Table 1

 Summary of defects in the immune defences

ImmunodeficiencyClinical features
MBL deficiency heterozygoteSusceptibility to Burkholderia complex
Specific pneumococcal antibody deficiencyRecurrent Streptococcus pneumoniae infections
T cell dysregulationAs seen in adult onset antibody deficiency
Arg131/Arg131 homozygous allotype for CD32Associated with poor outcome from Streptococcus pneumoniae bacteraemia