与Hb Bart's水肿胎相关镜像综合征母体并发症的病例对照研究

摘要

目的:评价Hb Bart's水肿胎(即α-地中海贫血1纯合子)是否并发镜像综合征和分娩孕周对母体并发症的影响.方法:回顾性分析Hb Bart's水肿胎并发镜像综合征13例(A组)、Hb Bart's水肿胎未并发镜像综合征61例(B组)和排除Bart's水肿胎和血红蛋白H病(HbH)、无产科合并症的孕妇74例(C组)临床资料,对3组的实验室检查结果和母体并发症情况进行比较.另外,将74例Hb Bart's水肿胎按照分娩孕周是否大于28周分成两个亚组,比较两亚组母体的发病情况.结果:①A组血细胞比容、血红蛋白、血小板和血清白蛋白均明显低于B、C组(P＜0.05),尿酸明显高于B、C组(P＜0.05)；而B组和C组以上指标比较,差异均无统计学意义(P＞0.05).②A组母体严重并发症(包括弥漫性凝血功能障碍、心力衰竭、急性肺水肿、急性肾功能衰竭和HELLP综合征)、胎盘早剥、产后出血、肝肾功能损害和胎盘粘连的发生率均明显高于B组和C组(P＜0.0167).B组与C组比较,上述并发症发生率除产后出血和胎盘粘连外,其余均无统计学意义(P＞0.05).③74例Hb Bart's水肿胎患者中,≥孕28周者,其有镜像综合征、母体有严重并发症、胎盘早剥、产后出血、肝肾功能损害和胎盘粘连的发生率均明显高于＜孕28周组(P＜0.05).结论:Hb Bart's水肿胎增加母体并发症的发生,其对母体的危害程度除了与是否并发镜像综合征有关,分娩孕周也是重要因素,临床上应重视Hb Bart's水肿胎的早期产前诊断,一旦确诊应尽早终止妊娠,尽可能降低母体风险.%Objective:To investigate the effect of Hb Bart's hydrops fetalis and gestational weeks at delivery on maternal complications. Methods: We retrospectively reviewed the clinical data of 13 cases of Hb Bart's hydrops fetalis with mirror syndrome (A group), 61 cases of Hb Bart's hydrops fetalis without mirror syndrome (B group) and 74 pregnant women without Hb Bart's hydrops fetalis or HbH fetus (C group), and compared laboratory finding and maternal complications among three groups. In addition, 74 cases of Hb Baits hydrops fetalis were divided into 2 subgroups according to delivery gestational weeks <28 and = 28. A subgroup analysis was performed to compare maternal complications. Results:? Compared with B or C group, the values of HCT, Hb, PLT and ALB were significantly lower in A group (P<0. 05), whereas U-RIC was significantly higher (P<0. 05). No significant difference was found between B and C group ( P> 0. 05). ? Compared with B or C group, the incidences of major maternal complications (disseminated or diffuse intravascular coagulation, congestive heart failure, acute pulmonary edema, acute kidney failure and HELLP syndrome), placental abruption, postpartum hemorrhage, liver or renal function impairment and pla-cental adherence were significantly higher in A group (P<0.0167). Except for postpartum hemorrhage and placental adherence, the incidences of other maternal complications were not significantly different between B and C groups. (3; The incidences of mirror syndrome, major maternal complications, placental abruption, postpartum hemorrhage, liver or renal function impairment and placenta! adherence were significantly higher in women with delivery at or beyond 28 weeks than less than 28 weeks (P<0.05). Conclusions:Hb Bart's hydrops fetalis increase the incidence of maternal complications, both mirror syndrome and delivery gesta-tional weeks play important roles in maternal severe complications. Great concern for the early prenatal diagnosis of Hb Barts hydrops fetalis should be given, once it is diagnosed, early termination of pregnancy can reduce maternal risks as far as possibly.