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1.
Pediatr Transplant ; 10(6): 744-6, 2006 Sep.
Article in English | MEDLINE | ID: mdl-16911501

ABSTRACT

Severe combined immunodeficiency (SCID) represents a group of rare, sometimes fatal, congenital disorders in which there is a combined absence of T-lymphocyte and B-lymphocyte function. Children with SCID die within two years of age, if untreated. The effective treatment for SCID is a hematopoietic stem cell transplantation (HSCT). It has been repeatedly described that in peripheral blood of infants with SCID maternal T cells can be found. Here we report a case of blood chimerism in a one-year-old boy with SCID.


Subject(s)
Chimerism , Severe Combined Immunodeficiency/genetics , Alleles , Humans , Infant , Male , Polymerase Chain Reaction
2.
Transplantation ; 81(9): 1298-305, 2006 May 15.
Article in English | MEDLINE | ID: mdl-16699458

ABSTRACT

BACKGROUND: Patients whose kidney grafts fail develop alloantibodies that react with many HLA molecules. We analyzed the epitope specificity of HLA class I alloantibodies in the sera of 55 patients who had been sensitized by kidney grafts, and investigated the immunogenicity of various polymorphic epitopes. METHODS: HLA class I alloantibodies were detected and characterized by flow cytometry (FlowPRA beads). Potential "immunizing epitopes" were identified by comparing the amino acid sequences of HLA class I antigens/alleles of the donor, recipient and the antibody-reactivity pattern. RESULTS: In the 55 anti-HLA class I-positive patients, 82 different antibody reactivity patterns were identified; all but 5 (94%) were determined by a "public epitope" of donor HLA-A and/or -B molecules. Forty-five of 50 patients who showed HLA-A Res-MMs with their donors produced HLA-A antibodies, but only 31 of 51 subjects with HLA-B Res-MMs produced HLA-B antibodies (P=0.001; O.R.=5.81). The antibody patterns were specific for a "single" epitope of the mismatched donor molecules in 91% of patients. Forty-three of the 120 (36%) mismatched HLA-A and/or -B epitopes were positively correlated with antibody production. The polymorphic determinants of higher immunogenic capacity were b80N (Bw6-associated) and ab82-83LR (Bw4-associated) public epitopes. CONCLUSIONS: The humoral immune response against a kidney graft mainly produces HLA class I antibodies specific for "public epitopes" of mismatched donor molecules. A "single" donor-epitope may determine the production of a spread antibody pattern. In renal transplantation, epitope matching is better than HLA antigen matching for avoiding or minimizing development of HLA antibodies.


Subject(s)
Epitopes/analysis , Histocompatibility Antigens Class I/immunology , Isoantibodies/blood , Kidney Transplantation/immunology , Antibody Specificity , Antigen-Antibody Reactions , Flow Cytometry , HLA-A Antigens/immunology , HLA-B Antigens/immunology , Histocompatibility Antigens Class I/genetics , Humans , Immunoglobulin G/blood , Reoperation , Treatment Failure
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