Tuesday, January 1, 2008
Selective IgA deficiency
An overview of the disease mean serum IgA below 0.05 g / L, and IgG and IgM content normal; It immune defect is the most common type. So in groups disease rate is about 1 / 700. The disease mostly distributed, some familial, and involving several generations. On six different parts of the national epidemiological survey, which showed that the disease ethnic and regional differences. Α heavy chain gene coding structure may not be a major cause of disease; B cell differentiation in the early formation of IgA mature B cells stagnation or IgA specific T cells by inhibiting activation, causing disruption mature B cells may be the cause of the disease. Clinical manifestations of most of the patients asymptomatic, and occasionally found in the inspection. Other cases often different levels of sinus and respiratory infections; In case of bronchial asthma generally heavier. Some patients with gastrointestinal symptoms, such as chronic diarrhea, malabsorption syndrome and atrophy of intestinal villi. Giardia infection flagellate's rare. Because of gastrointestinal and respiratory secretion not IgA, prone to allergic reactions. In addition, rheumatoid arthritis, systemic lupus erythematosus, thyroid Zhu Yan, such as pernicious anemia and immune disease free rate increase. Most treatment without treatment. For respiratory infections, gastrointestinal symptoms, allergies and autoimmune diseases should be treated accordingly. Γ globulin preparations with only trace IgA, it is not selective IgA alternative; In addition, IgA by the mucosal surface of local produce, a body may not be able to give IgA expected arrival of parts and the importation of high repeat allergic reaction. Therefore, generally do not have γ globulin treatment. Only in a serious infection and IgG2 accompanied by lack of resources, application of this preparation in order to be effective. Minorities IgA deficiency can be spontaneous remission. Auxiliary secretion and serum IgA levels decreased significantly; IgE and minority patients also reduce IgG2. As IgG2 can right some more carbohydrate antigen produced more antibodies, therefore these patients easily recurring sinus and lung infection, or even lead to obstructive pulmonary disease. However, the cycle of B cells generate IgA number is not small. The patient contact or IgA plasma γ globulin, will produce anti-IgA antibodies, If after further contact or blood transfusion would cause allergic reactions globulin. In addition, the patients will have anti-thyroglobulin, gastric parietal cells, smooth muscle, collagen and food antigen antibody. Anti - bovine serum albumin antibody-positive rate also increased, such as the detection of bovine serum IgA anti-mask the lack of IgA. Therefore, to switch to other anti-serum (rabbit) were measured, more reliable.
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