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AGAMMAGLOBULINEMIA: WHAT IS AGAMMAGLOBULINEMIA?

Read about agammaglobulinemia medical facts: what is the definition of agammaglobulinemia, frequency, mortality or morbidity, and related agammaglobulinemia diseases.

Definition: What is "Agammaglobulinemia"?

AGAMMAGLOBULINEMIA

Agammaglobulinemia is also called hypogammaglobulinemia. It is the most ordinary type of primary immunodeficiency which accounts to about 50% of all the cases. The three major kinds can be defined as: early onset, late onset & X-linked. It was only after more than 50 years since the clinical manifestation was first recorded in 1952 by a certain Bruton. He described that the defect on the molecule in XLA or X-linked agammaglobulinemia has been explicated. To honor Bruton, the responsible gene was named Btk which means Bruton tyrosine kinase. It is estimated that 90% of the patients with the early-onset kind of agammaglobulinemia & the lack of B cells have defects on their Btk genes. The late-onset type is often referred to as the CVID or Common Variable Immunodeficiency. The last type is the early-onset non-Bruton agammaglobuliemia with absent or just low serum immunoglobulin (or Ig). Most of the cases are representative of a very heterogenous group which includes Ig deficiency with an increase in immunoglobulin M or hyper-IgM syndrome. There are also some infants who have an initial reading of low Ig level which soon increases to the normal level. This is named as Transient Hypogammaglobulinemia of infancy. Currently, the production of defective antibodies & the low circulation of a number of B cells were descriptive of some female infants & some males who do not have any Btk defects. These records imply the participation of other genes.

Frequency

Agammaglobulinemia in the US occurs at about 1 in every 250,000 of the male population. Saudi Arabia manifests a rate of 250 in every 100,000 people. These individuals who have agammagloublinemia comprise the 16% of the total primary humoral immunodeficiency categories. Spain has reported that 1,079 cases have been registered between the dates of January 1980 & December 1995. 49 of these cases were recorded as XLA. Brazil is seen at 166 cases of patients who suffer from immunodeficiencies which have been diagnosed for over 15 years. 60.8% of the total number of cases for Brazil was primary humoral defects. Among these, XLA was the least common. South Africa came up with 56% or 52 out of the 93 cases are antibody deficiency predominated. Australia compares at 71% of all 500 cases. In Hongkong, the humoral defects were recorded at 50 out of 117 patients who were diagnosed with primary immunodeficiency.

Mortality or Morbidity

Patients of this disorder are at risk of recurring infections. Acute bacterial infections could result into pneumonia, meningitis or even the more fatal forms of bacterial infection. The chief causes of morbidity are sinusitis or chronic upper pulmonary disorder or the lower pulmonary disorder such as bronchiectasis.



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