Abstract
Although experiments in animals can tell us something about the mechanisms of protection against infection, the relevance to human disease is usually uncertain. The study of rare immunodeficiency disorders has enabled us to make some clear statements on the critical role of individual host defence mechanisms against infection. It is likely that new genetically determined defects in relatively minor aspects of host defence or inflammation (e.g. lymphokines) will soon be discovered and enlighten us on the efficiency of the many ‘back-up’ mechanisms for dealing with infecting organisms. The problem with many of the known severe immunodeficiency disorders, such as severe combined immunodeficiency, is that more than one cell type is involved, making it difficult to link the clinical pattern of infection with any specific defect. Furthermore, it can be difficult to locate the immune defect in patients who are chronically infected with an organism, since the infection itself may cause secondary ‘abnormalities’ in the immune system, as is seen in mucocutaneous candidiasis. Nevertheless, studies in patients with immunodeficiencies involving antibody production and neutrophil function have enabled us to link defence mechanisms to specific infections. Most of these studies have been undertaken on patients in advanced countries, and the results may not apply to individuals in the Third World who are more frequently exposed to a wider range of organisms. In this situation a relatively minor defect in an amplifying or back-up mechanism may be potentially lethal.
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References
Levinsky RJ, Tiedeman K. Successful bone marrow transplantation for reticular dysgenesis. Lancet. 1983; I: 671–673.
Perry VH, Gordon S. Macrophages and microglia in the nervous system. Trends Neurosci. 1988; 11: 273–277.
Dale DC, DuPont G, Wewerka JR, Bull JM, Chusid MJ. Chronic neutropenia. Medicine. 1979; 58: 128–144.
Medical Research Council Working Party on Leukaemia in Childhood. Analysis of treatment in childhood leukaemia. I. Prolonged predisposition to drug-induced neutropenia following craniospinal irradiation. Br Med J. 1975; 3: 563–566.
Hitzig WH. Familiare neutropenie mit dominanten erbgang and hypergammaglobulinamie. Hely Med Acta. 1959; 26: 779–784.
Asherson GL, Webster ADB. Diagnosis and treatment of immunodeficiency diseases. Oxford: Blackwell Scientific, 1980: 301–302.
Segal A, Walport M. Neutrophil and complement defects: recent advances. In Webster ADB, ed. Immunodeficiency and Disease. London: Kluwer Academic Publishers, 1988: 149–179.
Roos D. The molecular basis of chronic granulomatous disease. In: Gupta S, Griscelli C, eds. New Concepts of Immunodeficiency Diseases. Chichester: John Wiley, 1993: 311–352.
Nauseff WM, Root RK, Maleck HL. Biochemical and immunologic analysis of hereditary myeloperoxidase deficiency. J Clin Invest. 1983; 71: 1297–1307.
Parry MF, Root RK, Metcalf JA, Delaney KK, Kaplow LS, Richar WT. Myeloperoxidase deficiency. Prevalence and clinical significance. Ann Intern Med. 1981; 95: 293–301.
Root RK, Rosenthal AS, Balestra DJ. Abnormal bactericidal, metabolic and lysosomal functions of Chediak-Higashi syndrome. J Clin Invest. 1972; 51: 649–665.
Geha RS, Leung DYM. Hyper immunoglobulin E syndrome. In: Rosen FS, Seligmann M, eds Immunodeficiencies. Philadelphia, PA: Harwood Academic, 1993: 571–583.
Gallin JI, Fischer A, Lisowska-Grospierre B, Anderson DC, Springer TA. Leukocyte adhesion deficiency: molecular basis and functional consequences. Immunodeficiency Rev. 1988; 1: 38–54.
Davies KA, Toothill VJ, Savill J, Hotchin N, Peters AM, Pearson JD, Haslett C, Burke M, Law SKA, Mercer NFG, Walport MJ, Webster ADB. A 19-year-old man with leucocyte adhesion deficiency. In vitro and in vivo studies of leucocyte function. Clin Exp Immunol. 1991; 84: 223–231.
Hosea SW, Brown EJ, Hamburger MI, Frank MM. Opsonic requirements for intravascular clearance after splenectomy. N Engl J Med. 1981; 304: 245–250.
Hosea SW, Burch CG, Brown EG, Berg RA, Frank MM. Impaired immune response in splenectomised patients to polyvalent pneumococcal vaccine. Lancet. 1981; i; 804–807.
