Holtkamp W, Kuhn W, Kunze E, Nagel G A
Zentrum Innere Medizin, Universität Göttingen.
Z Geburtshilfe Perinatol. 1988 Nov-Dec;192(6):278-81.
Two pregnancies in a patient with Gaucher's disease type I are reported. Anemia and thrombocytopenia were augmented during pregnancy, but no effect on the spleen and liver was observed. The first delivery was associated with a severe post partum haemorrhagia (platelet count 56,000/mcl, hemoglobin before delivery 10.2 g/100 ml, postpartum 5.5 g/100ml). At the end of the second pregnancy the platelet count was 33,000/mcl. Platelet transfusions were given after induction of preterm labor and no bleeding complications were observed. The obstetric aspects of Gaucher's disease are reviewed. Post partum haemorrhagia is associated with thrombopenia and is observed in patients with platelet counts under 100,000/mcl. Platelet infusions are recommended before delivery in these patients. Thrombocytopenic patients should be carefully monitored during labor with crossmatched blood on standby.
本文报告了1例Ⅰ型戈谢病患者的两次妊娠情况。妊娠期间贫血和血小板减少加重，但未观察到对脾脏和肝脏的影响。第一次分娩时伴有严重的产后出血（血小板计数56,000/微升，分娩前血红蛋白10.2克/100毫升，产后5.5克/100毫升）。第二次妊娠末期血小板计数为33,000/微升。在引产术后输注了血小板，未观察到出血并发症。本文对戈谢病的产科问题进行了综述。产后出血与血小板减少有关，在血小板计数低于100,000/微升的患者中可见。建议在这些患者分娩前输注血小板。血小板减少的患者在分娩期间应仔细监测，并备有交叉配血的血液。
