Selective immunoglobulin A deficiency

People with this deficiency lack immunoglobulin A (IgA), a type of antibody that protects against infections of the mucous membranes lining the mouth, airways, and digestive tract.85–90% of IgA-deficient individuals are asymptomatic, although the reason for lack of symptoms is relatively unknown and continues to be a topic of interest and controversy.Patients have an increased susceptibility to pneumonia and recurrent episodes of other respiratory infections and a higher risk of developing autoimmune diseases in middle age.[3] IgA deficiency and common variable immunodeficiency (CVID) feature similar B cell differentiation arrests,[4] but it does not present the same lymphocyte subpopulation abnormalities.[citation needed] Types include: When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level in the blood.[18] As opposed to the related condition CVID, selective IgA deficiency is not associated with an increased risk of cancer.

SpecialtyImmunologyimmunodeficiencyhypogammaglobulinemiaimmunoglobulin Aantibodyanaphylaxisblood transfusionsintravenous immunoglobulinpneumoniarespiratory infectionsautoimmune diseasescommon variable immunodeficiencyB celldifferentiationlymphocytechromosomescongenital intrauterine infectionsphenotypeTNFRSF13Bautoimmune diseaseceliac diseasecancerdiabetesB cell deficiencyClinical and Experimental ImmunologyBibcodeOnline Mendelian Inheritance in ManAmerican Academy of Allergy, Asthma, and ImmunologyDiseasesDBMedlinePluseMedicineLymphoidcomplementPrimaryhumoralX-linked agammaglobulinemiaTransient hypogammaglobulinemia of infancyDysgammaglobulinemiaIgG deficiencyIgM deficiencyHyper IgM syndromeWiskott–Aldrich syndromeHyper-IgE syndromeICF syndromeT cell deficiencythymic hypoplasiaDi George's syndromeNezelof syndromeAtaxia–telangiectasiaPurine nucleoside phosphorylase deficiencySeverecombinedX-SCIDAdenosine deaminase deficiencyOmenn syndromeZAP70 deficiencyBare lymphocyte syndromeHIV/AIDSLeukopeniaLymphocytopeniaIdiopathic CD4+ lymphocytopeniaComplementdeficiencyC1-inhibitorAngioedemaHereditary angioedemaComplement 2 deficiencyComplement 4 deficiencyMBL deficiencyProperdin deficiencyComplement 3 deficiencyTerminal complement pathway deficiencyParoxysmal nocturnal hemoglobinuriaComplement receptor deficiencyCell surface receptorG protein-coupled receptorhormoneCongenital hypothyroidism 1Luteinizing hormone insensitivityLeydig cell hypoplasiaMale-limited precocious pubertyFollicle-stimulating hormone insensitivityXX gonadal dysgenesisGonadotropin-releasing hormone insensitivityABCD syndromeWaardenburg syndrome 4aHirschsprung's disease 2Nephrogenic diabetes insipidus 1PTGER2Aspirin-exacerbated respiratory diseaseJansen's metaphyseal chondrodysplasiaFamilial hypocalciuric hypercalcemiaFamilial exudative vitreoretinopathy 1Enzyme-linked receptorgrowth factorRobinow syndromePfeiffer syndromeKAL2 Kallmann syndromeApert syndromeAntley–Bixler syndromeCrouzon syndromeJackson–Weiss syndromeAchondroplasiaHypochondroplasiaThanatophoric dysplasiaMuenke syndromeDonohue syndromeRabson–Mendenhall syndromeCongenital insensitivity to pain with anhidrosisKIT PiebaldismGastrointestinal stromal tumorPersistent Müllerian duct syndrome IITGF beta receptorsEndoglinHereditary hemorrhagic telangiectasiaTGFBR1TGFBR2Loeys–Dietz syndromeGUCY2DLeber's congenital amaurosis 1JAK-STATType I cytokine receptorLaron syndromeCSF2RASurfactant metabolism dysfunction 4Congenital amegakaryocytic thrombocytopeniaTNF receptorTNFRSF1ATNF receptor associated periodic syndromeTNFRSF5Hyper-IgM syndrome type 3TNFRSF13CTNFRSF6Autoimmune lymphoproliferative syndrome 1ADonnai–Barrow syndromeCenani–Lenz syndactylismWorth syndromeFamilial exudative vitreoretinopathy 4Osteopetrosis 1LDLR Familial hypercholesterolemiaImmunoglobulin superfamilyAGM3, 6IntegrinGlanzmann's thrombastheniaJunctional epidermolysis bullosa with pyloric atresiaEDAR hypohidrotic ectodermal dysplasiaNevoid basal-cell carcinoma syndromeBMPR1ABMPR1A juvenile polyposis syndromeX-linked severe combined immunodeficiency