Analysis of 11 cases of moderate or severe neonatal hemophilia / 中国小儿急救医学

ABSTRACT

Objective:To analyze the clinical features of moderate or severe neonatal hemophilia, and improve the understanding of this disease.Methods:Eleven cases of neonates with moderate or severe hemophilia admitted to our NICU from January 2012 to June 2019 were enrolled.The clinical features, laboratory data, treatments and prognosis of these 11 neonates were retrospectively analyzed.Results:All the neonates were male, and were diagnosed hemophilia A. Seven neonates presented with intracranial hemorrhage including one case complicated with cerebral hernia.Only two of these neonates with intracranial hemorrhage had neurological abnormalities.One case presented with right adrenal hematoma, and one case presented with retroperitoneal hematoma.Jaundice was observed in nine cases, and seven cases, jaundice appeared within two days after birth, whose earliest was 12 hours after birth, and the highest total bilirubin was 388 μmol/L.All cases had prolonged activated partial thromboplastin time.All neonates had decreased activity of coagulation factor Ⅷ including eight moderate and three severe neonatal hemophilia A. Four cases had genetic testing.Three cases infused with fresh frozen plasma and cryoprecipitate and the rest treated with coagulation factorⅧ infusion.Ten cases improved after treatment, and one case abandoned treatment.Conclusion:Moderate or severe hemophilia is often complicated with intracranial hemorrhage or abdominal hemorrhage, often accompanied with hyperbilirubinemia.Imaging examination should be performed to exclude occult bleeding, and coagulation function and coagulation factor activity should be detected as soon as possible.Blood coagulation factors are infused according to the different expected factors of different bleeding sites.