IgA deficiency

IgA deficiency

a selective lack of immunoglobulin A (IgA). The most common type of immunoglobulin deficiency, it occurs in about 1 in 400 individuals. IgA is a major antibody in the saliva and in the mucous membranes of the intestines and the bronchi. It protects against bacterial and viral infections. IgA deficiency is inheritied as an autosomal-dominant or autosomal-recessive trait and is associated with autoimmune abnormalities. It is common in patients with rheumatoid arthritis and in those with systemic lupus erythematosus. Many individuals with this deficiency have normal numbers of peripheral blood lymphocytes with IgA receptors and normal amounts of other immunoglobulins. Normality accompanied by IgA deficiency suggests that the B lymphocytes of the patient may not secrete IgA. In some patients with this deficiency, T cells seem to depress the synthesis of IgA.
observations Common symptoms are respiratory allergies associated with chronic sinopulmonary infection; GI diseases, such as celiac disease and regional enteritis; autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and chronic hepatitis; and malignant tumors, such as squamous cell carcinoma of the lungs, reticulum cell sarcoma, and thymoma. However, symptoms of IgA deficiency are often lacking in patients whose immune system may compensate for low IgA levels with extra amounts of IgM. The age of onset varies. Some children with IgA deficiency may begin to synthesize IgA spontaneously when a recurrent infection wanes and their condition improves. Diagnoses of IgA-deficient patients depend on the results of tests that commonly show normal IgE and IgM levels while IgA levels are below 5 mg/dL in serum. The cell-mediated immune response and circulating B cell levels often appear normal, although tests may indicate autoantibodies and antibodies against IgG, IgM, and cow's milk. T cell interferon production may be decreased in some patients with IgA deficiency, increasing the chances of infection.
interventions There is no known cure for selective IgA deficiency. Treatment usually involves efforts to control associated diseases, such as respiratory and GI infections.
nursing considerations The patient with IgA deficiency should not receive gamma globulin because associated sensitization may cause anaphylaxis during administration of blood products. If the patient requires a blood transfusion, the risk of any harmful reaction can be reduced by using washed red blood cells. Using the crossmatched blood of an IgA-deficient donor in such a transfusion is considered safer because it completely eliminates the risk of an adverse reaction. IgA deficiency is a lifelong condition, and patients with this disorder are commonly instructed to identify its symptoms and to seek treatment promptly when the symptoms appear.

IgA deficiency,

n a selective lack of immunoglobulin A, which constitutes the most common type of immunoglobulin deficiency, appearing in about 1 in 400 individuals. Immunoglobulin A is a major protein antibody in the saliva and the mucous membranes of the intestines and bronchi. It protects against bacterial and viral infections. IgA deficiency is common in patients with rheumatoid arthritis and in patients with systemic lupus erythematosus.
References in periodicals archive ?
4%) had low immunoglobulin levels; of these, 1 patient had pan-hypogammaglobulinemia and 3 had IgA deficiency.
10) Furthermore, IgG to deamidated gluten peptide (DGP) and IgG to tissue transglutaminase (TTG) are used in cases of IgA deficiency under the new guidelines for diagnosis of celiac disease.
36) In patients with IgA deficiency, results from several of the serologic tests used to diagnose celiac disease may be negative, putting a higher emphasis on histologic findings.
The authors noted that IgA deficiency should be excluded in the presence of low absorbance values in the tTG assay.
Kalha and Sellin, however, argue that CVID is the second most common PID, second to selective IgA deficiency (4).
Anyone with IgA deficiency (a blood condition that can skew screening results) should undergo a total serum IgA test.
Selective IgA deficiency in children with recurrent parotitis of childhood.
Other exclusion criteria include conditions that would con-traindicate the use of IVIG such as a prior serious IVIG infusion reaction, known IgA deficiency, intolerance of volume load, and nephrotic syndrome.
However, as these assays depend on the detection of IgA antibodies, they will obviously be negative in individuals with IgA deficiency.
An analysis of 349 positive antiendomysial antibody (EMA) tests over a 17-month period showed that 51% of patients had not received a concurrent serum IgA test to evaluate for IgA deficiency, and 10% of patients were overlooked for a follow-up intestinal biopsy, reported Kelly McGowan of the University of Calgary (Alta.