CONGENITAL FACTOR-XIII DEFICIENCY WITH TREATMENT OF FACTOR-XIII CONCENTRATE AND NORMAL VAGINAL DELIVERY

The outcome of all pregnancies complicated by congenital factor XIII (F XIII) deficiency has resulted in abortion without replacement therapy. We experienced a case with this disease and succeeded in normal vaginal delivery after treatment with weekly F XIII concentrate (Fibrogammin® P, Behringwerke AG) during pregnancy. This 20-year-old woman, gravida 1, para 0, was found to have an F XIII level of 0% at age 6 and was treated with F XIII concentrate occasionally when she suffered from massive bleeding. In 1986 she became pregnant and was hospitalized at 6 weeks’ gestation because of genital bleeding. Subsequent to this episode, F XIII concentrate was administered every week. At 37 weeks’ gestation a 2,646-gram girl with a 1-min Apgar score of 9 was delivered. Postpartum blood loss was 260 ml. One year after delivery neither the mother nor the infant were found to have hepatitis B, nonA, nonB hepatitis, ATL or HIV. F XIII concentrate proved effective in such cases without any risk of viral infection. © 1990 S. Karger AG, Basel.