Ceuppens JL, Baroja ML, van Vaek F, Anderson CL. Defect in the membrane expression of high affinity 72-kD Fcy receptors on phagocytic cells in four healthy subjects. J Clin Invest. 1988; 82: 571–578.
Bernichou G, Kanellopoulos JM, Wallow C, Bove F, Defraissy JF. Interferon-y restores T lymphocyte proliferation of non-responders to IgGI anti-CD3 via the induction of Fcy’ receptors on monocytes. Eur J Immunol. 1987; 17: 1175–1181.
Qualman SJ, Gupta PK, Mendelsohn G. Intracellular Escherichia coli in urinary malakoplakia. A reservoir of infection and its therapeutic implications. Am J Clin Pathol. 1984; 81: 35–42.
Abdou NI, Na Pombejara C, Sayawa A, Ragland C, Stechscwulte DJ, Nilsson U, Gourley W, Wattanabe I, Lindsey NJ, Allen MS. Malokoplakia: evidence for monocyte lysosomal abnormality correctable by cholinergic agonist in vitro and in vivo. New Engl J Med. 1977; 297: 1413–1419.
Komiyama A, Ichikawa M, Kanda H, Aoyama K, Yasui K, Yamazaki M, Kawai H, Miyagawa Y, Akabane T. Defective interleukin-1 production in a familial monocyte disorder with a combined abnormality of mobility and phagocytosis-killing. Clin Exp Immunol. 1988; 73: 500–504.
Dobbins WO. Whipple’s disease: an historical perspective. Q J Med. 1985; 56: 523–531.
Dobbins WO. Is there an immune deficit in Whipple’s disease? Dig Dis Sci. 1981; 26: 247–252.
Bjerknes R, Laerum OP, Oegaards S. Impaired bacterial degradation by monocytes and macrophages from a patient with treated Whipple’s disease. Gastroenterology. 1985; 89: 1139–1151.
Feurle GH, Dörken B, Schöpf E, Lenhard V. HLA-B27 and defects in the T-cell system in Whipples disease. Eur J Clin Invest. 1979; 9: 385–389.
Nathan CF, Murray HW, Wiebe ME, Ruben BY. Identification of interferon-y as the lymphokine that activates human macrophage oxidative metabolism and antimicrobial activity. J Exp Med. 1983; 158: 670–689.
Nathan CF, Kaplan G, Levis WR, Nusra A, Witmer MD, Sherwin SA, Job CK, Horowitz CR, Steinman RM, Cohn ZA. Local and systemic effects of intradermal recombinant interferon-y in patients with lepromatous leprosy. N Engl J Med. 1986; 315: 6–15.
Denis M, Forget A, Pelletier M, Skamene E. Pleiotropic effects of the Bcg gene. III. Respiratory burst in Bcg-congenic macrophages. Clin Exp Immunol. 1988; 73: 370–375.
Winston DJ, Terito MC, Ho WG, Miller MJ. Gale RP, Golde DW. Alveolar macrophage dysfunction in human bone marrow transplant recipients. Am J Med. 1982; 73: 859–866.
Murray HW, Rubin BY. Masur H, Roberts RB. Impaired production of lymphokines and immune (gamma) interferon in the acquired immunodeficiency syndrome. N Engl J Med. 1984; 310: 883–887.
Murray HW, Gellene RA, Libby DM, Rothermel CD, Rubin BY. Activation of tissue macrophages from AIDS patients: in vitro response of AIDS alveolar macrophages to lymphokines and interferon-gamma. J Immunol. 1985; 135: 2374–2377.
Amman AJ. Immunological aberrations in purine nucleoside phosphorylase deficiencies. In: Enzyme Defects and Immune Dysfunction. Ciba Foundation Symposium Series, 68. Amsterdam. Elsevier, 1979: 55–69.
Zegers BJM, Stoop JW. Metabolic causes of immune deficiency: mechanisms and treatment. In: Webster ADB, ed. Immunodeficiency and Disease. London: Kluwer Academic, 1988: 113–131.
Hong R. The DiGeorge anomaly. In: Rosen FS, Seligmann M, eds. Immunodeficiencies. Philadelphia, PA: Harwood Academic, 1993: 167–176.
