Mannan binding lectin deficiency and concomitant immunodefects
a Tampere University Hospital, Tampere, Finland:
Department of Clinical Microbiology, b Department of Paediatrics, c Orion Corporation, Espoo, Finland, d Medical School, University of Tampere, Tampere, Finland
Correspondence to: Dr Janne Aittoniemi, Department of Clinical Microbiology, Tampere University Hospital, PO Box 2000, FIN-33521 Tampere, Finland.
Accepted 20 October 1997
OBJECTIVE
To determine the prevalence of a
mannan binding lectin (MBL) deficiency in children with increased
susceptibility to infections and to investigate whether other
coexisting immunodeficiencies affecting opsonisation are needed to
render MBL deficiency clinically significant.
PATIENTS AND METHODS343 serum samples were
collected from 266 children with repeated infections, a single episode
of severe infection, or prolonged symptoms relating to infection. The
concentrations of MBL, immunoglobulin G (IgG), M (IgM), A (IgA), and
IgG subclasses (IgG1-4) were analysed.
RESULTSMBL deficiency was found in nine children
(3.2%), seven of whom had repeated infections or a single episode of
severe infection. In two, initial symptoms and signs suggestive of
infection eventually led to the diagnosis of an autoimmune
diseaseStill's disease in one and pauciarticular juvenile rheumatoid
arthritis in the other. Among the children with MBL deficiency and
infections, concomitant IgG subclass deficiency was detected in five
and a transient low level of one or two IgG subclasses in two.
Prevalence of an IgG subclass deficiency in children with MBL
deficiency was higher than in those without (56% and 22%, respectively).
CONCLUSIONSMBL deficiency alone is not an
independent risk factor for infection but may be manifested in
association with another humoral immunodeficiency affecting
opsonisation. Among children with MBL deficiency, those with juvenile
rheumatoid arthritis were overrepresented. This calls for further study.
© 1998 by Archives of Disease in Childhood
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