Takagi H, Imai A, Kawabata I, Sumi H, Shiraki S, Tamaya T
Department of Obstetrics and Gynecology, Gifu University School of Medicine, Japan.
J Med. 1989;20(2):163-70.
In this report, the maternal and fetal risks in a patient with paroxysmal nocturnal hemoglobinuria (PNH) were evaluated. A patient with PNH carried a pregnancy to successful delivery, with a healthy male infant, and suffered from postpartum cerebral subcortical, but not hepatic, thrombosis followed by recovery. An elevation of serum levels of lactate dehydrogenase isoenzyme, alpha-hydroxybutyrate dehydrogenase, was observed during pregnancy. The level was ameliorated to the non-pregnant level by delivery. However, hemoglobin concentrations during pregnancy and post-partum were at least 8 g/dl. This is a first report that shows tight coupling between an onset of intravenous hemolysis and pregnancy. The management of pregnancies in such patients is also discussed.
在本报告中，对一名阵发性夜间血红蛋白尿（PNH）患者的母婴风险进行了评估。一名PNH患者成功妊娠并分娩出一名健康男婴，产后出现脑皮质下血栓形成（而非肝血栓形成），随后康复。妊娠期间观察到血清乳酸脱氢酶同工酶、α-羟丁酸脱氢酶水平升高。分娩后该水平恢复到非妊娠水平。然而，妊娠期间和产后的血红蛋白浓度至少为8 g/dl。这是首份显示静脉溶血发作与妊娠之间紧密关联的报告。文中还讨论了此类患者的妊娠管理。
