EC Paediatrics

Hemophilia is a complex gene mutation blood disorder, that does not allow the clotting of the blood in the typical way it should, due to the insufficiency of blood clotting proteins also known as clotting factors. Hemophilia A is caused by the deficiency of clotting factor VIII (FVIII) and Hemophilia B is caused by the deficiency of clotting factor IX (FXI), which is present in the X chromosome. These are the key factors that help in the clotting of the blood. We could say that hemophilia A is a blood disorder that is genetic. The treatment is mainly blood clotting factor replacement therapy. We present a case of a 5-day-old neonate male with Hemophilia A, subdural hematoma with no known parental history of Hemophilia. Also highlights how the timely initiation of factor VIII (FVIII) transfusion along with other immediate interventions like the decompression of the subdural space could positively impact the outcome.

 Keywords: Neonate; Hemophilia A; Intracranial Hemorrhage (ICH); Subdural Hematoma (SDH); Factor VIII (FVIII); External Ventricular Drain (EVD)

1. Singleton TC and Keane M. “Diagnostic and therapeutic challenges of intracranial hemorrhage in neonates with congenital hemophilia: a case report and review”. The Ochsner Journal3 (2012): 249-253.
2. Zanon E and Pasca S. “Intracranial haemorrhage in children and adults with haemophilia A and B: a literature review of the last 20 years”. Blood Transfusion5 (2019): 378-384.
3. Sadek SAH., et al. “Intracranial Hemorrhage in a Newborn with Hemophilia”. Annals of Saudi Medicine5-6 (2000): 433-435.
4. Ferreira N., et al. “Neonatal hemophilia: a rare presentation”. Pediatric Reports4 (2015): 6338.
5. Kader Ş., et al. “A newborn with moderate hemophilia A with severe intracranial and extracranial hemorrhage: A case report”. Dicle Tıp Dergisi 20 (2017): 293-298.
6. Moreira A and Das H. “Acute Life-Threatening Hemorrhage in Neonates with Severe Hemophilia A: A Report of 3 Cases”. Journal of Investigative Medicine High Impact Case Reports (2018).
7. Puvabanditsin Surasak and Kased., et al. “Neonatal Hemophilia A”. Consultant (2021).