Pediatric Cardiology

, Volume 9, Issue 2, pp 95–101

Spectrum of Di George syndrome in patients with truncus arteriosus: Expanded Di George syndrome

  • Dorothy J. Radford
  • Lisa Perkins
  • Rosa Lachman
  • Y. H. Thong
Article

Summary

A study of 26 patients with truncus arteriosus showed a high prevalence of facial dysmorphism, aortic arch abnormalities, extracardiac malformations, and significant prenatal risk factors. There was little evidence of parathyroid or thymic abnormalities. However, there was laboratory evidence of immune deficiency, especially T-helper lymphocytes, and clinical evidence of predilection to infection. These findings suggest that patients with truncus arteriosus belong to the spectrum of the Di George syndrome.

Key words

Truncus arteriosus Di George syndrome Immunodeficiency Prenatal risk factors 

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Copyright information

© Springer-Verlag New York Inc 1988

Authors and Affiliations

  • Dorothy J. Radford
    • 1
  • Lisa Perkins
    • 2
  • Rosa Lachman
    • 2
  • Y. H. Thong
    • 3
  1. 1.Department of CardiologyPrince Charles HospitalBrisbaneAustralia
  2. 2.Immunology Laboratory, Department of PathologyMater HospitalSouth Brisbane
  3. 3.Department of Child HealthUniversity of Queensland, Mater Children's HospitalSouth BrisbaneAustralia

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