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

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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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Abbreviations

CKD:

Chronic kidney disease

HUS:

Haemolytic uremic syndrome

MARP:

Million of age-related population

RRT:

Renal replacement therapy

STEC:

Shigatoxin-producing Escherichia coli

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Acknowledgments

The present study was supported by a research grant from “Progetto Alice ONLUS Associazione per la lotta alla SEU”.

We thank A. Caprioli, S. Morabito and G. Scavia (National Institute of Health, Rome), E. Torresani, M. Arghittu and R. Colombo (Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milano). We would like to acknowledge the effort, dedication and commitment of the members of the North Italian HUS Network: S. Sardini (Asola), M. D’Agostino, A. Gervasoni (Bergamo), A. Bonazza, F. Pagani, G. Riva (Brescia), S. Grossi (Brescia), A. Pellegatta (Busto Arsizio), A. Reciputo (Cinisello Balsamo), C. Baldioli (Cittiglio), E. Milanesi (Cremona), E. Cama, A. Corti (Desenzano del Garda), F. Russo (Desio), B. Balduzzi (Esine), A. Rosco (Garbagnate), C. Sciuto (Lecco), C. Zambetti (Lodi), B. Osnaghi, L. Parola (Magenta), A. Bonomini, P. Pedroni (Manerbio), M. Casciana, R. Ceruti (Mantova), I. Frugnoli (Melzo), A. Bettinelli (Merate), P. Carlucci, R. Celano, M. Facchini, V. Goj, G. Pattarino, A. Plebani, F. Salvini, P. Tommasi (Milano), ML. Melzi (Monza), S. Andreoni, A. Monzani (Novara), G. Bossi (Pavia), P. Accorsi (Pieve di Coriano), D. Casnaghi (Rho), L. Cafarelli (Saronno), N. Altamura (Sesto San Giovanni), A. Bussolini (Tradate), S. Binda, L. Morando, A. Negri (Varese), C. Deicher (Vigevano), P. Casella, B. Roman (Vimercate).

Gianluigi Ardissino, Center for HUS Control, Prevention and Management, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Compliance with ethical standards

Funding

The present study was supported by a research grant from “Progetto Alice. ONLUS Associazione per la lotta alla SEU”.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors. All data were analysed retrospectively; patient’s (or parents’) consent to the use of sensitive personal data was required and provided at time of hospitalisation. The study was notified to the local review board.

Co-authors’ contributions

Gianluigi Ardissino, MD, PhD, conceptualised and designed the study, drafted the initial manuscript, and revised and approved the final version.

Stefania Salardi performed molecular biology analysis and revised and approved the final version.

Elisa Colombo performed molecular biology analysis and revised and approved the final version.

Sara Testa conceptualised and designed the study, revised and approved the final version.

Nicolò Borsa-Ghiringhelli performed molecular biology analysis and revised and approved the final version.

Fabio Paglialonga designed the data collection instruments and supervised data collection, revised and approved the final version.

Valentina Paracchini performed data collection, analysed the data and approved the final version.

Francesca Tel performed data collection, revised and approved the final version.

Ilaria Possenti performed data collection, edited and approved the final version.

Cristina Felice Civitillo performed data collection, revised, edited and approved the final version.

Mirco Belingheri performed data collection, edited and approved the final version.

Stefano Sardini analysed the data, revised and approved the final version.

Rossella Ceruti performed data collection, edited and approved the final version.

Carlo Baldioli performed data collection edited and approved the final version.

Paola Tommasi performed data analysis, revised and approved the final version.

Luciana Parola performed data collection, revised and approved the final version.

Fiorella Russo performed data collection, edited and approved the final version.

Silvana Tedeschi supervised molecular biology analysis, revised and approved the final version of the manuscript.

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Correspondence to Gianluigi Ardissino.

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Communicated by Peter de Winter

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Ardissino, G., Salardi, S., Colombo, E. et al. Epidemiology of haemolytic uremic syndrome in children. Data from the North Italian HUS network. Eur J Pediatr 175, 465–473 (2016). https://doi.org/10.1007/s00431-015-2642-1

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  • DOI: https://doi.org/10.1007/s00431-015-2642-1

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