Sahil D Doshi1, Therese Bittermann, Thomas D Schiano, David Seth Goldberg. 1. 1 Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. 2 Division of Gastroenterology, Department of Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, PA. 3 Division of Liver Diseases, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY. 4 Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics and Epidemiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. 5 Leonard Davis Institute of Health Economics, University of Pennsylvania, Philadelphia, PA.
Abstract
BACKGROUND: Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used Organ Procurement and Transplantation Network/United Network for Organ Sharing data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma. RESULTS: The study cohort included 620 patients with PCLD, 18 240 patients with hepatocellular carcinoma, and 98 567 patients with CLF. Compared with CLF patients, PCLD patients had significantly lower bilirubin and international normalized ratio at waitlisting, and less ascites and encephalopathy. However, they were significantly more likely to have severe chronic kidney disease. Moreover, patients with PCLD were more than 70% more likely to be transplanted compared with patients with CLF (odds ratio, 1.72; 95% confidence interval, 1.46-2.02) and had significantly longer posttransplant survival (P < 0.001). PCLD patients with exceptions were 5.7 times more likely to be transplanted than those without (odds ratio, 5.67; 95% confidence interval, 3.95-8.15) and measures of hepatic/renal dysfunction were inversely associated with the receipt of exceptions. CONCLUSIONS: Despite having more preserved liver synthetic function than patients with CLF on the waitlist, patients with PCLD are preferentially transplanted because they frequently receive exception points in an unstandardized fashion.
BACKGROUND:Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used Organ Procurement and Transplantation Network/United Network for Organ Sharing data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma. RESULTS: The study cohort included 620 patients with PCLD, 18 240 patients with hepatocellular carcinoma, and 98 567 patients with CLF. Compared with CLFpatients, PCLD patients had significantly lower bilirubin and international normalized ratio at waitlisting, and less ascites and encephalopathy. However, they were significantly more likely to have severe chronic kidney disease. Moreover, patients with PCLD were more than 70% more likely to be transplanted compared with patients with CLF (odds ratio, 1.72; 95% confidence interval, 1.46-2.02) and had significantly longer posttransplant survival (P < 0.001). PCLD patients with exceptions were 5.7 times more likely to be transplanted than those without (odds ratio, 5.67; 95% confidence interval, 3.95-8.15) and measures of hepatic/renal dysfunction were inversely associated with the receipt of exceptions. CONCLUSIONS: Despite having more preserved liver synthetic function than patients with CLF on the waitlist, patients with PCLD are preferentially transplanted because they frequently receive exception points in an unstandardized fashion.
Authors: Loes van Keimpema; Frederik Nevens; René Adam; Robert J Porte; Panagiotis Fikatas; Thomas Becker; Preben Kirkegaard; Herold J Metselaar; Joost P H Drenth Journal: Transpl Int Date: 2011-09-29 Impact factor: 3.782
Authors: F Temmerman; L Missiaen; B Bammens; W Laleman; D Cassiman; C Verslype; J van Pelt; F Nevens Journal: Aliment Pharmacol Ther Date: 2011-07-26 Impact factor: 8.171
Authors: Loes Van Keimpema; Daan B De Koning; Bart Van Hoek; Aad P Van Den Berg; Martijn G H Van Oijen; Robert A De Man; Frederik Nevens; Joost P H Drenth Journal: Liver Int Date: 2011-01 Impact factor: 5.828
Authors: Robert M Merion; Douglas E Schaubel; Dawn M Dykstra; Richard B Freeman; Friedrich K Port; Robert A Wolfe Journal: Am J Transplant Date: 2005-02 Impact factor: 8.086
Authors: E Waanders; H Venselaar; R H M te Morsche; D B de Koning; P S Kamath; V E Torres; S Somlo; J P H Drenth Journal: Clin Genet Date: 2010-01-20 Impact factor: 4.438
Authors: M Glanemann; W O Bechstein; A R Müller; J M Langrehr; M Knoop; R Raakow; S Jonas; P Neuhaus Journal: Clin Transplant Date: 1998-06 Impact factor: 2.863
Authors: Lesley A Inker; Brad C Astor; Chester H Fox; Tamara Isakova; James P Lash; Carmen A Peralta; Manjula Kurella Tamura; Harold I Feldman Journal: Am J Kidney Dis Date: 2014-03-16 Impact factor: 8.860