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Pediatric Nephrology

Open Access 09-04-2024 | Hemolytic Uremic Syndrome | Original Article

Outcome 10 years after Shiga toxin-producing E. coli (STEC)-associated hemolytic uremic syndrome: importance of long-term follow-up

Authors: Alejandra Rosales, Sarah Kuppelwieser, Thomas Giner, Johannes Hofer, Magdalena Riedl Khursigara, Dorothea Orth-Höller, Wegene Borena, Gerard Cortina, Therese Jungraithmayr, Reinhard Würzner, for the German-Austrian H. U. S.-Study Group

Published in: Pediatric Nephrology

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Abstract

Background

Hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury in children. HUS is known as an acute disease followed by complete recovery, but patients may present with kidney abnormalities after long periods of time. This study evaluates the long-term outcome of Shiga toxin-producing Escherichia coli-associated HUS (STEC-HUS) in pediatric patients, 10 years after the acute phase of disease to identify risk factors for long-term sequelae.

Methods

Over a 6-year period, 619 patients under 18 years of age with HUS (490 STEC-positive, 79%) were registered in Austria and Germany. Long-term follow-up data of 138 STEC-HUS-patients were available after 10 years for analysis.

Results

A total of 66% (n = 91, 95% CI 0.57–0.73) of patients fully recovered showing no sequelae after 10 years. An additional 34% (n = 47, 95% CI 0.27–0.43) presented either with decreased glomerular filtration rate (24%), proteinuria (23%), hypertension (17%), or neurological symptoms (3%). Thirty had sequelae 1 year after STEC-HUS, and the rest presented abnormalities unprecedented at the 2-year (n = 2), 3-year (n = 3), 5-year (n = 3), or 10-year (n = 9) follow-up. A total of 17 patients (36.2%) without kidney abnormalities at the 1-year follow-up presented with either proteinuria, hypertension, or decreased eGFR in subsequent follow-up visits. Patients needing extracorporeal treatments during the acute phase were at higher risk of presenting symptoms after 10 years (p < 0.05).

Conclusions

Patients with STEC-HUS should undergo regular follow-up, for a minimum of 10 years following their index presentation, due to the risk of long-term sequelae of their disease. An initial critical illness, marked by need of kidney replacement therapy or plasma treatment may help predict poor long-term outcome.

Graphical abstract

Appendix
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Metadata
Title
Outcome 10 years after Shiga toxin-producing E. coli (STEC)-associated hemolytic uremic syndrome: importance of long-term follow-up
Authors
Alejandra Rosales
Sarah Kuppelwieser
Thomas Giner
Johannes Hofer
Magdalena Riedl Khursigara
Dorothea Orth-Höller
Wegene Borena
Gerard Cortina
Therese Jungraithmayr
Reinhard Würzner
for the German-Austrian H. U. S.-Study Group
Publication date
09-04-2024
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-024-06355-z