How to cure hemophilia B?

summary

Hemophilia B, also known as factor IX deficiency or plasma thromboplastin deficiency, is a coagulation disorder first distinguished from hemophilia A by aggeller in 1952. The incidence rate of type A and B is about 5.4:1. How to cure hemophilia B? Next, I'd like to share my views with you.

How to cure hemophilia B?

First, in addition to the high risk of spleen transplantation, there are two types of rejection in patients, which need long-term immunosuppressive therapy, and the cost is extremely expensive. After transplantation, without exception, the survival time of transplanted spleen is short, and there are varying degrees of fibrosis and atrophy. For the healthy people who provided spleen, the possibility of dangerous infection after splenectomy was increased.

Second, the average activity of factor VIII in each bag of cryoprecipitate frozen (- 20 ℃) preparation is 100 u, which can increase the plasma concentration of factor VIII to more than 50% of the normal level. The utility model has the advantages of large efficiency and small capacity. It can be stored at - 20 ℃ for more than 25 days. Suitable for light and medium-sized patients.

Third: factor VIII and "concentrate" are freeze-dried products, and the activity of each unit of factor VIII and "concentrate" is equivalent to the average activity in 1 ml of fresh human plasma. Each bottle contained 200 U of factor Ⅷ. The activity of factor Ⅷ was increased by 2% when injected with 1 U of factor Ⅷ per kg body weight. However, the activity of factor Ⅷ was only increased by 0.5% - 1% when injected with 1 U of factor Ⅷ per kg body weight. The half-life of factor VIII and "factor VIII" in circulation is short, so it is necessary to supplement every 12 hours to maintain high factor level and control bleeding.

matters needing attention

(1) "Prevention and nursing of bleeding", pay special attention to avoid trauma. When you go to the hospital, you should explain the condition to the doctors and nurses, and avoid intramuscular injection as far as possible. Do a good job in family security, try to avoid using sharp tools, such as needles, scissors, knives, etc（ 2) At ordinary times in the case of no bleeding, appropriate exercise, to reduce the recurrence of the disease. But when there is active bleeding, the activity should be limited to avoid aggravating the bleeding.