OBJECTIVES:
Primary immune deficiency is relatively rare.
Patients present with recurrent or
persistent infections or
infections with opportunistic pathogens. We investigated
patients who presented during the years 2005-7 with recurrent or
persistent infections or
infections with opportunistic organisms, for underlying immune
deficiency.
DESIGN:
Descriptive study.
SETTING:
Department of
Immunology,
Medical Research Institute, Colombo. STUDY
POPULATION 257
patients referred to the Department of
Immunology,
Medical Research Institute, Colombo, with a
history of
recurrent infections, for evaluation of possible immune
deficiency. MEASUREMENTS Appropriate evaluation of
immunological competence of the humoral and
cell mediated
immune systems.
RESULTS:
There were 8
patients with agammaglobulinaemia (X linked agammaglobulinaemia and autosomal recessive agammaglobulinaemia), 2
patients each with
ataxia telangiectasia,
IgA deficiency and
hyper-IgE syndrome, 3
patients with
common variable immune deficiency (CVID), and 1
patient each with Griscelli
syndrome,
hyper-IgM syndrome and X linked
severe combined immune deficiency (SCID).
CONCLUSIONS:
Primary immune deficiency must be included in the evaluation of
patients with
recurrent infections, and timely intervention can prevent
morbidity and
mortality.