The disease caused by deficiency of Congenital Factor IX is known as Hemophilia B. Hemophilia B is an inherited or genetic disorder caused in humans due to the absence of congenital Factor IX which is a clotting protein essential for the process of coagulation. If there is a deficiency of clotting factor IX, the blood loses its ability to clot which results in excessive bleeding even from a minor cut.
The genes are passed from parents to their offspring but sometimes it can occur due to spontaneous mutation. According to the US Centers for Disease Control and Prevention, Hemophilia B occurs in 1 in 5000 live births making it four times less common than Hemophilia A.
The required gene for Hemophilia is the X chromosome. Hemophilia B is inherited as an X-linked recessive disorder where males are affected the most. Females inherit two X chromosomes, one from their father and one from their mother. Whereas males inherit an X chromosome from their mother and a Y chromosome from their father. Therefore, if the mother is suffering from Hemophilia, she becomes a carrier of it.
SYMPTOMS OF HEMOPHILIA B
The symptoms of Hemophilia B can vary with the seriousness of the disease. Some of the common symptoms include-
- Blood in urine
- Bleeding from nose
- Acute pain and swelling in joints
- Excessive bleeding from a minor cut
- Bleeding after surgery or tooth extractions
- Bleeding without any cause
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DIAGNOSIS FOR HEMOPHILIA B
A Coagulation study that includes a series of tests is advised by health care providers if the patient displays any one of the above-mentioned symptoms. Testing for the diagnosis of Hemophilia B includes-
ü Bleeding Time Test
ü Level of Fibrinogen
ü Prothrombin Time Test
ü Partial thromboplastin time Test
ü Serum factor IX activity
The primary treatment for Hemophilia B is the infusion of the missing clotting factor. If patients suffering from this disease need surgery, they must be given factor IX concentrate beforehand. Sometimes Prophylaxis, which is a routine treatment regime, is advised for patients with severe cases of this disease. Antibiotics that can be taken orally are also prescribed by doctors.
Patients are asked to regularly monitor their PT/INR ratio to adjust the doses of blood thinners such as WARFARIN (COUMADIN). PATIENT SELF TESTING is one of the easiest ways they can test their PT/INR level comfortably at home. PT/INR meters are an affordable, portable, and accurate device that gives the reading within seconds.