Journal of Trauma and Injury

The Korean Society of Traumatology (대한외상학회)
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Atypical Hemolytic Uremic Syndrome after Traumatic Rectal Injury: A Case Report

  • Kang, Ji-Hyoun (Division of Rheumatology, Department of Internal Medicine, Chonnam National University Hospital, Chonnam National University Medical School) ;
  • Lee, Donghyun (Division of Trauma Surgery, Department of Surgery, Chonnam National University Hospital, Chonnam National University Medical School) ;
  • Park, Yunchul (Division of Trauma Surgery, Department of Surgery, Chonnam National University Hospital, Chonnam National University Medical School)
  • Received : 2020.11.02
  • Accepted : 2021.02.01
  • Published : 2021.12.30
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Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, life-threatening condition of thrombotic microangiopathy characterized by thrombocytopenia, microangiopathic hemolytic anemia, and renal impairment. The mechanisms underlying aHUS remain unclear. Herein, we present the first case in the literature of aHUS after a traumatic injury. A 55-year-old male visited the emergency department after a traumatic injury caused by a tree limb. Abdominal computed tomography revealed a rectal wall defect with significant air density in the perirectal space and preperitoneum, implying rectal perforation. Due to the absence of intraperitoneal intestinal perforation, we performed diverting sigmoid loop colostomy. An additional intermittent simple repair was performed due to perianal and anal injuries. One day postoperatively, his urine output abruptly decreased and serum creatinine level increased. His platelet level decreased, and a spiking fever occurred after 2 days. The patient was diagnosed with acute renal failure secondary to aHUS and was treated with fresh frozen plasma replacement. Continuous renal replacement therapy (CRRT) was also started for oliguria and uremic symptoms. The patient received CRRT for 3 days and intermittent hemodialysis thereafter. After hemodialysis and subsequent supportive treatment, his urine output and renal function improved. The hemolytic anemia and thrombocytopenia also gradually improved. Dialysis was terminated on day 22 of admission and the patient was discharged after recovery. This case suggests that that a traumatic event can trigger aHUS, which should be considered in patients who have thrombocytopenia and acute renal failure with microangiopathic hemolytic anemia. Early diagnosis and appropriate management are critical for favorable outcomes.

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References

  1. Schmidtko J, Peine S, El-Housseini Y, Pascual M, Meier P. Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab. Am J Kidney Dis 2013;61:289-99. https://doi.org/10.1053/j.ajkd.2012.07.028
  2. Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood 2006;108:1267-79.
  3. Laurence J. Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol 2012;10(10 Suppl 17):1-12.
  4. Constantinescu AR, Bitzan M, Weiss LS, Christen E, Kaplan BS, Cnaan A, et al. Non-enteropathic hemolytic uremic syndrome: causes and short-term course. Am J Kidney Dis 2004;43:976-82. https://doi.org/10.1053/j.ajkd.2004.02.010
  5. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med 2009;361:1676-87. https://doi.org/10.1056/NEJMra0902814
  6. Besbas N, Karpman D, Landau D, Loirat C, Proesmans W, Remuzzi G, et al. A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int 2006;70:423-31. https://doi.org/10.1038/sj.ki.5001581
  7. Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS). Pediatr Nephrol 2008;23:1749-60. https://doi.org/10.1007/s00467-008-0935-6
  8. Laurence J, Haller H, Mannucci PM, Nangaku M, Praga M, Rodriguez de Cordoba S. Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol 2016;14(Suppl 11):2-15.
  9. Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol 2010;5:1844-59. https://doi.org/10.2215/CJN.02210310
  10. Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaime F, Dragon-Durey MA, Ngo S, et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 2013;8:554-62. https://doi.org/10.2215/CJN.04760512
  11. Jokiranta TS. HUS and atypical HUS. Blood 2017;129:2847-56. https://doi.org/10.1182/blood-2016-11-709865
  12. Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol 2016;31:15-39. https://doi.org/10.1007/s00467-015-3076-8
  13. Raina R, Krishnappa V, Blaha T, Kann T, Hein W, Burke L, et al. Atypical hemolytic-uremic syndrome: an update on pathophysiology, diagnosis, and treatment. Ther Apher Dial 2019;23:4-21. https://doi.org/10.1111/1744-9987.12763
  14. Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 2001;60:831-46. https://doi.org/10.1046/j.1523-1755.2001.060003831.x
  15. Huber-Lang M, Gebhard F, Schmidt CQ, Palmer A, Denk S, Wiegner R. Complement therapeutic strategies in trauma, hemorrhagic shock and systemic inflammation - closing Pandora's box?. Semin Immunol 2016;28:278-84. https://doi.org/10.1016/j.smim.2016.04.005
  16. Weitz M, Amon O, Bassler D, Koenigsrainer A, Nadalin S. Prophylactic eculizumab prior to kidney transplantation for atypical hemolytic uremic syndrome. Pediatr Nephrol 2011;26:1325-9. https://doi.org/10.1007/s00467-011-1879-9
  17. Nester C, Stewart Z, Myers D, Jetton J, Nair R, Reed A, et al. Pre-emptive eculizumab and plasmapheresis for renal transplant in atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 2011;6:1488-94. https://doi.org/10.2215/CJN.10181110
  18. Krishnappa V, Gupta M, Elrifai M, Moftakhar B, Ensley MJ, Vachharajani TJ, et al. Atypical hemolytic uremic syndrome: a meta-analysis of case reports confirms the prevalence of genetic mutations and the shift of treatment regimens. Ther Apher Dial 2018;22:178-88. https://doi.org/10.1111/1744-9987.12641