Haemolytic uraemic syndrome

Last reviewed: 2 Jun 2022
Last updated: 22 Jun 2022

Summary

Definition

History and exam

Key diagnostic factors

  • diarrhoea, especially bloody diarrhoea
  • childhood, especially age <5 years
More key diagnostic factors

Other diagnostic factors

  • known community outbreak of Shiga toxin-producing E coli
  • history of ingestion of food that may have been contaminated with Shiga toxin-producing E coli
  • unusual adverse effect following treatment with ciclosporin, some chemotherapy agents, targeted cancer agents, and quinine
  • pregnancy or postpartum status
  • unusual adverse effect following bone marrow transplant
  • family history of possible HUS-like syndrome
Other diagnostic factors

Risk factors

  • ingestion of contaminated food or water
  • known community outbreak of toxicogenic E coli
  • exposure to infected individuals in institutional settings
  • genetic predisposition (atypical HUS)
  • bone marrow transplant
  • exposure to ciclosporin, some chemotherapy agents, targeted cancer agents, and quinine
  • pregnancy- or postpartum-related
More risk factors

Diagnostic investigations

1st investigations to order

  • FBC
  • peripheral blood smear
  • renal function/creatinine
  • serum electrolytes (sodium, potassium, chloride and bicarbonate, calcium and phosphorus)
  • prothrombin time (PT), PTT
  • LDH
  • haptoglobin
  • stool culture on sorbitol-MacConkey agar to detect Shiga toxin-producing Escherichia coli
  • polymerase chain reaction (PCR) to detect Shiga toxin 1/Shiga toxin 2
  • proteins involved in complement regulation
More 1st investigations to order

Investigations to consider

  • urinalysis
  • ADAMTS13 level
  • LFTs
  • serum amylase, lipase, glucose
More investigations to consider

Treatment algorithm

ACUTE

Shiga toxin-producing Escherichia coli (STEC) HUS

atypical HUS

secondary HUS: not due to Streptococcus pneumoniae

secondary HUS: due to 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.

  • Haemolytic uraemic syndrome images
  • Differentials

    • Thrombotic thrombocytopenic purpura (TTP)
    • Malignant hypertension
    • Systemic lupus erythematosus (SLE)
    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
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer