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Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase.

Publication ,  Journal Article
Levy, Y; Hershfield, MS; Fernandez-Mejia, C; Polmar, SH; Scudiery, D; Berger, M; Sorensen, RU
Published in: J Pediatr
August 1988

We report a 5-year-old girl with adenosine deaminase (ADA) deficiency who was asymptomatic during the first years of life. At 3 years of age, she developed chronic and recurrent sinopulmonary infections, and at 4 1/2 years of age she had one major infection with Streptococcus pneumoniae (bacteremia and septic arthritis of the hip). Immunologic evaluation at 5 years of age revealed persistent lymphopenia, decreased helper-suppressor T cell ratios, and low proliferative responses to mitogens. The IgG, IgM, and IgA levels were normal; the IgG2 level was low normal or below normal. The patient had specific antibodies against toxoids and viral antigens but failed to produce antibodies against Haemophilus influenzae type b and pneumococcal polysaccharides. Although no symptoms of allergy were present, she had persistent eosinophilia and elevated IgE levels. The patient had 0.6% of normal ADA activity in erythrocytes and approximately 1% of normal ADA activity in peripheral blood mononuclear cells. Beginning at 6 years of age, she was treated with weekly injections of polyethylene glycol-modified bovine ADA. This treatment was well tolerated and effectively reversed the biochemical consequence of ADA deficiency. Concomitantly, she improved clinically and her T lymphocyte numbers and blastogenic responses to mitogens in vitro became normal. The late onset of clinical symptoms and relatively benign clinical course in this patient emphasize the need to consider ADA deficiency in a broad spectrum of immunodeficient children.

Duke Scholars

Published In

J Pediatr

DOI

ISSN

0022-3476

Publication Date

August 1988

Volume

113

Issue

2

Start / End Page

312 / 317

Location

United States

Related Subject Headings

  • T-Lymphocytes
  • Respiratory Tract Infections
  • Recurrence
  • Pediatrics
  • Nucleoside Deaminases
  • Leukocyte Count
  • Immunologic Deficiency Syndromes
  • Humans
  • Female
  • Erythrocytes
 

Citation

APA
Chicago
ICMJE
MLA
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Levy, Y., Hershfield, M. S., Fernandez-Mejia, C., Polmar, S. H., Scudiery, D., Berger, M., & Sorensen, R. U. (1988). Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase. J Pediatr, 113(2), 312–317. https://doi.org/10.1016/s0022-3476(88)80271-3
Levy, Y., M. S. Hershfield, C. Fernandez-Mejia, S. H. Polmar, D. Scudiery, M. Berger, and R. U. Sorensen. “Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase.J Pediatr 113, no. 2 (August 1988): 312–17. https://doi.org/10.1016/s0022-3476(88)80271-3.
Levy Y, Hershfield MS, Fernandez-Mejia C, Polmar SH, Scudiery D, Berger M, et al. Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase. J Pediatr. 1988 Aug;113(2):312–7.
Levy, Y., et al. “Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase.J Pediatr, vol. 113, no. 2, Aug. 1988, pp. 312–17. Pubmed, doi:10.1016/s0022-3476(88)80271-3.
Levy Y, Hershfield MS, Fernandez-Mejia C, Polmar SH, Scudiery D, Berger M, Sorensen RU. Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase. J Pediatr. 1988 Aug;113(2):312–317.
Journal cover image

Published In

J Pediatr

DOI

ISSN

0022-3476

Publication Date

August 1988

Volume

113

Issue

2

Start / End Page

312 / 317

Location

United States

Related Subject Headings

  • T-Lymphocytes
  • Respiratory Tract Infections
  • Recurrence
  • Pediatrics
  • Nucleoside Deaminases
  • Leukocyte Count
  • Immunologic Deficiency Syndromes
  • Humans
  • Female
  • Erythrocytes