Abstract
The cases of pregnant patients with the congenital coagulation factors deficiency, or its reduction, who underwent successful deliveries are very uncommon, so the management of hemorrhage during pregnancy and labor has not been established. As we experienced 6 cases (10 pregnancies) of the congenital coagulation disorders, we analysed the change of coagulation factors during pregnancy and clarified the change of hemostatic mechanism during pregnancy and the management of labor.
The activity of factor VIII in plasma increased during pregnancy, namely 108±10.2% in non-pregnant women, 150±53% in pregnant women at 3rd month, 173±47% at 5th month, 250±76% at 8th month. Also in plasma of factor VIII deficiency patients, such as von Willebrand's disease, mild case of hemophilia A carrier, its activity increased during pregnancy and exceeded 100% at 10th month, so they underwent successful deliveries without any replacement therapy. But in one case of severe hemophilia A carrier, factor VIII activity in plasma didn't increase over 20% of normal, so she, who underwent Cesarean section because of CPD, was treated with AHF replacement therapy. In this case hemorrhage from uterine cavity was normal, but hemorrhage from the operative wound couldn't be ceased easily.
We concluded as follows:
1. Even in patients of the congenital coagulation factors deficiency decreased activity of coagulation factors increases during pregnancy.
2. Even in these patients hemorrhage from uterine cavity can be ceased by the biological ligation of uterine muscle and by the extrinsic coagulation mechanism of tissue thromboplastin in placenta and decidua.
3. Hemorrhage from the wound of laceration or incision can't be ceased without replacement therapy.
4. Therofore careful, intentional and vaginal delivery is the absolute principle. Cesarean section should be elected for obstetric reasons only.
5. The heredity to newborn infant should be considered.
