Medical Genetics Lymphadenopathy and autoimmunity may be present Susceptibility to major gram-positive pathogens and opportunistic nfections is increased. Most patients 70%)develop chronic liver disease by age 30Medical Genetics Lymphadenopathy and autoimmunity may be present. Susceptibility to major gram-positive pathogens and opportunistic infections is increased. Most patients (> 70%) develop chronic liver disease by age 30