Use of plasma-derived factor X concentrate in neonates and infants with congenital factor X deficiency.
Karen L ZimowskiCatherine E McGuinnYasmina L AbajasCorinna L SchultzShipra KaickerGlaivy BatsuliPublished in: Journal of thrombosis and haemostasis : JTH (2020)
All four patients presented in the first week of life with severe bleeding. Following treatment of the acute bleed, prophylactic pdFX was initiated at an average of 29 days of life and a dose of 69 IU/kg every 48 hours. Incremental recovery (IR) in three infants averaged 1.42 IU/dL per IU/kg (min-max: 1.06-1.67 IU/dL per IU/kg). One patient experienced thrombotic complications in the setting of sepsis. After a median follow-up of 26.5 months, no patient has experienced breakthrough bleeding episodes. Our study supports the use of pdFX in neonates and infants and suggests that higher pdFX dosing of 70 to 80 IU/kg every 48 hours based on the smallest available vial size is feasible. Because of variability in IR, close monitoring of FX activity should be used to guide dosing in this age group.
Keyphrases
- case report
- end stage renal disease
- atrial fibrillation
- intensive care unit
- ejection fraction
- liver failure
- low birth weight
- prognostic factors
- acute kidney injury
- drug induced
- peritoneal dialysis
- early onset
- respiratory failure
- patient reported outcomes
- preterm infants
- septic shock
- extracorporeal membrane oxygenation
- smoking cessation