Barrett DJ, Amman AI, Wara DW, Cowan MJ, Fisher TJ, Stiehm ER. The clinical and immunologic spectrum of the DiGeorge syndrome. J Clin Lab Immunol. 1981; 6: 1–6.
Webster ADB. Lymphocyte disorders in immunodeficiency. In: Peters TJ, ed. Subcellular Pathology of Systemic Disease. London: Chapman and Hall Medical, 1987: 304–320.
Fisher D, Martin B, van der Weyden R, Snyderman R, Kelley WN. A role for adenosine deaminase in human monocyte maturation. J Clin Invest. 1976; 58: 399–407.
Morgan G, Levinsky RJ, Hugh-Jones K, Fairbanks LD, Morris GS, Simmonds A. Heterogeneity of biochemical, clinical and immunological parameters in severe combined immunodeficiency due to adenosine deaminase deficiency. Clin Exp Immunol. 1987; 70: 491–499.
Griscelli C, Lisowska-Grospierre B. Combined immunodeficiency with defective expression in MHC class II genes. In: Gupta S, Griscelli C, eds. New Concepts of Immunodeficiency Diseases. Chichester: John Wiley, 1993: 177–190.
Noguchi M, Rosenblatt HM, Adelstein S, McBride OW, Leonard WJ. Interleukin-2 receptor y chain mutation results in X-linked severe combined immunodeficiency in humans. Cell. 1993; 73: 147–157.
Fischer A, Griscelli C, Friedrich W, Kubanek B, Levinsky R, Morgan G, Vossen J, Wagemaker G, Landais P. Bone marrow transplantation for immunodeficiencies and osteopetrosis. European Survey 1968–1985. Lancet. 1986; ii: 1080–1083.
Waldmann TA. Ataxia telangiectasia. A multi-system hereditary disease with immunodeficiency, impaired organ maturation, X-ray hypersensitivity, and a high incidence of neoplasia. Ann Intern Med. 1983; 99: 367–379.
Gatti RA. Ataxia-telangiectasia: genetic studies. In: Gupta S, Griscelli C, eds. New Concepts of Immunodeficiency Diseases. Chichester: John Wiley, 1993: 203–229.
Blaese RM, Strober W, Waldmann TA. Immunodeficiency in the Wiskott Aldrich syndrome. In: Bergsma D, Good RA, Finstad J, Paul NW, eds. Immunodeficiency in Man and Animals. Sunderland, USA: Sinauer, 1975: 250–254.
Rosenstein Y, Park JK, Bierer BE, Burakoff SJ. The Wiskott-Aldrich syndrome: An immunodeficiency associated with defects of the CD43 molecule. In: Gupta S, Griscelli C, eds. New Concepts of Immunodeficiency Diseases. Chichester: John Wiley, 1993: 249–268.
Remold-O’Donnell E, Kenney DM, Parkman R, Cairns L, Savage B, Rosen FS. Characterization of a human lymphocyte surface sialoglycoprotein that is defective in Wiskott Aldrich syndrome. J Exp Med. 1984; 159: 1705–1723.
Purtilo DT, Cassel CK, Young JP, Harper R, Stephenson SR, Landing BH, Jawter GF. X-linked recessive progressive combined variable immunodeficiency (Duncan’s disease). Lancet. 1975; i: 935–940.
Sakamoto K, Freed HJ, Purtilo DT. Antibody responses to Epstein-Barr virus in families with the X-linked lymphoproliferative syndrome. J Immunol. 1980; 125: 921–925.
Sullivan JL, Woda BA. X-linked lymphoproliferative syndrome. In: Rosen FS, Seligman M, eds. Immunodeficiencies. Harwood Academic, 1993: 585–600.
Grossi CE, Crist WM, Abo T, Velardi A, Cooper MD. Expression of the Chediak Higashi lysosomal abnormality in human peripheral blood lymphocyte populations. Blood. 1985; 65: 837–844.
Vilmer E, Lenoir GM, Virelizier JL, Griscelli C. Epstein-Barr serology in immunodeficiencies: an attempt to correlate immune abnormalities in Wiskott Aldrich and Chediak Higashi syndromes and ataxia telangiectasia. Clin Exp Immunol. 1984; 55: 249–256.
