{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T08:58:46Z","timestamp":1790413126566,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T00:00:00Z","timestamp":1783987200000},"content-version":"vor","delay-in-days":13,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Primary hyperoxaluria (PH) is a rare autosomal recessive disorder characterized by excessive oxalate production, leading to nephrolithiasis, nephrocalcinosis, and kidney failure. Data from large pediatric cohorts remain limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This national, multicenter, retrospective study included 88 pediatric patients with PH followed in 15 centers in T\u00fcrkiye between 2010 and 2024. Clinical, genetic, and treatment\u2010related data were collected via a web\u2010based registry, and disease progression and outcomes were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The median age at diagnosis was 2.6 (IQR: 0.5\u20138.4) years in PH1, with an approximate diagnostic delay of 2\u2009years. At diagnosis, nearly half (49.4%) had kidney failure. Genetic diagnosis was performed in 59 (71.1%) patients. The most frequently detected variants were p.Val324Glyfs (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200912, 20.3%) and p.Gly170Arg (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20099, 15.2%). During a median follow\u2010up of 58\u2009months (IQR: 24\u2013109), dialysis was initiated at 51.8% and mortality reached 27.7%, particularly among patients presenting with kidney failure. Pyridoxine was used in 55.4% of patients, although response rates were limited, partly due to late diagnosis and non\u2010genotype\u2010guided use. Transplantation was performed in 24.1% of patients. Sequential liver\u2013kidney transplantation provided favorable long\u2010term renal outcomes and remains the most effective treatment for advanced disease by correcting the underlying metabolic defect. Patients with PH2 and PH3 exhibited a milder clinical course, preserved kidney function, and no mortality during follow\u2010up.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Delayed diagnosis remains a major challenge in pediatric PH. Early recognition, genotype\u2010guided therapy, and timely implementation of appropriate transplantation strategies are essential to improve outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/petr.70401","type":"journal-article","created":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T11:42:32Z","timestamp":1784029352000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical Characteristics, Genetic Spectrum, and Treatment Outcomes in Children With Primary Hyperoxaluria: Results From a National Turkish Registry"],"prefix":"10.1111","volume":"30","author":[{"given":"Elifsu G\u00f6zde","family":"Akb\u00f6r\u00fc","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Department of Pediatrics Gazi University  Ankara T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emre","family":"Levento\u011flu","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pediatrics, Division of Nephrology Gazi University  Ankara T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seda","family":"P\u0131narba\u015f\u0131","sequence":"additional","affiliation":[{"name":"Department of Pediatric Nephrology Diyarbak\u0131r Children Hospital  Diyarbak\u0131r T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehtap Akbal\u0131k","family":"Kara","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pediatric Nephrology Gaziantep University  Gaziantep T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beltinge Demircio\u011flu","family":"K\u0131l\u0131\u00e7","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pediatric Nephrology Gaziantep University  Gaziantep T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y\u0131lmaz","family":"Tabel","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pediatric Nephrology \u0130n\u00f6n\u00fc University  Malatya T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2644-5628","authenticated-orcid":false,"given":"Aysun Karabay","family":"Beyaz\u0131t","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pediatric Nephrology \u00c7ukurova University  Adana T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nurdan","family":"Y\u0131ld\u0131z","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Department of Pediatric Nephrology Marmara University  \u0130stanbul 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