongue (hypovitaminosis), etc.
               Thus, the basis of chronic bronchopulmonary pathology is organic and functional changes in the bronchopulmonary system and the subsequent development of hypoxemia and hypoxia. Now there is reason to talk about the distortion of the immunological status and surfactant deficiency in chronic bronchopulmonary disease. However, their role in the deep pathological, progressive changes has not been fully established. We can only assume their genetically determined deficiency, which causes chronization and progression of the process.

              The clinic is manifested as symptoms of a general nature and directly related with the bronchopulmonary system. It must be remembered that chronic bronchopulmonary pathology in 80% of cases occurs in the first 3 years of life and approximately 50% - in the first year of life, when its diagnosis at the initial stage is difficult. It is necessary to clarify the frequency of respiratory episodes in a child, and their duration. With increasing frequency of these episodes, their duration and the probability of chronization of the process increase.
               Coughing is a reflex act aimed at self-purification of the respiratory tract of mucus, pus, blood, foreign body, and other particles, which are normally not present in the bronchial tree. In chronic respiratory pathology there is occurrence of  the proliferation of the bronchial mucosa in relation to the inflammatory reaction, hypersecretion of glands, a violation of peristalsis, the accumulation of mucus and pus, compression of the bronchi and trachea by enlarged lymph nodes. Cough, mostly wet, accompanied by sputum, in the presence of emphysema is severe, unproductive, in remission - dry.
                Sputum is puromucous or purulent, more in the morning. It is not a three-layer, as in adults, in small quantities (30-50 ml), it is difficult to obtain it, because in most cases children can not expectorate. For bacteriological examination sputum is taken during bronchoscopic lavage or after digital pressure with a spatula on the tongue root, which causes reflex cough with expectoration.
              Cyanosis appears at exacerbation of chronic respiratory pathology or with the development of chronic pulmonary vascular disease, when it is constant and, depending on the activity of the pathological process, only its intensity changes. Often there is observed increase in body temperature, the phenomenon of hyperhidrosis.
              We must always pay attention at the form of a chest. Its deformation is evident in moderate and severe cases. It indicates a significant area of the pathological process in the lungs and long-term hypoxemia. "Chicken", "pigeon" chest, depressed sternum are signs of chronic bronchopulmonary diseases in preschool age; barrel - in schoolchildren. The asymmetry of the chest, deformation, a marked decrease in the size of a half part in measuring by centimeter tape - all this makes it possible to analyze during the inspection the severity, duration and localization of process.


                Shortness of breath at chronic bronchopulmonary disease is mixed, sometimes expiration. Deformation of the fingers in the form of drum sticks and nails in the form of watch glass reflects the duration of hypoxemia.


                    Percussion reveals bandbox sound (emphysema), at marked sclerotic changes shortening is detected.
                   Auscultation (at exacerbation) reveals diversity of dry and moist rales. Constant local fine bubbling moist rales is the criteria of chronic bronchopulmonary disease.
                   Cardiovascular system: expansion of the right heart, accent of the second tone in the pulmonary artery, weak tones and functional systolic murmur, the ECG changes shows myocardial hypoxia.                             
                Digestive system. Appetite is reduced, the liver is increased in size, its function is disrupted, there are disorders in hydrolysis and absorption of food, in severe cases, a child retards in the mass and growth. The skin is dry, visible pallor, reduced flexibility, opacity and the fragility of the hair.
           Sometimes there is short-term allergic rash, once rising of high temperature (due to a violation of drainage of purulent sputum). Headache, fatigue, irritability, aggression, memory decline indicate CNS violation. Pulmonary hemorrhage is very rare in children.
            X-rays changes are different: increased lung pattern, its deformation, cyst enlightenments, volume reduction of segment, lobe, mediastinal shift toward pathology, disateleсtasis (airless areas alternate with emphysema). Characteristic for this disease is that they are sustainable. They can only increase at exacerbation, but remain in remission.

Left side chronic bronchopulmonary                 Direct radiogram of chest.
disease.                                                                Decreasing of left lung field,
                                                                             shadowing in the middle areas.

Detail of chest radiograph at peribronchial pneumosclerosis in patient with chronic obstructive bronchitis: lung pattern is reinforced and deformed, there are clearly distinguished bronchial lumen, bordered by thickened walls.

Detail of chest radiographs in a direct projection at chronic bronchopulmonary disease: at low part of the right lung field lung pattern is reinforced and deformed, its radial direction can not be seen.

