{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T02:57:23Z","timestamp":1790909843998,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,4,9]],"date-time":"2025-04-09T00:00:00Z","timestamp":1744156800000},"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":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Primary hyperoxaluria type 1 (PH1) is a very rare inherited metabolic disorder characterized by excessive oxalate production due to mutation variants in the alanine\u2010glyoxylate aminotransferase gene (AGXT). Approximately 4% of PH1 cases are diagnosed after kidney transplantation. Most post\u2010transplant recurrences of PH1 are associated with poor graft outcomes. Lumasiran, a novel RNA interference (RNAi) therapeutic for PH1, was recently discovered with promising results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This report describes a pediatric case of PH1 diagnosed post\u2010kidney transplantation with graft dysfunction who was treated with extensive hemodialysis and lumasiran as rescue therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patient was able to stop hemodialysis with the improvement of her kidney function and plasma oxalate after the fourth dose of lumasiran.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This case underscores the importance of maintaining a high index of suspicion for PH1 in patients with congenital anomalies of the kidney and urinary tract (CAKUT) or unexplained end\u2010stage kidney disease (ESKD) cases, even post\u2010transplantation. It also demonstrates the potential efficacy of lumasiran in managing PH1 post\u2010transplantation when combined with intensive hemodialysis and supportive care. However, more studies with prolonged follow\u2010up periods are necessary to establish the long\u2010term efficacy and safety of lumasiran in the treatment of PH1 without the requirement for liver transplantation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.70079","type":"journal-article","created":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T00:29:11Z","timestamp":1744244951000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Hidden in <scp>CAKUT<\/scp>: Post\u2010Transplant Diagnosis of Primary Hyperoxaluria Type 1 and Rescue Management Using Lumasiran"],"prefix":"10.1111","volume":"29","author":[{"given":"Hebatallah","family":"Bahbah","sequence":"first","affiliation":[{"name":"Division of Pediatric Nephrology and Kidney Transplant Multiorgan Transplant Center, King Fahad Specialist Hospital  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Azzam","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology and Kidney Transplant Multiorgan Transplant Center, King Fahad Specialist Hospital  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ammar","family":"Hamed","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology and Kidney Transplant Multiorgan Transplant Center, King Fahad Specialist Hospital  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4451-1198","authenticated-orcid":false,"given":"Najla","family":"Aldaoud","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine King Fahd Specialist Hospital  Dammam Saudi Arabia"},{"name":"Department of Pathology and Microbiology Jordan University of Science and Technology  Irbid Jordan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6199-5174","authenticated-orcid":false,"given":"Alanoud","family":"Alshami","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology and Kidney Transplant Multiorgan Transplant Center, King Fahad Specialist Hospital  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,4,9]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1301564"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1681\/asn.2014070698"},{"key":"e_1_2_6_4_1","first-page":"119","article-title":"The Clinical Pattern of Primary Hyperoxaluria in Pediatric Patient at Queen Rania Abdulla Children Hospital","volume":"7","author":"Almardini R. 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