A condition marked by chronic abnormal dilation of bronchi and destruction of bronchial walls, bronchiectasis can occur throughout the tracheobronchial tree or can be confined to one segment or lobe. However, it’s usually bilateral and involves the basilar segments of the lower lobes. This disease has three forms: cylindrical (fusiform), varicose, and saccular (cystic). Bronchiectasis is irreversible once established.
Because of the availability of antibiotics to treat acute respiratory tract infections, the incidence of bronchiectasis has dramatically decreased in the past 20 years. Incidence is highest among Eskimos and the Maoris of New Zealand. It affects people of both sexes and all ages.
The different forms of bronchiectasis may occur separately or simultaneously. In cylindrical bronchiectasis, the bronchi expand unevenly, with little change in diameter, and end suddenly in a squared-off fashion. In varicose bronchiectasis, abnormal, irregular dilation and narrowing of the bronchi give the appearance of varicose veins. In saccular bronchiectasis, many large dilations end in sacs. These sacs balloon into pus-filled cavities as they approach the periphery and are then called saccules. (See Forms of bronchial dilatation, page 560.)
This disease results from conditions associated with repeated damage to bronchial walls and abnormal mucociliary clearance, which cause a breakdown of supporting tissue adjacent to airways. Such conditions include:
❑ cystic fibrosis
❑ immunologic disorders (agammaglobulinemia, for example)
❑ recurrent, inadequately treated bacterial respiratory tract infections, such as tuberculosis, and complications of measles, pneumonia, pertussis, or influenza
❑ obstruction (by a foreign body — most common in children, tumor, or stenosis) in association with recurrent infection
❑ inhalation of corrosive gas or repeated aspiration of gastric juices into the lungs
❑ congenital anomalies (uncommon), such as bronchomalacia, congenital bronchiectasis, immotile cilia syndrome, and Kartagener’s syndrome, a variant of immotile cilia syndrome characterized by situs inversus, bronchiectasis, and either nasal polyps or sinusitis.
In bronchiectasis, hyperplastic squamous epithelium denuded of cilia replaces ulcerated columnar epithelium. Abscess formation involving all layers of the bronchial wall produces inflammatory cells and fibrous tissue, resulting in dilation and narrowing of the airways. Mucus plugs or fibrous tissue obliterates smaller bronchioles, whereas peribronchial lymphoid tissue becomes hyperplastic. Extensive vascular proliferation of bronchial circulation occurs and produces frequent hemoptysis.
Initially, bronchiectasis may be asymptomatic. When symptoms do arise, they’re commonly attributed to other illnesses. The patient usually complains of frequent bouts of pneumonia or hemoptysis. The classic symptom, however, is a chronic cough that produces foul-smelling, mucopurulent secretions in amounts ranging from less than 10 ml/day to more than 150 ml/day.
Cough and sputum production are observed in greater than 90% of bronchiectasis patients. Characteristic findings include coarse crackles during inspiration over involved lobes or segments, occasional wheezing, dyspnea, sinusitis, weight loss, anemia, malaise, clubbing, recurrent fever, chills, and other signs of infection.
Advanced bronchiectasis may produce chronic malnutrition as well as right-sided heart failure and cor pulmonale due to hypoxic pulmonary vasoconstriction.
A history of recurrent bronchial infections, pneumonia, and hemoptysis in a patient whose chest X-rays show peribronchial thickening, areas of atelectasis, and scattered cystic changes suggest bronchiectasis.
In recent years, computed tomography scanning has supplanted bronchography as the most useful diagnostic test for bronchiectasis. It’s sometimes used with high-resolution techniques to better determine anatomic changes. Bronchoscopy doesn’t establish the diagnosis of bronchiectasis, but it does help to identify the source of secretions. Bronchoscopy can also be instrumental in pinpointing the site of bleeding in hemoptysis.
Other helpful laboratory tests include:
❑ sputum culture and Gram stain to identify predominant organisms
❑ complete blood count to detect anemia and leukocytosis
❑ pulmonary function tests to detect decreased vital capacity, expiratory flow rate, and hypoxemia. These tests also help determine the physiologic severity of the disease and the effects of therapy and help evaluate patients for surgery.
When cystic fibrosis is suspected as the underlying cause of bronchiectasis, a sweat electrolyte test is useful.
Treatment includes antibiotics, given orally or I.V., for 7 to 10 days or until sputum production decreases. Bronchodilators, combined with postural drainage and chest percussion, help remove secretions if the patient has bronchospasm and thick, tenacious sputum. Bronchoscopy may be used to remove obstruction and secretions. Hypoxia requires oxygen therapy; severe hemoptysis commonly requires lobectomy, segmental resection, or bronchial artery embolization if pulmonary function is poor. Long-term antibiotic therapy isn’t appropriate because it may predispose the patient to serious gram-negative infections and resistant organisms.
Provide supportive care and help the patient adjust to the permanent changes in lifestyle that irreversible lung damage necessitates. Thorough teaching is vital.
❑ Administer antibiotics as ordered, and explain all diagnostic tests. Perform chest physiotherapy, including postural drainage and chest percussion designed for involved lobes, several times a day. The best times to do this are early morning and just before bedtime. Instruct the patient to maintain each position for 10 minutes, and then perform percussion and tell him to cough. Show family members how to perform postural drainage and percussion. Also teach the patient coughing and deep-breathing techniques to promote good ventilation and the removal of secretions.
❑ Advise the patient to stop smoking, if appropriate, to avoid stimulating secretions and irritating the airways. Refer him to a local self-help group.
❑ Provide a warm, quiet, comfortable environment, and urge the patient to rest as much as possible. Encourage balanced, high-protein meals to promote good health and tissue healing and plenty of fluids (2 to 3 qt [2 to 3 L]) per day to hydrate and thin bronchial secretions). Give frequent mouth care to remove foul-smelling sputum. Teach the patient to dispose of all secretions properly. Instruct him to seek prompt attention for respiratory infections.
❑ Tell the patient to avoid air pollutants and people with upper respiratory tract infections. Instruct him to take medications (especially antibiotics) exactly as prescribed.
❑ To help prevent this disease, treat bacterial pneumonia vigorously and stress the need for immunization to prevent childhood diseases.
Review other book chapters online related to Kartagener syndrome:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Professional Guide to Diseases (Eighth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2005 ISBN: 1-58255-370-X 
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