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European Journal of Pediatrics

, Volume 148, Issue 6, pp 518–522 | Cite as

DiGeorge syndrome with hypogammaglobulinaemia: a patient with excess suppressor T cell activity treated with fetal thymus transplantation

  • M. Mayumi
  • H. Kimata
  • Y. Suchiro
  • S. Hosoi
  • S. Ito
  • Y. Kuge
  • K. Shinomiya
  • H. Mikawa
Immunology/Allergology

Abstract

A male infant with DiGeorge syndrome had hypogammaglobulinaemia with a normal number of B cells. CD3(+) T cells were reduced and the CD4(+)/CD8(+) ratio was reversed. Proliferative responses of T cells to mitogens and to allogeneic cells were low. The pokeweed mitogen (PWM)-induced B cell differentiation assay revealed a higher than normal suppressor T cell activity. This suggests that some T cells had differentiated into functionally mature cells resulting in an imbalance of regulatory T cell functions and that excess suppressor activity might play a role in hypogammaglobulinaemia. Fetal thymus transplantation improved both cellular and humoral immunity. The patient's susceptibility to viral and bacterial infections, proliferative response of T cells and serum Ig concentration returned to normal. The excess suppressor activity seen before transplantation disappeared. Hypocalcaemia did not improve. These results show that fetal thymus transplantation was effective not only in reconstituting cellular immunity but also in normalizing the imbalance of regulatory T cell functions in this patient with DiGeorge syndrome.

Key words

DiGeorge syndrome hypogammaglobulinaemia Suppressor T cells Fetal thymus transplantation 

Abbreviations

MNC

mononuclear cells

ConA

concanavalin A

PHA

phytohaemagglutinin P

PWM

pokeweed mitogen

3H-TdR

tritiated thymidine

PBL

peripheral blood lymphocytes

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Copyright information

© Springer-Verlag 1989

Authors and Affiliations

  • M. Mayumi
    • 1
  • H. Kimata
    • 1
  • Y. Suchiro
    • 1
  • S. Hosoi
    • 1
  • S. Ito
    • 1
  • Y. Kuge
    • 1
  • K. Shinomiya
    • 1
  • H. Mikawa
    • 1
  1. 1.Department of Paediatrics, Faculty of MedicineKyoto UniversityKyotoJapan

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