{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T02:42:57Z","timestamp":1760150577599,"version":"build-2065373602"},"reference-count":20,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2023,12,21]],"date-time":"2023-12-21T00:00:00Z","timestamp":1703116800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinics and Practice"],"abstract":"<jats:p>Autosomal recessive polycystic kidney disease (ARPKD) is often associated with hepatobiliary disease in the form of hepatic fibrosis and\/or Caroli disease. Combined liver\u2013kidney transplantation (CLKT) is a transplant modality of choice in children with both end-stage renal disease (ESRD) and severe hepatic disease. However, there is no consensus on whether children with ARPKD-associated ESRD without severe hepatic disease can be treated with isolated kidney transplantation (KT) without the need for CLKT. We retrospectively studied the efficacy of isolated KT in children with ARPKD without severe hepatic disease, and followed the course of hepatic disease post KT. This is a single-center study of three children with ARPKD and ESRD who underwent isolated KT. None of them had severe hepatic disease at the time of KT. All children were clinically diagnosed with ARPKD in the immediate postnatal period. All had hepatic fibrosis of varying degrees and two had intrahepatic biliary duct (IHBD) dilatation. None had gastrointestinal (GI) bleed, portal hypertension or cholangitis. Two children had preemptive KT. Pre-transplant unilateral or bilateral native nephrectomy were performed for two children, and one underwent unilateral native nephrectomy at the time of KT. The median creatinine clearance at a median post-KT follow-up of 24 months was 60.3 mL\/min\/1.73 m2. The two-year graft and patient survival were both 100%. Post KT, all three patients continued to demonstrate evidence of hepatic fibrosis and IHBD on sonogram; however, none of them were either evaluated for or required liver transplantation given normal synthetic liver function and absence of portal hypertension or other severe hepatobiliary disease. There were no adverse events observed such as cholangitis, GI bleed, or multiorgan failure. Hence, an excellent short-term graft and patient survival was demonstrated in this study of children with ARPKD and mild to moderate hepatic disease who received isolated KT. Long-term follow-up and larger studies are important to assess the efficacy of isolated KT in this subset of children with ARPKD.<\/jats:p>","DOI":"10.3390\/clinpract14010003","type":"journal-article","created":{"date-parts":[[2023,12,22]],"date-time":"2023-12-22T01:19:42Z","timestamp":1703207982000},"page":"24-30","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Short-Term Outcome of Isolated Kidney Transplantation in Children with Autosomal Recessive Polycystic Kidney Disease: A Case Series and Literature Review"],"prefix":"10.3390","volume":"14","author":[{"given":"Ratna","family":"Acharya","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Nemours Children\u2019s Hospital, Orlando, FL 32827, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8441-0220","authenticated-orcid":false,"given":"Kiran","family":"Upadhyay","sequence":"additional","affiliation":[{"name":"Division of Pediatric Nephrology, Department of Pediatrics, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,12,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1002\/humu.20029","article-title":"PKHD1 mutations in autosomal recessive polycystic kidney disease (ARPKD)","volume":"23","author":"Bergmann","year":"2004","journal-title":"Hum. Mutat."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1053\/j.gastro.2012.09.056","article-title":"Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease","volume":"144","author":"Lukose","year":"2013","journal-title":"Gastroenterology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1097\/MPG.0b013e318291e72b","article-title":"Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis","volume":"57","author":"Rawat","year":"2013","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1111\/j.1399-3046.2012.01661.x","article-title":"Pediatric liver transplantation for fibropolycystic liver disease","volume":"16","author":"Ko","year":"2012","journal-title":"Pediatr. Transplant."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1034\/j.1600-6143.2002.20412.x","article-title":"Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease","volume":"2","author":"Khan","year":"2002","journal-title":"Am. J. Transplant."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1111\/tri.12098","article-title":"Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): Indication and outcome","volume":"26","author":"Brinkert","year":"2013","journal-title":"Transpl. Int."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1097\/SLA.0b013e31817fc2b8","article-title":"The combined organ effect: Protection against rejection?","volume":"248","author":"Rana","year":"2008","journal-title":"Ann. Surg."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1007\/s00467-014-2887-3","article-title":"Transplantation in autosomal recessive polycystic kidney disease: Liver and\/or kidney?","volume":"30","author":"Chandar","year":"2015","journal-title":"Pediatr. Nephrol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1034\/j.1399-3046.2003.00094.x","article-title":"Survival of childhood polycystic kidney disease following renal transplantation: The impact of advanced hepatobiliary disease","volume":"7","author":"Davis","year":"2003","journal-title":"Pediatr. Transplant."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1111\/j.1399-3046.2006.00644.x","article-title":"Optimizing outcomes for neonatal ARPKD","volume":"11","author":"Beaunoyer","year":"2007","journal-title":"Pediatr. Transplant."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2083","DOI":"10.1093\/ndt\/gfr588","article-title":"Kidney and liver transplantation in patients with autosomal recessive polycystic kidney disease: A multicentric study","volume":"27","author":"Chapal","year":"2012","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2217","DOI":"10.1007\/s00467-021-05043-6","article-title":"Transplantation outcomes in patients with primary hyperoxaluria: A systematic review","volume":"36","author":"Metry","year":"2021","journal-title":"Pediatr. Nephrol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1111\/j.1399-3046.2008.01041.x","article-title":"Combined liver-kidney transplantation in children: Indications and outcome","volume":"12","author":"Sutherland","year":"2008","journal-title":"Pediatr. Transplant."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1111\/j.1399-3046.2008.01046.x","article-title":"Indications for combined liver and kidney transplantation in children","volume":"13","author":"Chava","year":"2009","journal-title":"Pediatr. Transplant."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1097\/01.tp.0000241104.58576.e6","article-title":"Comparison of renal allograft outcomes in combined liver-kidney transplantation versus subsequent kidney transplantation in liver transplant recipients: Analysis of UNOS Database","volume":"82","author":"Simpson","year":"2006","journal-title":"Transplantation"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"283","DOI":"10.5500\/wjt.v10.i10.283","article-title":"Combined liver and kidney transplantation in children and long-term outcome","volume":"10","author":"Ranawaka","year":"2020","journal-title":"World J. Transplant."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1458","DOI":"10.1097\/01.TP.0000084203.76110.AC","article-title":"One hundred percent patient and kidney allograft survival with simultaneous liver and kidney transplantation in infants with primary hyperoxaluria: A single-center experience","volume":"76","author":"Millan","year":"2003","journal-title":"Transplantation"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2227","DOI":"10.1007\/s00467-017-3880-4","article-title":"Combined and sequential liver-kidney transplantation in children","volume":"33","author":"Grenda","year":"2018","journal-title":"Pediatr. Nephrol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1584","DOI":"10.1097\/TP.0b013e3181a4e710","article-title":"Renal function recovery in children undergoing combined liver kidney transplants","volume":"87","author":"Perera","year":"2009","journal-title":"Transplantation"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1097\/TP.0b013e3181b29f0c","article-title":"Improved outcomes of combined liver and kidney transplants in small children (<15 kg)","volume":"88","author":"Perera","year":"2009","journal-title":"Transplantation"}],"container-title":["Clinics and Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2039-7283\/14\/1\/3\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:40:00Z","timestamp":1760132400000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2039-7283\/14\/1\/3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,21]]},"references-count":20,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,2]]}},"alternative-id":["clinpract14010003"],"URL":"https:\/\/doi.org\/10.3390\/clinpract14010003","relation":{},"ISSN":["2039-7283"],"issn-type":[{"type":"electronic","value":"2039-7283"}],"subject":[],"published":{"date-parts":[[2023,12,21]]}}}