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July 1965, Vol 110, No. 1 >
Article | July 1965

Immunologic Incompetence in Ataxia-Telangiectasia FREE

IRA M. ROSENTHAL, MD; A. S. MARKOWITZ, PhD; RUTH MEDENIS, MD
[+] Author Affiliations

Received for publication Dec 29, 1964.

Reprint requests to 840 S Wood St, Chicago, Ill 60612 (Dr. Rosenthal).


Am J Dis Child. 1965;110(1):69-75. doi:10.1001/archpedi.1965.02090030075010.
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References
Comments

THE predisposition of some children with the syndrome of ataxia-telangiectasia to suffer from frequent severe sinopulmonary infections has led to an investigation of the immunologic competence of patients with this disease. Louis-Bar,1 Boder and Sedgwick,2,3 and Centerwall and Miller,4 have described the striking clinical features of the disease which include progressive ataxia with onset in early childhood and telangiectasia involving the bulbar conjunctivae and other areas. Peterson et al5 recently reported thymic abnormalities and immunological incompetence in this disease. In the sera of four of five patients studied low or absent levels of γ-1A globulin were found. Fireman et al6 have recently reported studies on six patients and found five with no detectable γ-1A globulin and a sixth patient with a serum concentration much less than normal. Young et al7 have also recently reported a selective absence of γ-1A globulin in three of four

