Liver transplantation in adult polycystic liver disease: the Ontario experience

被引:15
作者
Alsager, Mohammed [1 ]
Neong, Shuet Fong [2 ]
Gandhi, Radhika [1 ]
Teriaky, Anouar [1 ]
Tang, Ephraim [2 ]
Skaro, Anton [2 ]
Qumosani, Karim [1 ]
Lilly, Les [2 ]
Galvin, Zita [2 ]
Selzner, Nazia [2 ]
Bhat, Mamatha Pallavi [2 ]
Puka, Klajdi [3 ]
Brahmania, Mayur [1 ,4 ]
机构
[1] Western Univ, Div Gastroenterol & Multiorgan Transplant, London, ON, Canada
[2] Univ Hlth Network, Multiorgan Transplant Program, Toronto, ON, Canada
[3] Western Univ, Dept Epidemiol & Biostat, London, ON, Canada
[4] Western Univ, Univ Hosp, London Hlth Sci Ctr, Room A10-224, London, ON N6A 5A5, Canada
关键词
Polycystic liver disease; Liver transplantation; PCLD outcome; MANAGEMENT; RESECTION;
D O I
10.1186/s12876-021-01703-x
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
BackgroundLiver transplantation (LT) remains the curative treatment for symptomatic Polycystic Liver Disease (PCLD) patients and is associated with excellent survival rates. The aim of the study is to review the Ontario experience in LT for PCLD.MethodsA retrospective study was performed from pre-existing LT databases from the LT Units at Toronto General Hospital and London Health Sciences Center, which are the two LT programs in Ontario, Canada. This database contains demographic, clinical parameters and follow-up of all patients transplanted for PCLD. Data was extracted for patients who underwent LT between January 2000-April 2017 and included follow up until December 31st, 2018.ResultsA total of 3560 patients underwent LT, of whom 51 (1.4%) had PCLD and met inclusion criteria. 43 (84%) of these patients were female. The median physiologic Model for End Stage Liver Disease (MELD-Na) score at time of referral was 13 (IQR=7-22), however all patients required MELD-Na exception points to receive LT. The median age of transplant was 62 years (IQR=59-64) for male vs. 52 (IQR=45-56) for female patients. 33 (65%) of our cohort had PCLD while 9 (17.5%) had ADPKD and 9 (17.5%) had both diseases. 39 (76%) had LT due to symptoms of mass effect, while 8 (16%) had portal hypertensive complications. After a median follow-up of 6.3 (IQR=2.9-12.5) years, the probability of survival was 96% (95% CI: 90%, 100%). Log-rank test, comparing survival analysis between males and females did not show a statistically significant difference (p=0.26).ConclusionMost patients underwent LT for PCLD due to symptoms of mass effect with women being more likely than men to undergo LT. LT for PCLD had excellent long-term survival.
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页数:6
相关论文
共 22 条
[1]   Normalised intrinsic mortality risk in liver transplantation: European Liver Transplant Registry study [J].
Adam, R ;
Cailliez, V ;
Majno, P ;
Karam, V ;
McMaster, P ;
Calne, RY ;
O'Grady, J ;
Pichlmayr, R ;
Neuhaus, P ;
Otte, JB ;
Hoeckerstedt, K ;
Bismuth, H .
LANCET, 2000, 356 (9230) :621-627
[2]   Evolution of indications and results of liver transplantation in Europe. A report from the European Liver Transplant Registry (ELTR) [J].
Adam, Rene ;
Karam, Vincent ;
Delvart, Valerie ;
O'Grady, John ;
Mirza, Darius ;
Klempnauer, Jurgen ;
Castaing, Denis ;
Neuhaus, Peter ;
Jamieson, Neville ;
Salizzoni, Mauro ;
Pollard, Stephen ;
Lerut, Jan ;
Paul, Andreas ;
Carlos Garcia-Valdecasas, Juan ;
Juan Rodriguez, Fernando San ;
Burroughs, Andrew .
JOURNAL OF HEPATOLOGY, 2012, 57 (03) :675-688
[3]  
Ahearn Aaron, 2016, AMA J Ethics, V18, P126, DOI 10.1001/journalofethics.2016.18.2.nlit1-1602
[4]   Extended Liver Resection for Polycystic Liver Disease Can Challenge Liver Transplantation [J].
Aussilhou, Beatrice ;
Doufle, Ghislaine ;
Hubert, Catherine ;
Francoz, Claire ;
Paugam, Catherine ;
Paradis, Valerie ;
Farges, Olivier ;
Vilgrain, Valerie ;
Durand, Francois ;
Belghiti, Jacques .
ANNALS OF SURGERY, 2010, 252 (05) :735-741
[5]  
BERREBI G, 1982, CLIN GENET, V21, P342
[6]   Canadian liver transplant allocation for hepatocellular carcinoma [J].
Brahmania, Mayur ;
Marquez, Vladimir ;
Kneteman, Norman M. ;
Bhat, Mamatha ;
Marleau, Denis ;
Wong, Philip ;
Peletekian, Kevork M. ;
Burak, Kelly W. ;
Congly, Stephen E. .
JOURNAL OF HEPATOLOGY, 2019, 71 (05) :1058-1060
[7]   Polycystic liver disease: a clinical review [J].
Chandok, Natasha .
ANNALS OF HEPATOLOGY, 2012, 11 (06) :819-826
[8]   Everolimus does not further reduce polycystic liver volume when added to long acting octreotide: Results from a randomized controlled trial [J].
Chrispijn, Melissa ;
Gevers, Tom J. G. ;
Hol, Jeroen C. ;
Monshouwer, Rene ;
Dekker, Heleen M. ;
Drenth, Joost P. H. .
JOURNAL OF HEPATOLOGY, 2013, 59 (01) :153-159
[9]   Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management [J].
Cnossen, Wybrich R. ;
Drenth, Joost P. H. .
ORPHANET JOURNAL OF RARE DISEASES, 2014, 9
[10]  
Doshi SD, 2018, TRANSPLANTATION, V101, P1838, DOI [10.1097/TP.0000000000001711, DOI 10.1097/TP.0000000000001711.WAITLISTED]