Johns Hopkins University, Baltimore, Maryland, USA.
Gynecology and Obstetrics, Johns Hopkins University, Baltimore, Maryland, USA.
Fetal Diagn Ther. 2019;45(6):365-372. doi: 10.1159/000497472. Epub 2019 Mar 21.
Anhydramnios caused by early anuria is thought to be universally fatal due to pulmonary hypoplasia. Bilateral renal agenesis and early fetal renal failure leading to anhydramnios constitute early pregnancy renal anhydramnios (EPRA). There have been successful reports of amnioinfusions to promote lung growth in the setting of EPRA. Some of these successfully treated EPRA fetuses have survived the neonatal period, undergone successful dialysis, and subsequently received a kidney transplant. Conversely, there are no reports of untreated EPRA survivors. This early success of amnioinfusions to treat EPRA justifies a rigorous prospective trial. The objective of this study is to provide a review of what is known about fetal therapy for EPRA and describe the Renal Anhydramnios Fetal Therapy trial. We review the epidemiology, pathophysiology, and genetics of EPRA. Furthermore, we have performed systematic review of case reports of treated EPRA. We describe the ethical framework, logistical challenges, and rationale for the current single center (NCT03101891) and planned multicenter trial.
由于肺发育不良，早期无尿引起的羊水过少被认为普遍致命。双侧肾发育不全和导致羊水过少的早期胎儿肾衰竭构成了早期妊娠羊水过少（EPRA）。已经有成功的羊水输注报告，以促进 EPRA 中的肺生长。其中一些成功治疗的 EPRA 胎儿在新生儿期存活下来，接受了成功的透析，随后接受了肾移植。相反，没有未经治疗的 EPRA 幸存者的报告。羊水输注治疗 EPRA 的早期成功证明了严格的前瞻性试验是合理的。本研究的目的是综述 EPRA 胎儿治疗的已知内容，并描述肾脏羊水过少胎儿治疗试验。我们回顾了 EPRA 的流行病学、病理生理学和遗传学。此外，我们还对经过治疗的 EPRA 病例报告进行了系统评价。我们描述了当前单中心（NCT03101891）和计划多中心试验的伦理框架、后勤挑战和基本原理。
