the primary immunodeficiency (pi) disorders are abnormalities in development and maturation of the immune system. individuals with pi disease may experience frequent infections, which limit their abilities to exhibit physical and psychological well-being secondary to their illness. in this survey we compared health-related quality of life of primary immune deficient patients with healthy childr...

conclusions subcutaneous nodules in the absence of a local reaction at the site of bcg vaccination may be the sole manifestation of disseminated bcg disease. case presentation we report on an 11-months-old boy with prolonged fever and diffuse hyperpigmented subcutaneous nodules. due to lymphopenia, oral thrush and severe adverse reaction to bcg vaccination, the possibility of primary immunodefi...

coincidence of autoimmune diseases such as immune thrombocytopenic purpura (itp) with  immunodeficiencies has  been  reported  previously in  patients  who  suffered  from primary antibody deficiency (pad). but there is no original study on immunological profiles of itp patients to find out their probable immune deficiency. in this case-control study, itp patients’ humoral immunity was investig...

common variable immunodeficiency (cvid) and selective iga deficiency (sigad) are the most common primary antibody deficiencies. these two diseases may have coincidence in one family and sigad can progress to cvid which suggest common underlying genetic defects between sigad and cvid. this study was designed to find the prevalence of multiple cases in families of iranian patients with cvid or si...

primary immunodeficiency diseases (pid) consist of a group of long-term illnesses which had permanent psychiatric effects on the patients and their parents. this study was designed to find out the most important origins and aspects of stressor in parents of pid patients.to assess the  impact of  psychiatric aspects in parents  of pid  patients, a valid and reliable questionnaire was compiled ba...

conclusions disseminated bcg infection should be considered in the differential diagnosis of infants and young children with fever, hepatosplenomegaly, lymphadenopathy, and history of bcg vaccination. the pcr method has a high positivity rate and can serve as a useful tool for the rapid and specific identification of m. bovis bcg substrain infection. results from the total of 34 children hospit...

In this issue of Blood,Weller et al studied patients with rare immunodeficiencies to demonstrate a role for Toll-like receptors (TLRs) in homeostasis of human CD27+IgM+IgD+B cells.