{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T05:45:39Z","timestamp":1792820739466,"version":"4.5.0"},"reference-count":41,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2013,4,17]],"date-time":"2013-04-17T00:00:00Z","timestamp":1366156800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Improved neonatal medical care and renal replacement technology have improved the long\u2010term survival of patients with ARPKD. Ten\u2010yr survival of those surviving the first year of life is reported to be 82% and is continuing to improve further. However, despite increases in overall survival and improved treatment of systemic hypertension and other complications of their renal disease, nearly 50% of survivors will develop ESRD within the first decade of life. In addition to renal pathology, patients with <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> develop ductal plate malformations with cystic dilation of intra\u2010 and extrahepatic bile ducts resulting in CHF and Caroli syndrome. Many patients with <jats:styled-content style=\"fixed-case\">CHF<\/jats:styled-content> will develop portal hypertension with resulting esophageal varices, splenomegaly, hypersplenism, protein losing enteropathy, and gastrointestinal bleeding. Management of portal hypertension may require EBL of esophageal varices or porto\u2010systemic shunting. Complications of hepatic involvement can include ascending cholangitis, cholestasis with malabsorption of fat\u2010soluble vitamins, and rarely benign or malignant liver tumors. Patients with <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> who eventually reach ESRD, and ultimately require kidney transplantation, present a unique set of complications related to their underlying hepato\u2010biliary disease. In this review, we focus on new approaches to these challenging patients, including the indications for liver transplantation in <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> patients with severe chronic kidney disease awaiting kidney transplant. While survival in patients with <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> and isolated kidney transplant is comparable to that of age\u2010matched pediatric patients who have received kidney transplants due to other primary renal diseases, 64\u201380% of the mortality occurring in <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> kidney transplant patients is attributed to cholangitis\/sepsis, which is related to their hepato\u2010biliary disease. Recent data demonstrate that surgical mortality among pediatric liver transplant recipients is decreased to &lt;10% at one yr. The immunosuppressive regimen used for kidney transplant recipients is adequate for most liver transplant recipients. We therefore suggest that in a select group of <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> patients with recurrent cholangitis or complications of portal hypertension, combined liver\u2013kidney transplant is a viable option. Although further study is necessary to confirm our approach, we believe that combined liver\u2013kidney transplantation can potentially decrease overall mortality and morbidity in carefully selected <jats:styled-content style=\"fixed-case\">ARPKD<\/jats:styled-content> patients with <jats:styled-content style=\"fixed-case\">ESRD<\/jats:styled-content> and clinically significant <jats:styled-content style=\"fixed-case\">CHF<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/petr.12076","type":"journal-article","created":{"date-parts":[[2013,4,18]],"date-time":"2013-04-18T03:57:06Z","timestamp":1366257426000},"page":"328-335","source":"Crossref","is-referenced-by-count":52,"title":["New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney\u2013liver complications"],"prefix":"10.1111","volume":"17","author":[{"given":"Grzegorz","family":"Telega","sequence":"first","affiliation":[{"name":"Department of Pediatrics Children's Hospital Health System of Wisconsin and Medical College of Wisconsin Milwaukee WI USA"},{"name":"Children's Research Institute Children's Hospital Health System of Wisconsin and Medical College of Wisconsin Milwaukee WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Cronin","sequence":"additional","affiliation":[{"name":"Children's Research Institute Children's Hospital Health System of Wisconsin and Medical College of Wisconsin Milwaukee WI USA"},{"name":"Department of Transplant Surgery Children's Hospital Health System of Wisconsin and Medical College of Wisconsin Milwaukee WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellis D.","family":"Avner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Children's Hospital Health System of Wisconsin and Medical College of Wisconsin Milwaukee WI USA"},{"name":"Children's Research Institute Children's Hospital Health System of Wisconsin and Medical College of Wisconsin Milwaukee WI 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