Pinching AJ, ed. AIDS and HIV infection. Clin Immunol Allergy. London: WB Saunders; 1986; 6: 467–558.
Clerici M, Shearer GM. A TH1 -* TH2 switch is a critical step in the etiology of HIV infection. Immunol Today. 1993; 14: 107–111.
Eales LJ, Moshtael O, Pinching AJ. Microbicidal activity of monocyte derived macrophages in AIDS and related disorders. Clin Exp Immunol. 1987; 67: 227–235.
Hawkins RE, Rickman LS, Vermund SH, Carl M. Association of mycoplasma and human immunodeficiency virus infection: detection of amplified Mycoplasma fermentans DNA in blood. J Infect Dis. 1992; 165: 581–585.
Lachmann PJ. Complement. In: McGee JO’D, Isaacson PG, Wright NA, eds. Oxford Textbook of Pathology. Vol. 1. Oxford: Oxford University Press, 1993: 259–266.
Rother K. Summary of reported deficiencies. In: Rother K, Rother U, eds. Hereditary and Acquired Complement Deficiencies in Animals and Man. Basel: Karger, 1986: 202–211.
Winkelstein JA, Shin HS, Wood WB Jr. Heat labile opsonins to pneumococcus. III. The participation of immunoglobulin and of the alternate pathway of C3 activation. J Immunol. 1972; 108: 1681–1689.
Lambris JD. The multi-functional role of C3, the third component of complement. Immunol Today. 1988; 9: 387–393.
Cooper NR. Immune adherence by the fourth component of complement. Science. 1969; 165: 396–398.
Ward PA, Cochrane CG, Müller-Eberhard NJ. The role of serum complement in chemotaxis of leukocytes in vitro. J Exp Med. 1965; 122: 327–346.
Würzner R, Orren A, Lachmann PJ. Inherited deficiencies of the terminal components of human complement. In: Rosen FS, Seligmann M, eds Immunodeficiencies. Harwood Academic, 1993: 295–312.
Haeney MR, Thompson RA, Faulkner J, Mackintosh P, Ball AP. Recurrent bacterial meningitis in patients with genetic defects of terminal complement components. Clin Exp Immunol. 1980; 40: 16–24.
Levy J, Schlesinger M. Complement deficiency: C7, C8 and properdin. In: Gupta S, Griscelli C, eds. New Concepts in Immunodeficiency Diseases. Chichester: John Wiley, 1993: 269–292.
Lint TF, Gewurz H. Component deficiencies–the ninth component. In Rother K, Rother U, eds. Hereditary and Acquired Complement Deficiencies in Animals and Man. Basel: Karger, 1986: 307–310.
Griffiss JM, Bertram MA. Immunoepidemiology of meningococcal disease in military recruits. II. Blocking of serum bactericidal activity by circulating IgA early in the course of invasive disease. J Infect Dis. 1977; 136: 733–739.
Thompson RA, Lachmann P.I. A second case of human C3b inhibitor (KAF) deficiency. Clin Exp Immunol. 1977; 27: 23–39.
Vetrie DF, Vorechovsky I, Sideras P, Holland J, Davies A, Flinter F, Hammarström, Kinnon C, Levinsky R, Bobrow M, Smith CIE, Bentley DR. The gene involved in X-linked agammaglobulinaemia is a member of the src family of protein-tyrosine kinases. Nature. 1993; 361: 226–233.
Hermaszewski RA, Webster ADB. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 1993; 86: 31–42.
Spickett GP, Webster ADB, Farrant J. Cellular abnormalities in common variable immunodeficiency. In: Rosen FS, Seligmann M, eds. Immunodeficiencies. Philadelphia, PA: Harwood Academic, 1993: 111–126.
Murphy TF, Apicella MA. Nontypable Haemophilus influenzae: a review of clinical aspects, surface antigens and the human immune response to infection. Rev Infect Dis. 1987; 9: 1–15.
Lever AML, Gross J, Webster ADB. Serum factors for opsoniozation of non-typable H. influenzae. J Clin Microbiol. 1985; 20: 33–38.
Stroud RM, Nagaki K, Pickering RJ, Gewurz H, Good RA, Cooper MD. Sub-units of the first complement component in immunologic deficiency syndromes. Independence of C1s and Clq. Clin Exp Immunol. 1970; 7: 133–137.
