Antiepileptic hypersensitivity syndrome in children
O Bessmertny1, R C Hatton, R P Gonzalez-Peralta
1Department of Pharmacy Services, Shands at the University of Florida, Gainesville, USA. olgabes@aol.com
Insights
Childhood antiepileptic hypersensitivity syndrome (AHS) is a severe reaction to anticonvulsants. Prompt recognition of fever, rash, and liver issues is crucial for immediate drug withdrawal to prevent fatal outcomes.
Area of Science:
- Pediatric Pharmacology
- Clinical Toxicology
- Adverse Drug Reactions
Background:
- Antiepileptic hypersensitivity syndrome (AHS) is a severe, idiosyncratic reaction to aromatic anticonvulsants.
- This syndrome can lead to multiorgan dysfunction and potentially fatal outcomes in children.
- Understanding the clinical spectrum and prognosis of AHS in pediatric populations is essential for timely management.
Purpose of the Study:
- To evaluate the clinical manifestations and patient outcomes associated with childhood antiepileptic hypersensitivity syndrome (AHS).
- To identify key clinical indicators for early diagnosis and intervention.
- To assess the impact of treatment strategies on AHS outcomes in children.
Main Methods:
- Identification of pediatric cases with suspected AHS using an adverse drug event reporting system.
- Review of medical charts for children presenting with fever, rash, lymphadenopathy, and liver dysfunction.
- MEDLINE search conducted for 'antiepileptic hypersensitivity syndrome' from 1966 to October 1999.
Main Results:
- Fourteen children met AHS criteria, with a mean age of 10.4 years, primarily on phenytoin, carbamazepine, or phenobarbital.
- Common symptoms included rash and fever (100%), lymphocytosis (71.4%), elevated ESR (64.3%), elevated aminotransferases (64.3%), and lymphadenopathy (57.1%).
- All patients recovered after anticonvulsant discontinuation; one fatality occurred due to liver failure complications. Steroid therapy showed no significant difference in outcome.
Conclusions:
- Childhood AHS can be fatal if not promptly diagnosed and managed.
- The presence of fever, rash, and hepatotoxicity should strongly suggest AHS, necessitating immediate cessation of the offending anticonvulsant.
- Early recognition and withdrawal of the causative agent are critical for favorable outcomes in pediatric AHS.
Objective:
To assess clinical features and outcomes of childhood antiepileptic hypersensitivity syndrome (AHS). AHS is an idiosyncratic reaction to aromatic anticonvulsants that can result in severe multiorgan dysfunction and death.
Methods:
Children with suspected AHS (fever, rash, lymphadenopathy, liver dysfunction) were identified by an in-house computerized adverse drug event reporting system. The medical charts of children with suspected AHS were reviewed. A MEDLINE search (from 1966 to October 1999) was performed using the term antiepileptic hypersensitivity syndrome.
Results:
Fourteen of 36 children who experienced a rash, urticaria, pruritus, fever, or hepatotoxicity associated with aromatic anticonvulsants met the criteria for AHS (mean age 10.4 +/- 6.5 y; males to females 8:6, white to African-American to biracial 10:3:1). Eight patients were receiving phenytoin, six carbamazepine, and four phenobarbital alone or in combination. The mean time from exposure to development of symptoms was 23.0 +/- 14.8 days. In addition to rash and fever (present in all patients by definition), other common features of AHS were lymphocytosis (71.4%), elevated erythrocyte sedimentation rate (64.3%), elevated aminotransferases (64.3%), lymphadenopathy (57.1%), eosinophilia (42.8%, coagulopathy (42.8%), leukocytosis (35.7%), leukopenia (35.7%), hyperbilirubinemia (35.7%), and nephritis (7.1%). All children recovered except one, who died from complications of liver failure. Clinical outcome was simimlar between children who received systemic steroid therapy (n=5) and those who did not. Antiepileptics producing AHS were discontinued in all patients.
Conclusions:
AHS can be fatal in children if not promptly recognized. Fever, rash, and hepatotoxicity should serve as presumptive evidence for AHS, which requires immediate discontinuation of an offending anticonvulsant.
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