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Pediatric Nephrology Publisher: Springer-Verlag Heidelberg ISSN: 0931-041X DOI: 10.1007/s00467-003-1262-6 Issue: Volume 18, Number 12 Date: December 2003 Pages: 1229 - 1235

Original Article

Risk factors for poor renal prognosis in children with hemolytic uremic syndrome

Alessandra Gianviti1, 23 , Alberto E. Tozzi2, Laura De Petris1, Alfredo Caprioli2, Lucilla Rav?2, Alberto Edefonti3, Gianluigi Ardissino3, Giovanni Montini4, Graziella Zacchello4, Alfonso Ferretti5, Carmine Pecoraro5, Tommaso De Palo6, Angela Caringella6, Maurizio Gaido7, Rosanna Coppo7, Francesco Perfumo8, Nunzia Miglietti9, Ilse Ratsche10, Rosa Penza11, Giovambattista Capasso12, Silvio Maringhini13, Salvatore Li Volti14, Carmen Setzu15, Marco Pennesi16, Alberto Bettinelli17, Leopoldo Peratoner18, Ivana Pela19, Elio Salvaggio20, Giuliana Lama21, Salvatore Maffei22 and Gianfranco Rizzoni1

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(1) Division of Nephrology and Dialysis, Bambino Ges? Children s Hospital and Institute for Scientific Research, Rome, Italy

(2) Istituto Superiore di Sanit?, Rome, Italy (3) Dialysis Unit, De Marchi Pediatric Clinic, Milan, Italy (4) Pediatric Department, University of Padua, Padua, Italy (5) Division of Nephrology and Dialysis, Santobono Hospital, Naples, Italy (6) Division of Nephrology and Dialysis, Giovanni XXIII Hospital, Bari, Italy (7) Division of Nephrology and Dialysis, Regina Margherita Hospital, Turin, Italy (8) Division of Nephrology and Dialysis, G. Gaslini Hospital, Genoa, Italy (9) Pediatric Clinic, Spedali Civili, Brescia, Italy (10) Pediatric Clinic, University of Ancona, Ancona, Italy (11) Pediatric Clinic, University of Bari, Bari, Italy (12) Division of Nephrology and Dialysis, University of Naples, Naples, Italy (13) Division of Nephrology, Di Cristina Hospital, Palermo, Italy (14) Pediatric Clinic, University of Catania, Catania, Italy (15) Division of Nephrology, Brotzu Hospital, Cagliari, Italy

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(16) Burlo Hospital, Trieste, Italy (17) Pediatric Unit, San Leopoldo Mantic Hospital, Merate, Italy (18) Pediatric Division, Civile Hospital, Pordenone, Italy (19) Pediatric Clinic, Meyer Hospital, Florence, Italy (20) Pediatric Clinic, Catholic University, Rome, Italy (21) Pediatric Clinic, Federico II University, Naples, Italy (22) Pediatric Clinic, University of Perugia, Italy (23) Division of Nephrology and Dialysis, Bambino Ges? Children s Hospital and

Research Institute, Piazza Sant Onofrio 4, 00165 Rome, Italy

Received: 20 January 2003 Revised: 16 June 2003 Accepted: 18 June 2003 Published online: 31 October 2003

Abstract Many factors have been proposed as predictors of poor renal prognosis in children with hemolytic uremic syndrome (HUS), but their role is still controversial. Our aim was to detect the most reliable early predictors of poor renal prognosis to promptly identify children at major risk of bad outcome who could eventually benefit from early specific treatments, such as plasmapheresis. Prognostic factors identifiable at onset of HUS were evaluated by survival analysis and a proportional hazard model. These included age at onset, prodromal diarrhea (D), leukocyte count, central nervous system (CNS) involvement, and evidence of Shiga toxinproducing Escherichia coli (STEC) infection. Three hundred and eighty-seven HUS cases were reported; 276 were investigated for STEC infection and 189 (68%) proved positive. Age at onset, leukocyte count, and CNS involvement were not associated with the time to recovery. Absence of prodromal D and lack of evidence of STEC infection were independently associated with a poor renal prognosis; only 34% of patients D?STEC? recovered normal renal function compared with 65%?76% of D+STEC+, D+STEC? and D?STEC+ patients. In conclusion, absence of both D and evidence of STEC infection are needed to identify patients with HUS and worst prognosis, while D? but STEC+ patients have a significantly better prognosis.

Keywords Hemolytic uremic syndrome - Shiga toxin-producing Escherichia coli - Prognostic factors - Long-term outcome - Classification - Atypical hemolytic uremic syndrome

Alessandra Gianviti Email: gianviti@ Phone: +39-06-68592393 Fax: +39-06-68592602

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