Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab

Hemolytic Uremic Syndrome (HUS) is a constellation of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Shiga toxin-producing Escherichia coli- (STEC-) mediated HUS is a common cause of acute renal failure in children and can rarely result in severe neurological complicat...

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Autores principales: Jacob H. Umscheid, Collin Nevil, Rhythm Vasudeva, Mohammed Farhan Ali, Nisha Agasthya
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Publicado: Hindawi Limited 2021
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Acceso en línea:https://doaj.org/article/029f5c3b98274989aad0596628eb3c76
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spelling oai:doaj.org-article:029f5c3b98274989aad0596628eb3c762021-11-22T01:10:29ZTreatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab2090-681110.1155/2021/8053246https://doaj.org/article/029f5c3b98274989aad0596628eb3c762021-01-01T00:00:00Zhttp://dx.doi.org/10.1155/2021/8053246https://doaj.org/toc/2090-6811Hemolytic Uremic Syndrome (HUS) is a constellation of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Shiga toxin-producing Escherichia coli- (STEC-) mediated HUS is a common cause of acute renal failure in children and can rarely result in severe neurological complications such as encephalopathy, seizures, cerebrovascular accidents, and coma. Current literature supports use of eculizumab, a monoclonal antibody that blocks complement activation, in atypical HUS (aHUS). However, those with neurologic complications from STEC-HUS have complement activation and deposition of aggregates in microvasculature and may be treated with eculizumab. In this case report, we describe a 3-year-old boy with diarrhea-positive STEC-HUS who developed severe neurologic involvement in addition to acute renal failure requiring renal replacement therapy. He was initiated on eculizumab therapy, with clinical improvement and organ recovery. This case highlights systemic complications of STEC-HUS in a pediatric patient. The current literature is limited but has suggested a role for complement mediation in cases with severe complications. We review the importance of early recognition of complications, use of eculizumab, and current data available.Jacob H. UmscheidCollin NevilRhythm VasudevaMohammed Farhan AliNisha AgasthyaHindawi LimitedarticlePediatricsRJ1-570ENCase Reports in Pediatrics, Vol 2021 (2021)
institution DOAJ
collection DOAJ
language EN
topic Pediatrics
RJ1-570
spellingShingle Pediatrics
RJ1-570
Jacob H. Umscheid
Collin Nevil
Rhythm Vasudeva
Mohammed Farhan Ali
Nisha Agasthya
Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab
description Hemolytic Uremic Syndrome (HUS) is a constellation of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Shiga toxin-producing Escherichia coli- (STEC-) mediated HUS is a common cause of acute renal failure in children and can rarely result in severe neurological complications such as encephalopathy, seizures, cerebrovascular accidents, and coma. Current literature supports use of eculizumab, a monoclonal antibody that blocks complement activation, in atypical HUS (aHUS). However, those with neurologic complications from STEC-HUS have complement activation and deposition of aggregates in microvasculature and may be treated with eculizumab. In this case report, we describe a 3-year-old boy with diarrhea-positive STEC-HUS who developed severe neurologic involvement in addition to acute renal failure requiring renal replacement therapy. He was initiated on eculizumab therapy, with clinical improvement and organ recovery. This case highlights systemic complications of STEC-HUS in a pediatric patient. The current literature is limited but has suggested a role for complement mediation in cases with severe complications. We review the importance of early recognition of complications, use of eculizumab, and current data available.
format article
author Jacob H. Umscheid
Collin Nevil
Rhythm Vasudeva
Mohammed Farhan Ali
Nisha Agasthya
author_facet Jacob H. Umscheid
Collin Nevil
Rhythm Vasudeva
Mohammed Farhan Ali
Nisha Agasthya
author_sort Jacob H. Umscheid
title Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab
title_short Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab
title_full Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab
title_fullStr Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab
title_full_unstemmed Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab
title_sort treatment of shiga-toxin hus with severe neurologic features with eculizumab
publisher Hindawi Limited
publishDate 2021
url https://doaj.org/article/029f5c3b98274989aad0596628eb3c76
work_keys_str_mv AT jacobhumscheid treatmentofshigatoxinhuswithsevereneurologicfeatureswitheculizumab
AT collinnevil treatmentofshigatoxinhuswithsevereneurologicfeatureswitheculizumab
AT rhythmvasudeva treatmentofshigatoxinhuswithsevereneurologicfeatureswitheculizumab
AT mohammedfarhanali treatmentofshigatoxinhuswithsevereneurologicfeatureswitheculizumab
AT nishaagasthya treatmentofshigatoxinhuswithsevereneurologicfeatureswitheculizumab
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