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Selective immunoglobulin A (IgA) deficiency (SIgAD [1]) is a kind of immunodeficiency, a type of hypogammaglobulinemia.People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract.
The most common such immunodeficiency is inherited selective IgA deficiency, occurring between 1 in 100 and 1 in 1000 persons, depending on population. They are associated with increased vulnerability to infection, but can be difficult to detect (or asymptomatic) in the absence of infection.
B cells low but present or normal, but with reduction in 2 or more isotypes (usually IgG & IgA, sometimes IgM): common variable immunodeficiency (CVID), CD19 deficiency, TACI (TNFRSF13B) deficiency, BAFF receptor deficiency. Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes
Selective immunoglobulin A deficiency, Selective IgE deficiency disease, IgG deficiency, and Isolated primary immunoglobulin M deficiency. Dysgammaglobulinemia is a type of immune disorder characterized by a reduction in some types of gamma globulins , resulting in heightened susceptibility to some infectious diseases where primary immunity is ...
279.01 Selective IgA immunodeficiency; 279.02 Selective IgM immunodeficiency; 279.03 Other selective immunoglobulin deficiencies; 279.04 Congenital hypogammaglobulinemia; 279.05 Immunodeficiency with increased IgM; 279.06 Common variable immunodeficiency; 279.09 Other deficiency of humoral immunity; 279.1 Deficiency of cell-mediated immunity ...
Decreased or absent IgA due to an inherited inability to produce IgA is termed selective IgA deficiency and can produce a clinically significant immunodeficiency. [20] Anti-IgA antibodies, sometimes present in individuals with low or absent IgA, can result in serious anaphylactic reactions when transfused with blood products that incidentally ...
IgA deficiency is an exception, and is present in 1 in 500 people. Some of the more frequently seen forms of PID include common variable immunodeficiency, severe combined immunodeficiency, X-linked agammaglobulinemia, Wiskott–Aldrich syndrome, DiGeorge syndrome, ataxia–telangiectasia, [11]
IgG deficiency is often found in children as transient hypogammaglobulinemia of infancy, which may occur with or without additional decreases in IgA or IgM. IgG subclass deficiencies are also an integral component of other well-known primary immunodeficiency diseases, such as Wiskott–Aldrich syndrome and ataxia–telangiectasia.
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