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Immunological abnormalities in a patient with lysinuric protein intolerance

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Abstract

We report an 8-year-old girl with lysimuric protein intolerance and immunological abnormalities including impaired function of lymphocytes, the presence of LE cells, antinuclear antibodies, and hypergammaglobulinaemia. These abnormalities have not been reported before and may be due to an amino acid imbalance or protein malnutrition in cells or tissues. The coincidence of a pre-stage of systemic lupus erythematosus (SLE) and LPI is not excluded.

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Abbreviations

LPI:

lysinuric protein intolerance

SLE:

systemic lupus erythematosus

ANA:

antinuclear antibodies

PHA:

phytohaemagglutinin

Con A:

concanavalin A

ADCC:

antibody-dependent cell-mediated cytotoxicity

References

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  3. Walser M (1983) Urea cycle disorders and other hereditaty hyperammonemic syndromes. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds). The metabolic basis of inherited disease. McGraw-Hill, New York, pp 402–438

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Authors and Affiliations

  1. Division of Infectious Disease, Immunology and Allergy, Saitama Children's Medical Centre, 2100 Magome, 339, Iwatsuki, Saitama, Japan

    M. Nagata, M. Suzuki, G. Kawamura & S. Kono

  2. Division of Endocrinology and Metabolism, Saitama Children's Medical Centre, 2100 Magome, 339, Iwatsuki, Saitama, Japan

    N. Koda & S. Yamaguchi

  3. Research Development Department, Aiiku Maternal and Child Health Centre, 105, Tokyo, Japan

    K. Aoki

Authors
  1. M. Nagata
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  2. M. Suzuki
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  3. G. Kawamura
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  4. S. Kono
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  5. N. Koda
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  6. S. Yamaguchi
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  7. K. Aoki
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Nagata, M., Suzuki, M., Kawamura, G. et al. Immunological abnormalities in a patient with lysinuric protein intolerance. Eur J Pediatr 146, 427–428 (1987). https://doi.org/10.1007/BF00444955

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  • DOI: https://doi.org/10.1007/BF00444955

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