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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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2021/8053246 |
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| 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 |
| collection | DOAJ |
| 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 |
| id | doaj-art-029f5c3b98274989aad0596628eb3c76 |
| institution | Kabale University |
| issn | 2090-6811 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-029f5c3b98274989aad0596628eb3c762025-02-03T01:26:24ZengWileyCase Reports in Pediatrics2090-68112021-01-01202110.1155/2021/8053246Treatment of Shiga-Toxin Hus with Severe Neurologic Features with EculizumabJacob H. Umscheid0Collin Nevil1Rhythm Vasudeva2Mohammed Farhan Ali3Nisha Agasthya4University of KansasUniversity of KansasUniversity of KansasUniversity of KansasUniversity of KansasHemolytic 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.http://dx.doi.org/10.1155/2021/8053246 |
| spellingShingle | Jacob H. Umscheid Collin Nevil Rhythm Vasudeva Mohammed Farhan Ali Nisha Agasthya Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab Case Reports in Pediatrics |
| title | 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_short | Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab |
| title_sort | treatment of shiga toxin hus with severe neurologic features with eculizumab |
| url | http://dx.doi.org/10.1155/2021/8053246 |
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