Adenosine Deaminase Deficiency – Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs that work together to defend against germs. It helps your body to recognize these “foreign” invaders. Then its job is to keep them out, or if it can’t, to find and destroy them.

If your immune system cannot do its job, the results can be serious. Disorders of the immune system include

• Allergy and asthma – immune responses to substances that are usually not harmful
• Immune deficiency diseases – disorders in which the immune system is missing one or more of its parts
• Autoimmune diseases – diseases causing your immune system to attack your own body’s cells and tissues by mistake

NIH: National Institute of Allergy and Infectious Diseases

• Agammaglobulinemia (Medical Encyclopedia)
• Aging changes in immunity (Medical Encyclopedia)
• Chronic granulomatous disease (Medical Encyclopedia)
• Graft-versus-host disease (Medical Encyclopedia)
• Histiocytosis (Medical Encyclopedia)
• Hyperimmunoglobulin E syndrome (Medical Encyclopedia)
• Immune response (Medical Encyclopedia)
• Immunodeficiency disorders (Medical Encyclopedia)
• Selective deficiency of IgA (Medical Encyclopedia)

Adenosine deaminase (ADA) deficiency is an inherited disorder that damages the immune system and causes severe combined immunodeficiency (SCID). People with SCID lack virtually all immune protection from bacteria, viruses, and fungi. They are prone to repeated and persistent infections that can be very serious or life-threatening. These infections are often caused by “opportunistic” organisms that ordinarily do not cause illness in people with a normal immune system.The main symptoms of ADA deficiency are pneumonia, chronic diarrhea, and widespread skin rashes. Affected children also grow much more slowly than healthy children and some have developmental delay.Most individuals with ADA deficiency are diagnosed with SCID in the first 6 months of life. Without treatment, these babies usually do not survive past age 2. In about 10 percent to 15 percent of cases, onset of immune deficiency is delayed to between 6 and 24 months of age (delayed onset) or even until adulthood (late onset). Immune deficiency in these later-onset cases tends to be less severe, causing primarily recurrent upper respiratory and ear infections. Over time, affected individuals may develop chronic lung damage, malnutrition, and other health problems.