{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T16:22:46Z","timestamp":1779294166511,"version":"3.51.4"},"reference-count":13,"publisher":"American Academy of Pediatrics (AAP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2008,4,1]]},"abstract":"<jats:p>Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome is a rare X-linked disorder that usually presents in early childhood with immune enteropathy, diabetes mellitus, and other autoimmune complications. The disease is caused by mutations in the forkhead box P3 gene, a transcription factor that is essential for the development and function of regulatory T cells. This population of cells plays an essential role in controlling immune responses and preventing autoimmunity. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome is often initially treated with immunosuppressive drugs, but only allogeneic hematopoietic stem cell transplantation has offered the possibility of cure. We recently performed an unrelated donor transplant in a child with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome by using a reduced-intensity conditioning regimen. This transplant provided a rare opportunity to gain valuable insight into the regeneration of the immune system after transplantation. Clinical recovery was associated with the emergence of regulatory T cell populations, the majority of which expressed memory phenotype markers and raised important questions about the origin and longevity of the FOXP3+ regulatory T cell pool.<\/jats:p>","DOI":"10.1542\/peds.2007-1863","type":"journal-article","created":{"date-parts":[[2008,3,4]],"date-time":"2008-03-04T01:27:09Z","timestamp":1204594029000},"page":"e998-e1002","source":"Crossref","is-referenced-by-count":45,"title":["Immune Reconstitution and Recovery of FOXP3 (Forkhead Box P3)-Expressing T Cells After Transplantation for IPEX (Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-linked) Syndrome"],"prefix":"10.1542","volume":"121","author":[{"given":"Hong","family":"Zhan","sequence":"first","affiliation":[{"name":"Institute of Child Health"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jo","family":"Sinclair","sequence":"additional","affiliation":[{"name":"Institute of Child Health"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stuart","family":"Adams","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital, London, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catherine M.","family":"Cale","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital, London, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon","family":"Murch","sequence":"additional","affiliation":[{"name":"Warwick Medical School, Coventry, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucia","family":"Perroni","sequence":"additional","affiliation":[{"name":"Lucia Perroni, Human Genetics, Galliera Hospital, Genova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Graham","family":"Davies","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital, London, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Persis","family":"Amrolia","sequence":"additional","affiliation":[{"name":"Institute of Child Health"},{"name":"Great Ormond Street Hospital, London, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Waseem","family":"Qasim","sequence":"additional","affiliation":[{"name":"Institute of Child Health"},{"name":"Great Ormond Street Hospital, London, United Kingdom"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"417","published-online":{"date-parts":[[2008,4,1]]},"reference":[{"key":"2021120902123630700_R1","doi-asserted-by":"crossref","unstructured":"Wildin RS, Smyk-Pearson S, Filipovich AH. 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