What is Agammaglobulinemia?
Agammaglobulinemia , a geneticautoimmune disorder , is an inherit resistant organization disorderliness in which the eubstance ’s immune system is not capable to make enough antibodies to agitate off infection either bacterium or viral . Agammaglobulinemia is also know by the name of Bruton ’s Agammaglobulinemia or Adam - linked agammaglobulinemia . Since children with this condition do not have sufficient antibody hence they are more prostrate to frequentinfections . This term is regard to come in Male more than female .
Agammaglobulinemia is have due to a defect in the cistron BTK or Bruton ’s Tyrosine Kinase which is responsible for production of antibodies to oppose off infections . The symptom of Agammaglobulinemia get down to become apparent by the metre the tiddler is about a year old where the child start to have frequent infection due to Agammaglobulinemia .
What Causes Agammaglobulinemia?
As tell , Agammaglobulinemia is a genetic precondition and is lapse down from the parent to the tyke . This condition is triggered by an freakishness in the BTK gene on chromosome X which affects the organic structure ’s ability to bring about antibodies . The reason why this disease occur in males more than female is because the cistron fault is linked to the ecstasy chromosome and male person have one X chromosome while females have two X chromosomes and hence they are the carriers of this defective gene . It is an autosomal recessionary trait means that only one transcript of the defective gene is enough to get Agammaglobulinemia . In some cases , the cistron sport in the X chromosome occurs as a new mutation and the child has this condition despite take no family history of this Agammaglobulinemia .
What are the Symptoms of Agammaglobulinemia?
During the first six month of a child ’s life it is the mother ’s antibodies which protect the child from any infection but after six month it is the child ’s own antibodies which protect the tike from infection and in cases of Agammaglobulinemia where there is insufficient product of antibodies this is the time when the symptom of Agammaglobulinemia go to airfoil but in some case there may be no symptoms even after the shaver is about four or five years of eld but that is quite rare . Some of the symptoms of Agammaglobulinemia are :
These symptoms may also take place in children who do not have Agammaglobulinemia but kid with Agammaglobulinemia get infected more oft than the children without this disease condition and the austereness of the symptoms may also be more in youngster with Agammaglobulinemia .
How is Agammaglobulinemia Diagnosed?
To make a diagnosis of Agammaglobulinemia , the pediatrist will take a detailed history of the family inquiring as to whether anyone else in the family have had the same symptom while develop up . A blood work will then be performed to await at the immune system of the consistency and look for the antibody levels in the body . There are many tests like flow cytometry , quantitative immunoglobulins , and immunoelectrophoresis that can quantitate whether he body is producing enough antibody required to fight infections and this will give clinching grounds to sustain the diagnosing of Agammaglobulinemia .
How is Agammaglobulinemia Treated?
The treatment for Agammaglobulinemia depends on various factor such the age of the child , the overall health of the child , and the severeness of the status . The most preferred treatment for Agammaglobulinemia is to replace the antibodies in the line of descent . This is called da Gamma globulin therapy and this will provide antibodies directly to the child ’s body through blood and prevent the kid from getting frequent infection or in other words plow Agammaglobulinemia . It should be noted here that children with Agammaglobulinemia should not get live vaccinations as the kid may end up have the disease for which inoculation were given instead of foreclose it . Also it is highly important that the child should be treated appropriately and emergently for any viral or bacterial infections so as to prevent worsening of the child ’s condition due to Agammaglobulinemia .
What is the Prognosis of Agammaglobulinemia?
The overall medical prognosis of a child with Agammaglobulinemia is quite practiced and most of the children go on to survive healthy lives and participate in all normal activities , although it is of import to make an accurate and early diagnosis and start contiguous treatment for Agammaglobulinemia . If left untreated , Agammaglobulinemia can prove to be life threatening as without antibodies the children will not be able to be protected from potentially serious infection due to Agammaglobulinemia .
