![]() Infections caused by mycoplasma bacteria can lead to severe arthritis including joint swelling and pain, in children with primary agammaglobulinemia. (For more information on this disorder, choose “dermatomyositis” as your search term in the Rare Disease Database.) Areas of patchy, reddish skin may appear around the eyes, knuckles and elbows and occasionally on the knees and ankles. These symptoms may include muscle weakness, often in the hip and shoulder areas, and difficulty swallowing. ![]() Echovirus infection can cause a group of symptoms that closely resembles dermatomyositis. Infections by almost any of the enterovirus family and the poliomyelitis virus can result in unusually severe illness in children with agammaglobulinemia. Males with X-linked primary agammaglobulinemia usually begin to show signs of such infections only late in the first year of life, after the IgG antibodies from the mother have been depleted. These lymphocytes govern the production of antibodies. The major symptoms of agammaglobulinemia are serial bacterial infections resulting from failures in specific immune responses because of defects in B-lymphocytes. All of these disorders are characterized by a weakened immune system that must be strengthened by the administration of gammaglobulin in order to fight off infections. The types of agammaglobulinemia are: X-linked agammaglobulinemia (XLA), the much rarer X-linked agammaglobulinemia with growth hormone deficiency (about 10 cases reported), and autosomal recessive agammaglobulinemia (ARAG). The specialized precursor cells that produce gammaglobulins, fail to develop or function properly leading to the deficiency in the number of mature lymphocyte cells called B cells. They are essential if the immune system is to do its job of fighting off bacteria, viruses, and other foreign substances that threaten the body. Antibodies are proteins (immunoglobulins, (IgM), (IgG) etc) that are critical and key components of the immune system. Stay Informed With NORD’s Email NewsletterĪgammaglobulinemia is a group of inherited immune deficiencies characterized by a low concentration of antibodies in the blood due to the lack of particular lymphocytes in the blood and lymph.Find a Rare Disease Patient Organization.Rare Disease Cures Accelerator (RDCA-DAP). ![]()
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