Factor III diciency is an autosomal recessive trait and may be associated with maternal intracranial hemorrhage (Letsky, 2000). In their review, Kadir and associates (2009) cited an increased risk of recurrent miscarriage and placental abruption. It has also been reported to cause umbilical cord bleeding (Odame, 2014). Treatment is fresh frozen plasma. Naderi and colleagues (2012) described 17 successful pregnancies in women receiving weekly prophylaxis with Factor XIII concentrate.
Fibrinogen abnormalities-either qualitative or quantitativealso may cause coagulation abnormalities. Autosomally inherited abnormalities usually involve the formation of a functionally defective ibrinogen-commonly referred to as dyibrinogenemia (Edwards, 2000). Familial hypoibrinogenemia and sometimes aibrinogenemia are infrequent recessive disorders. In some cases, both are found-hypodysibrinogenemia (Deering, 2003). Our experience suggests that hypoibrinogenemia represents a heterozygous autosomal dominant state. he thrombinclottable protein level in these patients typically ranges from 80 to 110 mg/ dL when nonpregnant, and this increases by 40 or 50 percent in normal pregnancy. Those pregnancy complications that give rise to acquired hypoibrinogenemia, such as placental abruption, are more common with ibrinogen deiciency. Trehan and Fergusson (1991) and Funai and coworkers (1997) described successful outcomes in two afected women in whom ibrinogen or plasma inusions were given throughout pregnancy.
Conduction Analgesia with Bleeding Disorders
Most serious bleeding disorders would logically preclude the use of epidural or spinal analgesia for labor or delivery. If the bleeding disorder is controlled, however, conduction analgesia may be considered. Chi and colleagues (2009) reviewed intrapartum outcomes in 80 pregnancies in 63 women with an inherited bleeding disorder. These included those with factor XI deiciency, hemophilia carrier status, vWD, platelet disorders, or a deiciency of factor VII, Xl, or X. Regional block was used in 41. Of these, 35 had spontaneously normalized hemostatic dysfunction, and others were given prophylactic replacement therapy. The reviewers reported no unusual complications. Singh and associates (2009) reviewed 13 women with factor XI deiciency. Nine received neuraxial analgesia without complications, but only after fresh-frozen plasma was transfused to most to correct the activated partial thromboplastin time.
Several important regulatory proteins act as inhibitors at strategic sites in the coagulation cascade to maintain blood fluidity. Inherited deiciencies of these inhibitory proteins are caused by gene mutations. Because they may be associated with recurrent thromboembolism, they are collectively referred to as thrombophilias. These are discussed in Chapter 52 (p. 1005) and reviewed by the American College of Obstetricians and Gynecologists (2017b).
Aessopos A, Karabatsos F, Farmakis D, et al: Pregnancy in patients with welltreated 3-thalassemia: outcome for mothers and newborn infants. Am J Obstet Gynecol 180:360, 1999
American College of Obstetricians and Gynecologists: Hemoglobinopathies in pregnancy. Practice Bulletin No. 78, January 2007, Reairmed 2015 American College of Obstetricians and Gynecologists: Neural tube defects. Practice Bulletin No. 44, July 2003, Reairmed 2016a
American College of Obstetrics and Gynecologists: Prenatal diagnostic testing for genetic disorders. Practice Bulletin No. 162, May 2016b