Neurological involvement in children with hemolytic uremic syndrome

Abstract

Our objective was to establish the rate of neurological involvement in Shiga toxin-producing Escherichia coli–hemolytic uremic syndrome (STEC-HUS) and describe the clinical presentation, management and outcome. A retrospective chart review of children aged ≤ 16 years with STEC-HUS in Children’s Health Ireland from 2005 to 2018 was conducted. Laboratory confirmation of STEC infection was required for inclusion. Neurological involvement was defined as encephalopathy, focal neurological deficit, and/or seizure activity. Data on clinical presentation, management, and outcome were collected. We identified 240 children with HUS; 202 had confirmed STEC infection. Neurological involvement occurred in 22 (11%). The most common presentation was seizures (73%). In the neurological group, 19 (86%) were treated with plasma exchange and/or eculizumab. Of the 21 surviving children with neurological involvement, 19 (91%) achieved a complete neurological recovery. A higher proportion of children in the neurological group had renal sequelae (27% vs. 12%, P = .031). One patient died from multi-organ failure.Conclusion: We have identified the rate of neurological involvement in a large cohort of children with STEC-HUS as 11%. Neurological involvement in STEC-HUS is associated with good long-term outcome (complete neurological recovery in 91%) and a low case-fatality rate (4.5%) in our cohort. What is Known:• HUS is associated with neurological involvement in up to 30% of cases.• Neurological involvement has been reported as predictor of poor outcome, with associated increased morbidity and mortality. What is New:• The incidence of neurological involvement in STEC-HUS is 11%.• Neurological involvement is associated with predominantly good long-term outcome (90%) and a reduced case-fatality rate (4.5%) compared to older reports.

Hemolytic uremic syndrome with central nervous system manifestations, a case report and literature review

“…Costigan et al . [7] reported that seizures were the most common presentation of CNS involvement, manifesting within 48 hours of admission to the hospital in 73% of included cases in their study, with 25% presenting with status epilepticus as the initial presentation. They additionally reported that encephalopathy was noted in 45%, while the focal neurologic deficit was evident in only 18% of the included cases [7] .…”

“…The reported rate of brain involvement in patients with HUS widely varies between 10% and 52% due to the unstrict definition of neurologic involvement among reported studies. In a recent study that involved a retrospective analysis of 202 children aged <16 years with confirmed STEC-caused HUS from 2005 to 2018, they reported a rate of 11% based on defining neurologic involvement by seizures, encephalopathy, or focal neurologic deficit, and considering features such as irritability or lethargy to be nonspecific [7] . Although brain involvement is not as common as renal involvement, it's the most common cause of mortality and a major contributor to morbidity in cases with HUS [8] .…”

Assessment of Pediatric Hemolytic Uremic Syndrome Patients Hospitalized in Pediatric Intensive Care Unit

“…Neurological involvement is reported in approximately 30% of all types of HUS. [15] Seizures, irritability, lethargy, encephalopathy are the most common CNS findings. [15] In pediatric age group CNS involvement rate is 3-53%.…”

“…[15] Seizures, irritability, lethargy, encephalopathy are the most common CNS findings. [15] In pediatric age group CNS involvement rate is 3-53%. [16] Single centered study from Turkey showed that CNS involvement seen in 13% of HUS patients.…”

Enterohemorrhagic Escherichia coli and a Fresh View on Shiga Toxin-Binding Glycosphingolipids of Primary Human Kidney and Colon Epithelial Cells and Their Toxin Susceptibility

“…The hallmark of hemorrhagic colitis due to EHEC is the development of bloody diarrhea several days after the onset of non-bloody diarrhea and abdominal pain. More severe EHEC infections can progress to systemic life-threatening complications, such as HUS and neurological abnormalities of the central nervous system [21,62,63,[152][153][154][155]. Within a week of onset of EHEC-mediated diarrhea/colitis, HUS develops abruptly and manifests as thrombocytopenia, intravascular microangiopathic hemolytic anemia, and acute renal insufficiency with kidney as the most commonly affected organ [152,[156][157][158][159][160].…”