M. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Chronic bronchitis in children icon

M. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Chronic bronchitis in children




НазваM. Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A. V., Ph. D. Chronic bronchitis in children
Дата19.09.2012
Розмір445 b.
ТипДокументи


M.Gorky Donetsk National Medical University Department No. 2 of Pediatrics Head of the Department Dr. Churilina A.V., Ph.D. CHRONIC BRONCHITIS IN CHILDREN

  • Assistant professor Masyuta D.I.


Although adult chronic bronchitis is defined as 3 mo or more of productive cough each year for 2 or more consecutive yr, there is no such accepted standard for children.

  • Although adult chronic bronchitis is defined as 3 mo or more of productive cough each year for 2 or more consecutive yr, there is no such accepted standard for children.

  • Its very existence as a separate entity has been questioned, which emphasizes the importance of searching for an underlying immunologic or mucosal abnormality.



A chronic or frequently recurring productive cough usually indicates an underlying pulmonary or systemic disease.

  • A chronic or frequently recurring productive cough usually indicates an underlying pulmonary or systemic disease.

  • Affected patients should be evaluated for

    • immune deficiencies,
    • anatomic abnormalities,
    • asthma,
    • environmental disease,
    • upper airway infection with postnasal discharge,
    • cystic fibrosis,
    • ciliary dyskinesis, and
    • bronchiectasis.


Cough and wheezing are common, and in one study, all 22 reported patients with chronic bronchitis had evidence of allergic disease.

  • Cough and wheezing are common, and in one study, all 22 reported patients with chronic bronchitis had evidence of allergic disease.

  • Rarely, bronchial irritation may be secondary to the chronic inhalation of dust or noxious fumes.



Clinical Manifestations

  • The chief symptom is cough, with or without expectoration.

  • The child usually complains of chest soreness, and characteristically these signs and symptoms are worse at night.

  • Wheezing may also be prominent, and physical findings are similar to those of acute bronchitis.

  • Some patients cough up large, solid, hypereosinophilic mucoid "casts" of the airways, giving rise to the term plastic bronchitis. These casts may be related to metaplastic bronchial epithelium, elements of which, together with inflammatory cells and noncellular material, can be found on histologic examination.



Clinical Manifestations

  • Some patients cough up large, solid, hypereosinophilic mucoid "casts" of the airways, giving rise to the term plastic bronchitis.

  • These casts may be related to metaplastic bronchial epithelium, elements of which, together with inflammatory cells and noncellular material, can be found on histologic examination.



Course and Prognosis

  • The course and the prognosis depend on appropriate management or eradication of any underlying illness.

  • Complications are those of the underlying illness.



Treatment

  • When an underlying cause for chronic bronchitis is found, this should receive appropriate management.

  • Allergic management may be helpful even when no underlying cause can be discovered.

  • Autogenous vaccines or inhalation of antibiotics is not effective.



BRONCHIECTASIS

  • Bronchiectasis is a chronic inflammatory disease characterized by permanent dilatation of the subsegmental airways associated with

    • inflammatory destruction of bronchial and peribronchial tissue,
    • accumulation of exudative material in dependent bronchi, and in some cases,
    • distention of dependent bronchi.


Etiology

  • Some patients may have congenital bronchiectasis, possibly caused by an arrest in bronchial development leading to cyst formation and the destruction of the bronchial wall when the cysts become infected.

  • Alternatively, there may be defective development of the bronchial cartilaginous supports.

  • Tracheobronchomegaly is a rare congenital condition in which the distal trachea and main bronchi are grossly dilated; a similar condition may be associated with recurrent pneumonia.



Etiology

  • Most cases of bronchiectasis are acquired after birth, usually resulting from chronic pulmonary infection, but the mechanisms involved are poorly understood.

  • Obstruction of the bronchial tree followed by infection is one likely cause.

  • Measles, pertussis, and pneumonia are rare causes of bronchiectasis.

  • Cystic fibrosis is the most common underlying disease in children with generalized bronchial involvement.



