Transplantation of Thymus Tissue in Complete DiGeorge Syndrome
| العنوان: | Transplantation of Thymus Tissue in Complete DiGeorge Syndrome |
|---|---|
| المؤلفون: | Sherrie E. Schiff, M N Batchvarova, Henry E. Rice, Richard A. Koup, Rebecca H. Buckley, Barton F. Haynes, Amy Kloster, T M McLaughlin, Frances E. Ward, M L Markert, Daniel C. Douek, Laura P. Hale, Andreas Boeck, Samuel M. Mahaffey, D D Kostyu |
| المصدر: | New England Journal of Medicine. 341:1180-1189 |
| بيانات النشر: | Massachusetts Medical Society, 1999. |
| سنة النشر: | 1999 |
| مصطلحات موضوعية: | Male, Biopsy, T-Lymphocytes, medicine.medical_treatment, Receptors, Antigen, T-Cell, Thymus Gland, Lymphocyte Activation, Peripheral blood mononuclear cell, Immune system, Antigen, DiGeorge syndrome, DiGeorge Syndrome, medicine, Humans, Abnormalities, Multiple, medicine.diagnostic_test, business.industry, Infant, Newborn, Infant, General Medicine, medicine.disease, Transplantation, Thymus transplantation, Immunology, Leukocytes, Mononuclear, Female, Mitogens, business, Cell Division, Congenital disorder |
| الوصف: | The DiGeorge syndrome is a congenital disorder that affects the heart, parathyroid glands, and thymus. In complete DiGeorge syndrome, patients have severely reduced T-cell function.We treated five infants (age, one to four months) with complete DiGeorge syndrome by transplantation of cultured postnatal thymus tissue. Follow-up evaluations included immune phenotyping and proliferative studies of peripheral-blood mononuclear cells plus biopsy of the thymus allograft. Thymic production of new T cells was assessed in peripheral blood by tests for T-cell-receptor recombination excision circles, which are formed from excised DNA during the rearrangement of T-cell-receptor genes.After the transplantation of thymus tissue, T-cell proliferative responses to mitogens developed in four of the five patients. Two of the patients survived with restoration of immune function; three patients died from infection or abnormalities unrelated to transplantation. Biopsies of grafted thymus in the surviving patients showed normal morphologic features and active T-cell production. In three patients, donor T cells could be detected about four weeks after transplantation, although there was no evidence of graft-versus-host disease on biopsy or at autopsy. In one patient, the T-cell development within the graft was demonstrated to accompany the appearance of recently developed T cells in the periphery and coincided with the onset of normal T-cell function. In one patient, there was evidence of thymus function and CD45RA+CD62L+ T cells more than five years after transplantation.In some infants with profound immunodeficiency and complete DiGeorge syndrome, the transplantation of thymus tissue can restore normal immune function. Early thymus transplantation - before the development of infectious complications - may promote successful immune reconstitution. |
| تدمد: | 1533-4406 0028-4793 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b37965eb56f791dc4ba5ab657c60d54b https://doi.org/10.1056/nejm199910143411603 |
| رقم الأكسشن: | edsair.doi.dedup.....b37965eb56f791dc4ba5ab657c60d54b |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15334406 00284793 |
|---|