Liver transplantation in adult polycystic liver disease: the Ontario experience

作者：Mohammed O. Alsager, Shuet Fong Neong, R.K. Gandhi, Anouar Teriaky, Ephraim Tang, Anton Skaro, Karim Qumosani, Les Lilly, Zita Galvin, Nazia Selzner, Mamatha Bhat, Klajdi Puka, Mayur Brahmania · 发表于：BMC Gastroenterology · 年份：2021 · DOI：10.1186/s12876-021-01703-x · 被引用次数：18 · 研究领域：Genetic and Kidney Cyst Diseases、Organ Transplantation Techniques and Outcomes、Liver Disease and Transplantation

BACKGROUND: Liver 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. METHODS: A 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. RESULTS: A 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 fema...