Abstract:

Objective    To explore the value of baseline erythrocytic hydrogen sulfide production level in predicting the long-term survival of postural tachycardia syndrome（POTS） children treated with midodrine hydrochloride. Methods    Twenty-eight children（male 12，female 16） with POTS were included in the study，and all of them were diagnosed with POTS in Department of Pediatrics，Peking University First Hospital from 2009 to 2012. Their mean age was（11.5±2.5）years old. They were treated with midodrine hydrochloride. Twenty-eight children with POTS were divided into two groups according to the baseline erythrocytic hydrogen sulfide production level. GroupⅠconsisted of 10 POTS children whose erythrocytic hydrogen sulfide production level was less than or equal to 27.1 nmol/（min·108 erythrocytes），and group Ⅱ consisted of 18 POTS children whose erythrocytic hydrogen sulfide production level was higher than 27.1 nmol/（min·108 erythrocytes）. The mean follow-up time was（5.3±1.8）years. The orthostatic intolerance symptom score and the symptom-free survival were compared between the two groups by Kaplan-Meier curve. Results    After 5.5 years of follow-up，the symptom score of children in group Ⅰ was markedly higher than that of group Ⅱ（χ2=3.458，P＜0.05）. The symptom-free survival of group Ⅰ was lower than that of group Ⅱ （χ2=10.848，P＜0.05）. Conclusion    The baseline erythrocytic hydrogen sulfide production level is an useful indicator in predicting the long-term survival of POTS children treated with midodrine hydrochloride.

Key words: postural tachycardia syndrome, midodrine hydrochloride, erythrocytic hydrogen sulfide production, child

摘要：

目的    研究红细胞硫化氢（H2S）产率对盐酸米多君治疗儿童体位性心动过速综合征（POTS）远期疗效的预测价值。方法    选取2009—2012年在北京大学第一医院儿科就诊的POTS患儿28例， 男12例， 女16例， 平均年龄为（11.5±2.5）岁，均采用口服盐酸米多君治疗。以初诊时基线红细胞硫化氢产率将患儿分为两组： 组1患儿（10例）基线红细胞硫化氢产率≤27.1 nmol/（min·108个红细胞），组2患者（18例）基线红细胞硫化氢产率＞27.1 nmol/（min·108个红细胞），对上述患儿随访（5.3±1.8）年，研究两组患儿的症状评分，并通过Kaplan-Meier曲线，比较两组患儿的无症状存活率。结果    随访5.5年时，组1患儿症状积分高于组2症状积分且有统计学差异（χ2＝3.458，P＜0.05）。Kaplan-Meier曲线分析表明，组2患儿无症状存活率明显高于组1（χ2＝10.848，P＜0.05）。结论    基线红细胞硫化氢产率对于预测盐酸米多君治疗儿童体位性心动过速综合征远期疗效具有重要价值。

关键词: 体位性心动过速综合征, 盐酸米多君, 红细胞硫化氢产率, 儿童