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Hemolytic Uremic Syndrome
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • HUS
        • Definitions

          • Thrombotic microangiopathy typically occurring after gastrointestinal infection with Shiga toxin-producing bacteria (classically E. coli O157:H7)
          • Clinical triad of hemolytic anemia, thrombocytopenia, & acute renal failure following severe diarrhea
            • Affected organ systems: Kidneys > CNS (20-50%) > gastrointestinal tract, heart
          • Atypical form (HUS without coexisting disease) often due to dysregulation in alternative complement pathway
            • Diarrhea less common; higher mortality

        IMAGING

        • General Features

          DIFFERENTIAL DIAGNOSIS

            PATHOLOGY

            • General Features

              • Microscopic Features

                CLINICAL ISSUES

                • Presentation

                  • Demographics

                    • Natural History & Prognosis

                      • Treatment

                        Selected References

                        1. Buder K et al: Neurodevelopmental long-term outcome in children after hemolytic uremic syndrome. Pediatr Nephrol. 30(3):503-13, 2015
                        2. Keir LS: Shiga toxin associated hemolytic uremic syndrome. Hematol Oncol Clin North Am. 29(3):525-39, 2015
                        3. Loirat C et al: An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. ePub, 2015
                        4. Hofer J et al: Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Front Pediatr. 2:97, 2014
                        5. Kaplan BS et al: Current treatment of atypical hemolytic uremic syndrome. Intractable Rare Dis Res. 3(2):34-45, 2014
                        6. Gitiaux C et al: Brain magnetic resonance imaging pattern and outcome in children with haemolytic-uraemic syndrome and neurological impairment treated with eculizumab. Dev Med Child Neurol. 55(8):758-65, 2013
                        7. Glatstein M et al: Timing and utility of ultrasound in diarrhea-associated hemolytic uremic syndrome: 7-year experience of a large tertiary care hospital. Clin Pediatr (Phila). 49(5):418-21, 2010
                        8. Toldo I et al: Diffusion-weighted imaging findings in hemolytic uremic syndrome with central nervous system involvement. J Child Neurol. 24(2):247-50, 2009
                        9. Scholbach TM: Changes of renal flow volume in the hemolytic-uremic syndrome--color Doppler sonographic investigations. Pediatr Nephrol. 16(8):644-7, 2001
                        Related Anatomy
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                        Related Differential Diagnoses
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                        References
                        Tables

                        Tables

                        KEY FACTS

                        • Terminology

                          • Imaging

                            • Clinical Issues

                              TERMINOLOGY

                              • Abbreviations

                                • HUS
                              • Definitions

                                • Thrombotic microangiopathy typically occurring after gastrointestinal infection with Shiga toxin-producing bacteria (classically E. coli O157:H7)
                                • Clinical triad of hemolytic anemia, thrombocytopenia, & acute renal failure following severe diarrhea
                                  • Affected organ systems: Kidneys > CNS (20-50%) > gastrointestinal tract, heart
                                • Atypical form (HUS without coexisting disease) often due to dysregulation in alternative complement pathway
                                  • Diarrhea less common; higher mortality

                              IMAGING

                              • General Features

                                DIFFERENTIAL DIAGNOSIS

                                  PATHOLOGY

                                  • General Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              Selected References

                                              1. Buder K et al: Neurodevelopmental long-term outcome in children after hemolytic uremic syndrome. Pediatr Nephrol. 30(3):503-13, 2015
                                              2. Keir LS: Shiga toxin associated hemolytic uremic syndrome. Hematol Oncol Clin North Am. 29(3):525-39, 2015
                                              3. Loirat C et al: An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. ePub, 2015
                                              4. Hofer J et al: Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Front Pediatr. 2:97, 2014
                                              5. Kaplan BS et al: Current treatment of atypical hemolytic uremic syndrome. Intractable Rare Dis Res. 3(2):34-45, 2014
                                              6. Gitiaux C et al: Brain magnetic resonance imaging pattern and outcome in children with haemolytic-uraemic syndrome and neurological impairment treated with eculizumab. Dev Med Child Neurol. 55(8):758-65, 2013
                                              7. Glatstein M et al: Timing and utility of ultrasound in diarrhea-associated hemolytic uremic syndrome: 7-year experience of a large tertiary care hospital. Clin Pediatr (Phila). 49(5):418-21, 2010
                                              8. Toldo I et al: Diffusion-weighted imaging findings in hemolytic uremic syndrome with central nervous system involvement. J Child Neurol. 24(2):247-50, 2009
                                              9. Scholbach TM: Changes of renal flow volume in the hemolytic-uremic syndrome--color Doppler sonographic investigations. Pediatr Nephrol. 16(8):644-7, 2001