What is X-linked Agammaglobulinemia?

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X-linked a-gamma globulinemia,is also called as Bruton’s agammaglobulinemia or congenital agammaglobulinemia. It is a relatively rare inherited disease and was the first immunodeficiency disease ever identified.

Failure of immunoglobulin’s (antibodies) production is responsible for this disease. Since the gene responsible for this disease is associated with X chromosome this disease is generally noticed in male infants’ and is referred as “X-linked”.

In X-linked agammaglobulinemia, pre-B-lymphocytes fail to mature into B-lymphocytes (mature B-lymphocytes only produce antibodies). As a result antibodies production does not take place, and the child’s body is unable to fight off bacterial and some viral infections.

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Right after they are born, babies are protected from infections by immunity transmitted to them by their mothers. The child receives maternal antibodies through placenta and breast milk.

Within the next few months, their immune systems develop and begin to assume responsibility for fighting off infections. But sometimes, babies have immune deficiencies and they don’t have the ability to fight off routine infections on their own.

When the maternal antibodies are exhausted and supply is ceased, the child begins to suffer from severe recurrent infections. As they have normal T cells and cell mediated immunity, the child shows resistances to various diseases caused by intracellular pathogens, but fail to have humoral immunity.

Close analysis shows that the children with X-linked agammaglobulinemia do have normal number of precursor B cells, but they fail to develop into functional cells due to unknown reason. Defective gene in long arm of X chromosome (X-LA) is found responsible for this disease in males.

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