Background: The aim of this study was to assess the treatment response to conventional antiepileptic drugs and low-dose adrenocorticotropic hormone therapy for infantile spasms in children with Down syndrome. Methods: We retrospectively investigated the response and relapse rates, electroencephalography findings, patient characteristics during drug withdrawal, and developmental outcome in 10 children with Down syndrome treated for infantile spasms in our hospital. Results: All patients showed cessation of infantile spasms and achieved electroencephalographic normalization. Spasm relapse occurred in one of 10 patients (10%). Antiepileptic drugs have been withdrawn for seven of 10 patients (70%), none of whom have experienced seizure relapse since drug withdrawal. The median developmental quotient (n = 8) was 20.5, which shows that the developmental outcome was unfavorable. Low-dose adrenocorticotropic hormone therapy achieved a low seizure remission rate of 28.6%. Conclusions: Elucidation of the optimal treatment for infantile spasms in children with Down syndrome is needed to reduce the duration of infantile spasms and improve the developmental outcome.
The aim of this study was to compare the effectiveness of propofol-based sedation and midazo-lam sedation in same-day bidirectional endoscopy (BDE) in children. The charts of children (≤15 years old) who had undergone same-day BDE were retrospectively reviewed. Demographic data, indications, sedatives and their dosages, clinical outcomes, endoscopic findings, adverse events, and patient time requirements were compared in cases with propofol-based and midazolam se-dation. A total of 91 children [51 boys, mean age 13 years (range 9-15)] were enrolled. Propofol alone or in combination with midazolam and/or pentazocine was given in 51 (propofol-based sedation group) while midazolam alone or in combination with pentazocine was given in 40 (midazolam sedation group). The mean doses of propofol, midazolam and pentazocine were 96 mg (range 40–145 mg), 4.1 mg (range 3–5 mg) and 7.5 mg in the propofol group while the mean doses of midazolam and pentazocine were 6.2 mg (range 4–10 mg) and 15 mg in the midazolam group, respectively. The total procedure times and endoscopic findings between the two groups were similar, but the median patient time requirement in the propofol group was significantly shorter than that of the midazolam group (7.3 h vs. 8.4 h, P<0.001). No adverse events occurred in either group. Propofol-based sedation shortened patient time requirements in same-day BDE compared with midazolam sedation in children.
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