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HEMOPHILIA IN CHILDREN
Hemophilia is an inherited disorder in which there is inability to form an effective clot and hence causes prolonged bleeding in a patient. It is seen exclusively in males. A person with hemophilia does not bleed faster, only longer.
How common is hemophilia?
A: Hemophilia affects approximately 1 in 5,000 live male births.
How is hemophilia inherited ?
A: There are two types of hemophilia - Hemophilia A and Hemophilia B. Hemophilia A results from deficiency or abnormality of clotting factor in the blood called as "factor VIII".
Hemophilia B results from deficiency or abnormality of another clotting factor called as "factor IX". Females are usually carriers (i.e. they have the abnormal gene that causes hemophilia but do not have clinical manifestations). The male children usually manifest the disease. Sometimes hemophilia occurs due to a fresh mutation in the gene sequence in the child.
The following permutations-combinations can occur while inheriting hemophilia :-
1. Normal Mother + Father with hemophilia.
During each pregnancy, the female offspring has 100% chance of being a carrier and the male offspring is always normal. Hence, sons of hemophiliac fathers and normal mothers are normal.
2. Carrier Mother + Normal Father
During each pregnancy, there is 25% chance of a normal female, 25% chance of a carrier female, 25% chance of a normal male and 25% chance of an affected male.
3. Carrier Mother + Father with hemophilia
Each pregnancy has 25% chance of a female carrier, 25% chance a female with hemophilia, 25% chance of a normal male and 25% chance of an affected male.
4. Mother with hemophilia + Father with hemophilia
During each pregnancy there is 50% chance of an affected female and a 50% chance of an affected male (actual occurrence is rare).
5. Mother with Hemophilia + Normal Father
During each pregnancy there is 50% chance of a carrier female and a 50% chance of an affected male though the actual occurrence is rare.
What are the problems in a hemophilia child ?
A: Hemophilia are divided into severe, moderate and mild diseases depending on the level of functional factor VIII or factor IX. (normal levels are between 50% and 150%). Mild hemophilia patients (factor levels>5% and <50%) bleed only after injury, invasive procedure or surgery. Carriers of hemophilia are also sometimes found to have factor levels in the Mild range. Moderate hemophiliacs (factor levels - 2 to 5%) experience more bleeds, about once a month, usually after trauma, surgery or extreme exertion. They usually bleed in the muscles and joints. Once a bleeding occurs in a particular area, these patients may continue to have bleeding episodes in those areas. Severe hemophiliacs (factor level<1%) bleed spontaneously usually in joints, muscles and soft tissues.
Joint and muscle bleeding may cause pain, tingling, heat, swelling, limited range of motion etc. It commonly occurs in the ankles, elbows and knees but can also affect any joint in the body.
A: Hemophilia affects approximately 1 in 5,000 live male births.
How is hemophilia inherited ?
A: There are two types of hemophilia - Hemophilia A and Hemophilia B. Hemophilia A results from deficiency or abnormality of clotting factor in the blood called as "factor VIII".
Hemophilia B results from deficiency or abnormality of another clotting factor called as "factor IX". Females are usually carriers (i.e. they have the abnormal gene that causes hemophilia but do not have clinical manifestations). The male children usually manifest the disease. Sometimes hemophilia occurs due to a fresh mutation in the gene sequence in the child.
The following permutations-combinations can occur while inheriting hemophilia :-
1. Normal Mother + Father with hemophilia.
During each pregnancy, the female offspring has 100% chance of being a carrier and the male offspring is always normal. Hence, sons of hemophiliac fathers and normal mothers are normal.
2. Carrier Mother + Normal Father
During each pregnancy, there is 25% chance of a normal female, 25% chance of a carrier female, 25% chance of a normal male and 25% chance of an affected male.
3. Carrier Mother + Father with hemophilia
Each pregnancy has 25% chance of a female carrier, 25% chance a female with hemophilia, 25% chance of a normal male and 25% chance of an affected male.
4. Mother with hemophilia + Father with hemophilia
During each pregnancy there is 50% chance of an affected female and a 50% chance of an affected male (actual occurrence is rare).
5. Mother with Hemophilia + Normal Father
During each pregnancy there is 50% chance of a carrier female and a 50% chance of an affected male though the actual occurrence is rare.
What are the problems in a hemophilia child ?
A: Hemophilia are divided into severe, moderate and mild diseases depending on the level of functional factor VIII or factor IX. (normal levels are between 50% and 150%). Mild hemophilia patients (factor levels>5% and <50%) bleed only after injury, invasive procedure or surgery. Carriers of hemophilia are also sometimes found to have factor levels in the Mild range. Moderate hemophiliacs (factor levels - 2 to 5%) experience more bleeds, about once a month, usually after trauma, surgery or extreme exertion. They usually bleed in the muscles and joints. Once a bleeding occurs in a particular area, these patients may continue to have bleeding episodes in those areas. Severe hemophiliacs (factor level<1%) bleed spontaneously usually in joints, muscles and soft tissues.
Joint and muscle bleeding may cause pain, tingling, heat, swelling, limited range of motion etc. It commonly occurs in the ankles, elbows and knees but can also affect any joint in the body.
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