Early manifestation and recognition of C2 complement deficiency in the form of pyogenic infection in infancy
| Authors | |
|---|---|
| Year of publication | 2003 |
| Type | Article in Periodical |
| Magazine / Source | J Paediatr Child Health |
| MU Faculty or unit | |
| Citation | |
| Field | Immunology |
| Keywords | complement deficiency; C2 complement component; immunoglobulin deficiency; meningitis |
| Description | Objective: Although frequently asymptomatic, C2 complement component deficiency may lead to severe pyogenic infections or lupus-like illness. Here we describe infectious manifestations in infancy and childhood in our C2 deficient patients. Methodology: Retrospective study of clinical manifestation in 3 patients. C2 deficiency was proved both by undetectable serum C2 level and typical homozygous 28bp deletion of C2 gene. Results: All patients were hospitalised at least once by the age of 12 months, each had one episode of meningitis in infancy, one also had arthritis with septicemia. Infections of the respiratory tract were the causes of other hospitalisations. Two patients also suffered from frequent mild respiratory tract infections, in both decreased IgA and IgG2 or IgG3 levels were recorded. Conclusion: Our observation point to an early manifestation of C2 deficiency within the first year of life, with meningitis as the most severe complication. The severity of immunodeficiency may be influenced by concomitant deficiencies of immunoglobulin isotypes. |
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