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Cystic Fibrosis, Pulmonary
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Cystic fibrosis (CF)
            • Synonyms

              • Mucoviscidosis, fibrocystic disease of pancreas
            • Definitions

              • Autosomal recessive multisystem disorder caused by dysfunctional chloride ion transport across epithelial surfaces → thickening of secretions (e.g., mucus, digestive fluids, sweat)

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Nuclear Medicine Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              • Reporting Tips

                                                Selected References

                                                1. Berkhout MC et al: CT-abnormalities, bacteriology and symptoms of sinonasal disease in children with Cystic Fibrosis. J Cyst Fibros. ePub, 2016
                                                2. Ferris H et al: Computed tomography dose optimisation in cystic fibrosis: a review. World J Radiol. 8(4):331-41, 2016
                                                3. Kuo W et al: Multicentre chest computed tomography standardisation in children and adolescents with cystic fibrosis: the way forward. Eur Respir J. ePub, 2016
                                                4. Murphy KP et al: Imaging of cystic fibrosis and pediatric bronchiectasis. AJR Am J Roentgenol. 206(3):448-54, 2016
                                                5. Mets OM et al: Emphysema is common in lungs of cystic fibrosis lung transplantation patients: a histopathological and computed tomography Study. PLoS One. 10(6):e0128062, 2015
                                                6. Sanders DB et al: Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis. Ann Am Thorac Soc. 12(1):64-9, 2015
                                                7. van Beek EJ: Personalizing medicine. Quantification of cystic fibrosis using computed tomography. Am J Respir Crit Care Med. 191(10):1098-9, 2015
                                                8. Wielpütz MO et al: Imaging modalities in cystic fibrosis: emerging role of MRI. Curr Opin Pulm Med. 21(6):609-16, 2015
                                                9. Xu H et al: [Allergic bronchopulmonary aspergillosis: a report of four cases with literature review.] Zhonghua Er Ke Za Zhi. 53(7):532-6, 2015
                                                10. Calder AD et al: Scoring of chest CT in children with cystic fibrosis: state of the art. Pediatr Radiol. 44(12):1496-506, 2014
                                                11. Locke LW et al: Quantitative imaging of airway liquid absorption in cystic fibrosis. Eur Respir J. 44(3):675-84, 2014
                                                12. Fleck R et al: Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI. Pediatr Radiol. 43(10):1279-86, 2013
                                                13. Vidal V et al: Bronchial artery embolization in adults with cystic fibrosis: impact on the clinical course and survival. J Vasc Interv Radiol. 17(6):953-8, 2006
                                                14. Grum CM et al: Chest radiographic findings in cystic fibrosis. Semin Respir Infect. 7(3):193-209, 1992
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Cystic fibrosis (CF)
                                                          • Synonyms

                                                            • Mucoviscidosis, fibrocystic disease of pancreas
                                                          • Definitions

                                                            • Autosomal recessive multisystem disorder caused by dysfunctional chloride ion transport across epithelial surfaces → thickening of secretions (e.g., mucus, digestive fluids, sweat)

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Ultrasonographic Findings

                                                                    • Angiographic Findings

                                                                      • Nuclear Medicine Findings

                                                                        • Imaging Recommendations

                                                                          DIFFERENTIAL DIAGNOSIS

                                                                            PATHOLOGY

                                                                            • General Features

                                                                              • Gross Pathologic & Surgical Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          • Image Interpretation Pearls

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Berkhout MC et al: CT-abnormalities, bacteriology and symptoms of sinonasal disease in children with Cystic Fibrosis. J Cyst Fibros. ePub, 2016
                                                                                              2. Ferris H et al: Computed tomography dose optimisation in cystic fibrosis: a review. World J Radiol. 8(4):331-41, 2016
                                                                                              3. Kuo W et al: Multicentre chest computed tomography standardisation in children and adolescents with cystic fibrosis: the way forward. Eur Respir J. ePub, 2016
                                                                                              4. Murphy KP et al: Imaging of cystic fibrosis and pediatric bronchiectasis. AJR Am J Roentgenol. 206(3):448-54, 2016
                                                                                              5. Mets OM et al: Emphysema is common in lungs of cystic fibrosis lung transplantation patients: a histopathological and computed tomography Study. PLoS One. 10(6):e0128062, 2015
                                                                                              6. Sanders DB et al: Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis. Ann Am Thorac Soc. 12(1):64-9, 2015
                                                                                              7. van Beek EJ: Personalizing medicine. Quantification of cystic fibrosis using computed tomography. Am J Respir Crit Care Med. 191(10):1098-9, 2015
                                                                                              8. Wielpütz MO et al: Imaging modalities in cystic fibrosis: emerging role of MRI. Curr Opin Pulm Med. 21(6):609-16, 2015
                                                                                              9. Xu H et al: [Allergic bronchopulmonary aspergillosis: a report of four cases with literature review.] Zhonghua Er Ke Za Zhi. 53(7):532-6, 2015
                                                                                              10. Calder AD et al: Scoring of chest CT in children with cystic fibrosis: state of the art. Pediatr Radiol. 44(12):1496-506, 2014
                                                                                              11. Locke LW et al: Quantitative imaging of airway liquid absorption in cystic fibrosis. Eur Respir J. 44(3):675-84, 2014
                                                                                              12. Fleck R et al: Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI. Pediatr Radiol. 43(10):1279-86, 2013
                                                                                              13. Vidal V et al: Bronchial artery embolization in adults with cystic fibrosis: impact on the clinical course and survival. J Vasc Interv Radiol. 17(6):953-8, 2006
                                                                                              14. Grum CM et al: Chest radiographic findings in cystic fibrosis. Semin Respir Infect. 7(3):193-209, 1992