title: [Prophylactic use of a recombinant activated factor VII in delivery haemorrhage by caesarean in a woman with major factor VII deficiency: a case report].
abstract: Taking in charge the delivery of pregnant women with inherited major deficiency of factor VII (FVII) is poorly reported in literature. We report here the haemorrhagic prophylaxis of delivery by recombinant activated FVII (rFVIIa) in a 27-year-old women, gravida 1, para 0, with major deficiency FVII by missense mutation (p.Arg337Cys). Her parents, first germen, presented a FVII deficiency. She has four brothers and three sisters, of which only one brother has major FVII deficiency with hemorrhagic diathesis in childhood (hematochezia). At her birth, because of dystocia, a right sterno-cleido-mastoid muscle hematoma and left clavicle fracture occurred. The FVII concentration was 0.08 U/mL. At the age of fifteen, a surgery of appendicitis was performed with substitution by FVII from plasma donors without any haemorrhagic complication. Because of anatomic specificity (bifid uterus and vagina), caesarean was planned. After reviewing of the literature, caesarean was performed at 38th week of gestation with haemorrhagic prophylaxis consisting in administration of rFVIIa (eptacog alfa) at a dose of 20 μg/kg, 30 min before surgery, then every 3 h during 48 h. No haemorrhagic complication occurred. Thrombosis prophylaxis was ensured by enoxaparin (4000 UI a day subcutaneously started 6 h after surgery for 5 days). Clinical examination of the newborn was normal. In future, modalities of taking in charge have to be evaluated by prospective studies involving a sufficiently numerous group of woman with FVII major deficiency, or by retrospective studies with the means of national or European registers.
authors: CHU de Nancy, service d'hématologie biologique, Vandoeuvre-lès-Nancy, France.
authors: Jean-François
authors: JF
authors: Comes
authors: Jean
authors: J
authors: Devignes
authors: Olivier
authors: O
authors: Thiebaugeorges
authors: Marie-Elisabeth
authors: ME
authors: Briquel
authors: Thomas
authors: T
authors: Lecompte
mesh_terms: D000328:Adult; D002585:Cesarean Section / Q000009:adverse effects; D018890:Chemoprevention / Q000379:methods*; D036861:Delivery, Obstetric / Q000009:adverse effects; D005168:Factor VII Deficiency / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007744:Obstetric Labor Complications / Q000517:prevention & control; D010375:Pedigree; D019106:Postoperative Hemorrhage / Q000517:prevention & control*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000188:drug therapy* / Q000235:genetics; D011994:Recombinant Proteins / Q000627:therapeutic use; D012720:Severity of Illness Index
chemical_list: D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa