title: The dilemma of inherited dysfibrinogenemia during pregnancy.
abstract: Inherited dysfibrinogenemia is a rare disorder caused by mutations in the fibrinogen gene, described in approximately 400 families to date. We present the case of a 20-year-old woman at 7 weeks of pregnancy with a history of two first-trimester spontaneous abortions and a family history of thrombotic events. Her testing revealed evidence of dysfibrinogenemia, necessitating multidisciplinary management planning including Hematology, OB-GYN, Maternal-Fetal Medicine, Blood Bank Services and Anesthesia. Antenatal care included a combination of intravenous fibrinogen infusions to maintain fibrinogen levels above 100 mg/dl and anticoagulation with low molecular weight heparin. She had an uneventful full-term delivery and continued fibrinogen infusions and thromboprophylaxis for 6 weeks postpartum. The combination of fibrinogen infusions and anticoagulation maintained the balance between bleeding and clotting in our patient during pregnancy. We recommend a multidisciplinary team approach for the management of dysfibrinogenemia during pregnancy to provide successful pregnancy outcomes.
authors.affiliation: Department of Internal Medicine, Henry Ford Hospital, Detroit, Michigan 48202, USA. javier.munoz@me.com
authors.forename: Javier
authors.initials: J
authors.lastname: Munoz
authors.forename: Jessica
authors.initials: J
authors.lastname: Schering
authors.forename: Angela
authors.initials: A
authors.lastname: Lambing
authors.forename: Salena
authors.initials: S
authors.lastname: Neal
authors.forename: Gregory
authors.initials: G
authors.lastname: Goyert
authors.forename: Pooja M
authors.initials: PM
authors.lastname: Green
authors.forename: Amr
authors.initials: A
authors.lastname: Hanbali
authors.forename: Sundara
authors.initials: S
authors.lastname: Raman
authors.forename: Philip
authors.initials: P
authors.lastname: Kuriakose
mesh_terms: D000347:Afibrinogenemia / Q000097:blood / Q000151:congenital / Q000188:drug therapy* / Q000235:genetics; D000925:Anticoagulants / Q000627:therapeutic use*; D005260:Female; D005340:Fibrinogen / Q000627:therapeutic use*; D015241:Fibrinogens, Abnormal / Q000235:genetics; D006470:Hemorrhage / Q000517:prevention & control*; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use*; D006801:Humans; D007275:Injections, Intravenous; D050498:Live Birth; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000517:prevention & control*; D013927:Thrombosis / Q000517:prevention & control*; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D015241:Fibrinogens, Abnormal; D006495:Heparin, Low-Molecular-Weight; D005340:Fibrinogen