JOURNAL OF PEDIATRIC INFECTION, cilt.2, sa.1, ss.19-24, 2008 (ESCI)
One of the main function of the immune system is to protect against microorganisms and infections are the hallmark of immunodeficiencies. However, specific symptoms such as hypocalcaemia in DiGeorge syndrome, and bleeding due to thrombocytopenia in WAS or autoimmune manifestations may be presenting features. Other clinical phenotypes have been identified in PIDs including, lymphoproliferative or granulomatous diseases, angioedema, autoinflammation, and hemophagocytosis.