Curr Allergy Asthma Rep. 2005 Jul. 2007 Mar. Patients with IgA deficiency and abnormalities of chromosome 18 may present with the following: In most cases, IgA-deficiency appears to occur in a sporadic fashion, but familial inheritance is described in a mode of autosomal-recessive or autosomal-dominant pattern with variable or incomplete expression. The clinical syndrome of specific antibody deficiency in children. [Medline]. P R Health Sci J. Agents that can cause IgA deficiency with such mechanisms include sulfasalazine, hydantoin, cyclosporine, gold, fenclofenac, sodium valproate, and captopril. Most upper and lower respiratory tract infections are caused by relatively less virulent microbes, or patients present without overt causative pathogens. Selective IgA Deficiency Low IgA can be caused by selective IgA deficiency, when only IgA levels are low but other antibodies (IgG, IgM, IgD, and IgE) are normal [ 25 ]. [Medline]. Bethesda, MD 20894. Pediatr Therapeut. Reduced IgA levels establish the diagnosis. Such individuals may have a partial IgG deficiency despite apparently normal IgG levels in the blood, and so should be investigated in an immunology clinic. Karaca NE, Gulez N, Aksu G, Azarsiz E, Kutukculer N. Does OM-85 BV prophylaxis trigger autoimmunity in IgA deficient children?. 2010 Jan. 30(1):10-6. As many as 85-90% of patients with immunoglobulin (Ig)A deficiency are asymptomatic. Prevention and treatment information (HHS). This was suggested in a report of 2 mothers with IgA deficiency whose infants had excessive T-cell suppressor function specific for IgA and IgA deficiency. J Clin Immunol. Transfus Med. Griese M, Walda M, Meuser M, Reinhardt D. Monatsschr Kinderheilkd. Murphy TK, Storch EA, Lewin AB et al. Pediatr Infect Dis J. [Medline]. Schatorjé EJ, de Jong E, van Hout RW, GarcÃa Vivas Y, de Vries E. J Clin Immunol. [29] However, laboratory measures of IgA-IgG subclass antibodies against pneumococcal antigens are not readily available. Serum bactericidal antibody response to serogroup C polysaccharide meningococcal vaccination in children with primary antibody deficiencies. Janzi M, Kull I, Sjoberg R, et al. Wang N, Shen N, Vyse TJ, et al. Pediatr Allergy Immunol. 2008. Ann Allergy Asthma Immunol. Although IgA deficiency (IgAD) is currently recognised as the most frequent immunodeficiency in humans,1 individuals with IgAD are largely considered to be healthy and when discovered are usually not investigated further or followed up.2 The rare occasion when IgAD is a cause for concern is when these individuals require blood or blood products, in which case current practice ⦠doi: 10.1002/jcla.22146. [Medline]. 2012:[Medline]. Gathmann B, Binder N, Ehl S, Kindle G. The European internet-based patient and research database for primary immunodeficiencies: update 2011. Additives (IVIg products containing sucrose are most often associated with renal dysfunction, acute renal failure, and osmotic nephrosis, particularly with preexisting risk factors [eg, history of renal insufficiency, diabetes mellitus, age >65 y, dehydration, sepsis, paraproteinemia, nephrotoxic drugs]), Kistler-Nitschmann fractionation; pH 4, nanofiltration, 6% solution: 10% sucrose, < 20 mg NaCl/g protein, Cohn-Oncley fractionation, PEG precipitation, ion-exchange chromatography, pasteurization, Cohn-Oncley cold ethanol fractionation, cation and anion exchange chromatography, solvent detergent treated, nanofiltration, low pH incubation, Cohn-Oncley fractionation, caprylate-chromatography purification, cloth and depth filtration, low pH incubation, Does not contain carbohydrate stabilizers (eg, sucrose, maltose), contains glycine, Solvent/detergent treatment targeted to enveloped viruses; virus filtration using Pall Ultipor to remove small viruses including nonenveloped viruses; low pH incubation, Contains sorbitol (40 mg/mL); do not administer if fructose intolerant, Cohn-Oncley fraction II/III; ultrafiltration; pasteurization, (Baxter Bioscience for the American Red Cross), Cohn-Oncley cold ethanol fractionation, followed by ultracentrafiltration and ion exchange chromatography; solvent detergent treated, 5% solution: 0.3% albumin, 2.25% glycine, 2% glucose, 9/24/10: Withdrawn from market because of unexplained reports of thromboembolic events, Cohn-Oncley fraction II/III; ultrafiltration; low pH incubation; S/D treatment pasteurization, (Swiss Red Cross for the American Red Cross), Kistler-Nitschmann fractionation; pH 4 incubation, trace pepsin, nanofiltration, Per gram of IgG: 1.67 g sucrose, < 20 mg NaCl, Cold ethanol fractionation, octanoic acid fractionation, and anion exchange chromatography; pH 4 incubation and depth filtration, L-proline (approximately 250 mmol/L) as stabilizer; trace sodium; does not contain carbohydrate stabilizers (eg, sucrose, maltose). Constantin C, Huber WD, Granditsch G, et al. Measurement of IgG subclass levels in children younger than 4 years may simply reflect maturational delay. Pediatr Allergy Immunol. Carr TF, Koterba AP, Chandra R, et al. Cunningham-Rundles C, Brandeis WE, Pudifin DJ, Day NK, Good RA. Clin Diagn Lab Immunol. J Allergy Clin Immunol. People with IgG deficiency are more likely to get infections. Am J Rhinol Allergy. Plebani A, Ugazio AG, Meini A, et al. Prevalence of selective immunoglobulin A deficiency in healthy Turkish school children. Cheng YK, Decker PA, O'Byrne MM, Weiler CR. Costa Carvalho et al (2005) also reported the absence of a positive association between unresponsiveness to unconjugated pneumococcal vaccines (for polysaccharide antigens [Pneumovax]) and IgA and/or IgG subclass deficiencies in children with recurrent sinusitis. 25(29):5308-14. Fusaro AE, Fahl K, Cardoso EC, et al. [Full Text]. During normal development, synthesis of IgG2 and IgG4 lags that of IgG1 and IgG3. Parker AR, Skold M, Ramsden DB, Ocejo-Vinyals JG, López-Hoyos M, Harding S. Lab Med. Anti-convulsants can cause hypogammaglobulinemia or selective IgA deficiency. The concept that antibodies against polysaccharide antigens are mainly IgG2 (with some IgG4) and that most antibodies against protein antigens are predominantly IgG1 and IgG3 does not appear to be universally valid. 29(1):73-7. 2010 Sep. 30(5):761-5. Authors have indicated a need to assess antibody responses to polysaccharide vaccines (eg, Pneumovax) in patients with bronchiectasis of unknown etiology. Epub:[Medline]. Any abnormal physical findings may indicate various diseases to which patients with selective IgA deficiency have an increased susceptibility. Share cases and questions with Physicians on Medscape consult. Isolated deficiency of IgG4 is associated with symptoms and signs which are similar to those associated with other IgG subclass deficiencies. /viewarticle/949438
Chow MA, Lebwohl B, Reilly NR, Green PH. This site needs JavaScript to work properly. Ann Allergy Asthma Immunol. 2012 Apr 2. Chest. Individuals with a selective IgA subclass deficiency are usually asymptomatic. Patients appear to be healthy between infections. One report indicated that as many as 20% of children with recurrent or chronic infections due to encapsulated bacteria have IgG subclass deficiency. [Medline]. Persistence of HBV-DNA in children with chronic hepatitis B who seroconverted to anti-HBs antibodies after interferon-alpha therapy: correlation with specific IgG subclass responses to HBsAg.
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