Hemophilia B
, also known as factor IX deficiency or Christmas disease, is the second most common type of hemophilia. The disorder was first reported in the medical literature in 1952 in a patient with the name of Stephen Christmas. The most famous family with hemophilia B was that of Queen Victoria of England.
How is Factor 9 deficiency Treated?
Standard treatment is
infusion of factor IX concentrates to replace the defective clotting factor
. The amount infused depends upon the severity of bleeding, the site of the bleeding, and the size of the patient.
What causes factor IX deficiency?
Hemophilia B
, also called factor IX (FIX) deficiency or Christmas disease, is a genetic disorder caused by missing or defective factor IX, a clotting protein. Although it is passed down from parents to children, about 1/3 of cases are caused by a spontaneous mutation, a change in a gene.
Is hemophilia A or B worse?
Recent evidence suggests that
hemophilia B is clinically less severe than hemophilia A
, highlighting the need to discuss further therapeutic options for each type of hemophilia. The study, “Haemophilia B is clinically less severe than haemophilia A: further evidence,” was published in Blood Transfusion.
What is a factor IX test for?
The factor IX test is part of a larger screening test to find out which
type of bleeding disorder you have
. Factor IX is a protein that helps your blood clot. If you are lacking this protein, you may have a bleeding disorder called hemophilia B.
What activates factor IX?
Factor IX is activated as the result of
the cleavage of two peptide bonds, Arg
145
and Arg
180
–Ile
181
, to form a two-chain enzyme. The chains are linked by a disulfide bond, and thus, the Gla domain remains associated with the protease domain and maintains phospholipid association capability.
What is Factor 9 in the blood?
Factor IX is a protein produced naturally in the body. It
helps the blood form clots to stop bleeding
. Injections of factor IX are used to treat hemophilia B, which is sometimes called Christmas disease.
Is this the only substitution that causes Factor 9 hemophilia?
Our bodies have 12 clotting factors that work together in this process (numbered using Roman numerals from I through XII). Having too little of factors VIII (8) or IX (9) is what causes hemophilia. A person with hemophilia will lack only one factor, either
factor VIII or factor IX
, but not both.
How is Factor VIII deficiency treated?
Desmopressin
raises the levels of factor VIII in the blood, and you take it through an IV or as a nasal spray. Drugs known as antifibrinolytics, such as aminocaproic acid and tranexamic acid, can also help if you have a mild form of the disorder.
What is Factor 9 called?
Factor IX (
or Christmas factor
) (EC 3.4. 21.22) is one of the serine proteases of the coagulation system; it belongs to peptidase family S1. Deficiency of this protein causes haemophilia B.
What race is most affected by hemophilia?
The average age of persons with hemophilia in the United States is 23.5 years. Compared to the distribution of race and ethnicity in the U.S. population,
white race is more common
, Hispanic ethnicity is equally common, while black race and Asian ancestry are less common among persons with hemophilia.
What foods to avoid if you have hemophilia?
- large glasses of juice.
- soft drinks, energy drinks, and sweetened tea.
- heavy gravies and sauces.
- butter, shortening, or lard.
- full-fat dairy products.
- candy.
- foods containing trans fats, including fried. foods and baked goods (pastries, pizza, pie, cookies, and crackers)
Does hemophilia go away?
There is no cure for hemophilia at this time
, but there is medicine people can take. This medicine helps them stop bleeding so they can do most of the things everyone else does.
What is the normal level of factor IX?
Normal plasma levels of factor IX are
50 to 150 units/dl
. Patients with levels less than 1 unit/dl have severe disease, those with levels of 1 to 5 units/dl moderate disease, and those with levels greater than 5 units/dl mild disease. The half-life of factor IX is approximately 18 to 24 hours.
What is factor 10 called?
COAGULATION CASCADE | Factor X
Factor X (fX), also called
Stuart factor
, is a vitamin-K dependent serine protease zymogen that is activated in the first common step of the intrinsic and extrinsic pathways of blood coagulation.
What is Factor 8 called?
Factor VIII (
antihemophilic factor
) is the protein that is deficient or defective in patients with classical hemophilia and Von Willebrand syndrome. Factor VIII in plasma is thought to be associated in a complex with the highest molecular weight multimers of another glycoprotein, Von Willebrand protein.
