Abstract
Other than the physiologic hypogammaglobulinemia of infancy, 80% of the confirmed immunodeficiencies consist of four syndromes: transient hypogammaglobulinemia of infancy (THI), IgG subclass deficiency, partial antibody deficiency with impaired polysaccharide responsiveness (IPR), and selective IgA deficiency IgAD. None are life threatening, all can be readily managed, and many recover spontaneously. An exact incidence of these disorders is not known. A summary of immunodeficiency registries in four countries listed IgAD in 27.5% of the patients, IgG subclass deficiency in 4.8%, and THI in 2.3%. The 1999 US survey of primary immunodeficiencies conducted by the Immune Deficiency Foundation found that 17.5% of these patients had IgAD and 24% had IgG subclass deficiency, while THI and IPR were not listed. The Jeffrey Modell Foundation (2005) survey of their global centers in 2004 reported IgAD in 15.5%, subclass deficiencies in 8%, and THI in 2% of their patients.
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Keywords
- Primary Immunodeficiency
- Common Variable Immunodeficiency
- Antibody Deficiency
- Pneumococcal Polysaccharide Vaccine
- Subclass Deficiency
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References
al-Attas, R.A. and Rahi, A.H. (1998) Primary antibody deficiency in Arabs: first report from eastern Saudi Arabia. J. Clin. Immunol. 18, 368–371.
Ambrosino, D.M., Siber, G.R., Chilmonczyk, B.A., Jernberg, J.B. and Finberg, R.W. (1987) An immunodeficiency characterized by impaired antibody responses to polysaccharides. N. Engl. J. Med. 316, 790–793.
Blecher, T.E., Soothill, J.F., Voyce, M.A. and Walker, W.H. (1968) Antibody deficiency syndrome: a case with normal immunoglobulin levels. Clin. Exp. Immunol. 3, 47–56.
Blum, P.M., Hong, R. and Stiehm, E.R. (1982) Spontaneous recovery of selective IgA deficiency. Additional case reports and a review. Clin. Pediatr. 21, 77–80.
Buckley, R.H. (2002) Immunoglobulin G subclass deficiency: fact or fancy? Curr. Allergy Asthma Rep. 2, 356–360.
Castigli, E., Wilson, S.A., Garibyan, L., Rachid, R., Bonilla, F., Schneider, L. and Geha, R.S. (2005) TACI is mutant in common variable immunodeficiency and IgA deficiency. Nat. Genet. 37, 829–834.
Cunningham-Rundles, S. (2004) The effect of aging on mucosal host defense. J. Nutr. Health Aging 8, 20–25.
Dalal, I. and Roifman, C.H. (2006). Transient hypogammaglobulinemia of infancy, UpToDate. Available at http://www.uptodate.com.
Fudenberg, H.H. and Fudenberg, B.R. (1964) Antibody to hereditary human gamma-globulin (Gm) factor resulting from maternal-fetal incompatibility. Science 145, 170–171.
Gitlin, D. and Janeway, C.A. (1956) Agammaglobulinemia, congenital, acquired and transient forms. Prog. Hematol. 1, 318–329.
Granoff, D.M., Shackelford, P.G., Pandey, J.P. and Boies, E.G. (1986a) Antibody responses to Haemophilus influenzae type b polysaccharide vaccine in relation to Km(1) and G2m(23) immunoglobulin allotypes. J. Infect. Dis. 154, 257–264.
Granoff, D.M., Shackelford, P.G., Suarez, B.K., Nahm, M.H., Cates, K.L., Murphy, T.V., Karasic, R., Osterholm, M.T., Pandey, J.P. and Daum, R.S. (1986b) Hemophilus influenzae type B disease in children vaccinated with type B polysaccharide vaccine. N. Engl. J. Med. 315, 1584–1590.
Herrod, H.G. (1993) Management of the patient with IgG subclass deficiency and/or selective antibody deficiency. Ann. Allergy 70, 3–8.
Hostoffer, R. (2006). IgA deficiency, UpToDate. Available at http://www.uptodate.com.
Kalfa, V.C., Roberts, R.L. and Stiehm, E.R. (2003) The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency. Ann. Allergy Asthma Immunol. 90, 259–264.
Kanoh, T., Mizumoto, T., Yasuda, N., Koya, M., Ohno, Y., Uchino, H., Yoshimura, K., Ohkubo, Y. and Yamaguchi, H. (1986) Selective IgA deficiency in Japanese blood donors: frequency and statistical analysis. Vox Sang. 50, 81–86.
Koistinen, J. (1976) Familial clustering of selective IgA deficiency. Vox. Sang 30, 181–190.
Kowalczyk, D., Mytar, B. and Zembala, M. (1997) Cytokine production in transient hypogammaglobulinemia and isolated IgA deficiency. J. Allergy Clin. Immunol. 100, 556–562.
