Selective IgA deficiency

Fatima Al-AwadhSelective IgA deficiencyOBJECTIVESDefine the selective IgA deficiency.Recognize the structure and function of IgA.Mention the causes, pathophysiology, signs & symptoms, complications, diagnosis, Treatment, Prognosis and Prevention.State the effect of vaccination on this disorder.Selective Iga deficiencythe complete absence of the IgA class of immunoglobulins in the blood serum and secretions.a relatively mild genetic immunodeficiency.IgA is a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract. It is the most common of the primary antibody deficiencies.IgA deficiency is believed to affect as many as 1 in 700 people, but in most of these persons it causes no clinical problems.structure

Function of IgA

Causes The defect causing these deficiencies is not known in a majority of cases; rarely, the deficiencies may be caused by mutations of Ig heavy chain constant region genes.Pathophysiology

B lymphocytes are unable to produce Ig APathophysiologySigns & Symptoms8590% of IgA-deficient individuals are asymptomatic. Some patients with IgA deficiency have a tendency to develop recurrent:sinopulmonary infectionsgastrointestinal infections and disordersOtitis mediaSkin infectionAllergiesComplicationsAn autoimmune disorder such as rheumatoid arthritis.severe, even life-threatening, reactions to transfusions of blood and blood products because they develop anti-IgA antibody.DiagnosisPatient & family historyFamily history of selective IgA deficiency.Either chronic or recurrent infections, allergies, auto-immune diseases, chronic diarrhea, or some combination of these problems.Quantitative immunoglobulinsrapid and accurate measurement of the amounts of the immunoglobulins M, G, and A.tests of blood serum demonstrate absence of IgA with normal levels of the other major classes of immunoglobulins (IgG and IgM).

NORMAL results of Ig M, G and A IgG560 to 1800 mg/dLIgM45 to 250 mg/dLIgA100 to 400 mg/dLSerum Immunoelectrophoresisdetect the presence of certain antibody.It is used to detect if the patient have anti-IgA antibodies.ManagementPrognosisPreventionThere is no means of prevention of Selective IgA Deficiency. Because IgA Deficiency does not become detectable until approximately six months of age, prenatal and neonatal detection of this disorder is currently not possible. Vaccination and IgA deficiencyContraindicated VaccinesRisk-specific recommended VaccinesEffective VaccinesOPV2 BCG Yellow fever Other live vaccines appear to be safe Pneumococcal All vaccines likely effective. Immune response might be attenuated.ReferancesBasic Immunology, Abbas 3th ed.http://www.nlm.nih.gov/medlineplus/ency/article/001476.htmhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2821513/http://primaryimmune.org/wp-content/uploads/2011/04/Selective-IgA-Deficiency.pdfhttp://www.cdc.gov/vaccines/pubs/pinkbook/downloads/appendices/A/immuno-table.pdf

Thank you