Shiga toxin-producing Escherichia coli (STEC) HUS is most common in young children (<5 years), but it can be seen at any age, with decreasing frequency in older children and adults.[7]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.
http://www.ncbi.nlm.nih.gov/pubmed/11237831?tool=bestpractice.com
Although large outbreaks have been reported, it is more commonly seen in small clusters or sporadically.[8]Bell BP, Goldoft M, Griffin PM, et al. A multistate outbreak of Escherichia coli 0157:H7-associated bloody diarrhea and hemolytic uremic syndrome from hamburgers. The Washington experience. JAMA. 1994 Nov 2;272(17):1349-53.
http://www.ncbi.nlm.nih.gov/pubmed/7933395?tool=bestpractice.com
[9]Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. 2005 Mar 19-25;365(9464):1073-86.
http://www.ncbi.nlm.nih.gov/pubmed/15781103?tool=bestpractice.com
[10]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.
https://www.nejm.org/doi/full/10.1056/NEJM200006293422601
http://www.ncbi.nlm.nih.gov/pubmed/10874060?tool=bestpractice.com
The annual incidence in the US in children <5 years is 2 to 3 per 100,000.[11]Noris M, Remuzzi G. Hemolytic uremic syndrome. J Am Soc Nephrol. 2005 Apr;16(4):1035-50.
https://jasn.asnjournals.org/content/16/4/1035.long
http://www.ncbi.nlm.nih.gov/pubmed/15728781?tool=bestpractice.com
In Europe, the prevalence of atypical or recurrent HUS is 3.3 per million children <18 years of age.[12]Zimmerhackl LB, Besbas N, Jungraithmayr T, et al; European Study Group for Haemolytic Uraemic Syndromes and Related Disorders. Epidemiology, clinical presentation and pathophysiology of atypical and recurrent hemolytic uremic syndrome. Semin Thromb Hemost. 2006 Mar;32(2):113-20.
http://www.ncbi.nlm.nih.gov/pubmed/16575686?tool=bestpractice.com
HUS is diagnosed in approximately 15% of children with STEC infections, approximately 5 to 10 days after the onset of diarrhoea.[8]Bell BP, Goldoft M, Griffin PM, et al. A multistate outbreak of Escherichia coli 0157:H7-associated bloody diarrhea and hemolytic uremic syndrome from hamburgers. The Washington experience. JAMA. 1994 Nov 2;272(17):1349-53.
http://www.ncbi.nlm.nih.gov/pubmed/7933395?tool=bestpractice.com
[9]Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. 2005 Mar 19-25;365(9464):1073-86.
http://www.ncbi.nlm.nih.gov/pubmed/15781103?tool=bestpractice.com
[13]Adams N, Byrne L, Rose T, et al. Sociodemographic and clinical risk factors for paediatric typical haemolytic uraemic syndrome: retrospective cohort study. BMJ Paediatr Open. 2019 Dec 17;3(1):e000465.
https://bmjpaedsopen.bmj.com/content/3/1/e000465
http://www.ncbi.nlm.nih.gov/pubmed/31909217?tool=bestpractice.com
[14]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.
https://academic.oup.com/cid/article/70/8/1643/5498369
http://www.ncbi.nlm.nih.gov/pubmed/31125419?tool=bestpractice.com
In England, 539 confirmed cases involving Escherichia coli O157 infections were reported in 2019.[15]Public Health England. Shiga toxin-producing Escherichia coli (STEC) data: 2019. Apr 2022 [internet publication].
https://www.gov.uk/government/publications/escherichia-coli-e-coli-o157-annual-totals/shiga-toxin-producing-escherichia-coli-stec-data-2019
Of these, 29% required hospitalisation and 3% developed HUS.
Other forms of HUS are much less common. It is difficult to estimate their incidence because of the overlap in diagnostic criteria with other thrombotic microangiopathies, especially thrombotic thrombocytopenic purpura (TTP).