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Mannan binding lectin deficiency and concomitant immunodefects

J Aittoniemi1, M Baer, E Soppi

  • 1Department of Clinical Microbiology, Tampere University Hospital, Finland.

Archives of Disease in Childhood
|June 5, 1998
PubMed
Summary
This summary is machine-generated.

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Mannan-binding lectin (MBL) deficiency in children is not a sole risk factor for infections. It often co-occurs with other humoral immunodeficiencies, particularly in those with autoimmune conditions like juvenile rheumatoid arthritis.

Area of Science:

  • Immunology
  • Pediatrics
  • Clinical Medicine

Background:

  • Recurrent infections in children can indicate underlying immunodeficiencies.
  • Mannan-binding lectin (MBL) plays a role in the innate immune system's opsonisation function.
  • The clinical significance of MBL deficiency alone versus in combination with other deficiencies requires investigation.

Purpose of the Study:

  • To determine the prevalence of MBL deficiency in children with recurrent or severe infections.
  • To assess if MBL deficiency requires coexisting immunodeficiencies to become clinically significant.
  • To explore the association between MBL deficiency and autoimmune diseases in this pediatric cohort.

Main Methods:

  • Serum samples from 266 children with infection-related issues were analyzed.

Related Experiment Videos

  • Concentrations of MBL, immunoglobulins (IgG, IgM, IgA), and IgG subclasses were measured.
  • Statistical comparison of MBL deficiency prevalence and associated conditions was performed.

    • Main Results:

    • MBL deficiency was identified in 3.2% of the children studied.
    • Seven children with MBL deficiency experienced repeated or severe infections.
    • Five of these children also had IgG subclass deficiencies, and two had transient low IgG subclasses.
    • Two children with MBL deficiency were diagnosed with autoimmune diseases (Still's disease, juvenile rheumatoid arthritis).
    • The prevalence of IgG subclass deficiency was significantly higher in children with MBL deficiency (56%) compared to those without (22%).

    Conclusions:

    • MBL deficiency alone is not an independent risk factor for infections.
    • MBL deficiency may manifest clinically when associated with other humoral immunodeficiencies affecting opsonisation.
    • An overrepresentation of juvenile rheumatoid arthritis was noted among children with MBL deficiency, warranting further research.