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A Symptoms and Signs
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Cystic fibrosis should be suspected in a young adult with a history of chronic lung disease (especially bronchiectasis), pancreatitis, or infertility Cough, sputum production, decreased exercise tolerance, and recurrent hemoptysis are typical complaints Patients also often complain of facial (sinus) pain or pressure and purulent nasal discharge Steatorrhea, diarrhea, and abdominal pain are also common Digital clubbing, increased anteroposterior chest diameter, hyperresonance to percussion, and apical crackles are noted on physical examination Sinus tenderness, purulent nasal secretions, and nasal polyps may also be seen
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risk of cystic fibrosis related respiratory exacerbations and the need for intravenous antibiotics Pharyngitis, laryngitis, and voice alterations are common adverse effects Inhalation of hypertonic saline has been associated with small improvements in pulmonary function and fewer pulmonary exacerbations The beneficial effects of hypertonic saline in cystic fibrosis may derive from improved airway mucous clearance Antibiotics are used to treat active airway infections based on results of culture and susceptibility testing of sputum S aureus (including methicillin-resistant strains) and a mucoid variant of P aeruginosa are commonly present H influenzae, Stenotrophomonas maltophilia, and B cepacia (which is a highly drug-resistant organism) are occasionally isolated Azithromycin (500 mg orally three times a week) may slow progression of disease in patients with P aeruginosa The use of aerosolized antibiotics (inhalation tobramycin solution and others) for prophylaxis or treatment of lower respiratory tract infections is sometimes helpful Although some studies of inhaled antibiotics demonstrate reduced exacerbations and increased FEV1 in patients chronically infected with P aeruginosa, there is concern about the emergence of drug-resistant organisms, equipment contamination with B cepacia, and side effects such as bronchospasm Inhaled bronchodilators (eg, albuterol, two puffs every 4 hours as needed) should be considered in patients who demonstrate an increase of at least 12% in FEV1 after an inhaled bronchodilator Vaccination against pneumococcal infection and annual influenza vaccination are advised Screening of family members and genetic counseling are suggested Lung transplantation is currently the only definitive treatment for advanced cystic fibrosis Double-lung or heart-lung transplantation is required A few transplant centers offer living lobar lung transplantation to selected patients The 3-year survival rate following transplantation for cystic fibrosis is about 55% Investigational therapies for cystic fibrosis include antiinflammatory agents (eg, ibuprofen, pentoxifylline, antiproteases), protein modification agents (eg, milrinone, phenylbutyrate), ion transport agents (eg, amiloride), and gene therapy
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Bronchiolitis is a generic term applied to varied inflammatory processes that affect the bronchioles, which are small conducting and respiratory airways < 2 mm in diameter In infants and children, bronchiolitis is common and usually caused by respiratory syncytial virus or adenovirus infection In adults, bronchiolitis is encountered in a variety of clinical settings, including infections, connective tissue diseases, inhalational injuries, drug reactions, and allograft transplantation No single classification scheme has been widely accepted and, as a result, there is a confusing and overlapping array of terms to describe these disorders from the viewpoints of the clinician, the radiologist, and the pathologist The pathologic classification has two variants Constrictive bronchiolitis (also referred to as obliterative bronchiolitis, or bronchiolitis obliterans) is characterized by chronic inflammation, concentric scarring, and smooth muscle hypertrophy causing luminal obstruction These patients have airflow obstruction on spirometry, minimal radiographic abnormalities, and a progressive, deteriorating clinical course Proliferative bronchiolitis occurs when there is an organizing intraluminal exudate, consisting of fibroblasts, foamy macrophages, and other cells that obstruct the lumen When this exudate extends to the alveolar space, the pattern is referred to as bronchiolitis obliterans with organizing pneumonia (BOOP; now more commonly referred to as cryptogenic organizing pneumonitis [COP]) (see Table 9 15) The clinical classification approach divides patients into groups based primarily upon etiology Inhalational injuries, postinfectious, and drug-induced causes are identified by association with a known exposure or illness prior to the onset of symptoms Disorders associated with bronchiolitis include organ transplantation, connective tissue diseases, and hypersensitivity pneumonitis Idiopathic cases are characterized by the insidious onset of dyspnea or cough, and include COP This disorder affects men and women between the ages of 50 and 70 years, typically with a dry cough, dyspnea, and constitutional symptoms present for weeks to months prior to presentation Pulmonary function testing typically shows a restrictive ventilatory defect The chest radiograph frequently shows bilateral patchy, groundglass or alveolar infiltrates, although other patterns have been described (see Table 9 15) Corticosteroids are effective in two-thirds of patients, and improvement can be prompt Therapy is initiated at 1 mg/kg/d for 1 3 months The dose is then tapered slowly to 20 40 mg/d, depending on the response, and weaned over the subsequent 3 6 months as tolerated Relapses are common if corticosteroids are stopped prematurely or tapered too quickly Other idiopathic disorders associated with bronchiolitis include respiratory bronchiolitis, which is a disorder of small airways in cigarette smokers It usually occurs without symptoms or physiologic evidence of lung disease In some patients, however, it can cause diffuse parenchymal infiltrates, a syndrome referred to as respiratory bronchiolitis associated interstitial lung disease (RB-ILD) and is often considered with desquamative interstitial pneumonia (DIP)
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