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Related Experiment Videos

Neonatal congenital Factor X deficiency.

S el Kalla1, N S Menon

  • 1Department of Pediatrics, Al Wasl Maternity & Pediatric Hospital, Dubai, United Arab Emirates.

Pediatric Hematology and Oncology
|October 1, 1991
PubMed
Summary

Congenital Factor X deficiency in neonates can cause severe bleeding, including intracranial hemorrhages. Replacement therapy with prothrombin complex concentrate can prevent major bleeding events in infants with this rare bleeding disorder.

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Area of Science:

  • Hematology
  • Pediatric Medicine
  • Genetics

Background:

  • Congenital Factor X deficiency is a rare inherited bleeding disorder.
  • Neonates with this condition present with bleeding soon after birth.

Observation:

  • Four neonates with Factor X deficiency experienced bleeding episodes, including intracranial hemorrhages.
  • One neonate with antenatal ventricular dilatation and postnatal hydrocephalus died from intracerebral hemorrhage.
  • Two surviving infants were treated with monthly prothrombin complex concentrate injections.

Findings:

  • Despite elevated prothrombin time and partial thromboplastin time, surviving infants remained free of major bleeding.
  • Troublesome petechiae and ecchymoses were observed in the treated infants.
  • Replacement therapy with prothrombin complex concentrate demonstrated efficacy in preventing severe bleeding.

Implications:

  • A proposed schedule for Factor X substitution therapy can guide management in infants and children.
  • Early diagnosis and consistent replacement therapy are crucial for managing severe Factor X deficiency.
  • This study highlights the importance of timely intervention to prevent life-threatening hemorrhages in affected neonates.

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