About Hemophilia

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Hemophilia

DEFINITION

Hemophilia is a bleeding disorder due to deficiency of a clotting factor.

There are two types of hemophilia:

Hemophilia A (classic hemophilia) is the shortage of factor VIII, which covers 80% of cases.

Hemophilia B (Christmas disease) is the lack of factor IX.

Bleeding patterns and consequences of these two types of hemophilia are the same.

CAUSE

Hemophilia is caused by several genes that are inherited disorders; inherited through the mother, but almost always attacked the boy.

SYMPTOMS

Severity of symptoms depends on the influence of gene abnormalities that occur on the activity of factor VII and factor IX. If the activity is less than 1%, there will be severe and recurrent bleeding episodes for no apparent reason.

If the activity at 5%, the symptoms are mild. Infrequent bleeding episodes without definite cause, but the surgery or injury can cause uncontrolled bleeding, which can be fatal.

The first bleeding episode usually occurs before age 18 months, which often occurs after a minor injury. Children prone to bruising. Even the injection into the muscle can cause bleeding which in turn led to extensive bruising (hematoma).

Recurrent bleeding into joints and muscles can ultimately lead to crippling deformities. Bleeding can lead to swelling of the tongue base that clogs the airways and respiratory symptoms occur.

Minor collisions on the head can lead to bleeding in the skull, which can cause brain damage and death.

Diagnosis

If a boy experiencing abnormal bleeding, it is believed he suffered from hemophilia.

Blood tests can find a slowdown in the freezing process. If there is a slowdown, then to confirm the diagnosis and determine the type and severity, upon examination conducted activity of factor VII and factor IX.

TREATMENT

People with hemophilia should avoid circumstances that can cause bleeding. They should really pay attention to teeth care to not have to undergo a tooth extraction.

The mild hemophiliacs who have undergone surgery or tooth extraction will be given the drug desmopressin to correct the clotting system that is only temporary, so no need for transfusion.

Patients should also avoid medications such as aspirin, warfarin, heparin, and certain pain medication (eg anti-inflammatory non-steroid), which can exacerbate bleeding disorders.

Treatment usually includes transfusions to replace clotting factor deficiencies. These factors are found in plasma and in greater amounts found in the plasma concentrations.

Some people form antibodies against factor VIII and factor IX are transfused, so transfusions ineffective.

If the antibodies were found in the blood sample, then the dose is increased or given plasma concentrates of different clotting factors or given drugs to reduce levels of antibodies.

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