Etiology

  • Other predisposing factors include

    • aspiration of a foreign body, often a nonopaque one,
    • enlarged bronchopulmonary nodes owing to tuberculosis,
    • recurrent and chronic lung infections, sarcoidosis,
    • neoplasm,
    • lung abscess,
    • localized cysts,
    • emphysema with compression of the other lung,
    • allergy, and
    • asthma.


Inhaled foreign body. Note the torch bulb in the right main bronchus



Inhaled foreign body. Note the torch bulb in the right main bronchus



Radiolucent foreign body in right main bronchus. Note the increased radiolucency of the right lung as a result of air trapping



Foreign body in right main bronchus (tooth)







Slapping the back to dislodge a foreign body



Etiology

  • Patients with immunodeficiency syndromes, especially panhypogammaglobulinemia, may have bronchiectasis, usually after repeated attacks of bacterial pneumonia and bronchitis.

  • Recurrent aspiration pneumonitis in familial dysautonomia can lead to bronchiectasis.

  • Primary ciliary dyskinesis results in chronic pulmonary infection which eventually leads to bronchiectasis.



Etiology

  • Gastroesophageal reflux with chronic aspiration may be a cause of bronchiectasis.

  • Patients with congenital heart disease may develop bronchiectasis secondary to infection related to compression of an airway by an abnormally positioned or very large blood vessel, including those used in shunting procedures.



Etiology

  • Reversible bronchiectasis or pseudobronchiectasis occurs commonly after pertussis. Shortly after or during these illnesses, the bronchi may appear cylindrically dilated on bronchography, but if these studies are repeated months later, the changes have disappeared.



Pathology

  • The first destructive change is a loss of ciliated epithelium, which is regenerated as cuboidal and squamous epithelium.

  • Concurrently, the elastic tissue within the bronchial walls disappears and thickening occurs because of

    • interstitial edema,
    • fibrosis, and
    • round cell infiltration.


Pathology

  • In adjacent parenchymal and peribronchial tissue, multiple abscesses may develop, and there is usually characteristic obstructive endarteritis of the small pulmonary vessels.

  • Generally, bronchiectasis follows a segmental distribution, except in cystic fibrosis.



Pathology

  • The right middle lobe segments, the basal segments of the lower lobes and the lingular segments of the left upper lobe are most frequently affected.

  • The right lower lobe is commonly involved in aspiration of a foreign body, and the right middle lobe is most frequently affected by hilar lymphadenopathy.



Clinical Manifestations

  • In symptomatic cases, cough is invariably present and produces copious mucopurulent sputum during acute respiratory infections.

  • Cough is more marked in the morning as there is a change of posture when the child wakes up.

  • The sputum usually is swallowed by young children.

  • Physical activity or change in position, particularly while reclining, often initiates a bout of coughing.



Clinical Manifestations

  • Recurrent infections of the lower respiratory tract are common; they tend to persist and are difficult to control.

  • Anorexia, irritability, and poor weight gain are also common.

  • Fever is much less common.



Clinical Manifestations

  • Later in the course, during acute exacerbations, hemoptysis may occur, varying in severity from blood streaked sputum to exsanguinating hemorrhage.

  • Bronchiectasis characteristically follows an intermittently improving and relapsing course.

  • Physical findings are absent or few.

  • Moist or musical rales may be heard or elicited by cough.



Clinical Manifestations







Clinical Manifestations

  • During acute exacerbations physical signs of atelectasis or diffuse pneumonitis are often present.

  • With extensive bronchiectasis, there is persistent dyspnea, and physical development is retarded

  • Ventilatory and diffusion studies may reveal more widespread or severe pulmonary involvement than suspected otherwise.



Roentgenography

  • Although there are no pathognomonic findings for bronchiectasis on standard chest radiographs, marked linear streaking ("railroad tracks") with loss of volume ("crowding") is highly suggestive.

  • Bronchogram shows delineated dilated deformed bronchi.

  • Bronchography has long been the gold standard for diagnosing bronchiectasis, but it is has been largely replaced by computed tomography (CT).



Post-infection bronchiectasis



  • Kartagener’s syndrom



Computed Tomography

  • This imaging technique is not quite as sensitive as bronchography but is considerably safer and can be done sequentially to follow the patient's course.

