Cystic fibrosis: CT assessment of lung involvement in children and adults

Thomas H. Helbich, Gertraud Heinz-Peer, Irmgard Eichler, Patrick Wunderbaldinger, Manfred Götz, Claudia Wojnarowski, Robert C. Brasch, Christian J. Herold

Research output: Contribution to journalArticlepeer-review

173 Scopus citations


PURPOSE: To compare a computed tomographic (CT)-based scoring system with nonimaging indexes of pulmonary status in patients with cystic fibrosis. MATERIALS AND METHODS: Pulmonary CT findings were assessed in 117 patients with cystic fibrosis, with cases classified according to three groups by age; 0-5 years, 6-16 years, and 17 years and older. Images were examined for specific abnormalities, and the severity and anatomic extent of each sign were used to generate a score. Scores in each category and the global score for each patient were correlated with pulmonary function test results, clinical status, serum immunoglobulin levels, and genotype, all obtained within 2 weeks of CT. RESULTS: The most frequent individual CT abnormalities were bronchiectasis in 94 (80.3%), peribronchial wall thickening in 89 (76.1%), mosaic perfusion in 71 (63.9%), and mucous plugging in 56 (51.3%) patients. The percentage of patients with specific CT findings and the overall CT scores increased-significantly (P < .05) with progressively increasing age groups. All CT findings and the overall CT scores correlated significantly (P < .05) with the pulmonary function test results, serum immunoglobulin levels, and clinical scores. No relationship was observed between genotype and CT scores. CONCLUSION: Scoring of CT studies in patients with cystic fibrosis seems to offer a reliable way to monitor disease status and progression and may provide a reasonable tool to assess treatment interventions.

Original languageEnglish (US)
Pages (from-to)537-544
Number of pages8
Issue number2
StatePublished - Nov 1999
Externally publishedYes


  • Bronchi, CT
  • Bronchiectasis
  • Children, respiratory system
  • Emphysema, pulmonary
  • Fibrosis, cystic
  • Lung, CT
  • Lung, interstitial disease

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging


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