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Gastrointestinal
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Cystic Fibrosis, Gastrointestinal Tract
Cystic Fibrosis, Gastrointestinal Tract
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      TERMINOLOGY

      • Definitions

        • Cystic fibrosis (CF): Autosomal recessive multisystem disorder caused by dysfunctional chloride ion transport across epithelial surfaces
          • Presenting symptoms of CF in infants & young children are most commonly GI related

      IMAGING

      • General Features

        • Radiographic Findings

          • Fluoroscopic Findings

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Levitte S et al: Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis. Pediatr Radiol. 51(8):1369-77, 2021
                                  2. Konrad J et al: Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators. Paediatr Respir Rev. ePub, 2020
                                  3. Toledano MB et al: The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study. PLoS One. 14(4):e0212779, 2019
                                  4. Dos Santos ALM et al: cystic fibrosis: clinical phenotypes in children and adolescents. Pediatr Gastroenterol Hepatol Nutr. 21(4):306-14, 2018
                                  5. Assis DN et al: Gastrointestinal disorders in cystic fibrosis. Clin Chest Med. 37(1):109-18, 2016
                                  6. Demeyer S et al: Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr. 175(7):881-94, 2016
                                  7. Kelly T et al: Gastrointestinal manifestations of cystic fibrosis. Dig Dis Sci. 60(7):1903-13, 2015
                                  8. Lavelle LP et al: Cystic fibrosis below the diaphragm: abdominal findings in adult patients. Radiographics. 35(3):680-95, 2015
                                  9. Leung DH et al: Baseline ultrasound and clinical correlates in children with cystic fibrosis. J Pediatr. 167(4):862-868.e2, 2015
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      TERMINOLOGY

                                      • Definitions

                                        • Cystic fibrosis (CF): Autosomal recessive multisystem disorder caused by dysfunctional chloride ion transport across epithelial surfaces
                                          • Presenting symptoms of CF in infants & young children are most commonly GI related

                                      IMAGING

                                      • General Features

                                        • Radiographic Findings

                                          • Fluoroscopic Findings

                                            • CT Findings

                                              • MR Findings

                                                • Ultrasonographic Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Levitte S et al: Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis. Pediatr Radiol. 51(8):1369-77, 2021
                                                                  2. Konrad J et al: Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators. Paediatr Respir Rev. ePub, 2020
                                                                  3. Toledano MB et al: The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study. PLoS One. 14(4):e0212779, 2019
                                                                  4. Dos Santos ALM et al: cystic fibrosis: clinical phenotypes in children and adolescents. Pediatr Gastroenterol Hepatol Nutr. 21(4):306-14, 2018
                                                                  5. Assis DN et al: Gastrointestinal disorders in cystic fibrosis. Clin Chest Med. 37(1):109-18, 2016
                                                                  6. Demeyer S et al: Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr. 175(7):881-94, 2016
                                                                  7. Kelly T et al: Gastrointestinal manifestations of cystic fibrosis. Dig Dis Sci. 60(7):1903-13, 2015
                                                                  8. Lavelle LP et al: Cystic fibrosis below the diaphragm: abdominal findings in adult patients. Radiographics. 35(3):680-95, 2015
                                                                  9. Leung DH et al: Baseline ultrasound and clinical correlates in children with cystic fibrosis. J Pediatr. 167(4):862-868.e2, 2015