Prophylactic use of a recombinant activated factor VII in delivery haemorrhage by caesarean in a woman with major factor VII deficiency: a case report

2011 ◽  
Vol 69 (6) ◽  
pp. 713-719
Author(s):  
Jean-François Comes ◽  
Jean Devignes ◽  
Olivier Thiebaugeorges ◽  
Marie-Elisabeth Briquel ◽  
Thomas Lecompte
Keyword(s):  
Case Report ◽  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Factor Vii Deficiency ◽  
Activated Factor Vii ◽  
Prophylactic Use

scholarly journals Case Report: Factor VII Deficiency Presented With Cephalohematoma After Birth

2021 ◽  
Vol 9 ◽  
Author(s):  
Yuan-Chun Lo ◽  
Ching-Tien Peng ◽  
Yin-Ting Chen
Keyword(s):  
Replacement Therapy ◽  
Intracranial Hemorrhage ◽  
Hypovolemic Shock ◽  
Rehabilitation Program ◽  
Factor Vii ◽  
High Dose ◽  
Homozygous Mutation ◽  
Recombinant Activated Factor Vii ◽  
Factor Vii Deficiency ◽  
Activated Factor Vii

Introduction: Factor VII deficiency is a rare inherited autosomal recessive bleeding disorder with a global prevalence of 1/500,000. Most cases remain asymptomatic, and cases with severe clinical presentation are rarely reported.Case Presentation: A newborn male with no relevant maternal antenatal history, delivered via vacuum-assisted cesarean section, presented with a large cephalohematoma after delivery. Poor appetite, pale appearance, and bulging fontanelles were observed 2 days later, progressing to hypovolemic shock. Further imaging examination revealed a large intracranial hemorrhage. Serial laboratory examination revealed remarkable coagulopathy with prolonged prothrombin time and factor VII deficiency (<1%, severe type). The patient was genetically confirmed to have the FVII:c 681+1 G>T homozygous mutation. Brain hemorrhage was resolved with high-dose factor VII replacement therapy with recombinant activated factor VII. However, repeated hemothorax and intracranial hemorrhage were detected. Therefore, the patient was under regular factor VII supplementation with a rehabilitation program for cerebral palsy.Conclusions: A case of factor VII deficiency with large cephalohematoma and intracranial hemorrhage after birth is described herein, which was treated with high-dose replacement therapy. Variants of the FVII:c 681+1 G>T (IVS6+1G>T) homozygous genotype may present with a severe phenotype at the neonatal stage. We aim to share a unique neonatal presentation with a certain genotype and treatment experience with initial replacement therapy, followed by regular prophylactic dosage.

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