Hemophilia B – What is it, Causes, Symptoms and Treatments!

Hemophilia B – What it is, Causes, Symptoms and Treatments of this condition. In addition, Hemophilia B is characterized by a deficiency in factor IX clotting activity, which results in prolonged removal after injury, tooth extractions, or surgery, and delayed or recurrent bleeding  before complete wound healing. The age at diagnosis and the frequency of bleeding episodes are related to the level of factor IX clotting activity.

People with Hemophilia B bleed easily, and blood  takes longer to clot. People with Hemophilia B may experience spontaneous or internal bleeding and often have painful, swollen joints due to bleeding  into the joints. This rare but serious condition can have life-threatening complications.

Causes of Hemophilia B: Hemophilia B is caused by an X-linked hereditary recessive trait, with the defective gene located on the X chromosome. Females have two copies of the X chromosome. If the factor IX gene on one chromosome does not work, the gene on the other chromosome can do the work of making enough factor IX.

Males have only one X chromosome. If the factor IX gene is missing from a boy’s X chromosome, he will have Hemophilia B. For this reason, most people with Hemophilia B are male. If a woman has a defective factor IX gene, she is considered a carrier. This means that the defective gene can be passed on to your children.

Boys born to these women have a 50% chance of having Hemophilia B. Your daughters have a 50% chance of being carriers. All female children with hemophilia carry the defective gene.

Symptoms of Hemophilia B: The extent of symptoms of Hemophilia B depends on the severity of your factor deficiency. People with mild disabilities may bleed in the event of trauma. People with a severe disability can bleed for no reason. This is called “spontaneous bleeding ”. In children with Hemophilia B , these symptoms may occur around age 2 years.

Spontaneous bleeding  can cause the following:

• Blood in the urine;
• Blood  in the stool;
• Deep bruises;
• Large unexplained bruises;
• excessive bleeding  ;
• bleeding  gums;
• frequent nosebleeds ;
• Joint pain ;
• tight joints;
• Irritability (in children).

Diagnoses of Hemophilia B: The diagnosis of Hemophilia B is established in individuals with low factor IX clotting activity. Identification of a pathogenic variant F9 hemiline on molecular genetic testing in a male proband confirms the diagnosis. Identification of a heterozygous F9 pathogenic variant on molecular genetic testing in a symptomatic female confirms the diagnosis.

Treatments For Hemophilia B: Treatment for Hemophilia B includes replacement of the missing clotting factor. You will receive factor IX concentrates. To prevent a bleeding attack , people with Hemophilia B and their families can be taught to give factor IX concentrates at home at the first signs of bleeding . People with severe forms of the disease may need regular preventive infusions.

If you have severe hemophilia B , you also need to take factor IX concentrate before surgery or certain types of dental work. You must get the hepatitis B vaccine. People with hemophilia are more likely to get hepatitis B because they can receive blood products.

Some people with hemophilia B  develop antibodies against factor IX. These antibodies are called inhibitors. Inhibitors attack factor IX so that it no longer works.