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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·10~8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(χ~2=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(χ~2=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。
Objective To investigate the long-term efficacy of erythrocyte hydrogen sulfide (H2S) in the treatment of children with orthostatic tachycardia syndrome (POTS). Methods 28 POTS children, 12 males and 16 females, were selected for pediatric treatment at Peking University First Hospital from 2009 to 2012. The average age was (11.5 ± 2.5) years. All patients were treated with oral midodrine hydrochloride. Baseline erythrocyte hydrogen sulfide production rate at first visit was used to divide the patients into two groups: group 1 (n = 10) had baseline H2S yields of ≤27.1 nmol / (min · 10-8 erythrocytes), group 2 (n = 18) Cases) Baseline erythrocyte hydrogen sulfide production rate> 27.1 nmol / (min · 108 red blood cells), the follow-up of children (5.3 ± 1.8) years, the two groups of children symptom score, and by Kaplan-Meier curve, comparing two Asymptomatic survival rate in children. Results At the follow-up of 5.5 years, symptom scores of children in group 1 were significantly higher than those of group 2 (χ ~ 2 = 3.458, P <0.05). Kaplan-Meier curve analysis showed that the asymptomatic survival rate in group 2 was significantly higher than that in group 1 (χ ~ 2 = 10.848, P <0.05). Conclusion Baseline erythrocyte hydrogen sulfide production rate is of great value in predicting the long-term efficacy of midodrine hydrochloride in children with orthostatic tachycardia syndrome.