Severe Combined Immunodeficiency

Immunodeficiency

“…Imaging studies may indicate the impact of immunodeficiency on body organs and systems, including the brain, thymus, heart, lymph nodes, blood vessels, lungs, sinuses, liver, spleen, kidneys, gastrointestinal tract, muscles, bones, and joints 2,6,8. Infants with suspected immunodeficiency should be screened through a blood test called T-cell receptor excision circle screening 14,15. T lymphocytes (T cells) may be reduced from thymic dysfunction, causing SCID, predisposing the infant to life-threatening infection 14,15…”

“…Infants with suspected immunodeficiency should be screened through a blood test called T-cell receptor excision circle screening 14,15. T lymphocytes (T cells) may be reduced from thymic dysfunction, causing SCID, predisposing the infant to life-threatening infection 14,15…”

“…Acute infection should be treated promptly after an evaluation of blood cultures 8–11. People most prone to severe infection, such as in SCID and asplenia, may receive prophylactic antibiotics 14,15. Intravenous immune globulin or subcutaneous immune globulin is the standard replacement therapy in patients with PID and SID 2,6,33…”

“…16,20 Since B-lymphocyte antibody production requires proper T-lymphocyte functioning, most T-lymphocyte immunodeficiencies cause a dual B-lymphocyte and T-lymphocyte PID disorder. 14 Examples of PID disease classifications include B-lymphocyte immunodeficiency (adaptive), T-lymphocyte immunodeficiency (adaptive), severe combined immunodeficiency deficiency (adaptive), phagocyte disorders (innate), and complement defects (innate). 6,[13][14][15] The type of infection and clinical presentation will vary based on a specific PID.…”

“…14 Examples of PID disease classifications include B-lymphocyte immunodeficiency (adaptive), T-lymphocyte immunodeficiency (adaptive), severe combined immunodeficiency deficiency (adaptive), phagocyte disorders (innate), and complement defects (innate). 6,[13][14][15] The type of infection and clinical presentation will vary based on a specific PID. 6,[13][14][15] Bacterial infections are common with B-lymphocyte deficiencies, while viral, fungal, and bacterial infections are common in dual B-lymphocyte and T-lymphocyte deficiencies.…”