Health status and sociodemographic characteristics of adults receiving a cystic fibrosis diagnosis after age 18 years.

OBJECTIVES: An increasing percentage of cystic fibrosis (CF) diagnoses are occurring in adulthood. The purpose of this study was to explore how age at diagnosis may be associated with selected disease and sociodemographic characteristics. DESIGN: The 1996 Cystic Fibrosis Foundation (CFF) Patient Registry data were analyzed to test for associations between age at diagnosis and selected variables. All cases involved individuals > or = 18 years who were represented in the CFF Patient Registry for 1996. Patients were assigned to one of two groups: those diagnosed with CF after age 18 years (n = 786) and those diagnosed before 18 years (n = 6,641). RESULTS: In 1996, the incidence of late diagnosis was 7.8%, and the prevalence was 10.9%. The mean age of late diagnosis was 27 years. Respiratory symptoms most frequently led to late diagnosis. Patients receiving a late CF diagnosis were less likely to have alleles for Delta F508. There was no correlation between age at diagnosis and percent predicted FEV(1), although patients in the late-diagnosis group were an average of 10 years older than those in the early-diagnosis group. Late diagnosis was associated with fewer complications, fewer hospitalizations, less oxygen use, fewer courses of home IV treatment, and less enzyme use. Women were most often diagnosed late. Men displayed more diversity in conditions leading to diagnosis. Psychosocially, those patients receiving late diagnoses were more likely to be college graduates, married, and employed full time. For those adults who died in 1996, there was a positive association between their age at diagnosis and age at death. CONCLUSION: Those patients diagnosed with CF as adults differ, both medically and psychosocially, from those diagnosed at a younger age; these differences have implications for diagnosis, treatment, and education.

High-resolution CT in the acute exacerbation of cystic fibrosis: evaluation of acute findings, reversibility of those findings, and clinical correlation.

OBJECTIVE: The aims of this study were threefold: to compare high-resolution CT (HRCT) of adult patients with cystic fibrosis (CF) during acute exacerbations with asymptomatic patients with CF, to evaluate reversibility of HRCT abnormalities after exacerbations, and to correlate HRCT with clinical parameters. SUBJECTS AND METHODS: Nineteen symptomatic and eight asymptomatic patients were prospectively evaluated by HRCT and pulmonary function tests (PFTs). Symptomatic patients were reassessed 2 weeks after the exacerbation. Studies were independently reviewed by two radiologists using a modified Bhalla scoring system, noting the presence, extent, and severity of bronchiectasis, peribronchial thickening, mucus plugging, and atelectasis or consolidation. Modifications to the Bhalla system included evaluation of the presence and profusion of centrilobular nodules and air-fluid levels within bronchiectatic cavities. The highest possible score was 24 points. Higher scores indicated greater severity. Mosaic perfusion was noted but not included in the modified Bhalla HRCT score. Total modified Bhalla HRCT score and components of the HRCT score were correlated with corresponding PFT parameters. RESULTS: Bronchiectasis, peribronchial thickening, mucus plugging, centrilobular nodules, and mosaic perfusion were identified in symptomatic and asymptomatic patients. Air-fluid levels in bronchiectatic cavities, identified in two patients, represented the only finding limited to acute exacerbation. Reversible findings included air-fluid levels (100%), centrilobular nodules (36%), mucus plugging (33%), and peribronchial thickening (11%). Total HRCT severity scores of symptomatic and asymptomatic patients correlated with forced vital capacity (FVC) (r = .44, p = .01) and forced expiratory volume at 1 sec (FEV1) (r = .34, p = .04). Severity of bronchiectasis correlated with FVC (r = .50, p = .004) and FEV1 (r = .40, p = .02). Mucus plugging and centrilobular nodules did not correlate with PFT parameters. In the symptomatic patients, improvement in HRCT score correlated with changes in FEV1/FVC (r = .39, p = .049). CONCLUSION: Air-fluid levels in bronchiectatic cavities were the only parenchymal finding shown by HRCT that was limited to the acute exacerbation of CF in our study population. However, this finding was rare, being seen in two of 19 patients. Mucus plugging, centrilobular nodules, and peribronchial thickening were potentially reversible findings in symptomatic patients. HRCT accurately revealed disease severity in patients with CF. We also found that changes in HRCT scores correlated with clinical improvement as determined by PFTs.

Hemoptysis as the presenting symptom in bronchiolitis obliterans organizing pneumonia.

