OP89 – 3062: Escherichia coli 055 associated D+haemolytic uraemic syndrome with severe neurological phenotype in southern England

Abstract

Objective Diarrhoea associated HUS with neurological involvement in the context of E. Coli 0157 is well described. Spreading microvascular thrombosis caused by combined effects of lipopolysaccharide, cytokine, enhanced shear stress, and verotoxin is purported to play a major role in the development of central nervous dysfunction. Methods We report part of a retrospective case series of 42 patients with D+ HUS. 6 had E Coli055 associated HUS and presented over 7 months in 2014 from a circumscribed geographical area of Southern England. Results 4 boys and 2 girls with median age 40.5 months (range 19m to 60m). Of these 5 had neurological involvement within 2–5 days of start of illness in the form of: generalised seizures – 2, encephalopathy – 2 and focal seizure+hemiplegia – 1. Only the child with hemiplegia had residual neurology at discharge and 6 months follow up. Of the 5, 2 were too unwell to have an MRI scan and the other three had high signal in the basal ganglia or deep white matter or both. EEG in all patients was encephalopathic. All 5 with neurological disease required peritoneal dialysis or haemodialysis or both. 2 required inotropic support. In total 42 patients were admitted with HUS between July 2004 and Jan 2015. 9/42 (21.4%) developed cerebral involvement. 3 children were positive for E. Coli 0157 and 1 was Rotavirus positive. 9 children had atypical HUS and were treated with Ecluzimab, which is a monoclonal antibody directed against complement protein, C5. Conclusion To our knowledge, this is the first case series of D+HUS with E. Coli 055; causing a severe phenotype with neurological involvement in most children in our cohort. Encouragingly only one child has sustained medium term neurological deficit. Epidemiogical and public health investigations are ongoing to try and explain this cohort from a small geographical area.