Congenital factor X deficiency

Congenital factor X deficiency: This disease is rare and is an autosomal recessive inheritance. Patients' parents are often married to close relatives, and both men and women can develop the disease. Since factor X may be related to the function of the internal and external coagulation system, and may also have similar symptoms of factor Ⅶ deficiency, the degree of bleeding is related to the concentration of factor X. Homozygous types generally have bleeding symptoms, and the concentration of factor X in the heterozygous type is about 20% to 50% without bleeding tendency. In laboratory tests, prothrombin time (PT), partial thromboplastin time (PTT), and snake venom time were all prolonged, and the latter could be distinguished from factor Ⅶ deficiency. Factor X supplementation is the main treatment, and concentrated preparations of stored plasma, PPSB or factor X can be used. Infusion of 10 to 15 ml of plasma per kg of body weight. The effective hemostatic concentration of factor X is about 5% to 10%. The hemostatic concentration of severe bleeding is about 15% to 20%.

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