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Síndrome hemolítico-urêmica

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 15 Apr 2025
Last updated: 22 Jun 2022

Summary

Definition

History and exam

Key diagnostic factors

  • diarreia, especialmente diarreia hemorrágica
  • infância, especialmente <5 anos de idade
Full details

Other diagnostic factors

  • surto na comunidade conhecido de E coli produtora da toxina Shiga
  • história de ingestão de alimentos que podem ter sido contaminados com E coli produtor da toxina Shiga
  • efeito adverso incomum após o tratamento com ciclosporina, alguns agentes quimioterápicos, agentes direcionados ao câncer e quinina
  • estado de gravidez ou pós-parto
  • efeito adverso incomum após transplante de medula óssea
  • história familiar de possível síndrome tipo síndrome hemolítico-urêmica (SHU)
Full details

Risk factors

  • ingestão de alimentos ou de água contaminada
  • surto na comunidade conhecido de E coli toxogênica
  • exposição a indivíduos infectados em ambientes institucionais
  • predisposição genética (SHU atípica)
  • transplante de medula óssea
  • exposição à ciclosporina, alguns agentes quimioterápicos, agentes específicos para câncer e quinina
  • relacionada à gravidez ou ao pós-parto
Full details

Diagnostic investigations

1st investigations to order

  • Hemograma completo
  • esfregaço de sangue periférico
  • função renal/creatinina
  • eletrólitos séricos (sódio, potássio, cloreto e bicarbonato, cálcio e fósforo)
  • tempo de protrombina (TP), TTP
  • lactato desidrogenase (LDH)
  • haptoglobina
  • coprocultura em ágar MacConkey-sorbitol para detectar Escherichia coli produtora de toxina Shiga
  • reação em cadeia da polimerase para detectar toxina Shiga 1/toxina Shiga 2
  • proteínas envolvidas na regulação do complemento
Full details

Investigations to consider

  • urinálise
  • nível de ADAMTS13
  • TFHs
  • amilase sérica, lipase, glicose
Full details

Treatment algorithm

ACUTE

SHU associada a Escherichia coli produtora de toxina Shiga (STEC)

SHU atípica

SHU secundária: não devida a Streptococcus pneumoniae

SHU secundária: decorrente de S pneumoniae

Contributors

Authors

Sharon Andreoli, MD

Byron P. and Francis D. Hollet Professor of Pediatrics, Pediatric Nephrology

Indiana University School of Medicine

Indianapolis

IN

Disclosures

SA owns stock in Merck and Pfizer, and has been a consultant for Reata Pharmaceuticals. SA is on the Editorial Board of Pediatric Nephrology and the Journal of Pediatrics.

Myda Khalid, MD

Associate Professor of Pediatric Nephrology

Indiana University School of Medicine

Indianapolis

IN

Disclosures

MK declares that she has no competing interests.

Acknowledgements

Dr Sharon Andreoli and Dr Myda Khalid would like to gratefully acknowledge Dr Ann Zimrin and Dr John Hess, previous contributors to this topic.

Disclosures

AZ and JH declare that they have no competing interests.

Peer reviewers

Rebecca Connor, MD

Chief Fellow

Section of Hematology and Oncology

Department of Internal Medicine

Wake Forest University Baptist Medical Center

Winston-Salem

NC

Disclosures

RC declares that she has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Fakhouri F, Zuber J, Frémeaux-Bacchi V, et al. Haemolytic uraemic syndrome. Lancet. 2017 Aug 12;390(10095):681-96. Abstract

Banatvala N, Griffin PM, Greene KD, et al. The United States national prospective hemolytic uremic syndrome study: microbiologic, serologic, clinical and epidemiologic findings. J Infect Dis. 2001 Apr 1;183(7):1063-70. Abstract

Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. 2005 Mar 19-25;365(9464):1073-86. Abstract

Wong CS, Jelacic S, Habeeb RL, et al. The risk of the hemolytic-uremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med. 2000 Jun 29;342(26):1930-6.Full text  Abstract

McKee RS, Schnadower D, Tarr PI, et al; Pediatric Emergency Medicine Collaborative Research Committee and Pediatric Emergency Research Canada. Predicting hemolytic uremic syndrome and renal replacement therapy in Shiga toxin-producing Escherichia coli-infected children. Clin Infect Dis. 2020 Apr 10;70(8):1643-51.Full text  Abstract

Boyce TG, Swerdlow DL, Griffin PM. Escherichia coli O157:H7 and the hemolytic uremic syndrome. N Engl J Med. 1995 Aug 10;333(6):364-8. Abstract

Rathbone J, Kaltenthaler E, Richards A, et al. A systematic review of eculizumab for atypical haemolytic uraemic syndrome (aHUS). BMJ Open. 2013 Nov 4;3(11):e003573.Full text  Abstract

Loirat C, Fakhouri F, Ariceta G, et al. An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol. 2016 Jan;31(1):15-39. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Síndrome hemolítico-urêmica images

  • Differentials

    • Púrpura trombocitopênica trombótica (PTT)
    • Hipertensão maligna
    • Lúpus eritematoso sistêmico (LES)
    More Differentials

  • Guidelines

    • Guidelines on hemolytic uremic syndrome by Indian Society of Pediatric Nephrology: key messages
    • Hemolytic uremic syndrome in a developing country: consensus guidelines
    More Guidelines
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