title: Congenital factor XIII deficiency with treatment of factor XIII concentrate and normal vaginal delivery.
abstract: 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.
authors.affiliation: Department of Obstetrics and Gynecology, Hamamatsu University School of Medicine, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kobayashi
authors.forename: T
authors.initials: T
authors.lastname: Terao
authors.forename: T
authors.initials: T
authors.lastname: Kojima
authors.forename: J
authors.initials: J
authors.lastname: Takamatsu
authors.forename: T
authors.initials: T
authors.lastname: Kamiya
authors.forename: H
authors.initials: H
authors.lastname: Saito
mesh_terms: D000328:Adult; D005176:Factor XIII / Q000235:genetics / Q000378:metabolism / Q000627:therapeutic use*; D005177:Factor XIII Deficiency / Q000151:congenital / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007743:Labor, Obstetric; D010375:Pedigree; D011247:Pregnancy; D011248:Pregnancy Complications
chemical_list: D005176:Factor XIII