Neonatal congenital Factor X deficiency.
Four neonates with congenital Factor X deficiency presented soon after birth with bleeding episodes. Two of the newborns had intracranial hemorrhages; one of them also had antenatal ventricular dilatation and postnatal hydrocephalus and died of massive intracerebral hemorrhage at four months. One patient was lost for follow up. The two surviving infants were followed up for four years and two years respectively, while on replacement therapy with three injections of 40 units/kg prothrombin complex a month. In spite of markedly elevated prothrombin time and partial thromboplastin time, these two infants remain free of major bleeding manifestations except for troublesome petechiae and ecchymoses. A schedule for substitution therapy with Factor X is proposed for infants and children to prevent bleeding in severe Factor X deficiency.
el Kalla
S
S
Department of Pediatrics, Al Wasl Maternity & Pediatric Hospital, Dubai, United Arab Emirates.
Menon
N S
NS
D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002543:Cerebral Hemorrhage / Q000209:etiology; D003241:Consanguinity; D005171:Factor X Deficiency / Q000150:complications / Q000151:congenital*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010314:Partial Thromboplastin Time; D011517:Prothrombin Time
D001779:Blood Coagulation Factors; C025667:prothrombin complex concentrates