          At bronchographic observation (which is obligate) there are the deformation of the bronchi, cylindrical and saccular bronchiectasis, reducing the angle of the bronchi branching.


Bronchiectasis. Coloured bronchogram (X-ray) of a human lung showing bronchiectasis.Bronchiectasis is a lung disorder in which the bronchi and bronchioles (airways of the lungs, red) are permanently dilated and distorted. In this case, some of the bronchi have terminal bulbous enlargements, a condition known as fusiform (saccular) bronchiectasis.

Normal bronchogram.                                Varicose bronchoectasis.                           

Representative CT scan image from a person with advanced CBPD shows emphysematous changes and subpleural blebs.

                Identification of chronic bronchopulmonary diseases at the level of deforming bronchitis without bronchiectasis may be conventionally considered  because the stabilization of the pathological process in these conditions is more likely. Clinical experience shows that bronchiectasis is less evident than deforming bronchitis.

                           Deforming bronchitis.
                Deformations of the bronchi may be different: the broadening or narrowing of the lumen, presence of granulation and follicular polyps, false cysts, bronchiectasis (cylindrical, saccular, mixed), which lead to varying degrees of obstruction of small bronchi. Chronic process in the lung tissue in combination with deforming bronchitis or bronchiectasis becomes stable, leads to irreversible changes in the affected area of lungs.
              It is believed that chronic bronchopulmonary diseases are characterized by the local endobronchitis. However, at the severe forms with involvement in the pathological process of many segments of the left and right lungs endobronchitis becomes diffuse.

               Modern classification of chronic pulmonary disease involves the next indicators:
1. Localization and extent of pathological process (segment or segments, lobe).
2. The nature of bronchial lesions:
- Deformation
- Bronchiectasis: cylindrical, saccular, varicose, mixed
- Bronchostenosis
3. Nature of endobronchitis:
- Catarrhal
- Purulent
- Local
- Diffuse
4. Current:
- With occasional exacerbations
- With frequent exacerbations
- Continuously recurring
5. Severity:
- Mild
- Moderate
- Severe
6. Period of illness:
- Exacerbation
- Recovery
- Remission
7. The degree of respiratory failure.
8. Complications:
- Pulmonary (emphysema, obstructive syndrome,
  abscess, pulmonary hemorrhage, pleurisy, pyopneumothorax etc.)
- Extrapulmonary (pulmonary heart, amyloidosis, brain abscess)

                 Diagnostic criteria of chronic bronchopulmonary disease:
1. Anamnesis:
a) direct link with a previous acute pneumonia;
b) recurrent inflammation in the same area of the lungs.
2. Clinical:
a) cough (mostly wet);
b) sputum (mucous-purulent);
c) hyperhydrosis, intermittent fever;
d) deformation of the thorax (in particular, flattening on the side of lesion;
d) the presence of stable local fine bubbling moist rales.
3. Radiographic:
a) strengthening and deformation of the pulmonary pattern;
b) thickening of the bronchial walls;
a) reducing of fibrosic lung segments;
d) infiltration of lung tissue in the focus of the lesion in the acute phase.
4. Bronchologic:
a) Bronchoscopy - a localized endobronchitis;
b) Bronchography:
- Cylindrical or saccular bronchiectasis;
- Curvature, the convergence of bronchial trunks (deforming bronchitis)
- Bronchostenosis.


WHO Strategy for prevention and control of Chronic Respiratory Diseases:
The Global Strategy for the prevention and control of noncommunicable diseases, developed in direct response to the global threat posed by noncommunicable diseases and endorsed by the Fifty-Third World Health Assembly, cites chronic respiratory disease as one of the four priority disease groups to be addressed.
Factors contributing to the burden of CRDs

Goal of the strategy
Objectives of the strategy
General principles

Strategic directions

A - Basic:
1.      Pediatrics. Textbook. / O. V. Tiazhka, T. V. Pochinok, A. N. Antoshkina et al. / edited by O. Tiazhka – Vinnytsia : Nova Knyha Publishers, 2011 – 584 pp. : il.
2.      ISBN 978-966-382-355-3Nelson Textbook of Pediatrics, 19th Edition Kliegman, Behrman. Published by Jenson & Stanton, 2011, 2608.  ISBN: 978-080-892-420-3.
3.      Illustrated Textbook of Paediatrics, 4th Edition.  Published by  Lissauer & Clayden, 2012, 552 p. ISBN: 978-072-343-566-2.
4.      Denial Bernstein. Pediatrics for medical Students. – Second edition, 2012. – 650 p.

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