{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T06:49:07Z","timestamp":1791614947506,"version":"4.3.4"},"reference-count":15,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T00:00:00Z","timestamp":1791504000000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"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":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Familial hypomagnesemia with hypercalciuria and nephrocalcinosis is a rare autosomal recessive tubulopathy caused by mutations in\n                      <jats:italic>CLDN16<\/jats:italic>\n                      or\n                      <jats:italic>CLDN19<\/jats:italic>\n                      , leading to progressive chronic kidney disease. While kidney transplantation is the definitive treatment, the safety of using living\u2010related heterozygous carrier donors remains debated due to potential subclinical tubular dysfunction. We report the 2\u2010year follow\u2010up of a pediatric patient with FHHNC and a\n                      <jats:italic>CLDN19<\/jats:italic>\n                      mutation who received a graft from his heterozygous carrier mother.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Report<\/jats:title>\n                    <jats:p>\n                      A male patient presented at 3\u2009months of age with nephrocalcinosis, hypercalciuria, and hypomagnesemia. Genetic analysis confirmed a homozygous CLDN19 (G179S) mutation. Consistent with the\n                      <jats:italic>CLDN19<\/jats:italic>\n                      phenotype, the patient exhibited severe ocular involvement, including high myopia and macular coloboma. He progressed to end\u2010stage kidney disease by age 15 and underwent living\u2010related kidney transplantation from his mother (a heterozygous carrier) at age 16. Pre\u2010operative donor evaluation showed normal renal function without proteinuria, hypercalciuria, nephrolithiasis, or nephrocalcinosis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      At 24\u2009months post\u2010transplantation, graft function remained stable with a serum creatinine of 1.22\u2009mg\/dL and a 24\u2010h creatinine clearance of 94\u2009mL\/min\/1.73\u2009m\n                      <jats:sup>2<\/jats:sup>\n                      . Metabolic abnormalities were fully corrected; serum magnesium stabilized at 1.89\u2009mg\/dL without supplementation, the urinary calcium\/creatinine ratio normalized (2.6 to 0.06), and proteinuria decreased from 2580\u2009mg\/day to 324\u2009mg\/day. The heterozygous donor maintained stable renal function (24\u2010h creatinine clearance 86\u2009mL\/min\/1.73\u2009m\n                      <jats:sup>2<\/jats:sup>\n                      ) without proteinuria, nephrolithiasis, or metabolic complications during follow\u2010up.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Given the shortage of deceased donor organs, heterozygous parents may be considered suitable donors for children with FHHNC, provided they undergo comprehensive genetic, biochemical, and radiological evaluation to exclude evidence of tubular dysfunction. However, this conclusion should be limited to carefully selected donors, and longer\u2010term follow\u2010up is required to establish the safety of kidney donation in heterozygous CLDN19 carriers.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/petr.70495","type":"journal-article","created":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T06:13:19Z","timestamp":1791612799000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Kidney Transplantation From a Heterozygous\n                    <scp>\n                      <i>CLDN19<\/i>\n                    <\/scp>\n                    Carrier in a Child With\n                    <scp>FHHNC<\/scp>\n                    : A 2\u2010Year Follow\u2010Up"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5212-9282","authenticated-orcid":false,"given":"Hakan","family":"Erdo\u011fan","sequence":"first","affiliation":[{"name":"Department of Pediatric Nephrology, Bursa Faculty of Medicine City Hospital, University of Health Sciences  Bursa T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7212-2419","authenticated-orcid":false,"given":"Mahmut Esat","family":"Dan\u0131\u015fo\u011flu","sequence":"additional","affiliation":[{"name":"Department of Urology, Kidney Transplantation Ac\u0131badem Bursa Hospital  Bursa T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7107-431X","authenticated-orcid":false,"given":"H\u00fcseyin","family":"\u00c7elik","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Kidney Transplantation Ac\u0131badem Bursa Hospital  Bursa T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6650-3880","authenticated-orcid":false,"given":"Murat","family":"\u015eaml\u0131","sequence":"additional","affiliation":[{"name":"Department of Urology and Kidney Transplantation Ac\u0131badem Bursa Hospital  Bursa T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0065-0340","authenticated-orcid":false,"given":"B\u00fclent","family":"Oktay","sequence":"additional","affiliation":[{"name":"Department of Urology and Kidney Transplantation Ac\u0131badem Bursa Hospital  Bursa 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