X-linked agammaglobulinemia (XLA), or Bruton agammaglobulinemia, is an inherited immunodeficiency disease caused by mutations in the gene coding for Bruton tyrosine kinase (BTK). BTK is critical to the maturation of pre?B cells to differentiating mature B cells. No curative therapy exists for X-linked agammaglobulinemia (XLA), or Bruton agammaglobulinemia.
Treatment of X-linked agammaglobulinemia
Treatment for XLA is IVIG. Typical doses are 400-600 mg/kg/mo given every 3-4 weeks. Doses and intervals can be adjusted based on individual clinical responses. Therapy should begin at age 10-12 weeks. Maintenance of an IgG trough level of 500-800 mg/dL is recommended. Therapy should be started at age 10-12 weeks. Currently, no evidence supports that one particular brand or route of administration (IV vs SC) is better than the other.
Major Research on X-linked agammaglobulinemia in United Kingdom
The research being carried out throughout the country can be stated as NIHR BioResource - Rare Disease: PID - Primary Immune Disorders, NIHR BioResource - Rare Disease: SPEED - Specialist Pathology: Evaluating Exomes in Diagnostics and NIPSIGEN - Clinical translation of non-invasive prenatal diagnosis .
Disease Statistics of X-linked agammaglobulinemia
The disease statistics can be observed as 60,270,708 of the total population are prone to the disease.