In severe cases of immunodeficiency with total absence of the immune system (termed severe combined immunodeficiency - SCID) symptoms are present early on after birth with diarrhea, failure to gain weight, skin rashes and respiratory infections such as pneumonia. In most cases however, primary immunodeficiency results from antibody deficiency disorders either global with no ability to make antibodies or more frequently, partial or selective immunodeficiency with the inability of the child to make antibodies against certain infections.
In Antibody Deficiency Disorders symptoms appear either in early childhood or recognized only later on during adulthood. Due to the frequent use of antibiotics, symptoms are not as severe or life threatening as one would expect in a case of a deficient immune system. Symptoms are usually the result of recurrent respiratory infections such as chronic sinus infection, chronic purulent ear infections, chronic sputum productive cough particularly when not explained by tobacco smoking, or history of recurrent pneumonia.
Occasionally, the presenting symptoms may include deep seated infections such as meningitis, deep abscesses or bone or joint infection. Many of these patients may have proven history of asthma and/or hay fever. The recognition of these conditions is crucial in trying to avoid potential life threatening infections. Once the condition is recognized treatment may include chronic prophylactic antibiotics and potentially intravenous immunoglobulin treatment as a replacement therapy. The vast majority of patients with antibody deficiency disorders will do well on such treatment.
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