REFERENCES

1 +
Louis-Bar, D.:  Sur un syndrome progressif, comprenat des télangiectasies capillaries cutanées et conjonctivales symétriques a disposition naevoide et des troubles cérébelleux , Confin Neurol 4:32, 1941;.
2 +
Boder, E., and Sedgwick, R. P.:  Ataxia-Telangiectasia, Oculocutaneous Telangiectasia and Frequent Pulmonary Infections , Pediatrics 21:526, 1958;.
3 +
Boder, E., and Sedgwick, R. P.: " Ataxia-Telangiectasia: Review of 101 Cases ," in Cerebellum, Posture and Cerebral Palsy, G. Walsh, ed., Little Club Clin Develop Med 8:100, 1963;.
4 +
Centerwall, W. R., and Miller, M. M.:  Ataxia, Telangiectasia, and Sinopulmonary Infections , Amer J Dis Child 95:385, 1958;.
5 +
Peterson, R. D. A.; Kelly, W. D.; and Good, R. A.:  Ataxia-Telangiectasia, Its Association With Defective Thymus, Immunological-Deficiency Disease, and Malignancy , Lancet 1: 1186, 1964;.
6 +
Fireman, P.; Boesman, M.; and Gitlin, D.:  Ataxia-Telangiectasia, Dysgammaglobulininemia With Deficient Gamma-1-A (beta 2-A)-globulin , Lancet 1:1193, 1964;.
7 +
Young, R. R.; Austen, K. F.; and Moser, H. W.:  Abnormalities of Serum Gamma 1A Globulin and Ataxia Telangiectasia , Medicine 43:423, 1964;.
8 +
Moorhead, P. S., et al:  Chromosome Preparations of Leukocyte Cultures From Human Peripheral Blood , Exp Cell Res 20:613, 1960;.
9 +
Fireman, P.; Vannier, W. E.; and Goodman, H. C.:  Association of Skin Sensitizing Antibody with β2A Globulins in Sera From Ragweed-Sensitive Patients , J Exp Med 117:603, 1963;.
10 +
Eisen, A. H., et al:  Immunologic Deficiency in Ataxia Telangiectasia , New Eng J Med 272:18, 1965;.
11 +
Good, R. A., and Varco, R. L.:  Successful Homograft of Skin in Child With Agammaglobulinemia , JAMA 157:713, 1955;.
12 +
Good, R. A., et al:  Transplantation Studies in Patients With Agammaglobulinemia , Ann NY Acad Sci 64:882, 1957;.
13 +
Schubert, W. K., et al:  Homograft Rejection in Children With Congenital Immunological Defects: Agammaglobulinemia and Aldrich Syndrome , Transplant Bull 26:125, 1960;.
14 +
Gitlin, D.; Rosen, F. S.; and Janeway, C. A.:  Thymus and Other Lymphoid Tissues in Congenital Agammaglobulinemia: II. Delayed Hypersensitivity and Homograft Survival in Child With Thymic Alymphoplasia , Pediatrics 33:711, 1964;.
15 +
Miller, J. F. A. P.:  Thymus and Development of Immunologic Responsiveness , Science 144:1544, 1964;.
16 +
Miller, J. F. A. P.: Immunological Function of Thymus, Lancet 2:748, 1961;.
17 +
Good, R. A., et al:  Role of Thymus in Development of Immunologic Capacity in Rabbits and Mice , J Exp Med 116: 773, 1962;.
18 +
Good, R. A., et al: "Studies on Role of Thymus in Developmental Biology, With Consideration of Association of Thymus Abnormalities and Clinical Disease ," in Third International Symposium on Immunopathology, Grabar, P. and Meischer, P. (eds.), Basel: Schwabe & Co., 1963;, p 177.
19 +
Szanto, P.: Personal communication to the authors.
20 +
Habel, K. J.:  Immunological Determinations of Polyoma Virus Oncogenesis , J Exp Med 115:181, 1962;.
21 +
Miller, J. F. A. P.; Grant, G. A.; and Roe, F. J. C.:  Effects of Thymectomy on Induction of Skin Tumors by 3, 4-Benzopyrene , Nature 199:920, 1963;.
22 +
Archer, O. K.; Sutherland, D. E. R.; and Good, R. A.:  Appendix of Rabbit: Homologue of Bursa in Chicken , Nature 200:337, 1963;.
23 +
Zollinger, R. M., Jr., et al:  Effect of Thymectomy on Skin Homograft Survival in Children , New Eng J Med 207:707, 1964;.
24 +
Schulman, I., and Abildgaard, C.: To be published.
25 +
Osoba, D., and Miller, J. F. A. P.:  Evidence for Humoral Thymus Factor Responsible for Maturation of Immunological Faculty , Nature 199:653, 1963;.
26 +
Osoba, D., and Miller, J. F. A. P.:  Lymphoid Tissues and Immune Responses of Neonatally Thymectomized Mice Bearing Thymus Tissue in Millipore Diffusion Chamber , J Exp Med 119:177, 1964;.
27 +
Levey, R. H.; Trainin, N.; and Law, L. W.:  Evidence for Function of Thymic Tissue in Diffusion Chambers Implanted in Neonatally Thymectomized Mice , J Nat Cancer Inst 31:199, 1963;.
28 +
Gowans, J. L., et al:  Initiation of Immune Responses by Small Lymphocytes , Nature 196:651, 1962;.

