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

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.

[1]  B. Kerem,et al.  Correlation between genotype and phenotype in patients with cystic fibrosis. , 1994, The New England journal of medicine.

[2]  J. Remy,et al.  Pulmonary involvement in progressive systemic sclerosis: sequential evaluation with CT, pulmonary function tests, and bronchoalveolar lavage. , 1993, Radiology.

[3]  D. McCauley,et al.  Cystic fibrosis: scoring system with thin-section CT. , 1991, Radiology.

[4]  J. Kattwinkel,et al.  A new prognostic score and clinical evaluation system for cystic fibrosis. , 1973, The Journal of pediatrics.

[5]  M. Candusso,et al.  Cystic Fibrosis in Children: HRCT Findings and Distribution of Disease , 1996, Journal of thoracic imaging.

[6]  K S Lee,et al.  CT of the chest: minimal tube current required for good image quality with the least radiation dose. , 1995, AJR. American journal of roentgenology.

[7]  C. Grum,et al.  Spirometry and chest roentgenographic appearance in adults with cystic fibrosis. , 1992, Chest.

[8]  R. Stern,et al.  Cultures of thoracotomy specimens confirm usefulness of sputum cultures in cystic fibrosis. , 1984, The Journal of pediatrics.

[9]  D M Hansell,et al.  Bronchiolitis obliterans: high resolution CT findings and correlation with pulmonary function tests. , 1993, Clinical radiology.

[10]  D M Hansell,et al.  Bronchiectasis: functional significance of areas of decreased attenuation at expiratory CT. , 1994, Radiology.

[11]  R E COOKE,et al.  A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis. , 1959, Pediatrics.

[12]  P. Schur,et al.  Immunoglobulin Subclasses in Normal Children , 1979, Pediatric Research.

[13]  G. T. te Meerman,et al.  Long term prognosis of patients with cystic fibrosis in relation to early detection by neonatal screening and treatment in a cystic fibrosis centre. , 1995, Thorax.

[14]  L. Tsui,et al.  Rapid nonradioactive detection of the major cystic fibrosis mutation. , 1990, American journal of human genetics.

[15]  S. Greenberg,et al.  The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. , 1976, Human pathology.

[16]  W. Wheeler,et al.  Progression of cystic fibrosis lung disease as a function of serum immunoglobulin G levels: a 5-year longitudinal study. , 1984, The Journal of pediatrics.

[17]  J. H. Comroe,et al.  A new method for measuring airway resistance in man using a body plethysmograph: values in normal subjects and in patients with respiratory disease. , 1956, The Journal of clinical investigation.

[18]  H. Shwachman,et al.  Long-term study of one hundred five patients with cystic fibrosis; studies made over a five- to fourteen-year period. , 1958, A.M.A. journal of diseases of children.

[19]  C. Ruzal-Shapiro.  Cystic fibrosis. An overview. , 1998, Radiologic clinics of North America.

[20]  A. Zapletal,et al.  Lung Function in Children and Adolescents: Methods, reference values , 1987 .

[21]  D. Hansell,et al.  High-resolution computed tomography in pulmonary cystic fibrosis. , 1989, The British journal of radiology.

[22]  B. Wood.  Cystic fibrosis: 1997. , 1997, Radiology.

[23]  A. Friedman,et al.  High-resolution CT in the acute exacerbation of cystic fibrosis: evaluation of acute findings, reversibility of those findings, and clinical correlation. , 1997, AJR. American journal of roentgenology.

[24]  D. Zurakowski,et al.  Cystic fibrosis: a system for assessing and predicting progression. , 1998, AJR. American journal of roentgenology.

[25]  N. Müller,et al.  Diseases of the bronchioles: CT and histopathologic findings. , 1995, Radiology.

[26]  J. Katz,et al.  Clinical features as predictors of functional status in children with cystic fibrosis. , 1986, The Journal of pediatrics.

[27]  G. Owen,et al.  Severity of chest disease in cystic fibrosis patients in relation to their genotypes. , 1992, Journal of medical genetics.

[28]  K. Conboy,et al.  Ultrafast computerized tomography of the chest in cystic fibrosis: A new scoring system , 1991, Pediatric pulmonology.

[29]  J. Peters,et al.  Evaluation of scoring system of the chest radiograph in cystic fibrosis: a collaborative study. , 1980, AJR. American journal of roentgenology.