Insulin-dependent diabetes mellitus and severe atopic dermatitis in a child with adenosine deaminase deficiency

L. D. Notarangelo, G. Stoppoloni, R. Toraldo, E. Mazzolari, A. Coletta, P. Airò, C. Bordignon, A. G. Ugazio

Research output: Contribution to journalArticlepeer-review

Abstract

We report a 2.3-year-old girl with complete lack of adenosine deaminase (ADA) activity who presented with severe atopic dermatitis and insulin-dependent diabetes mellitus but only mild recurrent infections. Abnormalities of immune function included profound depletion of CD8+ lymphocytes, hyperimmunoglobulinaemia E, and very low in vitro proliferative response to mitogens. Treatment with polyethylene glycol-conjugated ADA was followed by rapid amelioration of clinical and immunological conditions. The immunological and clinical features of this child suggest that the clinical spectrum of ADA deficiency may be broader than originally supposed.

Original languageEnglish
Pages (from-to)811-814
Number of pages4
JournalEuropean Journal of Pediatrics
Volume151
Issue number11
DOIs
Publication statusPublished - Nov 1992

Keywords

  • Adenosine deaminase
  • Auto-immunity
  • Diabetes
  • Immunodeficiency

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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