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References

1 +
Louis-Bar, D.:  Sur un syndrome progressif, comprenat des télangiectasies capillaries cutanées et conjonctivales symétriques a disposition naevoide et des troubles cérébelleux , Confin Neurol 4:32, 1941;.
2 +
Boder, E., and Sedgwick, R. P.:  Ataxia-Telangiectasia, Oculocutaneous Telangiectasia and Frequent Pulmonary Infections , Pediatrics 21:526, 1958;.
3 +
Boder, E., and Sedgwick, R. P.: " Ataxia-Telangiectasia: Review of 101 Cases ," in Cerebellum, Posture and Cerebral Palsy, G. Walsh, ed., Little Club Clin Develop Med 8:100, 1963;.
4 +
Centerwall, W. R., and Miller, M. M.:  Ataxia, Telangiectasia, and Sinopulmonary Infections , Amer J Dis Child 95:385, 1958;.
5 +
Peterson, R. D. A.; Kelly, W. D.; and Good, R. A.:  Ataxia-Telangiectasia, Its Association With Defective Thymus, Immunological-Deficiency Disease, and Malignancy , Lancet 1: 1186, 1964;.
6 +
Fireman, P.; Boesman, M.; and Gitlin, D.:  Ataxia-Telangiectasia, Dysgammaglobulininemia With Deficient Gamma-1-A (beta 2-A)-globulin , Lancet 1:1193, 1964;.
7 +
Young, R. R.; Austen, K. F.; and Moser, H. W.:  Abnormalities of Serum Gamma 1A Globulin and Ataxia Telangiectasia , Medicine 43:423, 1964;.
8 +
Moorhead, P. S., et al:  Chromosome Preparations of Leukocyte Cultures From Human Peripheral Blood , Exp Cell Res 20:613, 1960;.
9 +
Fireman, P.; Vannier, W. E.; and Goodman, H. C.:  Association of Skin Sensitizing Antibody with β2A Globulins in Sera From Ragweed-Sensitive Patients , J Exp Med 117:603, 1963;.
10 +
Eisen, A. H., et al:  Immunologic Deficiency in Ataxia Telangiectasia , New Eng J Med 272:18, 1965;.
11 +
Good, R. A., and Varco, R. L.:  Successful Homograft of Skin in Child With Agammaglobulinemia , JAMA 157:713, 1955;.
12 +
Good, R. A., et al:  Transplantation Studies in Patients With Agammaglobulinemia , Ann NY Acad Sci 64:882, 1957;.
13 +
Schubert, W. K., et al:  Homograft Rejection in Children With Congenital Immunological Defects: Agammaglobulinemia and Aldrich Syndrome , Transplant Bull 26:125, 1960;.
14 +
Gitlin, D.; Rosen, F. S.; and Janeway, C. A.:  Thymus and Other Lymphoid Tissues in Congenital Agammaglobulinemia: II. Delayed Hypersensitivity and Homograft Survival in Child With Thymic Alymphoplasia , Pediatrics 33:711, 1964;.
15 +
Miller, J. F. A. P.:  Thymus and Development of Immunologic Responsiveness , Science 144:1544, 1964;.
16 +
Miller, J. F. A. P.: Immunological Function of Thymus, Lancet 2:748, 1961;.
17 +
Good, R. A., et al:  Role of Thymus in Development of Immunologic Capacity in Rabbits and Mice , J Exp Med 116: 773, 1962;.
18 +
Good, R. A., et al: "Studies on Role of Thymus in Developmental Biology, With Consideration of Association of Thymus Abnormalities and Clinical Disease ," in Third International Symposium on Immunopathology, Grabar, P. and Meischer, P. (eds.), Basel: Schwabe & Co., 1963;, p 177.
19 +
Szanto, P.: Personal communication to the authors.
20 +
Habel, K. J.:  Immunological Determinations of Polyoma Virus Oncogenesis , J Exp Med 115:181, 1962;.
21 +
Miller, J. F. A. P.; Grant, G. A.; and Roe, F. J. C.:  Effects of Thymectomy on Induction of Skin Tumors by 3, 4-Benzopyrene , Nature 199:920, 1963;.
22 +
Archer, O. K.; Sutherland, D. E. R.; and Good, R. A.:  Appendix of Rabbit: Homologue of Bursa in Chicken , Nature 200:337, 1963;.
23 +
Zollinger, R. M., Jr., et al:  Effect of Thymectomy on Skin Homograft Survival in Children , New Eng J Med 207:707, 1964;.
24 +
Schulman, I., and Abildgaard, C.: To be published.
25 +
Osoba, D., and Miller, J. F. A. P.:  Evidence for Humoral Thymus Factor Responsible for Maturation of Immunological Faculty , Nature 199:653, 1963;.
26 +
Osoba, D., and Miller, J. F. A. P.:  Lymphoid Tissues and Immune Responses of Neonatally Thymectomized Mice Bearing Thymus Tissue in Millipore Diffusion Chamber , J Exp Med 119:177, 1964;.
27 +
Levey, R. H.; Trainin, N.; and Law, L. W.:  Evidence for Function of Thymic Tissue in Diffusion Chambers Implanted in Neonatally Thymectomized Mice , J Nat Cancer Inst 31:199, 1963;.
28 +
Gowans, J. L., et al:  Initiation of Immune Responses by Small Lymphocytes , Nature 196:651, 1962;.

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