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Introduction: Refractory status epilepticus is a life-threatening disease in children wherein seizure movements don't response to first line anti convulsion drugs. This study reviewed risk factors, management and early outcome of children with refractory status epilepticus. Methods: Patients with refractory status epilepticus admitted in Tabriz children's hospital between 2003 and 2006 were reviewed prospectively for risk factors, management and outcome at time of discharge from hospital. Results: Of the total of 121 patients with status epilepticus, 47 patients (38.8%) were afflicted with refractory status epilepticus. The most common form of refractory status epilepticus was acute symptomatic (38.3%). Seizure caused by acute symptomatic [RR= 2.36, (CI 95%=1.25 – 4.44), P=0.006] was a risk factor for refractory status epilepticus development. 87.2% of patients with refractory status epilepticus were treated by midazolam infusion. There was no significant relation between non-response to midazolam, etiology (P=0.798) and type of seizure (P=0.273). Early outcome of children with refractory status epilepticus was significantly worse than those with status epilepticus (P=0.0001). Conclusion: About 38% of patients with status epilepticus develop refractory status epilepticus with an increased mortality and morbidity. Seizure caused by acute symptomatic is an important risk factor for developing refractory status epilepticus. In most cases, midazolam infusion results in end of seizure.

Keywords: Refractory status epilepticus, Intravenous midazolam, Mortality, Morbidity

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