{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T02:08:54Z","timestamp":1768529334319,"version":"3.49.0"},"reference-count":15,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T00:00:00Z","timestamp":1764460800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2025,12]]},"abstract":"ABSTRACT<\/jats:title>\n \n Background<\/jats:title>\n \n Schimke Immuno\u2010Osseous Dysplasia Is a Rare Autosomal Recessive Multisystem Disorder Caused by Biallelic Pathogenic Variants in the\n \n SMARCAL1<\/jats:italic>\n <\/jats:styled-content>\n Gene, Which Encodes a\n DNA<\/jats:styled-content>\n Annealing Helicase Essential for Replication Fork Stability and Genomic Maintenance. Loss of\n \n SMARCAL1<\/jats:italic>\n <\/jats:styled-content>\n Function Leads to Genomic Instability, Resulting in a Characteristic Clinical Triad of Disproportionate Short Stature, Steroid\u2010Resistant Nephrotic Syndrome and Immunodeficiency. Kidney Transplantation Is the Standard Treatment for End\u2010Stage Renal Disease in Schimke Immuno\u2010Osseous Dysplasia. However, the Underlying Genomic Fragility and Immunodeficiency Heighten the Risk of Post\u2010Transplant Complications, Particularly Epstein\u2013Barr Virus\u2010Associated Post\u2010Transplant Lymphoproliferative Disorder.\n <\/jats:p>\n <\/jats:sec>\n \n Method<\/jats:title>\n We Report the Case of a 7\u2010Year\u2010Old Girl With Genetically Confirmed Schimke Immuno\u2010Osseous Dysplasia Who Developed Epstein\u2013Barr Virus\u2010Positive Diffuse Large B\u2010Cell Lymphoma Eight Months Following Maternal Kidney Transplantation, Despite Reduced\u2010Intensity Immunosuppression. Rituximab Therapy Resulted in Complete Remission, but Required the Withdrawal of all Immunosuppressive Agents, Thereby Posing a Risk of Kidney Graft Loss.<\/jats:p>\n <\/jats:sec>\n \n Result<\/jats:title>\n In the Context of Primary Immunodeficiency and the Development of Post\u2010Transplant Lymphoproliferative Disorder in Such a Setting, the Patient Subsequently Underwent Haploidentical Hematopoietic Stem Cell Transplantation From the Mother, Using an \u03b1\u03b2 T\u2010Cell\u2010Depleted Graft and a Nephrotoxicity\u2010Sparing Conditioning Regimen. Full Donor Chimerism Was Achieved by Day 28. Immune Reconstitution Occurred Within One Year. At 27\u2009Months After Hematopoietic Stem Cell Transplantation She Remains in Remission With Preserved Kidney Function and no Ongoing Immunosuppression.<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n This Case Highlights the Feasibility of Haploidentical Hematopoietic Stem Cell Transplantation as a Curative Salvage Strategy for Immunosuppression\u2010Free Survival After Kidney Transplantation in Patients With Schimke Immuno\u2010Osseous Dysplasia.<\/jats:p>\n <\/jats:sec>","DOI":"10.1111\/petr.70234","type":"journal-article","created":{"date-parts":[[2025,12,1]],"date-time":"2025-12-01T04:44:53Z","timestamp":1764564293000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Successful Use of Haploidentical\n HSCT<\/scp>\n in a Child With Schimke Immuno\u2010Osseous Dysplasia Who Developed\n PTLD<\/scp>\n After Kidney Transplantation"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6949-8922","authenticated-orcid":false,"given":"Cemile","family":"Pehlivano\u011flu","sequence":"first","affiliation":[{"name":"Pediatric Nephrology Department Ko\u00e7 University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4475-0407","authenticated-orcid":false,"given":"Fatih","family":"Erbey","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology & Bone Marrow Transplantation Unit Ko\u00e7 University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-2416-9566","authenticated-orcid":false,"given":"\u00d6zlem","family":"\u00dcnl\u00fcgedik","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Ko\u00e7 University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0597-7350","authenticated-orcid":false,"given":"Ba\u015fak","family":"Akyollu","sequence":"additional","affiliation":[{"name":"Prof. Dr. M\u00fcnci Kalayo\u011flu Organ Transplant Center Ko\u00e7 University Hospital Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4802-0986","authenticated-orcid":false,"given":"Ebru","family":"Y\u0131lmaz","sequence":"additional","affiliation":[{"name":"KANKA, Pediatric Hematology\/Oncology & Bone Marrow Transplantation Center Erciyes University Faculty of Medicine Kayseri Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2015-3541","authenticated-orcid":false,"given":"Musa","family":"Karak\u00fck\u00e7\u00fc","sequence":"additional","affiliation":[{"name":"KANKA, Pediatric Hematology\/Oncology & Bone Marrow Transplantation Center Erciyes University Faculty of Medicine Kayseri Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7212-7041","authenticated-orcid":false,"given":"Banu Oflaz","family":"S\u00f6zmen","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology & Bone Marrow Transplantation Unit Ko\u00e7 University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0561-3343","authenticated-orcid":false,"given":"Sevgi Bilgi\u00e7","family":"Eltan","sequence":"additional","affiliation":[{"name":"Pediatric Allergy and Immunology Marmara University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6805-5313","authenticated-orcid":false,"given":"Nurdan","family":"Y\u0131ld\u0131z","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Department Marmara University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0312-2447","authenticated-orcid":false,"given":"Burak","family":"Ko\u00e7ak","sequence":"additional","affiliation":[{"name":"Prof. Dr. M\u00fcnci Kalayo\u011flu Organ Transplant Center Ko\u00e7 University Hospital Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4852-989X","authenticated-orcid":false,"given":"Ilmay","family":"Bilge","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology Department Ko\u00e7 University Faculty of Medicine Istanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,11,30]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng821"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2008.060095"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.044313"},{"key":"e_1_2_11_5_1","unstructured":"E.Lippner T.L\u00fccke C.Salgado C. 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