{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,5]],"date-time":"2025-08-05T12:51:14Z","timestamp":1754398274802},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"925","license":[{"start":{"date-parts":[[2002,11,1]],"date-time":"2002-11-01T00:00:00Z","timestamp":1036108800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2002,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Liver disease is rare in childhood, but important new developments have altered the natural history and outcome. It is important that clinicians are aware of these diseases and their management. Acute liver failure is most often due to viral hepatitis, paracetamol overdose, or inherited metabolic liver disease. The clinical presentation includes jaundice, coagulopathy, and encephalopathy. Early diagnosis is necessary to prevent complications such as cerebral oedema, gastrointestinal bleeding, and renal failure. Early supportive management, in particular intravenous N-acetylcysteine, may be effective but liver transplantation is usually the definitive treatment and thus early referral to a specialist unit for liver transplantation is mandatory. Chronic liver failure may be due to unresolved neonatal liver disease, either inherited biliary hypoplasia or extrahepatic biliary atresia, while in older children, autoimmune liver disease or cystic fibrosis are the commonest causes. Treatment includes specific medication, nutritional support, and liver transplantation, which now has a 90% survival with good quality life.<\/jats:p>","DOI":"10.1136\/pmj.78.925.660","type":"journal-article","created":{"date-parts":[[2002,12,20]],"date-time":"2002-12-20T17:44:09Z","timestamp":1040406249000},"page":"660-667","source":"Crossref","is-referenced-by-count":25,"title":["Managing liver failure"],"prefix":"10.1093","volume":"78","author":[{"given":"D A","family":"Kelly","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2002,11,1]]},"reference":[{"key":"2023042418012625900_R1","first-page":"77","article-title":"Fulminant hepatitis and acute liver failure","volume-title":"Diseases of the liver and biliary system in children","author":"Alonso","year":"1999"},{"key":"2023042418012625900_R2","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1136\/bmj.304.6835.1169","article-title":"Fulminant hepatitis B in infants born to anti-Hbe hepatitis B carrier mothers","volume":"304","author":"Beath","year":"1992","journal-title":"BMJ"},{"key":"2023042418012625900_R3","doi-asserted-by":"crossref","first-page":"1252","DOI":"10.1002\/hep.510290418","article-title":"Mutations in the basic core promotor and the precore region of hepatitis B virus and their selection in children with fulminant and chronic hepatitis B","volume":"29","author":"Friedt","year":"1999","journal-title":"Hepatology"},{"key":"2023042418012625900_R4","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1097\/00005176-199801000-00015","article-title":"Neonatal hemochromatosis: outcome of pharmacologic and surgical techniques","volume":"26","author":"Sigurdsson","year":"1998","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R5","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1016\/0140-6736(92)92685-9","article-title":"Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase","volume":"340","author":"Lindstetd","year":"1992","journal-title":"Lancet"},{"key":"2023042418012625900_R6","doi-asserted-by":"crossref","first-page":"S49","DOI":"10.1007\/PL00014321","article-title":"Indications and outcome of liver transplantation in tyrosinemia type1","volume":"158","author":"Mohan","year":"1999","journal-title":"Eur J Pediatr"},{"key":"2023042418012625900_R7","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1097\/00005176-199804000-00024","article-title":"Generalised mitochondrial cytopathy is an absolute contraindication to orthotopic liver transplantation in childhood","volume":"26","author":"Thomson","year":"1998","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R8","doi-asserted-by":"crossref","first-page":"4743","DOI":"10.1182\/blood.V90.12.4743","article-title":"Treatment of familial hemophagocytic lymphohistiocytosis with bone marrow transplantation from HLA genetically nonidentical donors","volume":"90","author":"Jabado","year":"1997","journal-title":"Blood"},{"key":"2023042418012625900_R9","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1053\/jhep.2001.22131","article-title":"Autoimmune hepatitis\/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study","volume":"33","author":"Gregorio","year":"2001","journal-title":"Hepatology"},{"key":"2023042418012625900_R10","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1034\/j.1399-3046.2000.00125.x","article-title":"Liver transplantation: to do or not to do?","volume":"4","author":"Kelly","year":"2000","journal-title":"Pediatric Transplantation"},{"key":"2023042418012625900_R11","doi-asserted-by":"crossref","first-page":"1377","DOI":"10.1136\/gut.27.11.1377","article-title":"Wilson\u2019s disease: clinical presentation and use of prognostic index","volume":"27","author":"Nazer","year":"1986","journal-title":"Gut"},{"key":"2023042418012625900_R12","first-page":"1557","article-title":"Pilot-controlled trial of the extracorporeal liver assist device in acute liver failure","volume":"6","author":"Ellis","year":"1996","journal-title":"Hepatology"},{"key":"2023042418012625900_R13","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1055\/s-2007-1007248","article-title":"Fulminant hepatic failure: pediatric aspects","volume":"16","author":"Bhaduri","year":"1996","journal-title":"Semin Liver Dis"},{"key":"2023042418012625900_R14"},{"key":"2023042418012625900_R15","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/S0140-6736(99)03492-3","article-title":"The frequency and outcome of biliary atresia in the UK and Ireland","volume":"355","author":"McKiernan","year":"2000","journal-title":"Lancet"},{"key":"2023042418012625900_R16","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1136\/adc.85.1.2","article-title":"Alpha-1 antitrypsin deficiency","volume":"85","author":"Primhak","year":"2001","journal-title":"Arch Dis Child"},{"key":"2023042418012625900_R17","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1002\/hep.510260121","article-title":"Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity","volume":"26","author":"Bull","year":"1997","journal-title":"Hepatology"},{"key":"2023042418012625900_R18","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1016\/S0959-437X(00)00078-2","article-title":"Inherited