Abstract
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.
Highlights
Shiga toxin-producing Escherichia coli (STEC) is a wellknown etiology for gastroenteritis and colitis; the toxin can traverse through the gastrointestinal system into the bloodstream causing injury to vascular endothelial cells [1, 2]. is results in a microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, known as hemolytic uremic syndrome (HUS) [1, 3]
HUS is a common cause of acute kidney injury in children, accounting for around 17% of cases and usually between the ages of 1 to 5 years (74%) [4]. is is secondary to toxin binding to the renal endothelial cells, but may extend to extrarenal tissue resulting in severe neurological complications of altered mental status, seizures, cerebrovascular accidents, and coma [5]
We present a case of a 3-year-old boy with diarrhea-positive Shiga toxin-producing Escherichia coli- (STEC-)HUS with severe renal and neurologic involvement who showed improvement of symptoms after eculizumab administration
Summary
Shiga toxin-producing Escherichia coli (STEC) is a wellknown etiology for gastroenteritis and colitis; the toxin can traverse through the gastrointestinal system into the bloodstream causing injury to vascular endothelial cells [1, 2]. is results in a microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, known as hemolytic uremic syndrome (HUS) [1, 3]. Eculizumab, a monoclonal antibody which inhibits complement pathway activation by inhibiting cleavage of C5, has been widely used and highly effective in the treatment of aHUS, yet its role in STEC-HUS is poorly defined [12,13,14,15,16]. We present a case of a 3-year-old boy with diarrhea-positive STEC-HUS with severe renal and neurologic involvement who showed improvement of symptoms after eculizumab administration.
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