摘要目的 观察葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症患者心脏体外循环围手术期治疗方法.方法 以2005年1月至2010年12月间在广西桂林医学院附属医院中进行心脏手术的10例G6PD缺乏症确诊患者作为病例组观察对象.同期选取性别、年龄、体质量、所患心脏手术疾病相匹配的20例非G6PD缺乏症患者作为对照组观察对象.病例组患者围手术期治疗措施集中于避免药物引起的急性溶血反应,减少手术应激,采用浅低温体外循环,加强血液保护等.观察指标包括患者围手术期呼吸机辅助时间、尿量、胸管引流量、输红细胞悬液量、输血浆量、术后第2天血红蛋白和血总胆红素水平,重症监护病房(ICU)停留时间等.结果 病例组呼吸机辅助时间[(8.6±5.7)h]、尿量[(1950±490)ml]、胸管引流量[(260±l20)ml]、输红细胞悬液量[(1.8±1.2)U]、输血浆量[(80±50)ml]、术后第2天血红蛋白[(99±12) g/L]和总胆红素水平[(27±11)μmol/L]与对照组比较[(9.3±4.5)h、(2100±670)ml、(253±146)ml、(1.3±1.0)U、(120±50)ml、(96±25)g/L、(24±8)μmol/L],差异均无统计学意义(t值分别为0.978、2.032、1.257、0.891、2.182、2.271、1.329,P均＞0.05),但病例组ICU停留时间[(2.6±0.6)d]较对照组[(1.8±1.5)d]明显增长(t=2.704,P＜ 0.05).结论 G6PD缺乏症患者心脏体外循环手术时,加强围手术期处理效果较好.

abstractsObjective To observe the perioperative management of cardiac surgery and extracorporeal circulation method in patients with glucose-6-phosphate dehydrogenase deficiency(G6PD).Methods Ten patients with G6PD deficiency underwent uneventful cardiac surgery procedures between January 2005 and December 2010.Twenty patients who had non-G6PD deficiency were as a control group,the selected conditions were the same gender,age,body mass,the risk of heart disease surgery.The preoperative management in patients with G6PD deficiency mainly focused on avoiding the drugs implicated in haemolysis,reducing the surgical stress,using moderate hypothermia extracorporeal circulation and enhancing blood conservation.Observed indicators included the assisted ventilation time,urine volume,the drainage volume of chest tube,the amount transfusion of red blood cells and plasma,the level of hemoglobin and serum total bilirubin in the 2nd day after surgery,ICU stay.Results Compared with the control group,patients with G6PD deficiency had no significant difference in duration of ventilation after the operation,drainage,urine,Hgb,bilirubin levels,and blood transfusion[(9.3 ± 4.5)h vs (8.6 ± 5.7)h,(2100 ±670)ml vs (1950 ± 490) ml,(253 ± 146)ml vs (260 ± 120)ml,(1.3 ± 1.0)U vs (1.8 ± 1.2)U,(96 ± 25)g/L vs (99 ± 12)g/L,and (24 ± 8)μmol/L vs (27 ± 1 l)μmol/L,t =0.978,2.032,1.257,0.891,2.182,2.271,and 1.329,all P ＞ 0.05].The duration of ICU discharge was significantly longer in the glucose-6-phosphate dehydrogenase deficient group[ (2.6 ± 0.6)d vs (1.8 ± 1.5)d,t =2.704,P ＜ 0.05].Conclusions Cardiac surgery can be performed safely in patients with G6PD deficiency with enhanced perioperative management.