{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T23:43:00Z","timestamp":1782603780946,"version":"3.54.5"},"reference-count":26,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":3106,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2007,3]]},"abstract":"<jats:sec>\n            <jats:title>Abstract<\/jats:title>\n            <jats:p>Cystic fibrosis (CF) is an inherited genetic defect in epithelial chloride transport that results a multisystem disease affecting the sweat glands and the pulmonary and digestive systems. Although pulmonary disease remains the primary cause of morbidity and mortality in these patients, up to 8% may develop focal biliary cirrhosis and portal hypertension. Liver transplantation is an accepted therapy for these patients. About 85% of CF patients develop pancreatic exocrine insufficiency and up to 34% will develop diabetes requiring insulin. We describe a series of 9 patients who underwent liver transplantation (6 transplantation of liver only [LO], and 3 combined en bloc liver-pancreas [LP] transplantation) for CF-related liver disease at our institution,. All 9 patients had pretransplant pancreatic exocrine insufficiency requiring enzyme supplementation. Of the 9, 5 patients (55%) had preoperative insulin dependent diabetes, including all 3 patients who underwent liver pancreas transplantation. Liver-pancreas transplants were performed en bloc. One-year patient survival for both LO and LP patients was 100%. Five-year patient survival was 83% for the LO patients and 100% for the LP patients. All LP patients are off insulin and do not require pancreatic enzyme supplementation. All patients receiving LO continue to need exogenous pancreatic enzyme supplementation. In the 6 patients who received LO, 2 were insulin dependent prior to surgery, and they remain insulin dependent after transplant. Of the 4 patients who received LO who were not insulin dependent pretransplant, 3 have now developed insulin-dependent diabetes. Patients receiving LO were more likely to be diabetic and require exogenous pancreatic enzymes after transplant than those receiving LP (83% vs. 0%, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.04, and 100% vs. 0%, <jats:italic toggle=\"yes\">P<\/jats:italic> = 0.01, respectively). In conclusion, patients with CF and end-stage liver disease requiring liver transplantation may benefit from combined liver-pancreas transplantation. <jats:italic toggle=\"yes\">Liver Transpl 13:406\u2013409, 2007.<\/jats:italic> \u00a9 2007 AASLD.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/lt.21070","type":"journal-article","created":{"date-parts":[[2007,2,22]],"date-time":"2007-02-22T23:05:54Z","timestamp":1172185554000},"page":"406-409","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":43,"title":["Combined En Bloc Liver Pancreas Transplantation for Children with CF"],"prefix":"10.1097","volume":"13","author":[{"given":"Kristin L.","family":"Mekeel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Max R.","family":"Langham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Regino","family":"Gonzalez-Perralta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Reed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan W.","family":"Hemming","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-16-20250124","first-page":"275","article-title":"Newonset diabetes mellitus in pediatric thoracic organ recipients receiving tacrolimusbased immunosuppression.","volume":"16","author":"Wagner","year":"1997","journal-title":"J Heart Lung Transplant"},{"issue":"Suppl 1","key":"R2-16-20250124","doi-asserted-by":"crossref","first-page":"S1","DOI":"10.1097\/00005176-199900001-00001","article-title":"Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group.","volume":"28","author":"Sokol","year":"1999","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"R3-16-20250124","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1055\/s-2007-1007316","article-title":"Hepatobiliary disease in cystic fibrosis.","volume":"14","author":"Colombo","year":"1994","journal-title":"Semin Liver Dis"},{"key":"R4-16-20250124","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1046\/j.1440-1746.2002.02671.x","article-title":"Transplantation for cystic fibrosis: outcome following early liver transplantation.","volume":"17","author":"Milkiewicz","year":"2002","journal-title":"J Gastroenterol Hepatol"},{"key":"R5-16-20250124","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1034\/j.1399-3046.2003.00021.x","article-title":"Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population.","volume":"7","author":"Molmenti","year":"2003","journal-title":"Pediatr Transplant"},{"key":"R6-16-20250124","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1016\/S0022-3468(03)00260-4","article-title":"Liver transplantation in children with cystic fibrosis: a longterm longitudinal review of a single centers experience.","volume":"38","author":"Fridell","year":"2003","journal-title":"J Pediatr Surg"},{"issue":"Suppl 27","key":"R7-16-20250124","first-page":"31","article-title":"Liver transplantation for hepatic cirrhosis in cystic fibrosis.","volume":"89","author":"NobleJamieson","year":"1996","journal-title":"J R Soc Med"},{"key":"R8-16-20250124","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/S0022-3476(96)70263-9","article-title":"Liver transplantation for cirrhosis in cystic fibrosis.","volume":"129","author":"NobleJamieson","year":"1996","journal-title":"J Pediatr"},{"key":"R9-16-20250124","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/S1262-3636(07)70189-1","article-title":"Diabetes: a major comorbidity of cystic fibrosis.","volume":"31","author":"Costa","year":"2005","journal-title":"Diabetes