Shuster Shirley, Ankawi Ghada, Licht Christoph, Reiser Jochen, Wang Xuexiang, Wei Changli, Chitayat David, Hladunewich Michelle
The Prenatal Diagnosis and Medical Genetics Program, Department of Obstetrics and Gynecology, Mount Sinai Hospital, University of Toronto, Toronto, ON M5G 1Z5, Canada.
Department of Medicine, Division of Nephrology, Sunnybrook Science Health Sciences Centre, University of Toronto, Toronto, ON M4N 3M5, Canada.
J Clin Med. 2018 Oct 4;7(10):324. doi: 10.3390/jcm7100324.
We report a case of a pregnant woman with nephrotic syndrome due to biopsy-proven focal segmental glomerulosclerosis (FSGS) whose fetus developed echogenic kidneys and severe oligohydramnios by 27 weeks of gestation. Maternal treatment with prednisone resulted in normalization of the amniotic fluid indices and resolution of fetal renal echogenicity. The newborn was noted to have transient renal dysfunction and proteinuria, resolving by 6 weeks postpartum. The transplacental passage of permeability factors is postulated to have caused both the fetal and newborn renal presentation, with significantly elevated levels of soluble urokinase-type plasminogen activator receptor (suPAR) noted in the cord blood. This case documents the transplacental maternal-fetal transmission of suPAR, demonstrating the potential for maternal-fetal transmission of deleterious, disease-causing entities, and adds to the differential diagnosis of fetal echogenic kidneys. Further, this is the first documentation of a fetal response to maternal systemic therapy.
我们报告一例经活检证实为局灶节段性肾小球硬化（FSGS）的肾病综合征孕妇，其胎儿在妊娠27周时出现肾回声增强和严重羊水过少。孕妇使用泼尼松治疗后，羊水指数恢复正常，胎儿肾回声增强消失。新生儿出现短暂性肾功能障碍和蛋白尿，产后6周恢复。推测通透性因子经胎盘传递导致了胎儿和新生儿的肾脏表现，脐血中可溶性尿激酶型纤溶酶原激活物受体（suPAR）水平显著升高。本病例记录了suPAR的经胎盘母婴传播，证明了有害致病实体母婴传播的可能性，并增加了胎儿肾回声增强的鉴别诊断。此外，这是胎儿对母体全身治疗反应的首次记录。
