Typical hemolytic and uremic syndrome (HUS) is the first cause of acute renal failure in children. Acute phase features and sequelea are unknown in infant aged 0 to 6 months.

 The aim of this study was to describe the course and evaluate the evolution of typical HUS in children aged 0 to 6 months.    

Material and methods:

  It was a retrospective, observational and multicenter study, including children aged 0 to 6 months who were diagnosed with HUS between 2000 and 2016, in France.

 All cases were registered by the Institut National de Veille Sanitaire. Neurological, renal and digestive outcomes were evaluated 1 month and 1 year after the diagnosis.

Results:

40 patients were included. The median age was 3 months (IQR, 1.25-5 months). Diarrhea preceding HUS was reported in 80% of patients, which was bloody in 62.5% of patients. The source of infection was identified in 47.5% of cases and was most commonly inter-individual (30.0%). During the acute phase, renal impairment was mild. Only 25.0% of patients required dialysis. 35.0% of patients presented with digestive complications, 27.5% with neurological symptoms and 10.0% with cardiac abnormalities. More than 60% of patients had renal consequences at 1 month and 1 year follow-up (64.8 and 63.5%, respectively) which mainly manifested as proteinuria. 12.1% of patients presented neurological symptoms at 1 year follow-up. Two patients had unexplained biliary cirrhosis during the follow-up and needed hepatic transplantation.

Conclusions:

 The acute clinical presentation of typical HUS in children of this age group (0-6 months) has not been studied in isolation.

 This work highlights the unique implications for this age group, in particular patients displayed poor renal and neurological outcomes. Long term follow up is necessary with research of proteinuria.