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Increased whole blood chemiluminescence in patients with Shwachman syndrome: therapy trial with thiamine and α-tocopherol

  • Immunology
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Abstract

Neutrophils purified from peripheral blood of patients with the Shwachman syndrome show enhanced chemiluminescence (CL) and depressed chemotaxis. Here we present data showing that the increased CL response can be demonstrated by using a whole blood CL assay. This assay is well-suited for studies in infants, because the blood sample volumes needed are small. Increase in CL was most distinct in the initial (1 min) activation induced by N-formyl-methionyl-leucyl-phenylalanine. The 1-min response is considered to derive from extracellular production of oxygen radicals. Such an extracellular oxygen radical production may render the patients susceptible to undue oxidant stress. We therefore treated the patients with two antioxidants, thiamine and α-tocopherol, for 3 months. This supplementation, however, failed to exert any significant effect on either whole blood CL or migration of the patients' neutrophils under agarose.

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Abbreviations

CL:

chemiluminescence

FMLP:

N-formylmethionyl-leucyl-phenylalanine

OZP:

opsonized zymosan particles

PBS:

phosphate-buffered saline

ZTS:

zymosan treated serum

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Authors and Affiliations

  1. Department of Bacteriology and Immunology, University of Helsinki, Haartmaninkatu 3, SF-00290, Helsinki, Finland

    M. Ristola, M. Leirisalo-Repo & H. Repo

  2. Second Department of Medicine, Helsinki University Central Hospital, Haartmaninkatu 4, SF-00290, Helsinki, Finland

    M. Ristola, M. Leirisalo-Repo & H. Repo

  3. Department of Paediatrics, Helsinki University Central Hospital, Stenbäckinkatu 11, SF-00290, Helsinki, Finland

    E. Savilahti

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  1. M. Ristola
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  2. E. Savilahti
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  3. M. Leirisalo-Repo
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  4. H. Repo
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Ristola, M., Savilahti, E., Leirisalo-Repo, M. et al. Increased whole blood chemiluminescence in patients with Shwachman syndrome: therapy trial with thiamine and α-tocopherol. Eur J Pediatr 150, 173–178 (1991). https://doi.org/10.1007/BF01963560

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  • DOI: https://doi.org/10.1007/BF01963560

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