Hypogammaglobulinemia – An Overview


All right, before we go any further, let’s start with the seemingly impossible task of pronouncing this condition: “hypo-gamma-globulin-emia.” Now that we have that part out of the way, we can talk a little bit more about this disorder.

Hypogammaglobulinemia, also known as Common Variable Immunodeficiency (CVID), is a disorder that falls into the category of the primary immunodeficiency diseases, meaning its main debilitating effect on the body is a weakening of the immune system that makes the individual susceptible to higher rates of infection and illness, among other symptoms. CVID is caused by a lack of B-lymphocytes, which results in low levels of immunoglobins (or antibodies), hence the effect on the immune system’s functionality.
Symptoms and Effects

Antibodies are a big deal. Think of them as the switchboard operator in your body’s line of defense against disease and infection. In addition to identifying foreign antigens, antibodies are also responsible for firing off the signals that tell your body which biological response if necessary to fight them off.
Because the disorder has a direct effect on antibody functions, those suffering from CVID tend to begin having bacterial infections when they are 7-9 months old. Infections that are typical of someone with CVID include respiratory illness, gastrointestinal disorders, autoimmune diseases, increased occurrence of malignancies and growth retardation.
Management and Treatment

Like many of the autoimmune disorders we have covered in the past, CVID is often treated with intravenous gammaglobulin (IVIg) every three to four weeks. Additionally, due to the constant threat of infections, antimicrobial therapies are usually weaved into a patient treatment plan along with any other antibiotics and physical therapies that help to treat illness and other physical ailments caused by the weakened immune system.

Because indications of CVID usually occur within the first year of an individual’s life, any sign of recurring bacterial infection is an indication that you should consult your pediatrician.

Sources:
Web MD – Hypogammaglobulinemia: http://emedicine.medscape.com/article/136471-overview
Mayo Clinic – Primary Immunodeficiency: http://www.mayoclinic.com/health/primary-immunodeficiency/DS01006
Ontario Association of Pediatrics – Hypogammaglobulinemia by Dr. Saul Greenberg: http://www.utoronto.ca/kids/Hypogam.htm

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