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Published in: European Journal of Pediatrics 4/2016

01-04-2016 | Original Article

Epidemiology of haemolytic uremic syndrome in children. Data from the North Italian HUS network

Authors: Gianluigi Ardissino, Stefania Salardi, Elisa Colombo, Sara Testa, Nicolò Borsa-Ghiringhelli, Fabio Paglialonga, Valentina Paracchini, Francesca Tel, Ilaria Possenti, Mirco Belingheri, Cristina Felice Civitillo, Stefano Sardini, Rossella Ceruti, Carlo Baldioli, Paola Tommasi, Luciana Parola, Fiorella Russo, Silvana Tedeschi

Published in: European Journal of Pediatrics | Issue 4/2016

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Abstract

Despite the severity of HUS and the fact that it represents a leading cause of acute kidney injury in children, the general epidemiology of HUS is all but well documented. The present study provides updated, population-based, purely epidemiological information on HUS in childhood from a large and densely populated area of northern Italy (9.6 million inhabitants, 1.6 million children). We systematically reviewed the files concerning patients with STEC-HUS and atypical HUS (aHUS) over a 10-year observation period (January 2003–December 2012). We included all incident cases with a documented first episode of HUS before the age of 18 years. We identified 101 cases of HUS during the 10 years. The overall mean annual incidence was 6.3 cases/million children aged <18 years (range 1.9–11.9), and 15.7/million of age-related population (MARP) among subjects aged <5 years; aHUS accounted for 11.9 % of the cases (mean incidence 0.75/MARP). The overall case fatality rate was 4.0 % (3.4 % STEC-HUS, 8.3 % aHUS).
Conclusion: Given the public health impact of HUS, this study provides recent, population-based epidemiological data useful for healthcare planning and particularly for estimating the financial burden that healthcare providers might have to face in treating HUS, whose incidence rate seems to increase in Northern Italy.
What is Known:
• HUS is a rare disease, but it represents the leading cause of acute kidney injury in children worldwide.
• STEC-HUS (also called typical, D + HUS) is more common compared to atypical HUS, but recent, population-based epidemiological data (incidence) are scanty.
What is New:
• Comprehensive, population-based epidemiological data concerning both typical and atypical HUS based on a long observational period.
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Metadata
Title
Epidemiology of haemolytic uremic syndrome in children. Data from the North Italian HUS network
Authors
Gianluigi Ardissino
Stefania Salardi
Elisa Colombo
Sara Testa
Nicolò Borsa-Ghiringhelli
Fabio Paglialonga
Valentina Paracchini
Francesca Tel
Ilaria Possenti
Mirco Belingheri
Cristina Felice Civitillo
Stefano Sardini
Rossella Ceruti
Carlo Baldioli
Paola Tommasi
Luciana Parola
Fiorella Russo
Silvana Tedeschi
Publication date
01-04-2016
Publisher
Springer Berlin Heidelberg
Published in
European Journal of Pediatrics / Issue 4/2016
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-015-2642-1

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