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This Article Full Text (PDF) Free All Versions of this Article: chest.06-3082v1 132/2/569    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Kaza, V. Articles by Safdar, Z. Search for Related Content PubMed PubMed Citation Articles by Kaza, V. Articles by Safdar, Z.

Correlation of Chest X-Ray pattern with Genotype, Age, and Gender in Adult Cystic Fibrosis -- A Single Center Study

¹Division of Pulmonary-Critical Care Medicine, Baylor College of Medicine, Houston, Texas, 77030, USA

safdar{at}bcm.edu

Abstract

IntroductionCystic fibrosis (CF) is a common lethal genetic disorder. Aim of this study was to determine the common chest radiographic patterns in adult CF, and correlate disease distribution on chest radiograph (CXR) with genotype, age, and gender.

Methods109 CF patients treated at Baylor Adult Cystic Fibrosis Center were identified. Intake CXR was reviewed and characterized as diffuse bilateral (DB), unilateral (UniL), upper lobe (UL), and lower lobe disease (LL) or relatively normal (N). Lack of intake CXR, and/or genotype excluded 41 patients from analysis.

ResultsOf 68 patients, 38 were homozygous for F508, and 30 were heterozygous. Mean age of the population was 30±8 (mean±SD; range 18-48). The most common CXR pattern was DB. 62% had DB, 28% had UL and 7% had LL predominance. This is in contrast to UL predominant CXR pattern commonly described in pediatric population. In 18 DB patients, archived pediatric films were available and average patient age was 15.7 years. DB pattern was present in 16 of 18 CXR's that antedated adult intake CXR's by average of 12.7 years. Homozygous F508 genotype was identified in 56% of patients and did not distinguish radiological phenotypes. There was no association between X-ray pattern and identified infecting/colonizing organisms and percentage predicted FEV1.

ConclusionsCF has commonly been reported as UL disease. However, in this study of adult patients common pattern observed was DB. A small subgroup analysis suggests that DB disease was not a pattern of disease evolution but may be present from disease onset.

Key Words: chest film • thoracic radiology • airway • respiratory care • clinical research • lung function