从产前诊断到产后后果:一个回顾性研究
Or Steg Saban1,2, Tal Weissbach2,3, Reuven Achiron2,3
1Pediatrics B Department, Sheba Medical Center, Ramat-Gan, Israel.
Archives of disease in childhood
|July 20, 2023
概括
在产前诊断的先天性肝内移植系统突变 (IHPSS) 通常会自发消失. 虽然大多数患有IHPSS的儿童存活下来,但有些人可能会经历成长失败或发育迟缓,特别是那些有多次分流的孩子.
科学领域:
- 儿科胃肠病学 儿科胃肠病学
- 胎儿医学 胎儿医学
- 血管形症 血管形症
背景情况:
- 先天性肝内移植系统突变 (IHPSS) 是一种罕见的血管异常,血液绕过肝脏.
- 产前诊断IHPSS是不常见的,这些病例的结果没有得到充分的记录.
- 现有的研究往往侧重于症状,产后诊断的患者.
研究的目的:
- 调查IHPSS在产前诊断的自然过程和结果.
- 为了比较单次和多次肝内转移的患者之间的产后结果.
主要方法:
- 通过超声波 (2006-2019) 诊断的胎儿IHPSS病例的回顾性分析,在一个单一的三级中心.
- 在儿科胃肠道病房诊断病例的未来随访.
- 结果的比较基于肝脏内转移的数量 (单次与多次).
主要成果:
- 包括26名儿童; 77%的儿童经历了生长限制.
- 自发的分流关闭发生在96%的患者中,平均年龄为7.5个月.
- 不能壮成长 (FTT) 在那些多次分流的人群中更常见 (62.5%与16.7%相比).
- 所有患者都存活下来,后续症状很小;轻微的发育延迟在26.9%的患者中被发现.
结论:
- 产前诊断的IHPSS通常在两年内自发关闭.
- 产前检测到IHPSS的儿童可能面临FTT和轻度发育迟缓的风险.
- 建议对这些患者进行定期的儿科胃肠病学监测.
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