{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T12:01:16Z","timestamp":1777636876967,"version":"3.51.4"},"reference-count":41,"publisher":"American Society of Hematology","issue":"2","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2011,1,13]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>FOXN1 deficiency is a primary immunodeficiency characterized by athymia, alopecia totalis, and nail dystrophy. Two infants with FOXN1 deficiency were transplanted with cultured postnatal thymus tissue. Subject 1 presented with disseminated Bacillus Calmette-Gu\u00e9rin infection and oligoclonal T cells with no naive markers. Subject 2 had respiratory failure, human herpes virus 6 infection, cytopenias, and no circulating T cells. The subjects were given thymus transplants at 14 and 9 months of life, respectively. Subject 1 received immunosuppression before and for 10 months after transplantation. With follow up of 4.9 and 2.9 years, subjects 1 and 2 are well without infectious complications. The pretransplantation mycobacterial disease in subject 1 and cytopenias in subject 2 resolved. Subject 2 developed autoimmune thyroid disease 1.6 years after transplantation. Both subjects developed functional immunity. Subjects 1 and 2 have 1053\/mm3 and 1232\/mm3 CD3+ cells, 647\/mm3 and 868\/mm3 CD4+ T cells, 213\/mm3 and 425\/mm3 naive CD4+ T cells, and 10 200 and 5700 T-cell receptor rearrangement excision circles per 100 000 CD3+ cells, respectively. They have normal CD4 T-cell receptor \u03b2 variable repertoires. Both subjects developed antigen-specific proliferative responses and have discon-tinued immunoglobulin replacement. In summary, thymus transplantation led to T-cell reconstitution and function in these FOXN1 deficient infants.<\/jats:p>","DOI":"10.1182\/blood-2010-06-292490","type":"journal-article","created":{"date-parts":[[2010,10,26]],"date-time":"2010-10-26T22:55:01Z","timestamp":1288133701000},"page":"688-696","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":108,"title":["First use of thymus transplantation therapy for FOXN1 deficiency (nude\/SCID): a report of 2 cases"],"prefix":"10.1182","volume":"117","author":[{"given":"M. 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