{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T11:03:48Z","timestamp":1792839828324,"version":"4.5.0"},"reference-count":41,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2013,1,1]],"date-time":"2013-01-01T00:00:00Z","timestamp":1356998400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2013,1,1]],"date-time":"2013-01-01T00:00:00Z","timestamp":1356998400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000051","name":"National Human Genome Research Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000051","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000098","name":"NIH Clinical Center","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000098","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","gastrojournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Gastroenterology"],"published-print":{"date-parts":[[2013,1]]},"DOI":"10.1053\/j.gastro.2012.09.056","type":"journal-article","created":{"date-parts":[[2012,10,3]],"date-time":"2012-10-03T14:45:54Z","timestamp":1349275554000},"page":"112-121.e2","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":161,"title":["Characteristics of Congenital Hepatic Fibrosis in a Large Cohort of Patients With Autosomal Recessive Polycystic Kidney Disease"],"prefix":"10.1053","volume":"144","author":[{"given":"Meral","family":"Gunay\u2013Aygun","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esperanza","family":"Font\u2013Montgomery","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Lukose","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maya","family":"Tuchman Gerstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie","family":"Piwnica\u2013Worms","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Choyke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kailash T.","family":"Daryanani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baris","family":"Turkbey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roxanne","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isa","family":"Bernardini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Murat","family":"Sincan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiongce","family":"Zhao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Netanya G.","family":"Sandler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annelys","family":"Roque","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel C.","family":"Douek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Graf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjan","family":"Huizing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joy C.","family":"Bryant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Parvathi","family":"Mohan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William A.","family":"Gahl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theo","family":"Heller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1053\/j.gastro.2012.09.056_bib1","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1111\/j.1651-2227.1996.tb14056.x","article-title":"Autosomal recessive polycystic kidney disease in 115 children: clinical presentation, course and influence of gender","volume":"85","author":"Zerres","year":"1996","journal-title":"Acta Paediatr"},{"key":"10.1053\/j.gastro.2012.09.056_bib2","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/s00467-002-1021-0","article-title":"Autosomal recessive polycystic kidney disease: outcomes from a single-center experience","volume":"18","author":"Capisonda","year":"2003","journal-title":"Pediatr Nephrol"},{"key":"10.1053\/j.gastro.2012.09.056_bib3","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1542\/peds.111.5.1072","article-title":"Autosomal recessive polycystic kidney disease: the clinical experience in North America","volume":"111","author":"Guay-Woodford","year":"2003","journal-title":"Pediatrics"},{"key":"10.1053\/j.gastro.2012.09.056_bib4","series-title":"Congenital hepatic fibrosis overview","author":"Gunay-Aygun","year":"2008"},{"key":"10.1053\/j.gastro.2012.09.056_bib5","doi-asserted-by":"crossref","first-page":"972","DOI":"10.2215\/CJN.07141009","article-title":"Correlation of kidney function, volume and imaging findings, and PKHD1 mutations in 73 patients with autosomal recessive polycystic kidney disease","volume":"5","author":"Gunay-Aygun","year":"2010","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1053\/j.gastro.2012.09.056_bib6","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.jpeds.2006.03.014","article-title":"Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health\/Office of Rare Diseases conference","volume":"149","author":"Gunay-Aygun","year":"2006","journal-title":"J Pediatr"},{"key":"10.1053\/j.gastro.2012.09.056_bib7","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1002\/ajmg.c.30225","article-title":"Liver and kidney disease in ciliopathies","volume":"151C","author":"Gunay-Aygun","year":"2009","journal-title":"Am J Med Genet C Semin Med Genet"},{"key":"10.1053\/j.gastro.2012.09.056_bib8","first-page":"1","article-title":"The ductal plate malformation","author":"Jorgensen","year":"1977","journal-title":"Acta Pathol Microbiol Scand Suppl"},{"key":"10.1053\/j.gastro.2012.09.056_bib9","first-page":"21","article-title":"A stereological study of intrahepatic bile ducts","volume":"82","author":"Jorgensen","year":"1974","journal-title":"Acta Pathol Microbiol Scand A"},{"key":"10.1053\/j.gastro.2012.09.056_bib10","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1002\/hep.1840160434","article-title":"Congenital diseases of intrahepatic bile ducts: variations on the theme \u201cductal plate malformation.