Antiepileptic hypersensitivity syndrome in children

OBJECTIVE: To assess clinical features and outcomes of childhood antiepilep tic hypersensitivity syndrome (AHS). AHS is an ideosyncratic reaction to ar omatic anticonvulsants that can result in severe multiorgan dysfunction and death. METHODs: Children with suspected AHS (fever, rash, lymphadenopathy, liver d ysfunction) were identified by an in-house computerized adverse drug event reporting system. The medical charts of children with suspected AHS were re viewed. A MEDLINE search (from 1996 to October 1999) was performed using th e term antiepileptic hypersensitivity syndrome. RESULTS: Fourteen of 36 children who experienced a rash, urticaria, pruritu s, fever, or hepatotoxicity associated with aromatic anticonvulsants met th e 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 phenyt oin, 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 ail patients by definition), oth er common features of AHS were lymphocytosis (71.4%), elevated erythrocyte sedimentation rate (64.3%), elevated aminotransferases (64.3%), lymphadenop athy (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 fai lure. Clinical outcome was similar between children who received systemic s teroid therapy (n = 5) and those who did not Antiepileptics producing AHS w ere 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, wh ich requires immediate discontinuation of an offending anticonvulsant.