Bronchiolitis obliterans organizing pneumonia (BOOP) is an uncommon but increasingly recognized pulmonary entity that usually presents with symptoms of dyspnea, cough, and fever. The medical literature describes rare cases of hemoptysis in BOOP, with very small quantities of blood expectorated. We describe two cases of BOOP, one idiopathic and one in association with rheumatoid arthritis, in which large-quantity hemoptysis was the primary presenting symptom.

New treatment modalities for cystic fibrosis.

Refinements in standard therapy for cystic fibrosis have led to dramatic increases in survival and quality of life over the past three decades. Standard therapy has consisted of oral and intravenous antibiotics, chest percussion with postural drainage, and aerosol bronchodilator therapy. The discovery of the cystic fibrosis gene and elucidation of the underlying biochemical defect have broadened our understanding of the pathophysiology of cystic fibrosis and provided a rationale for many new and innovative therapies. Modulation of airway epithelial ion transport may improve mucociliary clearance and delay colonization by infective organisms. Anti-inflammatory therapy may decrease lung injury that results from the host's attempt to limit airway infection. Supplementation of airway antiproteases may limit the destructive effects of unopposed proteases on pulmonary architecture. Genetic biotechnology has already produced agents that preserve pulmonary function and decrease infectious exacerbations by altering the viscoelastic properties of sputum from patients with cystic fibrosis. Both active and passive immunotherapy are currently being investigated as a measure to delay or combat endobronchial infection with Pseudomonas spp. Aerosolized aminoglycoside antibiotics are being increasingly employed to control pulmonary infection while minimizing systemic toxicity. These treatment modalities, combined with the prospects for gene therapy, provide a brighter outlook for the patient with cystic fibrosis than ever before.

Pulmonary complications of cystic fibrosis in adults.

The demographics of cystic fibrosis (CF) are continuously changing, with adults representing a growing percentage of the patient population, which is expected to reach 50% by the year 2000. Pulmonary complications are primarily responsible for the high morbidity and mortality in this disease. Although the radiographic findings are quite specific, the correct diagnosis may not be suggested in the adult patient because of a lack of familiarity with its pulmonary manifestations in this age group. High-resolution CT (HRCT) has contributed to our understanding of the radiographic findings, especially at the level of the small airways. The role of imaging, including chest radiography and HRCT, is discussed. Issues that remain controversial include imaging in the acute pulmonary exacerbation, and the routine use of imaging as part of clinical scoring and in monitoring responses to new treatment modalities.

The comparative effects of elastase-induced emphysema on costal and crural diaphragm and parasternal intercostal muscle contractility.

Chronically, hyperinflated human subjects with chronic obstructive pulmonary disease and animals with experimentally induced emphysema generate greater than expected levels of transdiaphragmatic pressure at high lung volume because of adaptive changes in the length-tension relationship of the costal diaphragm. The ability to lower intrathoracic pressure during inspiration depends on the mechanical action of all the inspiratory muscles acting in concert. However, the effect of chronic hyperinflation on the mechanical action of inspiratory muscles other than the costal diaphragm remains unknown. This study compares the effect of chronic hyperinflation in the form of elastase-induced emphysema on the contractile properties of the three major inspiratory muscles of the hamster (i.e., the costal and crural diaphragm and parasternal intercostals). Muscles were studied in vitro 6 months after emphysema was induced by intratracheal injection of elastase. Saline-injected animals served as controls. TLC in the elastase-injected hamsters was significantly greater than in controls (12.5 +/- 0.8 ml versus 9.0 +/- 0.3 ml, p < 0.002). Maximal tetanic tension, time to peak tension, maximal velocity of shortening, and the force-velocity relationship differed among the three muscles but for any given muscle were similar in control and emphysematous animals. In contrast, the fiber length optimal for tension generation (Lo) not only differed across muscles but was significantly shorter in the costal diaphragm of emphysematous animals compared with control animals. However, Lo of the parasternal intercostal and crural diaphragm was similar in both groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Pleural effusions in right-sided endocarditis: characteristics and pathophysiology.

The incidence, characteristics, and pathogenesis of pleural effusions in patients with right-sided endocarditis (RSE) are poorly defined. We have recently observed four patients with a history of intravenous drug abuse and bacteremia due to Staphylococcus aureus who had pleural effusions during an episode of RSE. We report the pleural fluid characteristics of five effusions in these four patients and attempt to define the pathogenesis of each. We found that (1) an exudative, sterile, serosanguineous, or bloody effusion is common in RSE, (2) empyema occurred in only one patient, and (3) transudative effusions due to CHF were not observed. Possible mechanisms of pleural fluid formation in RSE include parapneumonic effusion, septic pulmonary emboli with or without infarction, and empyema. Congestive heart failure does not appear to be a common cause of pleural effusion in pure right-sided endocarditis.