disorders of transport in the liver","volume":"10","author":"Thompson","year":"2000","journal-title":"Curr Opin Genet Dev"},{"key":"2023042418012625900_R19","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1093\/hmg\/4.6.1049","article-title":"Mapping of a locus for progressive familial intrahepatic cholestasis (Byler disease) to 18q21-q22, the benign recurrent intrahepatic cholestasis region","volume":"4","author":"Carleton","year":"1995","journal-title":"Hum Mol Genet"},{"key":"2023042418012625900_R20","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1002\/hep.510270126","article-title":"A 2 year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis associated liver disease","volume":"27","author":"Lindblad","year":"1998","journal-title":"Hepatology"},{"key":"2023042418012625900_R21","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/j.1432-2277.1997.tb00533.x","article-title":"Combined heart-lung-liver, and isolated liver transplantation for cystic fibrosis in children","volume":"10","author":"Couetil","year":"1997","journal-title":"Transpl Int"},{"key":"2023042418012625900_R22","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1097\/00005176-200001000-00017","article-title":"Non-alcoholic steatohepatitis in children","volume":"30","author":"Rashid","year":"2000","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R23","first-page":"189","article-title":"Complications and management of chronic liver disease","volume-title":"Diseases of the liver and biliary system in children","author":"Shepherd","year":"1999"},{"key":"2023042418012625900_R24","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1016\/0022-3468(91)90904-8","article-title":"The endoscopic and surgical management of portal hypertension in children: analysis of 124 cases","volume":"26","author":"Maksoud","year":"1991","journal-title":"J Pediatr Surg"},{"key":"2023042418012625900_R25","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1055\/s-2008-1066093","article-title":"Current therapeutic strategy in bleeding oesophageal varices in babies and children and long term results of endoscopic paravariceal sclerotherapy over twenty years","volume":"4","author":"Paquet","year":"1994","journal-title":"Eur J Pediatr Surg"},{"key":"2023042418012625900_R26","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1097\/00005176-200204000-00019","article-title":"Inferior vena cava occlusion and protein-losing enteropathy after liver transplantation in children","volume":"34","author":"Lee","year":"2002","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R27","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1097\/00005176-199909000-00004","article-title":"Role of transjugular portosystemic shunt in the treatment of portal hypertension in pediatric patients","volume":"29","author":"Heyman","year":"1999","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R28","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1097\/00005176-199907000-00007","article-title":"Propranolol n prevension of portal hypertensive haemorrhage in children: a pilot study","volume":"29","author":"Shashidhar","year":"1999","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R29","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1053\/lv.2000.6355","article-title":"Improvement of hepatorenal syndrome with extracorporeal albumin dialysis MARS: results of a prospective randomized, controlled clinical trial","volume":"6","author":"Mitzner","year":"2000","journal-title":"Liver Transplantation"},{"key":"2023042418012625900_R30","first-page":"293","article-title":"Liver transplantation","volume-title":"Diseases of the liver and biliary system in children","author":"Kelly","year":"1999"},{"key":"2023042418012625900_R31","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1002\/lt.500040607","article-title":"Efficacy of neoral in the immediate post operative period in children post liver transplantation","volume":"6","author":"Van Mourik","year":"1998","journal-title":"Liver Transplant Surgery"},{"key":"2023042418012625900_R32","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1016\/S0022-3468(97)90011-7","article-title":"Progress in paediatric liver transplantation: the Birmingham experience","volume":"32","author":"Talbot","year":"1997","journal-title":"J Pediatr Surg"},{"key":"2023042418012625900_R33","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1097\/00005176-200003000-00011","article-title":"Long term nutrition and neurodevelopmental outcome of liver transplantation in infants aged less than 12 months","volume":"30","author":"Van Mourik","year":"2000","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2023042418012625900_R34","first-page":"351","article-title":"Tacrolimus dual therapy versus cyclosporin-microemulsion triple therapy in paediatric liver transplantation: results from a multi-centre randomised trial","volume":"2","author":"Kelly","year":"2002","journal-title":"American Journal of Transplantation"},{"key":"2023042418012625900_R35","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1093\/ajcn\/65.2.534","article-title":"Orthotopic liver transplantation reverses the adverse nutritional changes of end stage liver disease in children","volume":"65","author":"Holt","year":"1997","journal-title":"Am J Clin Nutr"},{"key":"2023042418012625900_R36","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1002\/lt.500060223","article-title":"Maternal haemodynamics and pregnancy outcome in women with prior orthotopic liver transplantation","volume":"6","author":"Carr","year":"2000","journal-title":"Liver Transplantation"}],"container-title":["Postgraduate Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/78\/925\/660\/50041259\/postgradmedj-78-660.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/pmj\/article-pdf\/78\/925\/660\/50041259\/postgradmedj-78-660.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,24]],"date-time":"2023-04-24T18:04:50Z","timestamp":1682359490000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/pmj\/article\/78\/925\/660\/7045970"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,11,1]]},"references-count":36,"journal-issue":{"issue":"925","published-online":{"date-parts":[[2002,11,1]]},"published-print":{"date-parts":[[2002,11,1]]}},"URL":"https:\/\/doi.org\/10.1136\/pmj.78.925.660","relation":{},"ISSN":["0032-5473","1469-0756"],"issn-type":[{"value":"0032-5473","type":"print"},{"value":"1469-0756","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2002,11]]},"published":{"date-parts":[[2002,11,1]]}}}