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Congenital Aplasia of the Thymus Gland (DiGeorge's Syndrome)
Abstract
Two infants with congenital aplasia of the thymus were found to have normal polymorphonuclear-leukocyte function, immunoglobulins and antibody formation. Delayed hypersensitivity, allograft rejection and in vitro lymphocyte responses to phytohemagglutinin were impaired. Lymphoid tissue of these two patients and postmortem material from a third revealed a moderate deficiency of lymphocytes in the deep cortical regions of the lymph nodes and periarteriolar sheaths of spleen but normal cortical germinal-center activity and plasma cells. Thus, in thymic aplasia humoral immunity is normal, but cellular immunity is deficient, findings precisely the reverse of what is found in congenital agammaglobulinemia. The dichotomy found between the two types of immunity is consistent with experimental studies showing a separable cellular origin for humoral and cellular immunity.
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Cites
Estradiol metabolism in cirrhosis
Zumoff, B., Fishman, J., Gallagher, T. F., & Hellman, L. (1968) · Journal of Clinical Investigation · doi:10.1172/jci105709 · Cited by 80
Cited by
Velo‐cardio‐facial syndrome: A review of 120 patients
Goldberg, R., Motzkin, B., Marion, R., Scambler, P. J., & Shprintzen, R. J. (1993) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320450307 · Cited by 425
The spectrum of the DiGeorge syndrome
Conley, M. E., Beckwith, J. B., Mancer, J. F. K., & Tenckhoff, L. (1979) · The Journal of Pediatrics · doi:10.1016/s0022-3476(79)80207-3 · Cited by 401
Metadata
- Authors
- Kretschmer, R., Say, B., Brown, D., & Rosen, F. S.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1968-12-12
- Volume
- 279
- Issue
- 24
- Pages
- 1295-1301
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2323516722
- Cited by
- 173
- References
- 1
- FWCI
- 9.02
- Type
- journal article
- Language
- en
- Open access
- closed
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- Kretschmer, R., Say, B., Brown, D., & Rosen, F. S. (1968). Congenital Aplasia of the Thymus Gland (DiGeorge's Syndrome). New England Journal of Medicine. https://doi.org/10.1056/nejm196812122792401
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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