Haemolytic uraemic syndrome in children England, Wales, Northern Ireland, and Ireland: A prospective cohort study

Abstract

Haemolytic uraemic syndrome (HUS) caused by infection with Shiga toxin-producing Escherichia coli (STEC) is a relatively rare but potentially fatal multisystem syndrome clinically characterised by acute kidney injury. This study aimed to provide robust estimates of paediatric HUS incidence in England, Wales, Northern Ireland, and the Republic of Ireland by using data linkage and case reconciliation with existing surveillance systems, and to describe the characteristics of the condition. Between 2011 and 2014, 288 HUS patients were included in the study, of which 256 (89.5%) were diagnosed as typical HUS. The crude incidence of paediatric typical HUS was 0.78 per 100,000 person-years, although this varied by country, age, gender, and ethnicity. The majority of typical HUS cases were 1 to 4 years old (53.7%) and female (54.0%). Clinical symptoms included diarrhoea (96.5%) and/or bloody diarrhoea (71.9%), abdominal pain (68.4%), and fever (41.4%). Where STEC was isolated (59.3%), 92.8% of strains were STEC O157 and 7.2% were STEC O26. Comparison of the HUS case ascertainment to existing STEC surveillance data indicated an additional 166 HUS cases were captured during this study, highlighting the limitations of the current surveillance system for STEC for monitoring the clinical burden of STEC and capturing HUS cases.

Keywords: E. coli; Shiga-like toxin-producing; epidemiology; medical microbiology; paediatrics; surveillance.

Figure 1.

Venn diagram of HUS cases reported through the BPSU HUS study, the National Enhanced Surveillance System for STEC (NESSS), and the HPSC HUS surveillance register, 1 October 2011 to 31 October 2014 (n = 288).

Figure 2.

Number of haemolytic uraemic syndrome cases and incidence of HUS/100 000 person-years by age group and gender reported the BPSU HUS surveillance study, 1 October 2011 to 31 October 2014.