Mannan binding lectin deficiency and concomitant immunodefects

被引:43
作者
Aittoniemi, J
Baer, M
Soppi, E
Vesikari, T
Miettinen, A
机构
[1] Tampere Univ Hosp, Dept Clin Microbiol, FIN-33521 Tampere, Finland
[2] Tampere Univ Hosp, Dept Paediat, FIN-33521 Tampere, Finland
[3] Orion Corp Farmos, Espoo, Finland
[4] Tampere Univ, Sch Med, FIN-33101 Tampere, Finland
关键词
mannan binding lectin; IgG subclasses; immunodeficiency; opsonisation;
D O I
10.1136/adc.78.3.245
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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 methods-343 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 (IgG(1-4)) were analysed. Results-MBL 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 disease-Still'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). Conclusions-MBL 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.
引用
收藏
页码:245 / 248
页数:4
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