Common variable immunodeficiency: Description, Causes and Risk Factors:Abbreviation: CVID.Alternative Name: Acquired hypogammaglobulinemia, adult onset hypogammaglobulinemia.CVID is a group of rare genetic (primary) immunodeficiency disorders in which abnormalities in immune cell development (maturation) result in a decreased ability to appropriately produce antibodies in response to invading microorganisms, toxins, or other foreign substances. The symptoms of Common variable immunodeficiency usually become apparent during the second to the fourth decade of life.These disorders are caused by intrinsic or genetic defects in the immune system. Therefore, individuals who have primary immune deficiencies are born with the disorder.The disorder is characterized by a lack of antibody producing B-cells or plasma cells, low levels of most or all types of immunoglobulin, and recurrent bacterial infections.While the exact incidence of Common variable immunodeficiency is unknown, researchers estimate that about one out of 50,000 individuals develop the disorder.Although decreased serum levels of immunoglobulin G (IgG) and immunoglobulin A (IgA) are characteristic of the disorder, about 50% of CVID patients also have decreased immunoglobulin M (IgM) levels. In addition, about half of CVID patients experience some T-lymphocyte dysfunction.It is thought that Common variable immunodeficiency may result from a combination of genetic defects or from different disease genes (heterogenous). In many cases, there is no clear pattern of inheritance. However, in successive generations of some affected families (kindreds), there is evidence that CVID may be inherited as an autosomal recessive genetic trait. In addition, a rare acquired form of the disorder has been described in the medical literature. No clear pattern of inheritance has been observed. In most cases, there is no family history of immunodeficiency. However, when more than one family member is diagnosed with Common variable immunodeficiency, researchers believe it is the result of autosomal recessive inheritance.In some cases, individuals with CVID have an increased tendency to develop certain diseases characterized by abnormal tissue growths (neoplasms) that may be benign or malignant. In addition, some individuals with CVID may have an unusual susceptibility to certain autoimmune diseases. These disorders occur when the body's natural defenses against invading microorganisms mistakenly attack healthy tissue. The range and severity of symptoms and findings associated with CVID may vary from case to case.In general, the expected survival rate for male and female patients is 92% and 94%, respectively. Factors associated with fatality include low levels of IgG, poor T-cell responses to antigens, and a low percentage of B cells. Deaths related to Common variable immunodeficiency are usually the result of lymphoma. Other potential causes of death include right-sided heart failure secondary to chronic lung infection, liver failure, and malnutrition (resulting from gastrointestinal tract disease).Symptoms:The first signs of the deficiency are recurrent bacterial infections, which may occur as early as infancy or as late as the fourth decade of life. Common symptoms include recurrent infections of the ears, bronchi, sinuses, and lungs. Bronchiectasis (widening and scarring of the bronchial tubes) infections of the bronchi are severe. Patients with bronchiectasis may have a regular morning cough that produces yellow or green sputum. Many Common variable immunodeficiency patients have an enlarged spleen and lymph nodes. Other patients may develop painful inflammation of the knees, ankle, elbows, or wrist joints. Gastrointestinal symptoms may include abdominal pain, bloating, nausea, vomiting, diarrhea, or weight loss. Some patients develop autoantibodies, which are antibodies that mistakenly attack the body's tissues. Autoantibodies can destroy one or more types of body tissues, cause abnormal organ growth, or impair organ function. Autoantibodies commonly affect blood components (like red blood cells, connective tissues, and blood vessels), endocrine glands (like the thyroid or pancreas), as well as muscles, joints, and the skin.Diagnosis:A nephelometry blood test may be performed to diagnose CVID. The disorder is diagnosed after significantly decreased IgG and IgA levels are observed in the patient's blood. Some patients may also have decreased IgM levels. During the procedure, a sample of blood is taken from the patient. Anti-immunoglobulins are added to the blood sample. A medical instrument then measures the movement of particles in a substance that is caused by the interaction between immunoglobulins and anti-immunoglobulins. The test quickly and accurately measures the amount of IgM, IgG, and IgA in the patient's blood. Healthy individuals have 100-400 milligrams of IgA per deciliter of blood, 560-1,800 milligrams of IgG per deciliter of blood, and 45-250 milligrams of IgM per deciliter blood.Treatment:While there is currently no cure for Common variable immunodeficiency, various treatments may help relieve symptoms and resolve infections associated with the disease. IVIG (intravenous immunoglobulin) therapy is used most often to treat CVID patients.Immunoglobulin may be administered intravenously (injected into the vein) or subcutaneously (injected below the skin). Solutions of 3-12% intravenous immunoglobulin (IVIG) have been used on a regular basis to maintain a trough level of 400-500 milligrams/deciliter in adults. A dose of 400-600 milligrams/deciliter every two to four weeks is usually required. In patients with structural lung damage, a trough level of 700-800 milligrams/deciliter is generally required.The most common side effects of IVIG include backache, nausea, vomiting, chills, low-grade fever, myalgia (general feeling of discomfort), and fatigue. Adverse effects usually occur within 30 minutes of the infusion and typically last for several hours. Slowing the rate of infusion or interrupting the infusion for a few minutes can help prevent side effects. Adverse effects can be treated with antipyretics, diphenhydramine, or corticosteroids. Although anaphylactic reactions to immunoglobulin concentrates are rare, patients who have IgA deficiency have an increased risk for these effects. Long-term intravenous access is not recommended because it can increase the risk of infection.Patients with chronic sinusitis or lung disease may need long term treatment with broad spectrum antibiotics such as ampicillin (Principen®), tetracycline (Helidac Therapy®, Sumycin®, or Sumycin® Syrup), cephalexin (Biocef®, Keflex®, Keftab®, Panixine®, or Zartan®), trimethoprim/sulfmethoxazole (Bactrim® or Septra®), or ciprofloxacin (Cipro®).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.
A team of researchers from the universities of North Carolina and Emory in Atlanta moved one step closer to cure HIV forcing the AIDS-causing virus to reactivate meaning this virus becomes vulnerable to the immune system and medication. This is a significant...
A new pneumonia-like coronavirus from China emerged in the city of Wuhan with a population of around 11 million people. The US Centers for Disease Control and Prevention identified a seafood market as the center of the outbreak. The market was closed on the 1st of...
No Results Found
The page you requested could not be found. Try refining your search, or use the navigation above to locate the post.
When it is so hot outside you still can find hundreds of ways to cool yourself and drinking a mocktail is one of them. Here are few wonderful recipes for you to try. Kiwi Sour 1 oz orange juice 3 slices kiwi 0.75 oz demerara green tea syrup 0.75 oz lime juice 1 oz...
Many people are motivated for active sports in spring. However, before you buy a membership, you should check which exercises are useful to you and which ones should not be done in any way. Unfortunately, nowadays there are practically no people with an absolutely...
In childhood, many of us dreamed of learning to jump high. Now, after years, it became easier - Kangoo Jumps has appeared. This is one of the relatively new, but quickly gaining popularity types of fitness training. There are several advantages of jumpers. ...
Privacy & Cookies Policy
Necessary cookies are absolutely essential for the website to function properly. This category only includes cookies that ensures basic functionalities and security features of the website. These cookies do not store any personal information.
Any cookies that may not be particularly necessary for the website to function and is used specifically to collect user personal data via analytics, ads, other embedded contents are termed as non-necessary cookies. It is mandatory to procure user consent prior to running these cookies on your website.