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Published on: February 9, 2024
Lethal toxic encephalopathy due to childhood shigellosis or Ekiri syndrome
Babak Pourakbari1, Setareh Mamishi, Leila Kohan
1Pediatric Infectious Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Insights
Lethal toxic encephalopathy in children with shigellosis is rare but deadly. Brain edema may predict fatal outcomes, highlighting the need for early recognition and intervention.
Area of Science:
- Pediatrics
- Infectious Diseases
- Neurology
Background:
- Shigellosis can lead to lethal toxic encephalopathy (Ekiri syndrome), a rare condition with a high fatality rate.
- Limited data exist on predictive factors for this severe complication.
Purpose of the Study:
- To evaluate clinical and laboratory characteristics of children with lethal toxic encephalopathy due to shigellosis.
- To identify potential predictors of mortality in these patients.
Main Methods:
- Retrospective analysis of 1295 children with shigellosis over 10 years.
- Selection criteria included extreme toxicity, convulsions, rapid neurological deterioration, brain edema, and fatal outcome without sepsis or severe dehydration.
Main Results:
- Five children (0.4%) were diagnosed with lethal toxic encephalopathy.
- All affected children experienced rapid neurological deterioration and brain edema, despite intensive treatment.
- Brain edema was observed in all fatal cases.
Conclusions:
- Lethal toxic encephalopathy is a rare but devastating complication of shigellosis.
- The presence of brain edema may serve as a predictor of mortality.
- Early detection and management aimed at preventing brain edema are crucial for improving patient outcomes.
Abstract:
Lethal toxic encephalopathy due to shigellosis or Ekiri syndrome is a rare complication of shigellosis with a high fatality rate. Data are very limited on factors that can predict this encephalopathy, so we evaluated clinical and laboratory characteristics for these patients. In this study children with extreme toxicity and convulsions followed by rapid neurological deterioration resulting in brain edema and fatal outcome without sepsis and severe dehydration were selected as having lethal toxic encephalopathy. There were 1295 children with shigellosis during the 10 years of the study. Five children (0.4%) had lethal toxic encephalopathy due to shigellosis. Death occurred following rapid neurological detoriation resulting in brain edema despite intensive treatment. Evidence of brain edema may be a prediction factor for death. Early recognition of encephalopathy and measures to prevent brain edema may improve patient outcome.
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