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FANCONI'S ANEMIA IN CHILDREN
Fanconi anemia patients have a higher incidence of cancer especially blood cancer (leukemia) than the general population. With onset of bone marrow failure, patients may succumb to infection (due to decrease in white cells), may have spontaneous bleeds (due to decrease in platelets) and have anemia (leading to decrease in oxygen supply to tissues).
If a child is diagnosed as Fanconi anemia, can his brothers and sisters also be affected?
Fanconi anemia is an inherited anemia. Both parents must be carriers of the Fanconi anemia gene for their child to be born with this disorder. If both parents carry the gene the chances are one in four (25%) that other children will also inherit the disease. This pattern of inheritance is known as "autosomal recessive" in medical terminology.
Thus, once a child is diagnosed as Fanconi anemia, other siblings in the family should also undergo the chromosomal breakage test even though they are normal otherwise.
Fanconi anemia can also be diagnosed before a child is born. The diagnosis can be done by performing a procedure called as chorionic villus sampling (CVS) at 10th - 12th week of pregnancy or by amniocentesis at 15th to 17th week of pregnancy and testing for chromosomal breakage.
At what age does aplastic anemia occur in Fanconi anemia?
No one can predict when marrow failure may occur. However most often it occurs in first decade of life though it has been reported to occur for first time even after the age of 40 years.
What is the treatment for Fanconi anemia?
The treatment for Fanconi anemia consists of :
Bone marrow transplant
Androgen therapy
Synthetic growth factors (Hematopoietic growth factors)
Gene therapy
Bone marrow transplant corrects problems related to bone marrow (anemia, low white count, low platelet count, leukemia). However patients may still suffer from problems related to other systems of the body.
Androgens are artificial male hormones that stimulate production of one or more types of blood cells for extended period of time. Androgens prolong the life of many Fanconi anemia patients but are not cure. Androgens can cause serious side effects such as liver disease and can have masculinizing effects which diminish or disappear with dose adjustments.
Hematopoietic growth factors such as GM-CSF, G-CSF increase the white cell count in patients with Fanconi anemia.
One of the Fanconi anemia genes has been isolated with the help of gene therapy; the corrected gene is introduced in the body with help of injection which supplies the missing protein needed in the Fanconi anemia patient's body and blood system. However, gene therapy is still in the research phase.
Last updated on 01-12-2005
Fanconi anemia is an inherited anemia. Both parents must be carriers of the Fanconi anemia gene for their child to be born with this disorder. If both parents carry the gene the chances are one in four (25%) that other children will also inherit the disease. This pattern of inheritance is known as "autosomal recessive" in medical terminology.
Thus, once a child is diagnosed as Fanconi anemia, other siblings in the family should also undergo the chromosomal breakage test even though they are normal otherwise.
Fanconi anemia can also be diagnosed before a child is born. The diagnosis can be done by performing a procedure called as chorionic villus sampling (CVS) at 10th - 12th week of pregnancy or by amniocentesis at 15th to 17th week of pregnancy and testing for chromosomal breakage.
At what age does aplastic anemia occur in Fanconi anemia?
No one can predict when marrow failure may occur. However most often it occurs in first decade of life though it has been reported to occur for first time even after the age of 40 years.
What is the treatment for Fanconi anemia?
The treatment for Fanconi anemia consists of :
Bone marrow transplant Androgen therapy Synthetic growth factors (Hematopoietic growth factors) Gene therapyBone marrow transplant corrects problems related to bone marrow (anemia, low white count, low platelet count, leukemia). However patients may still suffer from problems related to other systems of the body.
Androgens are artificial male hormones that stimulate production of one or more types of blood cells for extended period of time. Androgens prolong the life of many Fanconi anemia patients but are not cure. Androgens can cause serious side effects such as liver disease and can have masculinizing effects which diminish or disappear with dose adjustments.
Hematopoietic growth factors such as GM-CSF, G-CSF increase the white cell count in patients with Fanconi anemia.
One of the Fanconi anemia genes has been isolated with the help of gene therapy; the corrected gene is introduced in the body with help of injection which supplies the missing protein needed in the Fanconi anemia patient's body and blood system. However, gene therapy is still in the research phase.
Last updated on 01-12-2005
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