CVID : diagnosis and prognosis

General principles of diagnosis, treatment, common variable immunodeficiency.

diagnosis

Suspected common variable immune deficiency (CVID) arises when examining children or adults with recurrent infections involving ears, sinuses, bronchi and lungs.

diagnosis is confirmed by the detection of low levels of immunoglobulins, including IgG, IgA and usually IgM.Patients completely immunized against polio, measles, diphtheria and tetanus antibodies to one or more of these vaccines may be very low or absent levels.

To determine the degree of immunity disorders performed immunization with other vaccines, such as pneumococcal.In some cases, these tests help the physician determine whether a patient will immunoglobulin replacement therapy.You can also determine the number of T-lymphocytes and their function tested in blood samples.Special laboratory techniques allow you to determine whether the produce B cells antibodies in vitro (tissue culture), and whether the normal function of T lymphocytes.

Genetics and Inheritance

Due to the fact that the genetic nature of Common Variable Immunodeficiency (CVID) is unclear, the exact mode of inheritance is not defined.In some cases, several family members is detected failure of one or more types of immunoglobulins.

For example, often a family member suffering from CVID, and the other has a selective immunoglobulin A deficiency over the past few years, an association of mutations in certain genes with CVID.For example, an autosomal recessive inheritance CVID was connected in one family with induced costimulation (ICOS), and in some families with a protein on B cells (CD19).Approximately 10% of CVID detected cell receptor mutations (TACI) two factors (BAFF or APRIL), necessary for normal growth and regulation of B cells.

not yet clear whether these mutations can cause impaired immunity, since some of them can be found in people with normal immunoglobulins.

Treatment Treatment of common variable immunodeficiency (CVID) is similar to the treatment of other disorders characterized by low levels of serum immunoglobulins.

In the absence of a significant breach of the T-lymphocytes, or organ damage immunoglobulin replacement therapy almost always reduces the severity of symptoms.Immunoglobulins are isolated from large amounts of human plasma.These preparations consist essentially of IgG and contain all important antibodies in healthy individuals are available.

Patients with chronic sinusitis or chronic lung disease may also need long-term treatment of broad-spectrum antibiotics.If you suspect an infection by microorganisms of the genera Mycoplasma and Chlamydia can be treated with antibiotics, which have a selective effect on these microorganisms.With the development of bronchiectasis
need physiotherapy and daily routines with the adoption of the provisions of the body to facilitate sputum from the lungs and bronchi.Patients with gastrointestinal symptoms or malabsorption should be evaluated for intestinal lamblia Giardia, rotavirus or some other infections of the gastrointestinal tract.Most patients with immunodeficiency and arthritis give favorable response to replacement therapy immunoglobulins.

forecast

Immunoglobulin replacement therapy combined with antibiotic therapy have greatly improved the prognosis for patients with common variable immunodeficiency (CVID).The goal of treatment - to protect the patient from infection and prevent chronic lung disease.

outlook for patients with CVID depends on the degree of damage to the lungs and other organs before diagnosis and the start of immunoglobulin replacement therapy, as well as the success of the introduction of immunoglobulins to prevent infections and antibiotics in the future.

Article courtesy of a worldwide organization IPOPI, working to improve the lives of people with primary immunodeficiency.
Copyright 2007 owned fund Immune Deficiency Foundation, USA."Guidelines for the primary immunodeficiency diseases for patients and their families", from which the material is taken under the license, was developed Immune Deficiency Foundation with support from Baxter Healthcare Corporation.

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