\u201d","volume":"16","author":"Desmet","year":"1992","journal-title":"Hepatology"},{"key":"10.1053\/j.gastro.2012.09.056_bib11","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1111\/j.1365-2559.1992.tb01031.x","article-title":"What is congenital hepatic fibrosis?","volume":"20","author":"Desmet","year":"1992","journal-title":"Histopathology"},{"key":"10.1053\/j.gastro.2012.09.056_bib12","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/S0025-6196(11)63624-0","article-title":"Ludwig symposium on biliary disorders\u2014part I","volume":"73","author":"Desmet","year":"1998","journal-title":"Mayo Clin Proc"},{"key":"10.1053\/j.gastro.2012.09.056_bib13","doi-asserted-by":"crossref","first-page":"514","DOI":"10.1136\/gut.19.6.514","article-title":"Congenital hepatic fibrosis: the long-term prognosis","volume":"19","author":"Kerr","year":"1978","journal-title":"Gut"},{"key":"10.1053\/j.gastro.2012.09.056_bib14","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1016\/S0022-3476(81)80320-4","article-title":"Congenital hepatic fibrosis in children","volume":"99","author":"Alvarez","year":"1981","journal-title":"J Pediatr"},{"key":"10.1053\/j.gastro.2012.09.056_bib15","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0168-8278(86)80073-3","article-title":"Hepatobiliary fibropolycystic disease: a clinical and histological review of 51 patients","volume":"2","author":"Summerfield","year":"1986","journal-title":"J Hepatol"},{"key":"10.1053\/j.gastro.2012.09.056_bib16","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/S0300-5089(21)00338-2","article-title":"Diseases of the intrahepatic biliary tree","volume":"2","author":"Caroli","year":"1973","journal-title":"Clin Gastroenterol"},{"key":"10.1053\/j.gastro.2012.09.056_bib17","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/ng833","article-title":"The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein","volume":"30","author":"Ward","year":"2002","journal-title":"Nat Genet"},{"key":"10.1053\/j.gastro.2012.09.056_bib18","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1086\/340448","article-title":"PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats","volume":"70","author":"Onuchic","year":"2002","journal-title":"Am J Hum Genet"},{"key":"10.1053\/j.gastro.2012.09.056_bib19","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1016\/S0070-2153(08)00813-2","article-title":"Ciliary dysfunction in developmental abnormalities and diseases","volume":"85","author":"Sharma","year":"2008","journal-title":"Curr Top Dev Biol"},{"key":"10.1053\/j.gastro.2012.09.056_bib20","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1097\/MPG.0b013e318228330c","article-title":"Congenital hepatic fibrosis and portal hypertension in autosomal dominant polycystic kidney disease","volume":"54","author":"O'Brien","year":"2012","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10.1053\/j.gastro.2012.09.056_bib21","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1007\/s004670050281","article-title":"Autosomal recessive polycystic kidney disease: long-term outcome of neonatal survivors","volume":"11","author":"Roy","year":"1997","journal-title":"Pediatr Nephrol"},{"key":"10.1053\/j.gastro.2012.09.056_bib22","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1093\/ndt\/16.8.1648","article-title":"Autosomal recessive polycystic kidney disease in adulthood","volume":"16","author":"Fonck","year":"2001","journal-title":"Nephrol Dial Transplant"},{"key":"10.1053\/j.gastro.2012.09.056_bib23","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/01.md.0000200165.90373.9a","article-title":"Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD)","volume":"85","author":"Adeva","year":"2006","journal-title":"Medicine (Baltimore)"},{"key":"10.1053\/j.gastro.2012.09.056_bib24","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1007\/BF00279301","article-title":"Cystic kidneys","volume":"68","author":"Zerres","year":"1984","journal-title":"Hum Genet"},{"key":"10.1053\/j.gastro.2012.09.056_bib25","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.ymgme.2009.10.010","article-title":"PKHD1 sequence variations in 78 children and adults with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis","volume":"99","author":"Gunay-Aygun","year":"2010","journal-title":"Mol Genet Metab"},{"key":"10.1053\/j.gastro.2012.09.056_bib26","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1681\/ASN.2008030287","article-title":"New equations to estimate GFR in children with CKD","volume":"20","author":"Schwartz","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"10.1053\/j.gastro.2012.09.056_bib27","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1080\/00365510510013596","article-title":"A cystatin C-based formula without anthropometric variables estimates glomerular filtration rate better than creatinine clearance using the Cockcroft-Gault formula","volume":"65","author":"Grubb","year":"2005","journal-title":"Scand J Clin Lab