The incidence of prematurity in Germany is about 10%and premature infants with haemophilia A (OMIM 306700) are in fact very rare.We report two new cases, one born in the 28th gestational week, weighing 1200 g with a f...The incidence of prematurity in Germany is about 10%and premature infants with haemophilia A (OMIM 306700) are in fact very rare.We report two new cases, one born in the 28th gestational week, weighing 1200 g with a factor VIII (FVIII)-level of 0.03 IU/ml treated with bolus injections of plasma derived FVIII concentrate (pdFVIII), and one born at week 30, weighing only 710 g with a factor level of <0.01 IU/ml and treated with recombinant FVIII concentrate (rFVIII).Recovery of FVIII was 96%in case 1 and 120%in case 2, FVIII half-life was 6 h and 8 h, respectively.During FVIII substitution, neither bleeding, thrombosis nor inhibitor development were noted in both infants.Conclusion: Immediate and frequent factor VIII substitution appears to be safe and effective for prophylaxis and treatment in premature haemophilic neonates.展开更多
文摘The incidence of prematurity in Germany is about 10%and premature infants with haemophilia A (OMIM 306700) are in fact very rare.We report two new cases, one born in the 28th gestational week, weighing 1200 g with a factor VIII (FVIII)-level of 0.03 IU/ml treated with bolus injections of plasma derived FVIII concentrate (pdFVIII), and one born at week 30, weighing only 710 g with a factor level of <0.01 IU/ml and treated with recombinant FVIII concentrate (rFVIII).Recovery of FVIII was 96%in case 1 and 120%in case 2, FVIII half-life was 6 h and 8 h, respectively.During FVIII substitution, neither bleeding, thrombosis nor inhibitor development were noted in both infants.Conclusion: Immediate and frequent factor VIII substitution appears to be safe and effective for prophylaxis and treatment in premature haemophilic neonates.
