Abstract
A 2½-month-old infant with complete DiGeorge syndrome was treated with bovine thymosin fraction V. Initial evaluation revealed normal numbers of blood lymphocytes, 29% EAC rosettes, 41% sIg lymphocytes and no T-rosettes. T-rosettes rose to 15% upon in vitro incubation with thymosin. There was no in vitro response to PHA, PWM, SLO, candida or allogeneic cells, and delayed skin reactivity to SKSD, candida and DNCB was absent. IgG, A, M and E were 175 mg%, 15 mg%, 40 mg% and 10 Iu/ml respectively. He received 10 mg/day of thymosin for 21 days, followed by 20 mg/day for an additional 17 days, and weekly injections of the same dose thereafter. During therapy the lymphocyte count did not change, but T-rosettes rose to 26% while EAC rosettes and sIg lymphocytes decreased to 14% and 10% respectively. Lymphocytes remained unreactive in vitro to PHA, PWM, candida and allogeneic cells, but reacted to SLO (S.I.=42). Delayed skin reactions remained negative. IgG, A and M were 120 mg%, 20 mg% and 62 mg% at 4 months of age, while IgE rose to 255 Iu/ml. No specific antibodies were detected. He died of sepsis at 4½ months of age. In the postmortem no thymus or parathyroids could be found, and the lymphoid tissue revealed depleted T-dependent areas with poorly developed germinal centers. Similar partial reconstitution of T-cell functions, presumably resulting from the maturation of null-cells, have been observed in other immunodeficiencies treated with thymic extracts.
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Bamzai, A., Kretschmer, R., Rothberg, R. et al. 681 THYMOSIN TREATMENT OF DI GEORGE SYNDROME. Pediatr Res 12 (Suppl 4), 477 (1978). https://doi.org/10.1203/00006450-197804001-00686
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DOI: https://doi.org/10.1203/00006450-197804001-00686
