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Pediatric Nephrology
Published in: Pediatric Nephrology 2/2024

20-07-2023 | Hemolytic Uremic Syndrome | Management Dilemma

Constipation and hemolytic uremic syndrome

Authors: Brendan Crawford, Paige Strebeck, Suzanne Saccente

Published in: Pediatric Nephrology | Issue 2/2024

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Abstract

Background

Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) classically presents with diarrhea. Absence of diarrheal prodrome increases suspicion for atypical HUS (aHUS). Inability to obtain a fecal specimen for culture or culture-independent testing limits the ability to differentiate STEC-HUS and aHUS.

Case-diagnosis/treatment

Our patient presented with abdominal pain and constipation, and evaluation of pallor led to a diagnosis of HUS. There was a complete absence of diarrhea during the disease course. Lack of fecal specimen for several days delayed testing for STEC. Treatment for atypical HUS was initiated with complement-blockade therapy. PCR-testing for Shiga toxin from fecal specimen later returned positive. Alternative complement-pathway testing did not identify a causative genetic variant or anti-Factor H antibody. A diagnosis of STEC-HUS was assigned, and complement-blockade therapy was stopped.

Conclusion

Diagnosis of aHUS remains a diagnosis of exclusion, whereby other causes of HUS are eliminated with reasonable certainty. Exclusion of STEC is necessary and relies on testing availability and recognition of testing limitations. Diarrhea-negative STEC-HUS remains a minority of cases, and future research is needed to explore the clinical characteristics of these patients.
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Metadata
Title
Constipation and hemolytic uremic syndrome
Authors
Brendan Crawford
Paige Strebeck
Suzanne Saccente
Publication date
20-07-2023
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 2/2024
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-023-06093-8

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