{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T02:34:48Z","timestamp":1768530888220,"version":"3.49.0"},"reference-count":22,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T00:00:00Z","timestamp":1694390400000},"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":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Schimke immuno\u2010osseous dysplasia (SIOD) is a rare systemic disease characterized by short stature, proteinuria, and recurrent infections. Patients usually have spondyloepiphyseal dysplasia, and progressive steroid\u2010resistant nephropathy that leads to kidney failure. However, their clinical course after kidney transplantation (KT) is not yet well known. Here, we present our experience with cases of SIOD treated at our institute.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Presentation<\/jats:title><jats:p>Since 2014, three children have been diagnosed with nephropathy resulting from SIOD. They presented with proteinuria in the nephrotic range at 7, 5, and 3\u2009years of age. Focal segmental glomerulosclerosis was confirmed and progressed to kidney failure approximately 2\u2009years after proteinuria was detected. These patients underwent living\u2010donor KT from their parents. After KT, Case 1 lost his graft within 7\u2009months due to multi\u2010organ failure caused by disseminated adenovirus infection and died. Case 2 experienced graft failure 5\u2009years after KT due to acute rejection from poor compliance. In Case 3, the allograft was still functioning 6\u2009years after KT with low\u2010dose tacrolimus single medication (trough level\u2009&lt;\u20095\u2009ng\/mL). Extra\u2010renal manifestations progressed regardless of KT, namely, right renal vein thrombosis and pulmonary hypertension in Case 1, severe bilateral hip dysplasia and Moyamoya syndrome in Case 2, and neutropenia and thrombocytopenia in Case 3, in addition to recurrent infection.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In SIOD patients, KT is complicated with recurrent infections due to their inherent immune dysfunction. Additionally, extra\u2010renal symptoms may render the patients morbid despite the recovery of kidney function.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14605","type":"journal-article","created":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T07:20:18Z","timestamp":1694416818000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Clinical course of post\u2010kidney transplant Schimke immuno\u2010osseous dysplasia"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5800-2090","authenticated-orcid":false,"given":"Hyun Ah.","family":"Woo","sequence":"first","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8003-3010","authenticated-orcid":false,"given":"Seong Heon","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8185-4408","authenticated-orcid":false,"given":"Yo Han","family":"Ahn","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0688-0278","authenticated-orcid":false,"given":"Sang Il","family":"Min","sequence":"additional","affiliation":[{"name":"Department of Surgery Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2285-3517","authenticated-orcid":false,"given":"Jongwon","family":"Ha","sequence":"additional","affiliation":[{"name":"Department of Surgery Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5428-6209","authenticated-orcid":false,"given":"Il\u2010Soo","family":"Ha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7556-1265","authenticated-orcid":false,"given":"Hae Il","family":"Cheong","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8323-5320","authenticated-orcid":false,"given":"Hee Gyung","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital and Seoul National University College of Medicine  Seoul Korea"},{"name":"Kidney Research Institute Seoul National University Medical Research Center  Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,9,11]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(71)90400-4"},{"key":"e_1_2_10_3_1","volume-title":"GeneReviews((R))","author":"Morimoto 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