{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T06:06:39Z","timestamp":1759730799783,"version":"3.41.2"},"reference-count":10,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,6,4]],"date-time":"2018-06-04T00:00:00Z","timestamp":1528070400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81670680"],"award-info":[{"award-number":["81670680"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2018,9]]},"abstract":"Abstract<\/jats:title>The choice of KT<\/jats:styled-content> only or CLKT<\/jats:styled-content> for infantile NPHP<\/jats:styled-content> with mild liver fibrosis is understudied. A 5\u2010year\u2010old girl was transferred to our center for KT<\/jats:styled-content> due to ESRD<\/jats:styled-content>. Her primary disease was infantile NPHP<\/jats:styled-content> with compound heterozygous NPHP<\/jats:styled-content>3 mutations: c.458A>C(p.Q153P)\/missense mutation and c.2032A>T(p. K678X)\/nonsense mutation. The patient had elevated liver enzymes and biopsy\u2010proven liver fibrosis. As liver synthesis was acceptable, only KT<\/jats:styled-content> was performed. However, liver fibrosis progressed at 1.5\u00a0years after transplantation, manifested with portal hypertension and hypersplenism. Common causes for portal hypertension were excluded, and the progression was attributed to NPHP<\/jats:styled-content>. AMR<\/jats:styled-content> attacked allograft at about 2\u00a0years post\u2010transplant. To solve both the liver and the kidney problems, CLKT<\/jats:styled-content> was performed. Her liver and kidney function recovered initially, but she unfortunately died of pneumonia and subsequent intracranial hemorrhage two weeks later. Nonsense mutation in NPHP<\/jats:styled-content>3 gene may be correlated with rapid progression of liver disease in infantile NPHP<\/jats:styled-content>. More studies are required to determine the role of CLKT<\/jats:styled-content> in these cases; however, combined transplantation may improve long\u2010term graft and patient survival.<\/jats:p>","DOI":"10.1111\/petr.13233","type":"journal-article","created":{"date-parts":[[2018,6,5]],"date-time":"2018-06-05T01:14:51Z","timestamp":1528161291000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Transplantation for infantile nephronophthisis with loss\u2010of\u2010function mutation in NPHP<\/scp>3: Lesson from a case"],"prefix":"10.1111","volume":"22","author":[{"given":"Huanxi","family":"Zhang","sequence":"first","affiliation":[{"name":"Organ Transplant Center The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiayue","family":"Luo","sequence":"additional","affiliation":[{"name":"Zhongshan School of Medicine Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Longshan","family":"Liu","sequence":"additional","affiliation":[{"name":"Organ Transplant Center The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Li","sequence":"additional","affiliation":[{"name":"Organ Transplant Center The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qian","family":"Fu","sequence":"additional","affiliation":[{"name":"Organ Transplant Center The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenfang","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pathology The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou 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China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liangzhong","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Pediatrics The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaofeng","family":"Zhu","sequence":"additional","affiliation":[{"name":"Organ Transplant Center The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1282-3341","authenticated-orcid":false,"given":"Changxi","family":"Wang","sequence":"additional","affiliation":[{"name":"Organ Transplant Center The First Affiliated Hospital of Sun Yat\u2010sen University Guangzhou China"},{"name":"Guangdong Provincial Key Laboratory on Organ Donation and Transplant Immunology Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,6,4]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-010-1585-z"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2008050456"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/nep.12563"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12330"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-3046.2003.00094.x"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2008030287"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2008.02416.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12593"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2008.02.017"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2011.284"}],"container-title":["Pediatric 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