Ahnen DJ, Brown WR. Campylobacter enteritis in immune-deficient patients. Ann Intern Med. 1982; 96: 187–188.
Klipstein FA, Engert RF, Short H, Schenk EA. Pathogenic properties of Campylobacter jejuni: assay and correlation with clinical manifestations. Infect Immunity. 1985; 50: 43–49.
Bernatowska E, Jose P, Davies H, Stephenson M, Webster D. Interaction of campylobacter species with antibody, complement and phagocytes. Gut. 1989; 30: 906–911.
Goodwin CS, Worsley B. Peptic ulcer disease and Helicobacter pylori infection. Curr Opin Gastroenterol. 1992; 8: 122–127.
Forsythe SJ, Dolby JM, Webster ADB, Cole JA. Nitrate-and nitrite-reducing bacteria in the achlorhydric stomach. J Med Microbiol. 1988; 25: 253–259.
Griffiss JM. Biologic function of the serum IgA system: modulation of complement mediated affector mechanisms and conservation of antigenic mass. Ann NY Acad Sci. 1983; 409: 697–707.
Belosevic M, Faubert GM, Dharampaul S. Antimicrobial action of antibodies against Giardia muris trophozoites. Clin Exp Immunol. 1994; 95: 485–489.
Sloper KS, Dourmashkin PR, Bird RB, Slavin G, Webster ADB. Chronic malabsorption due to cryptosporidiosis in a child with immunoglobulin deficiency. Gut. 1982; 23: 80–82.
Korthäuer U, Graf D, Mages HW, Brière F, Munoreedevi P, Malcolm S, Ugazio AG, Notarangelo LD, Levinsky RJ, Kroczek RA. Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM. Nature. 1993; 361: 539–543.
Unger BLP, Ward DJ, Fayer R, Quinn CA. Cessation of cryptosporidium associated diarrhoea in an acquired immunodeficiency syndrome patient after treatment with hyperimmune colostrum. Gastroenterology. 1990; 58: 2962–2965.
Taylor-Robinson D, McCormack WM. The genital mycoplasmas. N Engl J Med. 1980; 302: 1003–10, 1063–1067.
Roifman CM, Rao CP, Lederman HM, Lavi S, Quinn P, Gelfand EW. Increased susceptibility to mycoplasma infection in patients with hypogammaglobulinaemia. Am J Med. 1986; 80: 590–594.
Webster ADB, Furr PM, Hughes-Jones NC, Gorick BD, Taylor-Robinson D. Critical dependence on antibody for defence against mycoplasmas. Clin Exp Immunol. 1988; 71: 383–387.
McKinney RE Jr, Katz SL, Wilfert CM. Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. Rev Infect Dis. 1987; 9: 334–356.
Webster ADB. Echovirus disease in hypogammaglobulinaemia patients. Clin Rheum Dis. 1984; 10: 189–203.
Erlendsson K, Swartz T, Dwyer JM. Successful reversal of echovirus encephalitis in X-linked hypogammaglobulinaemia by intraventricular administration of immunoglobulin. N Engl J Med. 1985; 312: 351–353.
Cserr HF, Knopf PM. Cervical lymphatics, the blood—brain barrier and the immunoreactivity of the brain; a new view. Immunol Today. 1992; 13: 507–512.
Webster ADB, Rotbart HA, Warner T, Rudge P, Hyman N. Diagnosis of enterovirus brain disease in hypogammaglobulinemic patients by polymerase chain reaction. Clin Infect Dis. 1993; 17: 657–661.
Wyatt HV. Poliomyelitis in hypogammaglobulinaemia. J Infect Dis. 1973; 128: 802–806.
Hanson LA, Björkander J, Söderström R, Söderström T. Clinical significance of IgG subclass and IgA deficiency. In: Webster ADB, ed. Immunodeficiency and Disease. London: Kluwer Academic, 1988: 99–111.
Scadding GK, Lund VJ, Darby YC, Navas-Romero J, Seymour N, Turner MW. IgG subclass levels in chronic rhinosinusitis. Rhinology. 1994; 32: 15–19.
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Webster, A.D.B. (1994). The Pattern of Infection in Immunodeficiency. In: Sissons, J.G.P., Borysiewicz, L.K., Cohen, J. (eds) Immunology of Infection. Immunology and Medicine Series, vol 25. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-1430-1_8
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