Genotype and phenotype correlation in intracranial hemorrhage in neonatal factor VII deficiency among Thai children.

Congenital factor VII (FVII) deficiency is a rare inherited coagulopathy. The clinical manifestations and clinical findings vary widely, ranging from asymptomatic to life-threatening bleeding, including intracranial hemorrhage (ICH), with prolonged prothrombin time, normal partial thromboplastin time and normal platelet counts, which are confirmed by the low level of FVII assay. Treatment consists of fresh frozen plasma (FFP), prothrombin complex concentrates (PCCs), and recombinant activated FVII to treat bleeding and prophylactic therapy.

The use of recombinant activated factor VII to control bleeding in a preterm infant undergoing exploratory laparotomy.

The case of a preterm infant weighing 1120 g who successfully received recombinant activated factor VII (rFVIIa) without complication for control of a life-threatening bleeding event resulting from a ruptured umbilical artery is reported. After performing an exploratory laparotomy at 27 hours of age, hemorrhage from the surgical wound and various sites persisted. By 63 hours of age, the infant had received a total of 192 mL (171 mL/kg) of packed red blood cells, 115 mL (103 mL/kg) of fresh frozen plasma, 8 mL of cryoprecipitate, and 75 mL (67 mL/kg) of platelet concentrate without stabilization.