%0 Review
%T Successful perinatal management of a woman with congenital factor XIII deficiency using recombinant factor XIII: A case report and literature review.
%A Ito Y
%A Tsuji S
%A Kasahara M
%A Tokoro S
%A Murakami T
%A Takayama H
%J J Obstet Gynaecol Res
%V 50
%N 2
%D 2024 Feb 24
%M 37875278
%F 1.697
%R 10.1111/jog.15819
%X Factor XIII deficiency is an extremely rare autosomal recessive genetic disorder, occurring in 1 of 3-5 million people, and is associated with perinatal complications, such as habitual abortion and prolonged bleeding. Although plasma-derived factor XIII (Fibrogamin®) carries a risk of infection and contains very low concentrated forms of factor XIII (FXIII) used for a pregnant woman with congenital coagulation factor XIII deficiency, recombinant factor XIII (rFXIII, Novo Thirteen®; Tretten®, Novo Nordisk, Bagsvaerd, Denmark), which has no risk of infection and is highly concentrated, has emerged as a novel formulation. Herein, we report the first case of a Japanese pregnant woman with congenital coagulation factor XIII deficiency successfully managed by rFXIII. She had a good perinatal course without pregnancy-related complications and transfusion through the perinatal period.