Igα deficiency is characterised by early onset recurrent bacterial infections of lower respiratory tract and otitis media, profound hypoglammaglobulinemia, and a lack of circulating mature
B cells. One patient developed recurrent diarrhea and failure to thrive in the first month of life.
Diagnosis
Diagnostic recommendations
Additional Information
Diagnostic laboratories
Clinical:
Genetic:
Therapeutic options
- (Intravenous) immunoglobulins and antibiotic therapy. Oral poliovaccine should not be given because of the risk of paralytic disease.
- Agammaglobulinemia, eMedicine
Research programs, clinical trials
- Improved Healthcare for Patients with Primary Antibody Deficiencies through new Strategies Elucidating their Pathophysiology (IMPAD), IMPAD
- European Initiative for Primary Immunodeficiencies
- Immune Regulation in Patients with Common Variable Immunodeficiency and Related Syndromes, ClinicalTrials.gov