Invest"},{"key":"10.1053\/j.gastro.2012.09.056_bib28","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1148\/radiol.2311020963","article-title":"Spleen length in childhood with US: normal values based on age, sex, and somatometric parameters","volume":"231","author":"Megremis","year":"2004","journal-title":"Radiology"},{"key":"10.1053\/j.gastro.2012.09.056_bib29","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.2214\/ajr.171.6.9843315","article-title":"Normal liver, spleen, and kidney dimensions in neonates, infants, and children: evaluation with sonography","volume":"171","author":"Konus","year":"1998","journal-title":"AJR Am J Roentgenol"},{"key":"10.1053\/j.gastro.2012.09.056_bib30","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1148\/radiology.195.1.7892467","article-title":"Common bile duct in children: sonographic dimensions","volume":"195","author":"Hernanz-Schulman","year":"1995","journal-title":"Radiology"},{"key":"10.1053\/j.gastro.2012.09.056_bib31","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1056\/NEJMoa054341","article-title":"Volume progression in polycystic kidney disease","volume":"354","author":"Grantham","year":"2006","journal-title":"N Engl J Med"},{"key":"10.1053\/j.gastro.2012.09.056_bib32","doi-asserted-by":"crossref","first-page":"38","DOI":"10.2215\/CJN.00930306","article-title":"Normal values for renal length and volume as measured by magnetic resonance imaging","volume":"2","author":"Cheong","year":"2007","journal-title":"Clin J Am Soc Nephrol"},{"key":"10.1053\/j.gastro.2012.09.056_bib33","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1007\/s002470050114","article-title":"Liver volume in children measured by computed tomography","volume":"27","author":"Noda","year":"1997","journal-title":"Pediatr Radiol"},{"key":"10.1053\/j.gastro.2012.09.056_bib34","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1002\/lt.20188","article-title":"Estimation of standard liver volume for liver transplantation in the Korean population","volume":"10","author":"Yu","year":"2004","journal-title":"Liver Transpl"},{"key":"10.1053\/j.gastro.2012.09.056_bib35","first-page":"55","article-title":"Body length and organ weights of infants and children","volume":"9","author":"Coppoletta","year":"1933","journal-title":"Am J Pathol"},{"key":"10.1053\/j.gastro.2012.09.056_bib36","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.2214\/ajr.160.5.8470587","article-title":"Volume of the spleen in children as measured on CT scans: normal standards as a function of body weight","volume":"160","author":"Schlesinger","year":"1993","journal-title":"AJR Am J Roentgenol"},{"key":"10.1053\/j.gastro.2012.09.056_bib37","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1007\/BF02384985","article-title":"Standard splenic volume in children and young adults measured from CT images","volume":"27","author":"Watanabe","year":"1997","journal-title":"Surg Today"},{"key":"10.1053\/j.gastro.2012.09.056_bib38","first-page":"33","article-title":"Cystic renal diseases in children","volume":"18","author":"Gagnadoux","year":"1989","journal-title":"Adv Nephrol Necker Hosp"},{"key":"10.1053\/j.gastro.2012.09.056_bib39","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1046\/j.1440-1746.2001.02392.x","article-title":"Coagulation profile and platelet function in patients with extrahepatic portal vein obstruction and non-cirrhotic portal fibrosis","volume":"16","author":"Bajaj","year":"2001","journal-title":"J Gastroenterol Hepatol"},{"key":"10.1053\/j.gastro.2012.09.056_bib40","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1053\/j.gastro.2011.06.063","article-title":"Host response to translocated microbial products predicts outcomes of patients with HBV or HCV infection","volume":"141","author":"Sandler","year":"2011","journal-title":"Gastroenterology"},{"key":"10.1053\/j.gastro.2012.09.056_bib41","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1111\/j.1399-3046.2005.00456.x","article-title":"Predominant extrahepatic biliary disease in autosomal recessive polycystic kidney disease: a new association","volume":"10","author":"Goilav","year":"2006","journal-title":"Pediatr Transplant"}],"container-title":["Gastroenterology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0016508512014576?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0016508512014576?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,16]],"date-time":"2025-09-16T15:02:02Z","timestamp":1758034922000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0016508512014576"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,1]]},"references-count":41,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,1]]}},"alternative-id":["S0016508512014576"],"URL":"https:\/\/doi.org\/10.1053\/j.gastro.2012.09.056","relation":{},"ISSN":["0016-5085"],"issn-type":[{"value":"0016-5085","type":"print"}],"subject":[],"published":{"date-parts":[[2013,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Characteristics of Congenital Hepatic Fibrosis in a Large Cohort of Patients With Autosomal Recessive Polycystic Kidney Disease","name":"articletitle","label":"Article Title"},{"value":"Gastroenterology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1053\/j.gastro.2012.09.056","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}