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Brain & development · Mar 2004
Case ReportsSevere congenital factor X deficiency with intracranial bleeding in two siblings.
- Bahri Ermis, Rahmi Ors, Ayhan Tastekin, and Fatih Orhan.
- Department of Pediatrics, Atatürk University Faculty of Medicine, Erzurum, Turkey.
- Brain Dev. 2004 Mar 1;26(2):137-8.
AbstractCongenital factor X deficiency is a rare autosomal recessive disorder that usually presents with variable bleeding tendency, prolonged prothrombin time and partial thromboplastin time. Therefore, it may be misdiagnosed as hemorrhagic disease of the newborn. Factor X level should be investigated for the definite diagnosis. We first report a new family whose two infants presented with severe intracranial bleeding at different times and were found to have congenital factor X deficiency. Plasma replacement therapy was not found to be efficacious in these infants. In conclusion, a possible factor X deficiency should be considered when a newborn presents with intracranial bleeding.
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