Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome

We report on the case of a 7-year-old boy with Shiga toxin-producing Escherichia coli-related hemolytic uremic syndrome (STEC-HUS), initially presenting with abdominal pain as the only clinical feature and thus requiring differential diagnosis with a surgical emergency. Diagnosis of STEC-HUS was mad...

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Main Authors: Chiara Rosazza, Alberto M Cappellari, Cristiano Gandini, Elisa Scola, Gianluigi Ardissino
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2021/5587050
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author Chiara Rosazza
Alberto M Cappellari
Cristiano Gandini
Elisa Scola
Gianluigi Ardissino
author_facet Chiara Rosazza
Alberto M Cappellari
Cristiano Gandini
Elisa Scola
Gianluigi Ardissino
author_sort Chiara Rosazza
collection DOAJ
description We report on the case of a 7-year-old boy with Shiga toxin-producing Escherichia coli-related hemolytic uremic syndrome (STEC-HUS), initially presenting with abdominal pain as the only clinical feature and thus requiring differential diagnosis with a surgical emergency. Diagnosis of STEC-HUS was made with the appearance of bloody diarrhea and renal function impairment, and the clinical picture rapidly progressed to multiorgan failure. Relatively late and severe central nervous system (CNS) involvement was present, characterized by subacute encephalitis progressing to coma, which became apparent when the acute phase of thrombotic microangiopathy was resolving. Therefore, neurologic manifestations were thought to be related to reperfusion damage to the CNS and high-dose IV steroid pulse therapy was empirically administered. Following this therapeutic scheme, neurologic involvement resolved with no sequelae. This case offers several points of discussion on the clinical presentation and the diagnostic approach to STEC-HUS, on the related neurologic complications, and on a novel approach to their management.
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series Case Reports in Pediatrics
spelling doaj-art-5ebe3fa668d04343a7a84716b7595b532025-02-03T06:43:57ZengWileyCase Reports in Pediatrics2090-68032090-68112021-01-01202110.1155/2021/55870505587050Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic SyndromeChiara Rosazza0Alberto M Cappellari1Cristiano Gandini2Elisa Scola3Gianluigi Ardissino4Department of Pediatrics, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, Milan, ItalyDepartment of Neuroscience, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, University of Milan, Milan, ItalyFondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Anestesia e Terapia Intensiva Donna-Bambino, Milan, ItalyNeuroradiology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyCenter for HUS Prevention,Control and Management, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, ItalyWe report on the case of a 7-year-old boy with Shiga toxin-producing Escherichia coli-related hemolytic uremic syndrome (STEC-HUS), initially presenting with abdominal pain as the only clinical feature and thus requiring differential diagnosis with a surgical emergency. Diagnosis of STEC-HUS was made with the appearance of bloody diarrhea and renal function impairment, and the clinical picture rapidly progressed to multiorgan failure. Relatively late and severe central nervous system (CNS) involvement was present, characterized by subacute encephalitis progressing to coma, which became apparent when the acute phase of thrombotic microangiopathy was resolving. Therefore, neurologic manifestations were thought to be related to reperfusion damage to the CNS and high-dose IV steroid pulse therapy was empirically administered. Following this therapeutic scheme, neurologic involvement resolved with no sequelae. This case offers several points of discussion on the clinical presentation and the diagnostic approach to STEC-HUS, on the related neurologic complications, and on a novel approach to their management.http://dx.doi.org/10.1155/2021/5587050
spellingShingle Chiara Rosazza
Alberto M Cappellari
Cristiano Gandini
Elisa Scola
Gianluigi Ardissino
Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome
Case Reports in Pediatrics
title Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome
title_full Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome
title_fullStr Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome
title_full_unstemmed Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome
title_short Steroid Pulse Therapy for Severe Central Nervous System Involvement in Shiga Toxin-Producing Escherichia coli-Related Hemolytic Uremic Syndrome
title_sort steroid pulse therapy for severe central nervous system involvement in shiga toxin producing escherichia coli related hemolytic uremic syndrome
url http://dx.doi.org/10.1155/2021/5587050
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