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The longevity of patients with cystic fibrosis is increasing, and the median survival age is over 30 years Death occurs from pulmonary complications (eg, pneumonia, pneumothorax, or hemoptysis) or as a result of terminal chronic respiratory failure and cor pulmonale
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Davis PB Cystic fibrosis since 1938 Am J Respir Crit Care Med 2006 Mar 1;173(5):475 82 [PMID: 16126935] Elkins MR et al; National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis N Engl J Med 2006 Jan 19;354(3):229 40 [PMID: 16421364] Rowe SM et al Cystic fibrosis N Engl J Med 2005 May 12;352(19):1992 2001 [PMID: 15888700] Yankaskas JR et al Cystic fibrosis adult care: consensus conference report Chest 2004 Jan;125(1 Suppl):1S 39S [PMID: 14734689]
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Pulmonary Disorders
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to represent the spectrum of the smoking-related interstitial lung diseases Diffuse panbronchiolitis is an idiopathic disorder of respiratory bronchioles, more frequently diagnosed in Japan Men are affected about twice as often as women, two-thirds are nonsmokers, and most patients have a history of chronic pansinusitis Marked dyspnea, cough, and sputum production are frequent, and chest examination shows crackles and rhonchi Pulmonary function tests reveal obstructive abnormalities, and the chest radiograph shows a distinct pattern of diffuse, small, nodular shadows with hyperinflation It is important to consider both the clinical and pathologic classification schemes when evaluating bronchiolitis Both constrictive and proliferative pathology can be seen in postinfectious and connective tissue diseases, and an integration with the clinical history is important Compared with constrictive bronchiolitis, proliferative bronchiolitis is overall more common, more likely to show a restrictive or mixed ventilatory defect, more likely to have an abnormal chest radiograph, and more likely to be responsive to corticosteroids Constrictive bronchiolitis is relatively infrequent but can be seen in a variety of inhalation disorders; hypersensitivity pneumonitis; and chronic rejection in heart-lung, lung, and bone marrow transplant recipients There is often an obstructive defect with hyperinflation, and chest radiographs may be normal or show hyperinflation Constrictive bronchiolitis is relatively unresponsive to corticosteroids and is frequently progressive For both constrictive and proliferative bronchiolitis, open or thoracoscopic lung biopsy is required to make the definitive diagnosis in the majority of cases
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Oymak FS et al Bronchiolitis obliterans organizing pneumonia Clinical and roentgenological features in 26 cases Respiration 2005 May Jun;72(3):254 62 [PMID: 15942294] Ryu JH et al Bronchiolar disorders Am J Respir Crit Care Med 2003 Dec 1;168(11):1277 92 [PMID: 14644923] Schlesinger C et al The organizing pneumonias: an update and review Curr Opin Pulm Med 2005 Sep;11(5):422 30 [PMID: 16093817] Smyth RL et al Bronchiolitis Lancet 2006 Jul 22;368(9532):312 22 [PMID: 16860701]
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This section sets forth the evaluation and management of immunocompetent hosts separately from the approach to the evaluation and management of pulmonary infiltrates in immunocompromised hosts defined as patients with HIV disease, absolute neutrophil counts < 1000/mcL, current or recent exposure to myelosuppressive or immunosuppressive drugs, or those currently taking prednisone in a dosage of over 5 mg/d
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1 Community-Acquired Pneumonia
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ESSENTIALS OF DIAGNOSIS
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Symptoms and signs of an acute lung infection: fever or hypothermia, cough with or without sputum, dyspnea, chest discomfort, sweats, or rigors Bronchial breath sounds or rales are frequent auscultatory findings Parenchymal infiltrate on chest radiograph Occurs outside of the hospital or less than 48 hours after admission in a patient who is not hospitalized or residing in a long-term care facility for more than 14 days before the onset of symptoms
General Considerations
Community-acquired pneumonia is a common disorder, with approximately 2 3 million cases diagnosed each year in the United States It is the most deadly infectious disease in the United States and the sixth leading cause of death Mortality is estimated to be approximately 14% among hospitalized patients and less than 1% for patients who do not require hospitalization Important risk factors for increased morbidity and mortality from community-acquired pneumonia include advanced age, alcoholism, comorbid medical conditions, altered mental status, respiratory rate 30 breaths/min, hypotension (defined by systolic blood pressure < 90 mm Hg or diastolic blood pressure < 60 mm Hg), and blood urea nitrogen (BUN) > 30 mg/dL A predictor of patient risk and mortality from community-acquired pneumonia has been developed and validated by the Pneumonia Patient Outcomes Research Team (PORT) The PORT prediction scheme uses 19 clinical variables to stratify patients into five mortality risk classes (See Table 9 9) Patients under 50 years of age without those comorbid conditions and specific physical examination abnormalities listed in Table 9 9 are assigned to risk class I All other patients are assigned to risk categories based on the scoring system in Table 9 10 Thirty-day mortality by category is listed in Table 9 10 The PORT model can be used along with clinical judgment in the initial decision about whether to hospitalize a patient with community-acquired pneumonia In immunocompetent patients, the history, physical examination, radiographs, and sputum examination are neither sensitive nor specific for identifying the microbi-
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