Combined Factor V and VIII Deficiency- A Rare Case with Dental Management

Journal of oral medicine(2019)

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摘要
Familial multiple coagulation factor deficiency is a group of rare inherited bleeding disorders characterized by a simultaneous decrease in the levels of two or more coagulation factors. Among dual coagulation disorders CF5F8D is the most common type. Combined factor V and factor VIII is a rare autosomal recessive bleeding disorder characterized by concomitantly low levels of two coagulation factors, more common among Jews and Iranians. This condition was first described by Oeri et al. in 1954. The molecular mechanism of the association of the two factor deficiencies was discovered by Nichols et al as a mutation of LMANI gene. Approximately 70% of the patients have mutation in LMANI. It is rarely diagnosed due to the mild bleeding manifestations. Due to the rarity of combined factor deficiency, we present the case of a child with Familial multiple clotting factor deficiency, type 1.
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关键词
viii,dental management,rare case
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