Lakhanpal, S., O’Duffy, J.D., Homburger, H.A. and Moore, S.B. (1988) Evidence for linkage of IgA deficiency with the major histocompatibility complex. Mayo Clin. Proc. 63, 461–465.
Lefranc, G., Chaabani, H., Van Loghem, E., Lefranc, M.P., De Lange, G. and Helal, A.N. (1983) Simultaneous absence of the human IgG1, IgG2, IgG4 and IgA1 subclasses: immunological and immunogenetical considerations. Eur. J. Immunol. 13, 240–244.
Lemmon, J.K. and Knutsen, A.P. (2006). Clinical manifestations, diagnosis and treatment of IgG subclass deficiency, UpToDate. Available at http://www.uptodate.com.
Migone, N., Oliviero, S., de Lange, G., Delacroix, D.L., Boschis, D., Altruda, F., Silengo, L., DeMarchi, M. and Carbonara, A.O. (1984) Multiple gene deletions within the human immunoglobulin heavy-chain cluster. Proc. Natl. Acad. Sci. U.S.A. 81, 5811–5815.
Nathenson, G. (1971) Development of Gm antibodies following injection of anti-Rh gamma globulin. Transfusion 11, 302–306.
Oxelius, V.A., Laurell, A.B., Lindquist, B., Golebiowska, H., Axelsson, U., Bjorkander, J. and Hanson, L.A. (1981) IgG subclasses in selective IgA deficiency: importance of IgG2-IgA deficiency. N. Engl. J. Med. 304, 1476–1477.
Rockey, J.H., Hanson, L.A., Heremans, J.F. and Kunkel, H.G. (1964) Beta-2a Aglobulinemia in two healthy men. J. Lab. Clin. Med. 63, 205–212.
Roifman, C.M. (2004). Immunodeficiency disorders: general considerations. In: E.R. Stiehm, H.D. Ochs and J.A. Winkelstein (Eds),Immunologic Disorders in Infants and Children. Philadelphia, Elsevier: 391–393.
Saxon, A., Kobayashi, R.H., Stevens, R.H., Singer, A.D., Stiehm, E.R. and Siegel, S.C. (1980) In vitro analysis of humoral immunity in antibody deficiency with normal immunoglobulins. Clin. Immunol. Immunopathol. 17, 235–244.
Schur, P.H., Borel, H., Gelfand, E.W., Alper, C.A. and Rosen, F.S. (1970) Selective gamma-g globulin deficiencies in patients with recurrent pyogenic infections. N. Engl. J. Med. 283, 631–634.
Siegel, R.L., Issekutz, T., Schwaber, J., Rosen, F.S. and Geha, R.S. (1981) Deficiency of T helper cells in transient hypogammaglobulinemia of infancy. N. Engl. J. Med. 305, 1307–1313.
Sorensen, R.U. and Paris, K. (2006). Selective antibody deficiency with normal immunoglobulins (polysaccharide non-responses), UpToDate. Available at http://www.uptodate.com.
Stiehm, E.R., Ochs, H.D. and Winkelstein, J.A. (2004a). IgG subclass deficiencies. In: E.R. Stiehm, H.D. Ochs and J.A. Winkelstein (Eds), Immunologic Disorders in Infants and Children. Philadelphia, Elsevier: 393–398.
Stiehm, E.R., Ochs, H.D. and Winkelstein, J.A. (Eds)(2004b). Immunodeficiency disorders: general considerations. In Immunologic Disorders in Infants and Children. Philadelphia, Elsevier: 289–355.
Stiehm, E.R., Ochs, H.D. and Winkelstein, J.A. (Eds)(2004). Impaired polysacchride responsiveness (selective antibody deficiency). In Immunologic Disorders in Infants and Children. Philadelphia, Elsevier: 398–401.
West, C.D., Hong, R. and Holland, N.H. (1962) Immunoglobulin levels from the newborn period to adulthood and in immunoglobulin deficiency states. J. Clin. Invest. 41, 2054–2064.
Whelan, M.A., Hwan, W.H., Beausoleil, J., Hauck, W.W. and McGeady, S.J. (2006) Infants presenting with recurrent infections and low immunoglobulins: characteristics and analysis of normalization. J. Clin. Immunol. 26, 7–11.
Wood, P.M., Mayne, A., Joyce, H., Smith, C.I., Granoff, D.M. and Kumararatne, D.S. (2001) A mutation in Bruton’s tyrosine kinase as a cause of selective anti-polysaccharide antibody deficiency. J. Pediatr. 139, 148–151.
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Stiehm, R.E. (2007). The Four Most Common Pediatric Immunodeficiencies. In: Shurin, M.R., Smolkin, Y.S. (eds) Immune-Mediated Diseases. Advances in Experimental Medicine and Biology, vol 601. Springer, New York, NY. https://doi.org/10.1007/978-0-387-72005-0_2
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DOI: https://doi.org/10.1007/978-0-387-72005-0_2
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