Bodur Muhittin, Tutuncu Toker Rabia
Bursa Uludag University, Pediatric Neurology, Bursa, Turkey.
Clin Pediatr (Phila). 2025 Oct;64(9):1259-1263. doi: 10.1177/00099228251328136. Epub 2025 Mar 24.
In this study, we applied high-dose oral phenobarbital (PB) to children with recurrent and treatment-refractory seizures or recurrent status epilepticus and evaluated the effectiveness and safety of this treatment. We retrospectively reviewed patients' medical records who received oral high-dose PB treatment between January 2019 and July 2024. In this study, recurrent and treatment-refractory seizures was defined as the persistence of daily epileptic seizures or recurrent attacks of status epilepticus despite treatment with oral antiepileptic drugs or continuous intravenous midazolam therapy. High-dose oral PB therapy was performed on 11 patients (7 females and 4 males). The median age at the onset of epilepsy was 2 months (range: 0.06-132 months). The underlying disorders or comorbidity were genetic disorders (1q14 del, compound heterozygous for the , Wolf-Hirschhorn syndrome, ring chromosome 14 syndrome) in 4 patients, cerebral palsy in 2 patients, metabolic disorders (Zellweger syndrome, pyridoxine-dependent epilepsy) in 2 patients, traumatic brain injury and hypoxia, hemimegalencephaly, and ataxia and intellectual disability in 1 patient. The median age at initiation of high-dose PB therapy was 11 months (range: 2-203 months). The maximal dose of PB ranged from 6 to 14.7 mg/kg/d (median: 10 mg/kg/day). The maximal serum PB levels ranged from 33 to 56 µg/mL (median: 44 µg/mL). We evaluated the effectiveness of this treatment as follows: "effective" represented more than 50% seizure reduction, "ineffective" represented less than 50% seizure reduction, and "exacerbation" represented an increase in seizure frequency. In 7 of the 11 patients (63.6%), oral high-dose PB therapy was effective and was transiently effective in the other 4 patients. Adverse effects were noted in 6 patients (54.5%) during high-dose oral PB therapy: drowsiness in 5 patients and mild elevations in transaminases in 2 patients.
在本研究中，我们对患有复发性和难治性癫痫发作或复发性癫痫持续状态的儿童应用了大剂量口服苯巴比妥（PB），并评估了该治疗方法的有效性和安全性。我们回顾性分析了2019年1月至2024年7月期间接受口服大剂量PB治疗的患者的病历。在本研究中，复发性和难治性癫痫发作被定义为尽管接受了口服抗癫痫药物治疗或持续静脉注射咪达唑仑治疗，但仍持续每日癫痫发作或癫痫持续状态反复发作。对11例患者（7例女性，4例男性）进行了大剂量口服PB治疗。癫痫发作的中位年龄为2个月（范围：0.06 - 132个月）。潜在疾病或合并症包括：4例患者患有遗传性疾病（1q14缺失、 复合杂合子、沃尔夫 - 赫希霍恩综合征、14号环状染色体综合征），2例患者患有脑瘫，2例患者患有代谢性疾病（泽尔韦格综合征、吡哆醇依赖性癫痫），1例患者患有创伤性脑损伤和缺氧、半侧巨脑症以及共济失调和智力残疾。开始大剂量PB治疗的中位年龄为11个月（范围：2 - 203个月）。PB的最大剂量范围为6至14.7mg/kg/d（中位值：10mg/kg/天）。血清PB的最大水平范围为33至56μg/mL（中位值：44μg/mL）。我们对该治疗的有效性评估如下：“有效”表示癫痫发作减少超过50%，“无效”表示癫痫发作减少少于50%，“病情加重”表示癫痫发作频率增加。11例患者中有7例（63.6%）口服大剂量PB治疗有效，另外4例患者为短暂有效。在大剂量口服PB治疗期间，6例患者（54.5%）出现了不良反应：5例患者嗜睡，2例患者转氨酶轻度升高。
