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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

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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.

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.

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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.