Successful fetal liver transplantation in a child with severe combined immunodeficiency.
Following transplantation with allogeneic fetal liver, a boy with a severe combined immunodeficiency and adenosine-deaminase (A.D.A.) deficiency developed immunocompetent T and B cells in an orderly manner. Engraftment was indicated by appearance of A.D.A. activity and donor-lymphocyte and red-cell antigens. The child remained free of major infections until one year later when he developed an ultimately fatal nephrotic syndrome associated with immune-complex glomerulonephritis.
D000243:Adenosine Deaminase / Q000172:deficiency*; D000328:Adult; D000917:Antibody Formation; D001402:B-Lymphocytes / Q000276:immunology; D003601:Cytotoxicity Tests, Immunologic; D005260:Female; D005333:Fetus; D006680:HLA Antigens / Q000302:isolation & purification; D006801:Humans; D007104:Immune Adherence Reaction; D007136:Immunoglobulins / Q000302:isolation & purification; D007153:Immunologic Deficiency Syndromes / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D007958:Leukocyte Count; D016031:Liver Transplantation; D008231:Lymphopenia / Q000097:blood / Q000628:therapy; D008297:Male; D009700:Nucleoside Deaminases / Q000172:deficiency*; D011247:Pregnancy; D013601:T-Lymphocytes / Q000276:immunology; D014184:Transplantation, Homologous
D006680:HLA Antigens; D007136:Immunoglobulins; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase