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Gastrointestinal
Diagnosis
Abdominal Manifestations of Systemic Conditions
Metabolic or Inherited
Cystic Fibrosis
Cystic Fibrosis
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Cystic fibrosis (CF)

          • Synonyms

            • Mucoviscidosis

          • Definitions

            • Recessively inherited disorder of epithelial chloride transport caused by mutation of CF transmembrane conductance regulator (CFTR) gene
              • Pulmonary manifestations are primary cause of morbidity and mortality
              • CF increasingly seen to affect GI tract due to improved life expectancy

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Other Abdominal Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Lab

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  Selected References

                                  1. Dana J et al: Hepatic manifestations of cystic fibrosis. Curr Opin Gastroenterol. 36(3):192-8, 2020
                                  2. Lavelle LP et al: Cystic Fibrosis below the Diaphragm: abdominal Findings in Adult Patients. Radiographics. 35(3):680-95, 2015
                                  3. Keyzer C et al: Cystic fibrosis: unenhanced CT description of the appendix in asymptomatic adults. AJR Am J Roentgenol. 202(4):759-64, 2014
                                  4. Bilgin M et al: Ultrasonography and magnetic resonance imaging findings of abdominal complications of cystic fibrosis. Eur Rev Med Pharmacol Sci. 16 Suppl 4:48-51, 2012
                                  5. 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
                                  6. Haber HP: Cystic fibrosis in children and young adults: findings on routine abdominal sonography. AJR Am J Roentgenol. 189(1):89-99, 2007
                                  7. Robertson MB et al: Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 26(3):679-90, 2006
                                  8. Taylor CJ et al: The pancreas in cystic fibrosis. Paediatr Respir Rev. 3(1):77-81, 2002
                                  9. King LJ et al: Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 20(3):767-77, 2000
                                  10. 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

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Cystic fibrosis (CF)

                                          • Synonyms

                                            • Mucoviscidosis

                                          • Definitions

                                            • Recessively inherited disorder of epithelial chloride transport caused by mutation of CF transmembrane conductance regulator (CFTR) gene
                                              • Pulmonary manifestations are primary cause of morbidity and mortality
                                              • CF increasingly seen to affect GI tract due to improved life expectancy

                                          IMAGING

                                          • General Features

                                            • CT Findings

                                              • MR Findings

                                                • Ultrasonographic Findings

                                                  • Other Abdominal Findings

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Lab

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  Selected References

                                                                  1. Dana J et al: Hepatic manifestations of cystic fibrosis. Curr Opin Gastroenterol. 36(3):192-8, 2020
                                                                  2. Lavelle LP et al: Cystic Fibrosis below the Diaphragm: abdominal Findings in Adult Patients. Radiographics. 35(3):680-95, 2015
                                                                  3. Keyzer C et al: Cystic fibrosis: unenhanced CT description of the appendix in asymptomatic adults. AJR Am J Roentgenol. 202(4):759-64, 2014
                                                                  4. Bilgin M et al: Ultrasonography and magnetic resonance imaging findings of abdominal complications of cystic fibrosis. Eur Rev Med Pharmacol Sci. 16 Suppl 4:48-51, 2012
                                                                  5. 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
                                                                  6. Haber HP: Cystic fibrosis in children and young adults: findings on routine abdominal sonography. AJR Am J Roentgenol. 189(1):89-99, 2007
                                                                  7. Robertson MB et al: Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 26(3):679-90, 2006
                                                                  8. Taylor CJ et al: The pancreas in cystic fibrosis. Paediatr Respir Rev. 3(1):77-81, 2002
                                                                  9. King LJ et al: Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 20(3):767-77, 2000
                                                                  10. 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