Abstract
Immunodeficiency states in man are characterized by the inability of the organism to mount a normal immune response following antigenic stimulation. Since Bruton’s first description in 1952 (6) of a young boy who suffered from recurrent severe infections and who was found to be agammaglobulinemic — a disease now called “infantile X-linked agammaglobulinemia”-, a broad spectrum of immunodeficiency states with defects of humoral and/or cell-mediated immunity has been discovered (reviews: 3, 9, 12, 14). It should be recognized that, in addition to faulty or absent production of humoral antibodies and/or sensitized lymphocytes (“primary specific immunodeficiency”), lowered resistance to infectious disease may also be due to 1. immunoglobulin hypercatabolism or loss (e. g. protein-losing enteropathy); 2. exogenous physicochemical causes, e. g. cytotoxic or immunosuppressive agents, ionizing radiation; 3. concomitant diseases, e. g. metabolic disorders, such as Diabetes mellitus, viral diseases, or neoplasia of the lymphoreticular or hemopoietic system; or 4. dysfunction of phagocytic cell systems (e. g. chronic granulomatous disease). The present review is largely limited to findings pertaining to the group of “primary specific immunodeficiency states”. These rare but well-studied cases offer an opportunity for comparison of basic functional and morphological defects with current views and hypotheses of pathogenetic mechanisms.
Work supported by the Swiss National Foundation for Scientific Research
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
Barandun, S.: Bibl. haemat. (Basel) 17: 85 (1964).
Barandun, S., Riva, G., Spengler, G. A.. In: Bergsma, D., Good, R. A.: Immunologic Deficiency Diseases in Man (U. S. Natl. Foundation Original Ar-tic les Series IV, 40, 1968 ).
Barandun, S., Hess, M. W. Cottier, H.: Verh. dtsch. Ges. inn. Med. 78: 741 (1972).
van Bekkum, D. W.: Transpl. Rev. 9: 3 (1972).
Biggar, W. D., Park, B. H., Good, R. A.: Ann. Rev. Med. 24: 135 (1973).
Bruton, O. C.: Pediatrics 9: 722 (1952).
Cottier, H.: Trans. 6th Congr. Europ. Soc. Haemat., Copenhagen 1957, p. 41 ( Karger, Basel-New York, 1958 ).
Crabbe, P. A., Heremans, J. F.: Gut 7: 119 (1966).
Fudenberg, H., Good, R. A., Goodman, H. C., Hitzig, W., Kunkel, H. G., Roitt, I. M., Rosen, F. S., Rowe, D. S., Seligmann, M., Soothill, J. R.: Pediatrics 47: 927 (1971).
Di George, A. M.: J. Pediat. 67: 907 (1965).
Glanzmann, E., Riniker, P.: Wien. med. Wschr. 100: 35 (1950).
Good, R. A.: Amer. J. Path. 69: 484 (1972).
Good, R. A. , Kersey, J. , Spector, B. , Hansen, J. , Gajl, K.: Proc. Schering Symposium on Immunopathology, Cavtat 1973 (in press).
Hess, M. W., Schaedeli, J. , Cottier, H. : Verh. dtsch. Ges. Path. 55: 175 (1971) .
Hitzig, W. H.. In: Handbuch der Inneren Medizin, VII/1, 1973 (in press).
Hitzig, W. H., Barandun, S., Cottier, H.. In: Ergebnisse der inneren Medizin und Kinderheilkunde, p. 80 ( Springer, Berlin-Heidelberg-New York 1968 ).
Joel, D. D., Hess, M. W., Cottier, H.: J. exptl. Med. 135: 907 (1972).
Levin, A. S., Spitler, L. E., Fudenberg, H. H.: Ann. Rev. Med. 24: 175 (1973).
Lischner, H. W.: J. Pediat. 81: 1042 (1972).
Lischner, H. W., di George, A. M.: Lancet 2: 1044 (1969).
Miller, J. F. A. P.. In: New Concepts in Allergy and Clinical Immunology, p. 3, Proc. VII th Internatl. Congr. Allergology, 1970.
Miller, J. F. A. P., Mitchell, G. F.: Transpl. Rev. 1: 3 (1969).
Nézelof, C., Jammet, M. L., Lortholary, P., , B., Lamy, M.: Arch. franc. Pédiat. 21: 897 (1964).
Osoba, D.: Med. clin. North Amer. 56: 319 (1972).
Rosen, F. S., Craig, J. M., Vawter, G., Janeway, C. A.. In: Bergsma, D., Good, R. A.: Immunologic Deficiency Diseases in Man (U. S. Natl. Found. Original Articles Series IV, 67, 1968 ).
Schaffner, T., Cottier, H., Hess, M. W.: (in preparation 1974 ).
Schur, P. H., Borel, H., Gelfand, E. W., Alper, C. A., Rosen, F. S.: New Engl. J. Med. 283: 631 (1970).
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1975 Springer-Verlag/Wien
About this chapter
Cite this chapter
Hess, M.W., Cottier, H., Schaedeli, J., Barandun, S. (1975). Infections in Immunodeficiency States. In: Urbaschek, B., Urbaschek, R., Neter, E. (eds) Gram-Negative Bacterial Infections and Mode of Endotoxin Actions. Springer, Vienna. https://doi.org/10.1007/978-3-7091-8396-0_11
Download citation
DOI: https://doi.org/10.1007/978-3-7091-8396-0_11
Publisher Name: Springer, Vienna
Print ISBN: 978-3-7091-8398-4
Online ISBN: 978-3-7091-8396-0
eBook Packages: Springer Book Archive