  • A CT scan after inhalation of xenon may prove even more sensitive.



Computed Tomography





Diagnosis

  • Every patient with suspected or proved bronchiectasis should be evaluated for

    • sinusitis,
    • ciliary dyskinesis,
    • immune deficiency diseases,
    • tuberculosis,
    • asthma or other respiratory allergy, and
    • cystic fibrosis.


Diagnosis

  • If such a diagnosis cannot be made, these patients should

    • have bronchoscopy to exclude
      • bronchial stenosis,
      • strictures, tumors, and
      • foreign bodies and
    • possibly have bronchography to
      • document the bronchiectasis and
      • determine its extent and severity.


Diagnosis

  • A familial deficiency of bronchial cartilage has been proposed as an explanation of some cases of bronchiectasis in childhood and may be suggested by marked dilatation of the 2nd–4th-order bronchi during inspiration and apparent collapse during expiration.

  • Bronchoscopic washings and sputum samples should be cultured for routine pathogens.

  • Mycobacteria, and fungi, and a tuberculin skin test should be done.



Diagnosis

  • The right middle lobe syndrome consists of

    • subacute or chronic pneumonitis,
    • bronchial obstruction, and
    • atelectasis, and
  • it is generally caused by extrinsic compression of the middle lobe bronchus by hilar nodes, followed by peribronchitis and chronic infection.

  • Bronchiectasis may result.

  • On occasion, this syndrome is related to asthma or congenital anomalies of the bronchi.



Treatment

  • Therapy includes

    • elimination of all foci of respiratory infection,
    • effective mucus clearance (e.g., postural drainage), and when indicated,
    • antibiotic therapy.


Treatment

  • Postural drainage must be carried out intensively as long as secretions are being formed and is one of the most important aspects of management.



Antibiotic Therapy

  • The appropriate drug is selected on the basis of the antibiotic susceptibility of bacteria isolated from sputum or at bronchoscopy.

  • If no potential pulmonary pathogens are recovered, antibiotics should not be used.



Antibiotic Therapy

  • Systemic antibiotic therapy should be administered during acute exacerbations in courses of 2–3 wk.

  • Patients with cystic fibrosis may require more prolonged therapy.

  • Prolonged treatment for most other patients increases the risks of acquiring resistant flora and of drug reactions.



Antibiotic Therapy

  • Administering antibiotics by aerosol inhalation immediately after appropriate mucus clearance may also be helpful but should not be continued for excessively long periods, because this encourages the establishment of a drug-resistant bacterial flora.

  • Pseudomonas can be particularly troublesome.



Surgery

  • When localized severe disease progresses despite adequate medical management, segmental or lobar resection should be considered, even though the long-term results are often discouraging.

  • Some patients with lobar bronchiectasis, especially those with the right middle lobe syndrome, do very well after lobectomy.

  • Surgery may also be indicated when an intrinsic anatomic obstruction of the bronchus is found or when suppurative lesions result from aspiration of fragmented foreign bodies, especially such vegetal objects as grass fibers or fragments of peanut that elude bronchoscopic removal.



^ PRIMARY CILIARY DYSKINESIA (IMMOTE (DYSMOTILE) CILIA SYNDROME)

  • Kartagener described a group of children and adults with

    • situs inversus,
    • chronic sinusitis, and
    • airways disease
  • leading to bronchiectasis.



Pathogenesis

  • This triad of findings with a familial occurrence (Kartagener syndrome, immotile cilia syndrome) as well as a disorder with similar respiratory tract findings but no situs inversus is the result of absent ciliary and sperm tail motility.

  • The functional abnormality results from changes in the cilia and sperm tails, specifically the absence of arms on the nine peripheral microtubule doublets of the axoneme.



Pathogenesis

  • These arms are known to contain a cilia-specific ATPase, dynein, required for the differential sliding of microtubules that results in movement of cilia.

  • Motility may be absent, scattered, or uncoordinated, but all forms of dysmotility result in impaired mucociliary clearance, as demonstrated by inability to clear particles deposited on the respiratory epithelial surface.



Pathogenesis

  • The most likely pathogenic sequence is airway mucus retention and failure to clear pathogenic organisms, followed by chronic or frequently recurring respiratory tract infections and ultimately, injury to airway walls.

