Article: Prospective immunological profiling in a case of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX).
Clinical and experimental immunology
2004 Volume 137, Issue 2, Page(s) 373–378
Abstract: IPEX syndrome is a genetic autoimmune disease characterized by immune-mediated polyendocrinopathy, enteropathy, and X-linked inheritance. We describe a case of IPEX in which lymphocyte phenotypes were assessed at birth, before initiation of Cyclosporin A ...
Abstract | IPEX syndrome is a genetic autoimmune disease characterized by immune-mediated polyendocrinopathy, enteropathy, and X-linked inheritance. We describe a case of IPEX in which lymphocyte phenotypes were assessed at birth, before initiation of Cyclosporin A therapy, and at frequent intervals to 18 months of age. We performed flow cytometry for lymphocyte subtypes and for activation markers (HLA-DR, CD25, and CD69 or CD71). The ratios of both T to B cells and CD4+ to CD8+ cells were elevated at birth, but CD4+ cells were not activated. HLA-DR+ and CD25+ activated T-cells increased in association with two episodes of clinical deterioration: colitis and the onset of type I diabetes mellitus. These results indicate that measures of activation, particularly HLA-DR+ and CD25+ frequency, correlate well with the development of early active disease and may presage clinical episodes. Continuous maintenance of immunosuppression, once started, appears critical for prevention of permanent tissue damage. |
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MeSH term(s) | Follow-Up Studies ; Genetic Diseases, X-Linked/immunology ; Humans ; Immunophenotyping ; Infant, Newborn ; Lymphocyte Activation/immunology ; Male ; Polyendocrinopathies, Autoimmune/immunology ; Protein-Losing Enteropathies/immunology ; Syndrome ; T-Lymphocyte Subsets/immunology |
Language | English |
Publishing date | 2004-08 |
Publishing country | England |
Document type | Case Reports ; Journal Article |
ZDB-ID | 218531-3 |
ISSN | 1365-2249 ; 0009-9104 ; 0964-2536 |
ISSN (online) | 1365-2249 |
ISSN | 0009-9104 ; 0964-2536 |
DOI | 10.1111/j.1365-2249.2004.02537.x |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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