{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T23:30:45Z","timestamp":1768519845164,"version":"3.49.0"},"reference-count":14,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2016,9,26]],"date-time":"2016-09-26T00:00:00Z","timestamp":1474848000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">SIOD<\/jats:styled-content> is rare disorder related to <jats:styled-content style=\"fixed-case\">SMARCAL<\/jats:styled-content>1 or <jats:styled-content style=\"fixed-case\">SMARCAL<\/jats:styled-content>2 gene mutation, including (among other comorbidities) T\u2010cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11\u2010year\u2010old male with <jats:styled-content style=\"fixed-case\">SIOD<\/jats:styled-content>, who presented <jats:styled-content style=\"fixed-case\">ITP<\/jats:styled-content> at 2\u00a0years after renal transplantation with decrease in platelet count (from normal) to 56\u00a0000\/\u03bcL and then (gradually) to 2000\/\u03bcL. There was no effect of iv. methylprednisolone\/dexamethasone. As the presence of antibodies against <jats:styled-content style=\"fixed-case\">GPII<\/jats:styled-content>b\/<jats:styled-content style=\"fixed-case\">III<\/jats:styled-content>a, <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>b, and <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>a<jats:styled-content style=\"fixed-case\">II<\/jats:styled-content>a platelet glycoproteins was confirmed, patient was given 50\u00a0g of <jats:styled-content style=\"fixed-case\">IVIG<\/jats:styled-content> and then was put on plasmapheresis; however, both showed poor direct effect. As we were afraid to give rituximab (due to expected overimmunosuppression), we prescribed the oral <jats:styled-content style=\"fixed-case\">TPO<\/jats:styled-content>\u2010receptor agonist (eltrombopag). Patient responded after 17\u00a0days of therapy, to the final dose of 50\u00a0mg\/d (approx. 2\u00a0mg\/kg). The antiplatelet antibodies disappeared after four plasmapheresis. Overall, the therapy was continued for 7\u00a0weeks and was stopped at platelet count of 433\u00a0000\/\u03bcL. Platelet count remained stable in 8\u2010month follow\u2010up. Combination of plasmapheresis and <jats:styled-content style=\"fixed-case\">TPO<\/jats:styled-content>\u2010receptor agonist was effective in post\u2010renal transplant acute <jats:styled-content style=\"fixed-case\">ITP<\/jats:styled-content> in patient with <jats:styled-content style=\"fixed-case\">SIOD<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/petr.12828","type":"journal-article","created":{"date-parts":[[2016,9,27]],"date-time":"2016-09-27T04:20:00Z","timestamp":1474950000000},"page":"1148-1151","source":"Crossref","is-referenced-by-count":6,"title":["Eltrombopag (thrombopoietin\u2010receptor agonist) and plasmapheresis as rescue therapy of acute post\u2010renal transplant immune thrombocytopenia in a child with Schimke immuno\u2010osseous dysplasia\u2014case report"],"prefix":"10.1111","volume":"20","author":[{"given":"Ryszard","family":"Grenda","sequence":"first","affiliation":[{"name":"Department of Nephrology, Kidney Transplantation and Hypertension The Children's Memorial Health Institute  Warsaw Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wioletta","family":"Jarmu\u017cek","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Kidney Transplantation and Hypertension The Children's Memorial Health Institute  Warsaw Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna","family":"Latoszy\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Kidney Transplantation and Hypertension The Children's Memorial Health Institute  Warsaw Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylwester","family":"Prokurat","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Kidney Transplantation and Hypertension The Children's Memorial Health Institute  Warsaw Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacek","family":"Rubik","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Kidney Transplantation and Hypertension The Children's Memorial Health Institute  Warsaw Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,9,26]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng821"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004310050001"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2008.01013.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000133624.65946.40"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2005.10.002"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2006.03.010"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/1546\u20100096\u20109\u201027"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1532\/IJH97.E0213"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.31089"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23132"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-08-302984"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-06-225565"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2006.041426"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa073275"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpetr.12828","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpetr.12828","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.12828","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T02:13:08Z","timestamp":1696039988000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.12828"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9,26]]},"references-count":14,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2016,12]]}},"alternative-id":["10.1111\/petr.12828"],"URL":"https:\/\/doi.org\/10.1111\/petr.12828","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9,26]]}}}