Subject(s)
Anti-Retroviral Agents/therapeutic use , Chemoprevention/methods , Disease Transmission, Infectious/prevention & control , HIV Infections/prevention & control , HIV Infections/transmission , Post-Exposure Prophylaxis/methods , Transfusion Reaction , Anemia, Sickle Cell/therapy , Child , DNA, Viral/blood , Female , HIV/isolation & purification , HIV Antibodies/blood , HIV Antigens/blood , Humans , RNA, Viral/bloodABSTRACT
This report described a 6-month-old boy who presented with miliary tuberculosis and severe CD8 deficiency, which is most likely secondary to miliary tuberculosis since his CD8 was back to normal 6 month after therapy and primary CD8 deficiency, (ZAP 70 defect) was ruled out. To our knowledge this is the first paediatric case reported with severe CD8 deficiency secondary to miliary TB.
Subject(s)
CD8 Antigens/blood , Severe Combined Immunodeficiency/etiology , Tuberculosis, Miliary/complications , Antitubercular Agents/therapeutic use , CD8 Antigens/immunology , Humans , Infant , Male , Severe Combined Immunodeficiency/blood , Tuberculosis, Miliary/drug therapy , Tuberculosis, Miliary/immunologyABSTRACT
Members of the Toll-like receptor (TLR) and interleukin-1 receptor (IL-1R) superfamily share an intracytoplasmic Toll-IL-1 receptor (TIR) domain, which mediates recruitment of the interleukin-1 receptor-associated kinase (IRAK) complex via TIR-containing adapter molecules. We describe three unrelated children with inherited IRAK-4 deficiency. Their blood and fibroblast cells did not activate nuclear factor kappaB and mitogen-activated protein kinase (MAPK) and failed to induce downstream cytokines in response to any of the known ligands of TIR-bearing receptors. The otherwise healthy children developed infections caused by pyogenic bacteria. These findings suggest that, in humans, the TIR-IRAK signaling pathway is crucial for protective immunity against specific bacteria but is redundant against most other microorganisms.