Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Atypical Hemolytic Uremic Syndrome in a 13-year-old Lao Girl: A Case Report

  • Kedsatha, Philavanh (Department of Pediatrics, Children Hospital) ;
  • Cheong, Hae Il (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Choi, Yong (Department of Pediatrics, Seoul National University Children's Hospital)
  • Received : 2018.12.20
  • Accepted : 2019.01.26
  • Published : 2019.04.30
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Abstract

Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (antiCFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.

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References

  1. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med 2009;361:1676-87. https://doi.org/10.1056/NEJMra0902814
  2. Lee JM, Park YS, Lee JH, Park SJ, Shin JI, Park YH, et al. Atypical hemolytic uremic syndrome: Korean pediatric series. Pediatr Int 2015;57:431-8. https://doi.org/10.1111/ped.12549
  3. 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
  4. ragon-Durey MA, Loirat C, Cloarec S, Macher MA, Blouin J, Nivet H, et al. Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 2005;16:555-63. https://doi.org/10.1681/ASN.2004050380
  5. Lee BH, Kwak SH, Shin JI, Lee SH, Choi HJ, Kang HG, et al. Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1/CFHR3 deficiency. Pediatr Res 2009;66:336-40. https://doi.org/10.1203/PDR.0b013e3181b1bd4a
  6. Dragon-Durey MA, Sethi SK, Bagga A, Blanc C, Blouin J, Ranchin B, et al. Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome. J Am Soc Nephrol 2010;21:2180-7. https://doi.org/10.1681/ASN.2010030315
  7. chaefer F, Ardissino G, Ariceta G, Fakhouri F, Scully M, Isbel N, et al. Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome. Kidney Int 2018;94:408-18. https://doi.org/10.1016/j.kint.2018.02.029
  8. Sellier-Leclerc AL, Fremeaux-Bacchi V, Dragon-Durey MA, Macher MA, Niaudet P, Guest G, et al. Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome. J Am Soc Nephrol 2007;18:2392-400. https://doi.org/10.1681/ASN.2006080811
  9. inha A, Gulati A, Saini S, Blanc C, Gupta A, Gurjar BS, et al. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children. Kidney Int 2014;85:1151-60. https://doi.org/10.1038/ki.2013.373
  10. Hofer J, Janecke AR, Zimmerhackl LB, Riedl M, Rosales A, Giner T, et al. Complement factor H-related protein 1 deficiency and factor H antibodies in pediatric patients with atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 2013;8:407-15. https://doi.org/10.2215/CJN.01260212
  11. Brocklebank V, Johnson S, Sheerin TP, Marks SD, Gilbert RD, Tyerman K, et al. Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland. Kidney Int 2017;92:1261-71. https://doi.org/10.1016/j.kint.2017.04.028
  12. Jozsi M, Licht C, Strobel S, Zipfel SL, Richter H, Heinen S, et al. Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency. Blood 2008;111:1512-4. https://doi.org/10.1182/blood-2007-09-109876
  13. Khandelwal P, Gupta A, Sinha A, Saini S, Hari P, Dragon Durey MA, et al. Effect of plasma exchange and immunosuppressive medications on antibody titers and outcome in anti-complement factor H antibody-associated hemolytic uremic syndrome. Pediatr Nephrol 2015;30:451-7. https://doi.org/10.1007/s00467-014-2948-7
  14. Hisano M, Ashida A, Nakano E, Suehiro M, Yoshida Y, Matsumoto M, et al. Autoimmune-type atypical hemolytic uremic syndrome treated with eculizumab as first-line therapy. Pediatr Int 2015;57:313-7. https://doi.org/10.1111/ped.12469
  15. Ito N, Hataya H, Saida K, Amano Y, Hidaka Y, Motoyoshi Y, et al. Efficacy and safety of eculizumab in childhood atypical hemolytic uremic syndrome in Japan. Clin Exp Nephrol 2015;20:265-72.

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