Immunoglobulin deficiency syndrome

Immunoglobulin deficiency syndromes: Description, Causes and Risk Factors:Immunoglobulin deficiency syndromes are a group of immunodeficiency disorders in which the patient has reduced number of Immunoglobulinor lack of antibodies.Immunoglobulins (Ig) are antibodies. There are five major classes of antibodies: IgG, IgM, IgA, IgD, and IgE.
  • IgG is the most abundant of the classes of immunoglobulins. It is the antibody for viruses, bacteria, and antitoxins. It is found in most tissues and plasma.
  • IgM is the first antibody present in an immune response.
  • IgA is an early antibody for bacteria and viruses. It is found in saliva, tears, and all other mucous secretions.
  • IgD's activity is unknown.
  • IgE is present in the respiratory secretions. It is an antibody for parasitic diseases, hay fever, and allergic asthma.
All antibodies are made by B-cells (an immunologically important lymphocyte that is not thymus-dependent, is of short life, and resembles the bursa-derived lymphocyte of birds in that it is responsible for the production of immunoglobulins, i.e., it is the precursor of the plasma cell and expresses immunoglobulins on its surface but does not release them. It does not play a direct role in cell-mediated immunity). Any disease that harms the development or function of these cells will cause a decrease in the amount of antibodies produced. Since antibodies are essential in fighting infectious diseases, people with immunoglobulin deficiency syndromes become ill more often. However, the cellular immune system is still functional, so these patients are more prone to infection caused by organisms usually controlled by antibodies.There are two types of immunodeficiency diseases: primary and secondary. Secondary disorders occur in normally healthy bodies that are suffering from an underlying disease. Once the disease is treated, the immunodeficiency is reversed.Immunoglobulin deficiency syndromes are primary immunodeficiency diseases occurring because of defective B lymphocytes or antibodies. They account for 50% of all primary immunodeficiencies, and they are, therefore, the most prevalent type of immunodeficiency disorders.Immunoglobulin deficiencies are the result of congenital defects affecting the development and function of B lymphocytes (B-cells). There are two main points in the development of B-cells when defects can occur. First, B-cells can fail to develop into antibody-producing cells. Second, B-cells can fail to make a particular type of antibody or fail to switch classes during maturation. Initially, when B-cells start making antibodies for the first time, they make IgM. As they mature and develop memory, they switch to one of the other four classes of antibodies. Failures in switching or failure to make a sub class of antibody leads to immunoglobulin deficiency diseases.Immunoglobulin heavy chain deletion is a genetic disease in which part of the antibody molecule is not produced. It results in the loss of several antibody classes and subclasses, including most IgG antibodies and all IgA and IgE antibodies. The disease occurs because part of the gene for the heavy chain has been lost.Symptoms:Symptoms are persistent and frequent infections, diarrhea, failure to thrive, and malabsorption (of nutrients).An immunodeficiency disease is suspected when children become ill frequently, especially from the same organisms. Laboratory tests are performed to verify the diagnosis. Antibodies can be found in the blood. Depending on the type of immunoglobulin deficiency the laboratory tests will show a decrease or absence of antibodies or specific antibody subclasses.Patients with immunoglobulin deficiencies are also prone to some viral infections, including echovirus, enterovirus, and hepatitis B. They may also have a bad reaction to the attenuated version of the polio virus vaccine.Diagnosis:An immunodeficiency disease is suspected when children become ill frequently, especially from the same organisms. The profile of organisms that cause infection in patients with immunoglobulin deficiency syndrome is unique and is preliminary evidence for this disease. Laboratory tests are performed to verify the diagnosis. Antibodies can be found in the blood. Blood is collected and analyzed for the content and types of antibodies present. Depending on the type of immunoglobulin deficiency the laboratory tests will show a decrease or absence of antibodies or specific antibody subclasses.Treatment:Immunodeficiency diseases can not be cured. Patients are treated with antibiotics and immune serum. Immune serum is a source of antibodies. Antibiotics are useful for fighting bacteria infections. There are some drugs that are effective against fungi, but very few drugs that are effective against viral diseases.Bone marrow transplantation can, in most cases, completely correct the immunodeficiency.Patients with immunoglobulin deficiency syndromes must practice impeccable health maintenance and care, paying particular attention to optimal dental care, in order to stay in good health.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care. 

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