ESPN 51th Annual Meeting

ESPN 2018


 
Characteristics and evolution of typical hemolytic uremic syndrome in infants from 0 to 6 months: study of a French national cohort, from 2000 to 2016
HEMERY FLORIANE 1 FILA MARC 1 NOBILI FRANÇOIS 2 SELLIER ANNE LAURE 3 KWON THERESA 4 BOYER OLIVIA 5 BESSENAY LUCIE 6 PIETREMENT CHRISTINE 7 BOURDAT MICHEL GUYLHENE 8 CLOAREC SYLVIE 9 ROUSSEY GWENAELLE 10 TAQUE SOPHIE 11 NOVO ROBERT 12 BROUX FRANÇOISE 13 LAVOCAT MARIE PIERRE 14 TERZIC JOELLE 15 DE PARSCAU LOIC 16 GARNIER ARNAUD 17

1- CHU MONTPELLIER
2- CHU BESANÇON
3- CHU LYON
4- CHU ROBERT DEBRé, PARIS
5- CHU NECKER, PARIS
6- CHU CLERMONT FERRAND
7- CHU REIMS
8- CHU GRENOBLE
9- CHU TOURS
10- CHU NANTES
11- CHU RENNES
12- CHU LILLE
13- CHU ROUEN
14- CHU SAINT ETIENNE
15- CHU STRASBOURG
16- CHU BREST
17- CHU TOULOUSE
 
Introduction:

 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.