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Author: Brian Wu PhD. MD Candidate, Keck School of Medicine, Los Angeles, USA; Chief Editor: Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, September 2015.
A patient with a specific antibody deficiency cannot produce IgG molecules to the polysaccharides in encapsulated bacteria (such as Streptococcus pneumoniae, Staphylococcus aureus, and Haemophilus influenzae). This makes the patient vulnerable to recurrent bacterial lung infections (pneumonia), sinus infections, ear infections (otitis media), and others.
Specific antibody deficiency is also called selective antibody deficiency, partial antibody deficiency, and impaired polysaccharide responsiveness.
Specific antibody deficiency is usually diagnosed in preschool children and occurs in both girls and boys of all races.
The exact cause of specific antibody deficiency is not known but it is likely due to a genetic mutation. It may be due to a breakdown in communication between B cells and other cells in the immune system.
Some patients with specific antibody deficiency are asymptomatic because other components of their immune system are still functional. Other patients may present with:
The diagnosis of specific antibody deficiency is based on:
Treatment for specific antibody deficiency centres on:
Primary goals of treatment are to prevent bronchiectasis and scarring in the lungs from repeated respiratory infections and to maintain the overall quality of life.
The prognosis for patients with specific antibody deficiency is generally reasonable. Children have been known to outgrow specific antibody deficiency naturally and those who do not are still able to maintain a good quality of life with antibiotic and immunoglobulin therapy.
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