Deficiency of factor IX ( hemophilia c)

August 12, 2017 17:51 | Genetic Diseases

disease is transmitted in an autosomal type of inheritance, so that they suffer from both sexes.

There are latent (hidden), small and pronounced form of the disease.In the first form, which is more than 50% of all cases of hemophilia C, bleeding occur rarely, only with more injuries and operations.In mild disease, spontaneous bleeding minimum or no, but naturally there bleeding in trauma and surgical interventions.Minor surgery (removal of teeth, intestinal polyps, and others.) Are rarely accompanied by bleeding.For severe forms of the disease are characterized as moderate spontaneous bleeding (nosebleeds, easy bruising, etc.) As well as long and heavy bleeding in surgical interventions.Occasionally there are subcutaneous and intermuscular hematoma and acute bleeding in the joints.In this regard, the disease becomes some similarities with the easiest varieties of hemophilia A or B.

Women menstrual bleeding there is not always, and mostly only in the period of puberty.Extremely rare (in rare cases) rec

orded post-partum bleeding.For most women, including those with very low levels of factor XI in the plasma at delivery are required transfusion therapy and prevention.In contrast, injuries and surgical interventions in hemophilia C may be complicated by severe and life-threatening bleeding.Often such bleeding becomes a complete surprise to physicians, since nothing prevents suspected irregularities in the blood clotting system.

treatment.Reliable and stop bleeding prophylaxis provided by intravenous administration of a natural, fresh-frozen plasma or dry.Validity injected into the blood stream after the first factor XI transfusion is about 60 hours after repeated administration increases to 120 hours or more.Consequently, to ensure normal blood clotting postoperative enough intravenous plasma injections in the early days of every 48 hours, and then after 72 h.

From nonspecific treatments for this disease with the most successful use of e-aminocaproic acid, promoting the prevention andstop bleeding with small injuries and operations (cuts, tooth extraction).