  • Situs inversus occurs in about 50% of individuals with primary cilia dyskinesia (PCD).



Pathogenesis

  • One hypothesis states that normal rotation of viscera depends on the motion of ciliated gut cells early in development.

  • The absence of ciliary motility allows random rotation.



Clinical Manifestations

  • Individuals with PCD may have respiratory distress during the newborn period but may survive to adulthood without overt chronic sinusitis and airways disease symptoms.

  • A feature that is helpful in differentiating PCD from Cystic Fibrosis is repeated bouts of acute otitis media or chronic serous otitis.

  • Children diagnosed after several years of life often have been treated with tympanostomy tubes; conductive hearing loss is common.



Clinical Manifestations

  • Many children with PCD experience frequent wheezing and may have an initial diagnosis of asthma.

  • The hallmark symptom is a chronic, often loose or productive cough.

  • Sputum can range from mucoid to purulent.

  • The symptoms of acute sinusitis are occasionally encountered.

  • Pneumonia may supervene.



Clinical Manifestations

  • Lower respiratory tract symptoms can progress to weight loss, diminished exercise tolerance, and respiratory disability.

  • Respiratory failure in childhood is uncommon, as are lung complications such as pneumothorax and hemoptysis.

  • Lobar atelectasis, occurs frequently.

  • Males are frequently infertile and display absent or poor sperm motility.



Diagnosis

  • PCD should be suspected in children with chronic or recurring upper and lower respiratory tract symptoms, especially in the presence of substantial middle ear disease.

  • Radiographic or computed tomography (CT) imaging shows involvement of the paranasal sinuses.

  • Chest roentgenograms may demonstrate overinflation, bronchial wall thickening, and peribronchial infiltrates.

  • Bronchiectasis is best detected by CT scanning.



Diagnosis

  • The presence of a right-sided heart in a child with chronic respiratory tract symptoms is virtually diagnostic, but this configuration occurs in only 50% of these patients.

  • Pulmonary function testing of older children yields a typical obstructive pattern.



Diagnosis

  • Scrapings or brushings of nasal mucosa can be examined directly by light or preferably by phase-contrast microscopy for evidence of motility.

  • In most PCD tissue specimens, little or no ciliary motion is seen.

  • However, because substantial motility has been documented in scrapings of several individuals with absent dynein arms, light microscopic examination of living tissue can only be used as a screening tool.



Diagnosis

  • The gold standard is quantitative documentation of abnormal structural elements, such as missing dynein arms or random orientation of cilia in nasal or bronchial biopsies or scrapings with electron microscopy.

  • Concordance of ultrastructural abnormalities in cilia and sperm is not complete.

  • To avoid acquired ciliary changes, mucosal specimens should not be obtained until 2 wk after an acute respiratory tract infection.

  • Ultrastructural evaluation should be reserved for highly suspicious cases.



Treatment

  • Therapy is symptomatic.

  • Cough should be encouraged.

  • Chest physiotherapy assists the clearance of mucus.

  • Bronchodilators can be used for symptomatic wheezing or for documentation of reversible airway obstruction.



Antibiotic Therapy

  • Antibiotics should be prescribed for evidence of infection of sinuses or lower airways.

  • The choice of antibiotics is best dictated by identification and sensitivity testing of pathogenic organisms, often pneumococcus or untypable Haemophilus influenzae.

  • Oral antibiotic administration is usually effective.



Treatment

  • Children should be examined several times each year and followed by periodic chest radiographs and serial pulmonary function testing.

  • Sinus and middle ear symptoms refractory to medical therapy deserve consultation with an otolaryngologist.



Treatment

  • Surgical intervention may be helpful in selected cases.

  • Prevention of lung infection by measles and pertussis vaccines is highly desirable.

  • Additional preventive measures include avoidance of cigarette smoke and other airway irritants.



Prognosis

  • Progression of lung disease appears to be much slower for patients with PCD than for those with Cystic Fibrosis.

  • With proper treatment, disabling lung disease often can be avoided for long periods.

  • A normal lifespan is possible.



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