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Cystic Fibrosis
Laura E. Heyneman, MD; Gerald F. Abbott, MD, FACR
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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 causing mutations of CF transmembrane conductance regulator (CFTR) gene, which 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

            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

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
                                          2. Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
                                          3. Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
                                          4. 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
                                          5. Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
                                          6. Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
                                          7. Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
                                          8. 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
                                          9. 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 causing mutations of CF transmembrane conductance regulator (CFTR) gene, which 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

                                                    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

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
                                                                                  2. Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
                                                                                  3. Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
                                                                                  4. 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
                                                                                  5. Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
                                                                                  6. Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
                                                                                  7. Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
                                                                                  8. 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
                                                                                  9. Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005