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Selective Antibody Deficiency With Normal Immunoglobulins

by James Fernandez, MD, PhD

Selective antibody deficiency with normal immunoglobulins is characterized by an inadequate response to certain types of vaccines but not to others, even though people have normal or near normal levels of antibodies (immunoglobulins).

Selective antibody deficiency with normal immunoglobulins is one of the most common immunodeficiencies that causes frequent sinus and lung infections. People with this disorder have an inadequate immune response to certain vaccines (called polysaccharide vaccines), such as the pneumococcal vaccine.

Affected people have many sinus and lung infections and sometimes symptoms of allergies, such as a chronic runny and stuffy nose (rhinitis), a rash, and asthma. The severity of the disorder varies. Some children have a form of the disorder that resolves on its own over time.

Diagnosis

Children are not tested for this disorder until after age 2 years because younger children, including those without an immunodeficiency disorder, may have frequent sinus and lung infections and weak responses to certain vaccines. Testing consists of blood tests to measure levels of immunoglobulins and to evaluate how well the body produces immunoglobulins in response to vaccines. Normal levels of antibodies and an inadequate response to certain vaccines confirms the diagnosis.

Treatment

Children are vaccinated with the pneumococcal conjugate vaccine to prevent pneumococcal infections. Sinus and lung infections and allergy symptoms are treated. Occasionally, infections continue after treatment. Then, antibiotics (such as amoxicillin and trimethoprim/sulfamethoxazole) can be given. Rarely, when infections recur frequently despite use of these antibiotics, people are given immune globulin (antibodies obtained from the blood of people with a normal immune system).

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