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RATIONALE AND OBJECTIVES: Most radiologists are familiar with the classic chest radiographic findings of cystic fibrosis (CF) when these occur in children. We hypothesized that given the same findings, a diagnosis of CF would be less likely to be considered in an adult than in a child. METHODS: We compiled 30 pediatric and 28 adult CF chest radiographs and obtained two independent readings on each by different general radiologists among the eight who volunteered to participate as they performed their daily clinical work. The cases were presented to the readers so that they did not know which radiographs were part of the study. The association between the correct diagnosis of CF and whether the patient was an adult or a child was assessed using odds ratios and logistic regression, so that Brasfield score, Schwachman-Kulczycki score, and the patient's sex could also be considered as predictive of correct diagnosis. RESULTS: In 67% of the pediatric cases, at least one of the radiologists considered CF as a possible diagnosis, whereas they considered CF a possibility in only 50% of the adults. Both radiologists suggested the correct diagnosis in 40% of pediatric cases and only 14% of adult cases (p < .05). CONCLUSION: Because the radiographic findings were similar in the two groups of patients according to severity groupings, we believe CF was less commonly considered in the adult patient because of the traditional belief that CF is a childhood disease.

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The medical records of 142 patients with cystic fibrosis were reviewed. The patient group included 78 males and 64 females; three patients were black. Periods of observation ranged from two to 25 years (mean, 14.5 years). The analysis focused on clinical evaluation at age 18 years and included information gained at an earlier age. Evaluation at age 18 years was based on Shwachman and Kulczycki's (S-K) scoring system, Brasfield chest roentgenographic scoring system, pulmonary function measurements, height-adjusted weight percentile, sputum bacteriologic results, number of hospitalizations for treatment of pulmonary infections prior to the age of 18 years, time of onset of clubbing, and frequency of complications. There were no significant differences between the sexes in clinical features. Median survival from the time of diagnosis to the conclusion of the study period (1955 to 1984) was 22 years for females and 25 years for males (NS). Median length of survival beyond the age of 18 years was eight years for females and 12 years for males (NS). Stepwise logistic regression and Cox regression analysis applied to 11 variables identified the S-K clinical score at 18 years of age as the best predictor of survival to the age of 23 years. The median durations of survival after the age of 18 years for patients with clinical scores of 30 to 49, 50 to 64, and 65 to 75 at age 18 were five, seven and a half, and 12 years, respectively (p less than 0.0001). Low clinical score, low weight percentile, and Pseudomonas cepacia colonization of the lower respiratory tract at the age of 18 years indicated a poor prognosis. On the other hand, high clinical score, good weight percentile, and colonization with Staphylococcus aureus alone were likely to be found in patients with mild disease and an increased likelihood of long-term survival with preserved pancreatic function.

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In 3 male patients, chronic pulmonary sequelae followed influenza virus infection at 5, 24, and 42 months of age. Varying degrees of interstitial fibrosis, bronchial and bronchiolar erosions and metaplasia, obliterative bronchiolitis, and interstitial chronic inflammatory infiltrates were found on lung biopsy. Influenza A/Hong Kong/68 (H3N2) virus was isolated from the lung tissue of one patient 8 weeks after the onset of illness. This is the longest persistence of infectious virus in lung tissue yet reported. Persistent radiographic abnormalities included peribronchial thickening, interstitial densities, bronchiectasis, obliterative bronchiolitis, and segmental atelectasis. Pulmonary function tests showed an obstructive restrictive pattern, with mild improvement after bronchodilation and with deterioration after exercise. These observations suggest that influenza virus infection may be more serious in infants and young children than has been previously recognized and may contribute to the pathogenesis of unexplained interstitial pneumonitis, pulmonary fibrosis, obliterative bronchiolitis, and bronchiectasis.

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The majority (86.6%) of patients with cystic fibrosis were found to be carriers of Pseudomonas aeruginosa. None of them, however, carried P. aeruginosa in their nares. In contrast, none of the non-CF family members of the patients with CF were carriers of P. aeruginosa. For example, only 4 of 468 cultures from skin, throat, and nares of the family members were positive for P. aeruginosa. Isolations of P. aeruginosa from the same CF patients were often of the same pyocine type. No specific pyocine type of P. aeruginosa was predominant in patients with CF. Isolations of P. aeruginosa from siblings with CF may or may not be of the same pyocine type as that of the family proband. Colonization of a patient with CF by P. aeruginosa is not a threat to the non-CF members of the family.

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