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 ?
Charts were reviewed for certain comorbid conditions such as autoimmune thyroiditis, insulin dependent diabetes mellitus, Down's syndrome, epilepsy and IgA deficiency for two categories of CD.
Celiac disease is frequently associated with dermatitis herpetiformis, Down syndrome, selective IgA deficiency, and other conditions with autoimmune features such as type 1 diabetes mellitus, thyroid disease, and liver disease.
Keywords: Sensorineural hearing loss, primary immunodeficiency, pediatric, selective IgA deficiency
The subjects whose file records included deficient data (dTG-IgA and serum IgA levels could not be found in the file records of 28 subjects), who received a diagnosis of subclinical hypothyroidism (four subjects), who had selective IgA deficiency (one subject) and/or partial IgA deficiency (one subject) were excluded from the study.
Anti-pneumococcal polysaccharide antibodies of the IgG1 and IgG3 subclasses occur in persons who have IgA deficiency [29] or heavy chain constant region deletions of the [gamma]2 gene [30,31].
14 It is suggested that IgA deficiency has been associated with inheritance of C4B null allele (no C4B protein is produced).
You also want to do a total serum IgA to rule out IgA deficiency.
Screening is recommended for children with symptoms compatible with gluten-related disorders, children with close family members diagnosed with celiac disease, and children and youth with conditions known to be associated with celiac disease, such as type 1 diabetes, autoimmune thyroiditis, Down syndrome, dermatitis herpetiformis, psoriasis, and IgA deficiency (Tack et al.
The most common primary immunodeficiency disorders include selective IgA deficiency --which affects 1 in 500 children--Ig[G.
4%) had low immunoglobulin levels; of these, 1 patient had pan-hypogammaglobulinemia and 3 had IgA deficiency.
IgA deficiency is much more common in patients with CD than in the general population and can result in a false negative test for tTG and EMA.
Franco et al23 studied the association between IgA deficiency and respiratory atopy in young male adults.