- Hemophilia - a rare, but the disease sign
- Signs
Hemophilia - a rare hereditary disorder in which impaired coagulation, or clotting. After any injury bleeding in patients with hemophilia lasts much longer than in healthy people. They often develop internal bleeding, particularly in the joints.
In some cases, hemophilia is purchased - it occurs when the body produces antibodies
Antibodies - "soldiers' immunity
to coagulation factors.
In newborns with hemophilia protein necessary for normal blood clotting is missing or is at a very low level. These proteins are called coagulation factors.
Hemophilia, with very few exceptions, is found only in men.
What is blood clotting
Trauma (eg, cut) of a blood vessel triggers a complex chain of events that result in blood clotting. Coagulation - a reaction to bleeding, which prevents the loss of too much blood. Most blood loss can be life-threatening, and often causes damage to internal organs.
What is the coagulation factor
Coagulation factor is a group of substances, especially proteins, which work with platelets
Platelets - what they are for the body?
Helping blood to clot. When damaged blood vessels clotting factors help the platelets combine with each other to close those sites that have been damaged.
People with hemophilia blood does not clot quickly enough, as clotting factors have no, or very little. This can lead to greater blood loss and / or damage to the organs and tissues of the body. The result could be permanent damage or death of the patient. To stop the bleeding, hemophilia may need to enter the coagulation factors.
Types of hemophilia
There are two main types of hemophilia:
- Hemophilia A - is characterized by the absence or low levels of clotting factor VIII. Approximately 9 out of 10 people with hemophilia are diagnosed with this type of hemophilia.
- Hemophilia B is characterized by the absence or low levels of clotting factor IX.
Hemophilia can be mild, moderate, or severe, depending on the amount of blood coagulation factor. About 7 of the 10 persons with hemophilia A suffer from severe forms of the disease.
Causes
Hemophilia - a hereditary disease. It is caused by a defect in the genes that determine how the body makes blood clotting factors VIII and IX. These genes are located on the X chromosome.
If a woman has this defective gene ("hemophilia gene"), she can pass it on to their children. If she has a son, the likelihood that he will get sick with hemophilia, is 50%. When my daughter was born the likelihood that it will become a carrier of the hemophilia gene is also 50%.
A man with hemophilia, can not transmit the disease to his sons, but all his daughters will be carriers of hemophilia.
Very rarely girls are born with haemophilia. This can happen if her father had hemophilia and her mother is a carrier of the hemophilia gene.
Sometimes boys are born with hemophilia in women who are not carriers of the defective gene. In such cases, the genes are transferred child mutations occur, which is practically impossible to anticipate.
Symptoms
The main symptoms of hemophilia are frequent bruising, bruising and prolonged bleeding. Internal bleeding is common in people with severe hemophilia. Without rapid treatment of internal bleeding could result in damage to the joints, muscles or other body parts.
Most young children first signs of hemophilia are excessive bleeding during teething
Teething: hard times
, Bruising and bruising from even minor injuries, swelling in the joints, which can be caused by internal bleeding. In older children and adults, besides those already mentioned, the following symptoms of hemophilia: nosebleeds for no apparent reason, the presence of blood in urine
Blood in the urine - a reason for serious concern
(caused by bleeding in the kidney or bladder) and stool (caused by bleeding in the gut or stomach).
Bleeding in the joints - the most common problem of patients with hemophilia. Often bleeding begins a result of trauma and without treatment can continue for several days. However, people with haemophilia, learn to recognize early symptoms of hemophilia, and, as a rule, during seek medical help.