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

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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.

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Authors and Affiliations

  1. Department of Cardiology, Prince Charles Hospital, Rode Road, Chermside, Qld, 4032, Brisbane, Australia

    Dorothy J. Radford

  2. Immunology Laboratory, Department of Pathology, Mater Hospital, South Brisbane

    Lisa Perkins & Rosa Lachman

  3. Department of Child Health, University of Queensland, Mater Children's Hospital, South Brisbane, Australia

    Y. H. Thong

Authors
  1. Dorothy J. Radford
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  2. Lisa Perkins
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  3. Rosa Lachman
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  4. Y. H. Thong
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Radford, D.J., Perkins, L., Lachman, R. et al. Spectrum of Di George syndrome in patients with truncus arteriosus: Expanded Di George syndrome. Pediatr Cardiol 9, 95–101 (1988). https://doi.org/10.1007/BF02083707

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