Immunoglobulin A Deficiency Follow-up

Updated: Nov 14, 2016
  • Author: Marina Y Dolina, MD; Chief Editor: Michael A Kaliner, MD  more...
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Follow-up

Deterrence/Prevention

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  • Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued.
  • Secondary prevention relies primarily on vaccination to increase specific IgG and IgM (see Medication). In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi.
  • Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed).
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Complications

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  • Severe anaphylactic reactions to blood products
  • Recurrent sinopulmonary infections
  • Chronic diarrhea
  • Severe otitis media resulting in hearing loss; case reports of deaths
  • Malabsorption syndrome
  • Growth retardation secondary to malabsorption and chronic infection
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Prognosis

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  • In children aged 6 months to 4 years, IgAD may be transient and resolve permanently by age 5 years; in others, the syndrome may progresses to common variable immunodeficiency (CVID).
  • Adults with selective immunoglobulin A deficiency (SIgAD) are often asymptomatic; however, up to 90% have frequent bacterial respiratory tract infections.
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Patient Education

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  • Educate patients to recognize early signs of respiratory tract infections, such as increased phlegm, discolored phlegm, cough, or dyspnea.
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