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Grand Rounds |

Primary Immunodeficiency Diseases in Adults

Scott H. Sicherer, MD; Jerry A. Winkelstein, MD
JAMA. 1998;279(1):58-61. doi:10.1001/jama.279.1.58.
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Rosen FS, Cooper MD, Wedgwood RJP.The primary immunodeficiencies. N Engl J Med.1995;333:431-440.
Hermans PE, Diaz-Buxo JA, Stobo JD.Idiopathic late-onset immunoglobulin deficiency: clinical observations in 50 patients. Am J Med.1976;61:221-237.
Hausser C, Virelizier JL, Buriot D, Griscelli C.Common variable hypogamma-globulinemia in children: clinical and immunologic observations in 30 patients. AJDC.1983;137:833-837.
Cunningham-Rundles C.Clinical and immunologic analyses of 103 patients with common variable immunodeficiency. J Clin Immunol.1989;9:22-33.
Hermaszewski RA, Webster AD.Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. QJM.1993;86:31-42.
Sneller MC, Strober W, Eisenstein E, Jaffe JS, Cunningham-Rundles C.NIH conference: new insights into common variable immunodeficiency. Ann Intern Med.1993;118:720-730.
McKinney Jr RE, Katz SL, Wilfert CM.Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. Rev Infect Dis.1987;9:334-356.
Conley ME, Park CL, Douglas SD.Childhood common variable immunodeficiency with autoimmune disease. J Pediatr.1986;108:915-922.
Ament ME, Ochs HD, Davis SD.Structure and function of the gastrointestinal tract in primary immunodeficiency syndromes: a study of 39 patients. Medicine.1973;52:227-248.
Fasano MB, Sullivan KE, Sarpong SB.  et al. Sarcoidosis and common variable immunodeficiency: report of 8 cases and review of the literature. Medicine (Baltimore).1996;75:251-261.
Wright JJ, Wagner DK, Blaese RM, Hagengruber C, Waldmann TA, Fleisher TA.Characterization of common variable immunodeficiency: identification of a subset of patients with distinctive immunophenotypic and clinical features. Blood.1990;76:2046-2051.
Kornfeld SJ, Good RA, Litman GW.Atypical X-linked agammaglobulinemia. N Engl J Med.1994;331:949-951.
Ashman RF, Schaffer FM, Kemp JD.  et al. Genetic and immunologic analysis of a family containing five patients with common-variable immune deficiency or selective IgA deficiency. J Clin Immunol.1992;12:406-414.
Schaffer FM, Palermos J, Zhu ZB, Barger BO, Cooper MD, Volanakis JE.Individuals with IgA deficiency and common variable immunodeficiency share polymorphisms of major histocompatibility complex class III genes. Proc Natl Acad Sci U S A.1989;86:8015-8019.
Olerup O, Smith CI, Bjorkander J, Hammarstrom L.Shared HLA class II-associated genetic susceptibility and resistance, related to the HLA-DQB1 gene, in IgA deficiency and common variable immunodeficiency. Proc Natl Acad Sci U S A.1992;89:10653-10657.
Vorechovsky I, Zetterquist H, Paganelli R.  et al. Family and linkage study of selective IgA deficiency and common variable immunodeficiency. Clin Immunol Immunopathol.1995;77:185-192.
Roifman CM, Lederman HM, Lavi S, Stein LD, Levison H, Gelfand EW.Benefit of intravenous IgG replacement in hypogammaglobulinemic patients with chronic sinopulmonary disease. Am J Med.1985;79:171-174.
Sweinberg SK, Wodell RA, Grodofsky MP, Greene JM, Conley ME.Retrospective analysis of the incidence of pulmonary disease in hypogammaglobulinemia. J Allergy Clin Immunol.1991;88:96-104.
Cunningham-Rundles C, Siegal FP, Cunningham-Rundles S, Lieberman P.Incidence of cancer in 98 patients with common varied immunodeficiency. J Clin Immunol.1987;7:294-299.
French MA, Denis KA, Dawkins R, Peter JB.Severity of infections in IgA deficiency: correlation with decreased serum antibodies to pneumococcal polysaccharides and decreased serum IgG2 and/or IgG4. Clin Exp Immunol.1995;100:47-53.
Hanson LA, Soderstrom R, Nilssen DE.  et al. IgG subclass deficiency with or without IgA deficiency. Clin Immunol Immunopathol.1991;61:S70-S77.
Figueroa JE, Densen P.Infectious diseases associated with complement deficiencies. Clin Microbiol Rev.1991;4:359-395.
Hershfield MS, Mitchell BS.Immunodeficiency diseases caused by adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency.In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease . 7th ed. New York, NY: McGraw-Hill; 1995;2:1725-1768.
Shovlin CL, Simmonds HA, Fairbanks LD.  et al. Adult onset immunodeficiency caused by inherited adenosine deaminase deficiency. J Immunol.1994;153:2331-2339.
Ozsahin H, Arredondo-Vega FX, Santisteban I.  et al. Adenosine deaminase deficiency in adults. Blood.1997;89:2849-2855.
Sullivan KE, Mullen CA, Blaese RM, Winkelstein JA.A multiinstitutional survey of the Wiskott-Aldrich syndrome. J Pediatr.1994;125:876-885.
Lederman HM, Winkelstein JA.X-linked agammaglobulinemia: an analysis of 96 patients. Medicine (Baltimore).1985;64:145-156.
Schapiro BL, Newburger PE, Klempner MS, Dinauer MC.Chronic granulomatous disease presenting in a 69-year-old man. N Engl J Med.1991;325:1786-1790.
Good RA.Historical aspects of immunologic deficiency diseases.In: Kagan BM, Stiehm ER, eds. Immunologic Incompetence . Chicago, Ill: Year Book; 1971:149-177.
Fischer A, Landais P, Friedrich W.  et al. European experience of bone-marrow transplantation for severe combined immunodeficiency. Lancet.1990;336:850-854.
Hershfield MS.PEG-ADA replacement therapy for adenosine deaminase deficiency: an update after 8.5 years. Clin Immunol Immunopathol.1995;76:S228-S232.
Bordignon C, Notarangelo LD, Nobili N.  et al. Gene therapy in peripheral blood lymphocytes and bone marrow for ADA-immunodeficient patients. Science.1995;270:470-475.
Blaese RM, Culver KW, Miller AD.  et al. T lymphocyte-directed gene therapy for ADA-SCID: initial trial results after 4 years. Science.1995;270:475-480.
Holmes B, Quie PG, Windhorst DB, Good RA.Fatal granulomatous disease of childhood: an inborn abnormality of phagocytic function. Lancet.1966;1:1225-1228.
Finn A, Hadzic N, Morgan G, Strobel S, Levinsky RJ.Prognosis of chronic granulomatous disease. Arch Dis Child.1990;65:942-945.

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