Alajjuri Omar Ala', Samaha Mayar Essam, Honemeyer Ulrich, Mohammed Ghada, Mousa Noha A
College of Medicine, University of Sharjah, Sharjah, United Arab Emirates.
New Medical Center (NMC) Royal Hospital Sharjah, Sharjah, United Arab Emirates.
Front Med (Lausanne). 2022 Jun 29;9:870503. doi: 10.3389/fmed.2022.870503. eCollection 2022.
Bartter syndrome, a very rare inherited renal tubular disorder, characterized by urinary salt wastage, hypokalemia, polyuria, and metabolic alkalosis, may manifest antenatally as severe isolated polyhydramnios. Indomethacin is known to reduce salt wastage and subsequent polyhydramnios during pregnancy; however, it reduces the Ductus Arteriosus diameter among other potential complications, such as inhibition of gastrointestinal perfusion and increasing the risk of renal toxicity.
A 36-year-old multigravida presented with severe isolated polyhydramnios at 30 weeks of gestation. Based on a history of a previous pregnancy affected with Bartter syndrome, indomethacin was initiated. Amniotic fluid volume and Ductus Arteriosus diameter were monitored. As evidence lacks on optimal dose and duration of indomethacin, multiple-dose adjustments were made to reduce the amniotic fluid volume while maintaining normal Ductus Arteriosus diameter. Progressive polyhydramnios led to Cesarean section at 34+ weeks of gestation resulting in a healthy fetus diagnosed with Bartter syndrome in the early neonatal period.
We share our experience in the adjustment of the dose and duration of Indomethacin therapy in the treatment of severe polyhydramnios associated with antenatal Bartter syndrome. Amniotic fluid index, Ductus Arteriosus diameter, and umbilical artery doppler work together as key indicators to guide the success and safety of the therapy.
巴特综合征是一种非常罕见的遗传性肾小管疾病,其特征为尿盐丢失、低钾血症、多尿和代谢性碱中毒,可在产前表现为严重的单纯羊水过多。已知吲哚美辛可减少孕期盐丢失及随后的羊水过多;然而,它会减小动脉导管直径,并引发其他潜在并发症,如抑制胃肠道灌注和增加肾毒性风险。
一名36岁经产妇在妊娠30周时出现严重的单纯羊水过多。基于既往妊娠患有巴特综合征的病史,开始使用吲哚美辛。监测羊水量和动脉导管直径。由于缺乏关于吲哚美辛最佳剂量和疗程的证据,多次调整剂量以减少羊水量,同时维持动脉导管直径正常。进行性羊水过多导致在妊娠34 +周时行剖宫产,娩出一名健康胎儿,该胎儿在新生儿早期被诊断为巴特综合征。
我们分享了在治疗与产前巴特综合征相关的严重羊水过多时调整吲哚美辛治疗剂量和疗程的经验。羊水指数、动脉导管直径和脐动脉多普勒共同作为指导治疗成功与安全的关键指标。