Smith Kathleen A, Reynolds Monica L, Chang Emily H, Strauss Robert A, Straube Lacey E
Department of Anesthesiology, University of North Carolina School of Medicine, Chapel Hill, USA.
Department of Nephrology, University of North Carolina School of Medicine, Chapel Hill, USA.
Cureus. 2022 Jun 23;14(6):e26260. doi: 10.7759/cureus.26260. eCollection 2022 Jun.
Gitelman syndrome is an autosomal recessive inherited disorder that impairs the function of thiazide-sensitive sodium-chloride cotransporters in the distal convoluted tubule of the nephron. During labor and delivery, avoidance of sympathetic overactivity, meticulous hemodynamic monitoring, and expedited repletion of potassium and magnesium are required to avoid adverse outcomes. We present a parturient with severe Gitelman syndrome, requiring continuous electrolyte and fluid infusions, who underwent successful cesarean delivery. Potential severe morbidity was avoided with multidisciplinary planning and management.
吉特林综合征是一种常染色体隐性遗传性疾病,会损害肾单位远曲小管中噻嗪类敏感型氯化钠协同转运蛋白的功能。在分娩过程中,需要避免交感神经过度活跃、进行细致的血流动力学监测以及迅速补充钾和镁,以避免不良后果。我们报告了一位患有严重吉特林综合征的产妇,她需要持续输注电解质和液体,并成功进行了剖宫产。通过多学科规划和管理,避免了潜在的严重并发症。