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Hepatobiliary Cystic Fibrosis
Michael P. Federle, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Abbreviations

            • Cystic fibrosis (CF)
            • CF transmembrane conductance regulator (CFTR)
          • Synonyms

            • Mucoviscidosis
          • Definitions

            • Recessively inherited disorder of epithelial chloride transport
              • Affects hepatobiliary, pancreatic, and bowel functions in abdomen

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Imaging Recommendations

                    • Other Abdominal Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            • Lab

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Miguel M et al: Liver transplantation in children with cystic fibrosis: experience in our centre and preliminary results with a combined en bloc liver-pancreas graft. Eur J Pediatr Surg. 22(1):60-6, 2012
                                        2. Miller MR et al: Pulmonary function in individuals who underwent liver transplantation: from the US cystic fibrosis foundation registry. Liver Transpl. 18(5):585-93, 2012
                                        3. Ooi CY et al: Ursodeoxycholic acid in cystic fibrosis-associated liver disease. J Cyst Fibros. 11(1):72-3, 2012
                                        4. Chryssostalis A et al: Liver disease in adult patients with cystic fibrosis: a frequent and independent prognostic factor associated with death or lung transplantation. J Hepatol. 55(6):1377-82, 2011
                                        5. Costa PC et al: Cystic fibrosis-related liver disease: a single-center experience. Pediatr Rep. 3(3):e21, 2011
                                        6. Tümmler B et al: Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q. Thorax. 64(2):179-80, 2009
                                        7. Haber HP: Cystic fibrosis in children and young adults: findings on routine abdominal sonography. AJR Am J Roentgenol. 189(1):89-99, 2007
                                        8. Robertson MB et al: Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 26(3):679-90, 2006
                                        9. Taylor CJ et al: The pancreas in cystic fibrosis. Paediatr Respir Rev. 3(1):77-81, 2002
                                        10. King LJ et al: Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 20(3):767-77, 2000
                                        11. Soyer P et al: Cystic fibrosis in adolescents and adults: fatty replacement of the pancreas--CT evaluation and functional correlation. Radiology. 210(3):611-5, 1999
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Cystic fibrosis (CF)
                                                  • CF transmembrane conductance regulator (CFTR)
                                                • Synonyms

                                                  • Mucoviscidosis
                                                • Definitions

                                                  • Recessively inherited disorder of epithelial chloride transport
                                                    • Affects hepatobiliary, pancreatic, and bowel functions in abdomen

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Ultrasonographic Findings

                                                        • Imaging Recommendations

                                                          • Other Abdominal Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Microscopic Features

                                                                  • Lab

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Miguel M et al: Liver transplantation in children with cystic fibrosis: experience in our centre and preliminary results with a combined en bloc liver-pancreas graft. Eur J Pediatr Surg. 22(1):60-6, 2012
                                                                              2. Miller MR et al: Pulmonary function in individuals who underwent liver transplantation: from the US cystic fibrosis foundation registry. Liver Transpl. 18(5):585-93, 2012
                                                                              3. Ooi CY et al: Ursodeoxycholic acid in cystic fibrosis-associated liver disease. J Cyst Fibros. 11(1):72-3, 2012
                                                                              4. Chryssostalis A et al: Liver disease in adult patients with cystic fibrosis: a frequent and independent prognostic factor associated with death or lung transplantation. J Hepatol. 55(6):1377-82, 2011
                                                                              5. Costa PC et al: Cystic fibrosis-related liver disease: a single-center experience. Pediatr Rep. 3(3):e21, 2011
                                                                              6. Tümmler B et al: Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q. Thorax. 64(2):179-80, 2009
                                                                              7. Haber HP: Cystic fibrosis in children and young adults: findings on routine abdominal sonography. AJR Am J Roentgenol. 189(1):89-99, 2007
                                                                              8. Robertson MB et al: Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 26(3):679-90, 2006
                                                                              9. Taylor CJ et al: The pancreas in cystic fibrosis. Paediatr Respir Rev. 3(1):77-81, 2002
                                                                              10. King LJ et al: Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 20(3):767-77, 2000
                                                                              11. Soyer P et al: Cystic fibrosis in adolescents and adults: fatty replacement of the pancreas--CT evaluation and functional correlation. Radiology. 210(3):611-5, 1999