Skip to main content

Hemolytic Uremic Syndrome

  • Chapter
  • First Online:
  • 1384 Accesses

Abstract

There have been major advances in the understanding of pathogenesis and management of hemolytic uremic syndrome in the last decade. Since the consensus paper published in 2009 by the European Pediatric Study Group in 2009, there have been four prospective trials and multiple case reports on effective terminal blockade with Eculizumab, an international consensus on the diagnostic and therapeutic strategies for management has been published in 2015.

This is a preview of subscription content, log in via an institution.

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD   139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Kidney Disease Outcomes Quality Initiative. Kidney Disease Improving Global Outcomes (KDIGO) clinical practice guideline for acute kidney injury. Kidney Int Suppl. 2012;2:19–36.

    Article  Google Scholar 

  2. Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C, Loirat C, Pecoraro C, Taylor CM, Van de Kar N, Vandewalle J, Zimmerhackl LB, European Paediatric Study Group for HUS. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009;24(4):687–96.

    Article  Google Scholar 

  3. Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Frémeaux-Bacchi V, HUS International. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016;31(1):15–39.

    Article  Google Scholar 

  4. Mody RK, Gu W, Griffin PM, Jones TF, Rounds J, Shiferaw B, Tobin-D’Angelo M, Smith G, Spina N, Hurd S, Lathrop S, Palmer A, Boothe E, Luna-Gierke RE, Hoekstra RM. Postdiarrheal hemolytic uremic syndrome in United States children: clinical spectrum and predictors of in-hospital death. J Pediatr. 2015;166:1022–9.

    Article  Google Scholar 

  5. Agger M, Scheutz F, Villumsen S, Mølbak K, Petersen AM. Antibiotic treatment of verocytotoxin-producing Escherichia coli (VTEC) infection: a systematic review and a proposal. J Antimicrob Chemother. 2015;70:2440–6.

    Article  CAS  Google Scholar 

  6. Freedman SB, Xie J, Neufeld MS, Hamilton WL, Hartling L, Tarr PI, Alberta Provincial Pediatric Enteric Infection Team, Nettel-Aguirre A, Chuck A, Lee B, Johnson D, Currie G, Talbot J, Jiang J, Dickinson J, Kellner J, MacDonald J, Svenson L, Chui L, Louie M, Lavoie M, Eltorki M, Vanderkooi O, Tellier R, Ali S, Drews S, Graham T, Pang XL. Shiga toxin-producing escherichia coli infection, antibiotics, and risk of developing hemolytic uremic syndrome: a meta-analysis. Clin Infect Dis. 2016;62:1251–8.

    Article  Google Scholar 

  7. Kielstein JT, Beutel G, Fleig S, Steinhoff J, Meyer TN, Hafer C, Kuhlmann U, Bramstedt J, Panzer U, Vischedyk M, Busch V, Ries W, Mitzner S, Mees S, Stracke S, Nurnberger J, Gerke P, Wiesner M, Sucke B, Abu-Tair M, Kribben A, Klause N, Schindler R, Merkel F, Schnatter S, Dorresteijn EM, Samuelsson O, Brunkhorst R, Collaborators of the DS-HUSr. Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant. 2012;27:3807–15.

    Article  CAS  Google Scholar 

  8. Pape L, Hartmann H, Bange FC, Suerbaum S, Bueltmann E, Ahlenstiel-Grunow T. Eculizumab in typical hemolytic uremic syndrome (HUS) with neurological involvement. Medicine (Baltimore). 2015;94:e1000.

    Article  CAS  Google Scholar 

  9. Huemer M, Diodato D, Schwahn B, Schiff M, Bandeira A, Benoist JF, Burlina A, Cerone R, Couce ML, Garcia-Cazorla A, la Marca G, Pasquini E, Vilarinho L, Weisfeld-Adams JD, Kozich V, Blom H, Baumgartner MR, Dionisi-Vici C. Guidelines for diagnosis and management of the cobalamin-related remethylation disorders cblC, cblD, cblE, cblF, cblG, cblJ and MTHFR deficiency. J Inherit Metab Dis. 2017;40:21–48.

    Article  CAS  Google Scholar 

  10. Johnson S, Stojanovic J, Ariceta G, Bitzan M, Besbas N, Frieling M, Karpman D, Landau D, Langman C, Licht C, Pecoraro C, Riedl M, Siomou E, van de Kar N, Walle JV, Loirat C, Taylor CM. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol. 2014;29(10):1967–78.

    Article  Google Scholar 

  11. Noris M, Caprioli J, Bresin E, Mossali C, Pianetti G, Gamba S, Daina E, Fenili C, Castelletti F, Sorosina A, Piras R, Donadelli R, Maranta R, van der Meer I, Conway EM, Zipfel PF, Goodship TH, Remuzzi G. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol. 2010;5(10):1844–59.

    Article  CAS  Google Scholar 

  12. Durey MA, Sinha A, Togarsimalemath SK, Bagga A. Anti-complement-factor H-associated glomerulopathies. Nat Rev Nephrol. 2016;12(9):563–78.

    Article  CAS  Google Scholar 

  13. Sinha A, Gulati A, Saini S, Blanc C, Gupta A, Gurjar BS, Saini H, Kotresh ST, Ali U, Bhatia D, Ohri A, Kumar M, Agarwal I, Gulati S, Anand K, Vijayakumar M, Sinha R, Sethi S, Salmona M, George A, Bal V, Singh G, Dinda AK, Hari P, Rath S, Dragon-Durey MA, Bagga A, Indian HUS Registry. Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children. Kidney Int. 2014;85(5):1151–60. https://doi.org/10.1038/ki.2013.373.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Sethi, S.K. (2019). Hemolytic Uremic Syndrome. In: Sethi, S., Raina, R., McCulloch, M., Bunchman, T. (eds) Critical Care Pediatric Nephrology and Dialysis: A Practical Handbook. Springer, Singapore. https://doi.org/10.1007/978-981-13-2276-1_15

Download citation

  • DOI: https://doi.org/10.1007/978-981-13-2276-1_15

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-13-2275-4

  • Online ISBN: 978-981-13-2276-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics