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Plasma alpha-L-fucosidase activity in chronic inflammation and autoimmune disorders in a pediatric cohort of hospitalized patients

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Abstract

Human α-fucosidase (EC 3.2.1.51) is an enzyme (hydrolase) of particular biological and medical interest, as the inherited deficiency in its activity leads to fucosidosis, a pathology belonging to severe glycoprotein lysosomal storage disorders. Although its importance has increased in latest years, data about its plasma level in children with inflammatory disorders are still lacking. In the present study, plasma activity of α-L-fucosidase-1 (FUCA-1) and its potential association with chronic inflammatory pathologies was evaluated in hospitalized individuals, both pediatric and adult ones. A number of 201 Hungarian hospitalized patients, 144 children (1–13 years) and 57 adults (31–88 years), were enrolled in the study and underwent plasma assay of FUCA-1 activity, following the normal routine analytical run in the hospital service. Regression and Pearson tests were evaluated to investigate the relationship between FUCA-1 plasma levels and inflammatory disorders diagnosed with subjects recruited in the study. No correlation of FUCA-1 activity was observed in the pediatric patients with immune (p = 0.9677) or metabolic (p = 0.6974) disorders, but a correlation was reported when comparing clusters of chronic inflammatory and autoimmune disease vs. controls (p < 0.05). Furthermore, a relationship was found between FUCA-1 activity in plasma and inflammatory disorders and autoimmunity both in adults and in the pediatric cohort of patients (Pearson test, p = 0.000148). Alterations in plasma levels of FUCA-1 were significantly associated with chronic inflammatory and autoimmune disorders, both in children and adults. The result of the present study should encourage further research on FUCA-1 as a marker of chronic inflammation and autoimmunity.

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Authors and Affiliations

  1. Department of Pediatrics, Josa András County Hospital, Nyíregyháza, Hungary

    Ildikó Endreffy

  2. Council for Nutritional and Environmental Medicine, Toften 24, 8610, Mo i Rana, Norway

    Geir Bjørklund

  3. Department of Haematology, Josa András County Hospital, Nyíregyháza, Hungary

    László Szerafin

  4. Department of Neurological and Movement Sciences, University of Verona, Verona, Italy

    Salvatore Chirumbolo

  5. Department of Zoology, University of Concepcion, Concepción, Chile

    Mauricio A. Urbina

  6. Department of Pediatrics and Pediatric Health Care Center, Faculty of Medicine, University of Szeged, Szeged, Hungary

    Emőke Endreffy

Authors
  1. Ildikó Endreffy
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  2. Geir Bjørklund
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  3. László Szerafin
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  4. Salvatore Chirumbolo
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  5. Mauricio A. Urbina
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  6. Emőke Endreffy
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Contributions

All authors confirmed they have contributed to the intellectual content of this paper and have met the following three requirements: (a) significant contributions to the conception and design, acquisition of data, or analysis and interpretation of data; (b) drafting or revising the article for intellectual content; and (c) final approval of the published article.

Corresponding author

Correspondence to Geir Bjørklund.

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Ethical approval

The local ethics committee and medical board of Jósa András County Hospital approved the study before initiation.

Informed consent

Informed consent was obtained from all individual participants included in the study (from the parents of the children participants and directly from the adult participants).

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Endreffy, I., Bjørklund, G., Szerafin, L. et al. Plasma alpha-L-fucosidase activity in chronic inflammation and autoimmune disorders in a pediatric cohort of hospitalized patients. Immunol Res 65, 1025–1030 (2017). https://doi.org/10.1007/s12026-017-8943-x

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  • DOI: https://doi.org/10.1007/s12026-017-8943-x

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