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Neurological involvement in hemolytic-uremic syndrome
Insights
Neurological involvement in children with hemolytic-uremic syndrome (HUS) is common and associated with severe outcomes. Central nervous system complications indicate a poorer prognosis, including increased risk of death and chronic renal damage.
Area of Science:
- Pediatric Nephrology
- Pediatric Neurology
- Critical Care Medicine
Background:
- Hemolytic-uremic syndrome (HUS) is a serious condition primarily affecting children.
- Neurological complications can occur in HUS, but their impact on long-term outcomes requires further elucidation.
Purpose of the Study:
- To investigate the prevalence and characteristics of neurological involvement in children with HUS.
- To determine the association between neurological involvement and clinical outcomes in pediatric HUS patients.
Main Methods:
- Retrospective review of 44 children diagnosed with HUS at Milwaukee Children's Hospital.
- Analysis of neurological symptoms, physical examination findings, laboratory results, and neuroimaging.
- Assessment of short-term and long-term neurological recovery and overall patient outcomes.
Main Results:
- 34% of HUS patients (15/44) exhibited neurological involvement, with a mean age of 3.25 years.
- Seizures were common (12/15), often linked to hypertension, fever, hyponatremia, or hypocalcemia.
- Significant neurological sequelae included hemiparesis, visual defects, and cognitive impairment, with only 6/15 achieving complete recovery.
Conclusions:
- Neurological involvement in pediatric HUS is a significant indicator of disease severity.
- Children with HUS and neurological complications face higher risks of residual hypertension, chronic renal damage, and mortality.
- Early recognition and management of neurological symptoms are crucial for improving outcomes in severe HUS cases.
Abstract:
Of 44 children with hemolytic-uremic syndrome seen at Milwaukee Children's Hospital, 15 (34%) had neurological involvement. This group contained 8 boys and 7 girls, with a mean age of 3 1/4 years. Twelve patients had seizure within 48 hours of admission. Seizures were associated with hypertension, fever, hyponatremia, or hypocalcemia. Other neurological symptoms included altered consciousness, behavioral changes, diplopia, and dizziness. Hemiparesis (4 patients), eye involvement (7 patients), decerebrate posturing (2 patients), and ataxia (1 patient) were present on physical examination. Cerebrospinal fluid examination showed increased protein in 4 of 11 patients. Electroencephalograms were abnormal in all 9 patients tested. Computed tomographic and radionuclide scans showed evidence of vascular abnormalities in 4 of the 14 patients studied. Complete neurological recovery occurred in only 6 of the 15 children, while the remaining 6 demonstrated residual hemiparesis, seizures, and cortical visual defect. In those children with neurological involvement, there was a higher incidence of residual hypertension (49% versus 11%), chronic renal damage (40% versus 3.5%), and death (28% versus 0%), suggesting that central nervous system involvement indicates severe hemolytic-uremic syndrome.
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