镜像综合征研究现状

doi:10.3877/cma.j.issn.1673-5250.2021.03.006

镜像综合征(mirror syndrome)是指继发于胎儿水肿(HF)、胎盘水肿出现的母体水肿，母体水肿的进展与HF、胎盘水肿相关。镜像综合征罕见，其发病率目前尚未知，估算为1/(5 800～10 000)。目前已知包括同种免疫反应及非免疫性因素在内的多种病因，可能引起HF，进而导致母体镜像综合征的发生，但是该病的具体病理生理机制迄今尚未阐明。对于典型的母体镜像综合征的诊断并不困难，同时发现HF及母体水肿即可被诊断，导致HF及镜像综合征的病因学诊断对于该病的临床处理、判断预后及再发风险评估均至关重要。总体而言，发生镜像综合征孕妇的胎儿预后较差，导致的胎儿及新生儿死亡率很高。对该病的治疗方案选择，主要取决于导致HF的病因。HF治愈后，母体水肿可随之恢复，而且胎儿存活率也随之升高。母体发生镜像综合征后，若无有效缓解HF的措施，则建议对孕妇尽快终止妊娠。

关键词: 镜像综合征, 水肿，胎儿, 免疫性胎儿水肿, 非免疫性胎儿水肿, 子痫前期, 超声检查，产前

Mirror syndrome is normally defined as development of maternal edema in association with hydrops fetalis (HF) and placenta hydrops. The incidence rate of mirror syndrome is rare and estimated to be 1/(5 800-10 000). It is known that a variety of etiologies including alloimmune response and non-immune factors may cause HF and then lead to mirror syndrome, but the specific pathophysiological mechanism is still unclear. The diagnosis of mirror syndrome is not difficult when finding HF and maternal hydrops at the same time. The etiological diagnosis of HF and mirror syndrome is also very important for clinical treatment, prognosis and recurrence risk assessment. In general, the prognosis of mirror syndrome is poor with very high fetal and neonatal mortality. The treatment mainly depends on the cause of HF. If there are no effective measures to alleviate HF, it is recommended to terminate pregnancy as soon as possible.

Key words: Mirror syndrome, Hydrops fetalis, Immune hydrops fetalis, Non-immune hydrops fetalis, Pre-eclampsia, Ultrasonography, prenatal

表1 NIHF的病因与HF发生率及其发生机制

NIHF病因	HF发生率	HF发生机制
心血管功能异常	17%～35%	中心静脉压升高
染色体异常	7%～16%	心脏结构或功能异常，淋巴发育不良，骨髓细胞异常生成
血液系统异常	4%～12%	贫血，心排血量增高性心力衰竭，缺氧(甲型地中海贫血)
胎儿宫内感染	5%～7%	贫血，缺氧，内皮细胞受损，毛细血管通透性增加
胸部发育异常	6%	上腔静脉阻塞，或因胸内压增加引起静脉回流受阻
双胎输血综合征	3%～10%	血容量增加和中心静脉压升高
尿路异常	2%～3%	尿路积水，肾病综合征伴低蛋白血症
胃肠发育异常	0.5%～4%	静脉回流障碍，胃肠道梗阻和梗死伴蛋白质丢失和胶体渗透压下降
淋巴管发育异常	5%～6%	静脉回流障碍
胎儿肿瘤，包括绒毛血管瘤	2%～3%	贫血，心排血量增高性心力衰竭，低蛋白血症
骨骼发育不良	3%～4%	肝大，低蛋白血症，静脉回流障碍
各种胎儿综合征	3%～4%	各种胎儿综合征
遗传代谢病	1%～2%	脏器增大和静脉回流障碍，红细胞生成减少，贫血和(或)低蛋白血症
混杂因素	3%～15%	-
未知因素	15%～25%	-

表1 NIHF的病因与HF发生率及其发生机制

NIHF病因	HF发生率	HF发生机制
心血管功能异常	17%～35%	中心静脉压升高
染色体异常	7%～16%	心脏结构或功能异常，淋巴发育不良，骨髓细胞异常生成
血液系统异常	4%～12%	贫血，心排血量增高性心力衰竭，缺氧(甲型地中海贫血)
胎儿宫内感染	5%～7%	贫血，缺氧，内皮细胞受损，毛细血管通透性增加
胸部发育异常	6%	上腔静脉阻塞，或因胸内压增加引起静脉回流受阻
双胎输血综合征	3%～10%	血容量增加和中心静脉压升高
尿路异常	2%～3%	尿路积水，肾病综合征伴低蛋白血症
胃肠发育异常	0.5%～4%	静脉回流障碍，胃肠道梗阻和梗死伴蛋白质丢失和胶体渗透压下降
淋巴管发育异常	5%～6%	静脉回流障碍
胎儿肿瘤，包括绒毛血管瘤	2%～3%	贫血，心排血量增高性心力衰竭，低蛋白血症
骨骼发育不良	3%～4%	肝大，低蛋白血症，静脉回流障碍
各种胎儿综合征	3%～4%	各种胎儿综合征
遗传代谢病	1%～2%	脏器增大和静脉回流障碍，红细胞生成减少，贫血和(或)低蛋白血症
混杂因素	3%～15%	-
未知因素	15%～25%	-

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