Metab"},{"key":"R10-16-20250124","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1111\/j.1651-2227.1994.tb12956.x","article-title":"Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications.","volume":"83","author":"Lanng","year":"1994","journal-title":"Acta Paediatr"},{"key":"R11-16-20250124","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1002\/lt.20439","article-title":"Newonset diabetes after liver transplantation: from pathogenesis to management.","volume":"11","author":"Marchetti","year":"2005","journal-title":"Liver Transpl"},{"key":"R12-16-20250124","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1399-0012.1994.tb00933.x","article-title":"Restoration of exocrine pancreatic function following pancreasliverkidney transplantation in a cystic fibrosis patient.","volume":"8","author":"Stern","year":"1994","journal-title":"Clin Transplant"},{"key":"R13-16-20250124","doi-asserted-by":"crossref","first-page":"3567","DOI":"10.1016\/j.transproceed.2005.09.091","article-title":"Simultaneous liver and pancreas transplantation in patients with cystic fibrosis.","volume":"37","author":"Fridell","year":"2005","journal-title":"Transplant Proc"},{"key":"R14-16-20250124","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1097\/01.tp.0000167007.58199.9b","article-title":"A combined liverpancreas enbloc transplant in a patient with cystic fibrosis.","volume":"80","author":"Young","year":"2005","journal-title":"Transplantation"},{"key":"R15-16-20250124","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1399-0012.1990.tb00204.x","article-title":"Treatment of upper abdominal malignancies with organ cluster procedures.","volume":"4","author":"Mieles","year":"1990","journal-title":"Clin Transplant"},{"key":"R16-16-20250124","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1097\/00006676-199107000-00017","article-title":"Pancreaticoduodenal transplantation with enteric drainage following native total pancreatectomy for chronic pancreatitis: a case report.","volume":"6","author":"Gruessner","year":"1991","journal-title":"Pancreas"},{"key":"R18-16-20250124","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/S0041-1345(01)02729-4","article-title":"Combined liverpancreas transplantation: contribution of five cases.","volume":"34","author":"Aguirrezabalaga","year":"2002","journal-title":"Transplant Proc"},{"key":"R19-16-20250124","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1111\/j.1600-6143.2004.00588.x","article-title":"Combined en bloc liver and pancreas transplantation in patients with liver disease and type 1 diabetes mellitus.","volume":"4","author":"Pirenne","year":"2004","journal-title":"Am J Transplant"},{"key":"R20-16-20250124","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1034\/j.1399-3046.2003.00064.x","article-title":"Liver and intestinal transplantation in a child with cystic fibrosis: a case report.","volume":"7","author":"Fridell","year":"2003","journal-title":"Pediatr Transplant"},{"key":"R21-16-20250124","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/j.jcf.2004.08.001","article-title":"Clinical importance of cystic fibrosisrelated diabetes.","volume":"3","author":"Brennan","year":"2004","journal-title":"J Cyst Fibros"},{"key":"R22-16-20250124","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1002\/ppul.1142","article-title":"Presence of cystic fibrosisrelated diabetes mellitus is tightly linked to poor lung function in patients with cystic fibrosis: data from the European Epidemiologic Registry of Cystic Fibrosis.","volume":"32","author":"Koch","year":"2001","journal-title":"Pediatr Pulmonol"},{"key":"R23-16-20250124","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/S0168-8227(99)00058-3","article-title":"Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report.","volume":"45","author":"Moran","year":"1999","journal-title":"Diabetes Res Clin Pract"},{"key":"R24-16-20250124","first-page":"345","article-title":"Diabetes mellitus in patients with cystic fibrosis: the impact of diabetes mellitus on pulmonary function and clinical outcome.","volume":"6","author":"Rosenecker","year":"2001","journal-title":"Eur J Med Res"},{"key":"R25-16-20250124","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1111\/j.1399-0012.2005.00420.x","article-title":"Incidence and prevalence of diabetes mellitus in patients with cystic fibrosis undergoing lung transplantation before and after lung transplantation.","volume":"19","author":"Hadjiliadis","year":"2005","journal-title":"Clin Transplant"},{"key":"R26-16-20250124","doi-asserted-by":"crossref","first-page":"1145","DOI":"10.1016\/S0041-1345(96)00495-2","article-title":"Combined liver and pancreas transplantation induces pancreas allograft tolerance.","volume":"29","author":"Wang","year":"1997","journal-title":"Transplant Proc"},{"key":"R27-16-20250124","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1097\/00007890-199801270-00007","article-title":"Conversion of pancreas allograft rejection to acceptance by liver transplantation.","volume":"65","author":"Wang","year":"1998","journal-title":"Transplantation"}],"container-title":["Liver Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flt.21070","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01445473-200703000-00016","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T16:15:34Z","timestamp":1737735334000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01445473-200703000-00016"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,3]]},"references-count":26,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2007]]}},"URL":"https:\/\/doi.org\/10.1002\/lt.21070","relation":{},"ISSN":["1527-6465"],"issn-type":[{"value":"1527-6465","type":"print"}],"subject":[],"published":{"date-parts":[[2007,3]]}}}