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Cystic Fibrosis
Aletta Ann Frazier, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Cystic fibrosis (CF)
            • Synonyms

              • Mucoviscidosis
            • Definitions

              • Autosomal recessive disorder; mutations of CF transmembrane conductance regulator (CFTR) gene; regulates chloride transport
                • Abnormally viscous secretions from exocrine glands (salivary and sweat glands, pancreas, large bowel, tracheobronchial tree)
                • Multiorgan involvement; primarily lungs and pancreas
              • Most common fatal hereditary disease in Caucasians
              • Accounts for up to 25% of adult cases of bronchiectasis
              • Variable severity attributed to spectrum of CFTR mutations

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. McCallister A et al: Comparison of single breath hyperpolarized 129 Xe MRI with dynamic 19 F MRI in cystic fibrosis lung disease. Magn Reson Med. 85(2):1028-38, 2021
                                        2. Almughem FA et al: Cystic fibrosis: overview of the current development trends and innovative therapeutic strategies. Pharmaceutics. 12(7), 2020
                                        3. Benlala I et al: Quantification of MRI T2-weighted high signal volume in cystic fibrosis: a pilot study. Radiology. 294(1):186-96, 2020
                                        4. Chassagnon G et al: Quantification of cystic fibrosis lung disease with radiomics-based CT scores. Radiol Cardiothorac Imaging. 2(6):e200022, 2020
                                        5. Lattanzi C et al: Allergic bronchopulmonary aspergillosis in children with cystic fibrosis: an update on the newest diagnostic tools and therapeutic approaches. Pathogens. 9(9), 2020
                                        6. Renner S et al: Update on respiratory fungal infections in cystic fibrosis lung disease and after lung transplantation. J Fungi (Basel). 6(4), 2020
                                        7. Revel MP et al: Use of MRI to measure bronchial inflammation in cystic fibrosis. Radiology. 294(1):197-8, 2020
                                        8. Bergeron C et al: Cystic fibrosis: pathophysiology of lung disease. Semin Respir Crit Care Med. 40(6):715-26, 2019
                                        9. Burke AP et al: Bronchiectasis. In Practical Thoracic Pathology: Diseases of the Lung, Heart, and Thymus. Wolters Kluwer.137-41, 2017
                                        10. Nyilas S et al: Novel magnetic resonance technique for functional imaging of cystic fibrosis lung disease. Eur Respir J. 50(6), 2017
                                        11. Elborn JS: Cystic fibrosis. Lancet. 388(10059):2519-31, 2016
                                        12. Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
                                        13. Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
                                        14. Hasleton P et al: Congenital abnormalities and pediatric lung diseases, including neoplasms. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 97-100, 2013
                                        15. Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
                                        16. O'Connell OJ et al: Radiologic imaging in cystic fibrosis: cumulative effective dose and changing trends over 2 decades. Chest. 141(6):1575-83, 2012
                                        17. Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
                                        18. Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
                                        19. Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
                                        20. de Jong PA et al: Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 61(1):80-5, 2006
                                        21. Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Cystic fibrosis (CF)
                                                  • Synonyms

                                                    • Mucoviscidosis
                                                  • Definitions

                                                    • Autosomal recessive disorder; mutations of CF transmembrane conductance regulator (CFTR) gene; regulates chloride transport
                                                      • Abnormally viscous secretions from exocrine glands (salivary and sweat glands, pancreas, large bowel, tracheobronchial tree)
                                                      • Multiorgan involvement; primarily lungs and pancreas
                                                    • Most common fatal hereditary disease in Caucasians
                                                    • Accounts for up to 25% of adult cases of bronchiectasis
                                                    • Variable severity attributed to spectrum of CFTR mutations

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Angiographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. McCallister A et al: Comparison of single breath hyperpolarized 129 Xe MRI with dynamic 19 F MRI in cystic fibrosis lung disease. Magn Reson Med. 85(2):1028-38, 2021
                                                                              2. Almughem FA et al: Cystic fibrosis: overview of the current development trends and innovative therapeutic strategies. Pharmaceutics. 12(7), 2020
                                                                              3. Benlala I et al: Quantification of MRI T2-weighted high signal volume in cystic fibrosis: a pilot study. Radiology. 294(1):186-96, 2020
                                                                              4. Chassagnon G et al: Quantification of cystic fibrosis lung disease with radiomics-based CT scores. Radiol Cardiothorac Imaging. 2(6):e200022, 2020
                                                                              5. Lattanzi C et al: Allergic bronchopulmonary aspergillosis in children with cystic fibrosis: an update on the newest diagnostic tools and therapeutic approaches. Pathogens. 9(9), 2020
                                                                              6. Renner S et al: Update on respiratory fungal infections in cystic fibrosis lung disease and after lung transplantation. J Fungi (Basel). 6(4), 2020
                                                                              7. Revel MP et al: Use of MRI to measure bronchial inflammation in cystic fibrosis. Radiology. 294(1):197-8, 2020
                                                                              8. Bergeron C et al: Cystic fibrosis: pathophysiology of lung disease. Semin Respir Crit Care Med. 40(6):715-26, 2019
                                                                              9. Burke AP et al: Bronchiectasis. In Practical Thoracic Pathology: Diseases of the Lung, Heart, and Thymus. Wolters Kluwer.137-41, 2017
                                                                              10. Nyilas S et al: Novel magnetic resonance technique for functional imaging of cystic fibrosis lung disease. Eur Respir J. 50(6), 2017
                                                                              11. Elborn JS: Cystic fibrosis. Lancet. 388(10059):2519-31, 2016
                                                                              12. Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
                                                                              13. Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
                                                                              14. Hasleton P et al: Congenital abnormalities and pediatric lung diseases, including neoplasms. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 97-100, 2013
                                                                              15. Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
                                                                              16. O'Connell OJ et al: Radiologic imaging in cystic fibrosis: cumulative effective dose and changing trends over 2 decades. Chest. 141(6):1575-83, 2012
                                                                              17. Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
                                                                              18. Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
                                                                              19. Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
                                                                              20. de Jong PA et al: Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 61(1):80-5, 2006
                                                                              21. Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005