What is hemophilia? What is hemophilia?

Hemophilia is a bleeding disorder with hereditary coagulation disorders, including hemophilia A (factor deficiency), hemophilia B (factor deficiency), and hemophilia C (factor deficiency). Each type may occur separately or in combination.

Both hemophilia A and B are X-chromosome acute genetic ventricles, which manifest as a hereditary compartment in women and a disease in men. For example, when a woman carrying a gene marries a normal man, all the daughters born do not show illness, but half of the chance is that they are carriers of the hemophilia gene; Boys born are half the chance of being a political party and half of the chances of being a child. If a male patient is married to a normal woman, the sons born are normal, and the daughters born are all hemophilia transmitters.

Hemophilia C is an autosomal dominant or incomplete recessive inheritance that can be transmitted to both men and women.

1) Clinical manifestations The main manifestation of hemophilia is bleeding, which is characterized by: the bleeding site is extensive and severe, and it is not easy to stop bleeding, and the bleeding often lasts for several hours or even weeks; Lifetime minor injuries and prolonged bleeding after surgery; There is often spontaneous hemarthrosis and reversely causes hemophilic arthritis.

Diagnosis is confirmed by testing for clotting factors.

2)** Hemophilia is mainly hemostasis**: local hemostasis can be used with gelatin sponge, thrombin, epinephrine, etc.; Alternatives**: including transfusions of plasma, cryoprecipitate, factors, concentrates, and procoagulant complexes, which are effective for hemophilia A and B; 1-deamino8-D-arginine vasopressin intravenous infusion, effective against hemophilia A; Others** such as 6-aminocaproic acid, glucocorticoids, female contraceptives, peanut coatings, etc. can be used; Patients with this disease should avoid various surgeries as much as possible.

Hemophilia A is a hemorrhagic recessive genetic disorder associated with the X chromosome gene. About one in every 5,000-10,000 men has hemophilia A. Patients suffer from recurrent spontaneous bleeding that leads to joint damage or deformity and disability.

Relatives living donor spleen transplantation ** Hemophilia A began in the 60s of the 20th century. It is understood that after a domestic hospital performed spleen transplantation on a patient with hemophilia A**, the transplanted spleen of the patient who underwent surgery showed varying degrees of functional loss and atrophy.

According to experts, in addition to the high risks of the same spleen transplantation, there are inevitably two types of rejection in the patient's body, and long-term immunosuppressants are also required, which is extremely expensive. After transplantation, the transplanted spleen has a short survival time, and there are different degrees of fibrosis and atrophy. For healthy people who provide a spleen, postoperative surgery increases the likelihood of a dangerous infection after splenectomy.

At present, the internationally accepted method of hemophilia A is to use high-purified anti-hemophilia nail globulin, recombinant factor 8 and so on to replace**, this method has achieved good efficacy in developed countries, its cost is less than spleen transplantation, and the safety is high, and has become the main means of hemophilia A patients in European and American countries. Methods such as the use of self-stem cells to transfer factor 8 gene reinfusion ** hemophilia is also being explored.

In China, some doctors in some hospitals are still strongly recommending this surgery to hemophilia patients, and the crux of the problem is that before this operation, the vast majority of patients do not have a clear understanding of the postoperative risks and anti-rejection drugs, which involves the infringement of the patient's right to know. Therefore, our view is:

Resolutely oppose the use of relatives to transplant ** hemophilia with living spleen, and return the patient to the right to know.

What exactly is hemophilia?

It is a hemorrhagic disorder in which there is an inherited coagulation abnormality; The cause is likely to be due to genetic factors, or it may be a genetic mutation; In general, the symptoms are pain, impaired movement, and dysfunction at the bleeding site.

I think the main reason may be due to genetics, or it may be genetic mutations; The symptoms of hemophilia are mainly hemarthrosis or muscle bleeding, **bleeding and so on.

It's actually a bleeding disorder; The main reason is the lack of congenital clotting factors in the body; The joints may be painful and bleeding.

Hemophilia is a bleeding disorder in which there is an inherited coagulation disorder, often caused by a congenital deficiency of coagulation factors. Depending on the type of clotting factor deficiency, the disease can be divided into hemophilia A and hemophilia B. Hemophilia A is characterized by a deficiency of coagulation factor (factor 8) and hemophilia B is characterized by a deficiency of coagulation factor (factor 9), both of which are caused by mutations in the corresponding coagulation factor gene.

Among them, hemophilia A is the most common, accounting for about 85%.

Due to the lack of sufficient clotting factors in the body, the blood cannot clot properly in the damaged area, resulting in continuous bleeding, which can cause a series of symptoms. It is clinically characterized by spontaneous or minor bleeding from joints, muscles, visceral organs, and deep tissues.

There are no significant racial or regional differences in the incidence of hemophilia. In the male population, the incidence of hemophilia A is about 1 5000 and that of hemophilia B is about 1 25 000;Female hemophilia is extremely rare.