Chest
Clinical Investigations: CYSTIC FIBROSISPneumothorax in Cystic Fibrosis
Section snippets
Materials and Methods
The CFF supports and accredits CF care centers nationwide. These centers provide specialized care for persons with CF, and offer comprehensive diagnosis and treatment, as well as participation in clinical trials of experimental therapies. The CFF Patient Registry contains data on patients with CF who have been seen at one of the accredited CF care centers in the United States. This represents about 90% of all CF patients in the United States. The CFF has sponsored the patient registry since
Results
There were 965 patients (3.4% of total population) who had experienced at least one episode of pneumothorax. Two additional patients were reported to have had a pneumothorax, but there were no associated dates, and they were excluded from the study. There were a total of 1,180 entries with pneumothorax listed as a complication; most patients had only one recorded event (788 patients; 82%), although many (177 patients; 18%) had experienced more than one reported event, and one patient had 5
Discussion
Spontaneous pneumothorax is a well-known complication in patients with CF and is far more common than in the general population. In addition, this study shows that subsequent pneumothorax is common with more than one in five of the patients having experienced at least two events in separate years. It is impossible to know from this database whether the subsequent pneumothorax was more commonly ipsilateral or contralateral, but both have been described in the literature.1
The principle risk
Acknowledgments
The authors are grateful to Dr. Preston Campbell for making the CFF Patient Registry data available, and to Dr. James Yankaskas for his editorial assistance.
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This study was supported in part by a grant from the CFF.
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