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[Combined hereditary deficiency in factors VII and X revealed by a prolonged partial thromboplastin time]
G Boxus1, M Slacmeulder, J Ninane
1Service de pédiatrie, clinique Notre-Dame de Grâce, Gosselies, Belgique.
Background:
Congenital factors VII and X deficiency is rare. Association of both deficiencies is exceptional.
Case Report:
A 3 year-old boy, born to consanguinous Moroccan parents, had a prolonged partial thromboplastin time discovered fortuitously. This finding led to the diagnosis of combined factors VII and X deficiency. His siblings had the same deficiencies.
Conclusion:
Profound deficiencies in factors VII and X are inherited following an autosomal-recessive mode. These deficiencies may be asymptomatic, only discovered by prolonged partial thromboplastin time. They may also be revealed by intracranial bleeding and other severe hemorrhages. Treatment consists of administration of factor VII or PPSB.