Causes of Occurrence Hemophilia

The bumps and scratches are part of the life of every child. For most children, bicycles and clothing stray kick in a football game, a temporary bruise or a healing scab. However, for children with hemophilia, these normal traumas of childhood are grounds for concern.

                              figure via buzzle

Hemophilia is a rare bleeding disorder that prevents blood from clotting properly. Currently, about 17,000 people in the United States hemophilia. 1 in every 5,000 boys is born with hemophilia, girls are less affected by this genetic disease linked to sex. The man can not pass the hemophilia gene to their son, though all his daughters will be carriers of the gene. Each carrier child woman man has a probability of 50% of hemophilia after.

Definition
Human blood contains special proteins, called clotting factors. Identified by Roman numerals, clotting factors help stop bleeding and allow the treatment of blood vessels after injury. The last step in the clotting process (also called coagulation) is to establish the net "that closes the torn blood vessels and stops bleeding. This is part of the clotting factors VIII and IX. People with hemophilia are gaps in one of these factors because of abnormal genes and, consequently, their blood can not clot properly.

Hemophilia A, also known as factor VIII deficiency is the cause of approximately 80% of cases. Hemophilia B, which represents most of the other 20% of cases, a deficiency of factor IX. Patients were classified as mild, moderate or severe, based on the amount of factor in the blood.

Patients whose blood tests suggest severe hemophilia usually bleed frequently, and another patient with a benign form of bleeding usually rare. However, there are a number of gravity of each group. The reasons for this variability may relate to other clotting factors or differences in the various behaviors that constitute a threat of harm.

Signs and Symptoms

The symptoms of hemophilia vary depending on the degree of factor deficiency and the location of the hemorrhage. Some children are diagnosed with hemophilia during the first six months of life because they are unlikely to suffer damage, which could cause bleeding. For example, only 30% of men with hemophilia bleed excessively when circumcised and only 1% to 2% of children with hemophilia are bleeding in the skull (ie hemorrhage).

Once children with hemophilia begin crawling and cruising, parents may notice raised bruises on the abdomen, chest, buttocks and back. Sometimes, because bruises occur in unlikely places, parents may be suspected of child abuse before their child is diagnosed with hemophilia.

The child may be picky and do not want to reach for the bowl, walk or crawl. Other symptoms include:

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  extended the nose
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  excessive bleeding from biting down, lips or tongue
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  excessive bleeding after tooth extraction
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  excessive bleeding after surgery
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  blood in the urine (hematuria called)

The most common type of hemophilia bleeding associated muscles and joints. A child with hemophilia do not usually want to move the joints and muscles affected because of the pain and swelling. Rebleeding municipality can also lead to chronic damage.

Diagnosis

Your doctor may suspect your child has hemophilia, or there was a pattern of bruising and bleeding, especially if it is bleeding into the joints. Diagnosing the condition requires a set of blood tests, including blood cell counts (CBC), prothrombin time (PT), partial thromboplastin time (PTT) levels IX factor VIII and factor.

Treatment

Although hemophilia is a chronic and incurable (other than liver), can be successfully replacement therapy of coagulation factor - periodic infusions of clotting factor deficient in the blood of a child. the broker may be administered intravenously (IV) of pipeline in hematology clinic or at home by a nurse or visiting parents (and even older patients) who underwent special training. Team Hemophilia child (doctors called hematologists who specialize in treating blood diseases, doctors, nurses, nurses, social workers) will teach you how to prepare the concentrated clotting factor and when and how to present the vein of the child. When clotting factor is "managed" starts to work quickly and helps prevent joint damage.

Although these treatments are effective, they are also expensive. According to the National Heart, Lung, and Blood Institute, the majority of children in the U.S. starting regular infusions early in life will exhaust the limit of life insurance average $ 1000000 in the second decade of

via kidshealth