ABSTRACT
Recurrent infections of the upper respiratory tract are common in children with primary immunodeficiency. Management is with long-term prophylactic antibiotics and antifungals and, with an appropriate donor, bone marrow transplant has been very successful. Transient hypogammaglobulinaemia of infancy (THI) is a diagnosis of exclusion and a definitive diagnosis is only possible over time when the problem resolves. Activity against pneumococcal capsular polysaccharide appears best mediated by the IgG2 subclass which young children may be poor at generating. A small group of children, who present with severe infection and a paucity of lymphoid tissue will have X-linked agammaglobulinaemia. Children with antibody deficiency, THI and specific antibody deficiency with mild infections may be managed with prophylactic antibiotics including amoxicillin, azithromycin and cotrimoxazole. Autoimmune lymphoproliferative syndrome is an uncommon group of conditions associated with defects in the apoptosis pathway. UK national guidelines for Human Immunodeficiency Virus (HIV) testing list recurrent and troublesome ear infections and chronic parotitis.
