Abstract:
Primary immunodeficiencies (PIDs) remain largely
undiagnosed and underreported in South Africa.1
PIDs are inherited, non-communicable disorders that
involve a defect in one or more components of the immune
system. Immunodeficiencies comprise more than 250
heterogenous disorders that cause either an absent,
defective or weakened function of the immune system.2
These disorders are mostly associated with recurrent
infections that can present very early in childhood, but
depending on the defect, also in later childhood and even
in adulthood.