背景及原理:肝肺综合征(HPS)为晚期肝脏疾病的肺脏血管紊乱性疾病,与肺内血管扩张引起的血氧不足有关。无论肝病严重程度,HPS可以独立增加死亡率。肝移植可改善HPS生存率。低氧血症及肝移植情况进行了观察。我们评估了从1986年至2010年梅奥诊所就诊的106例HPS患者。使用Kaplan-Meier进行生存评估。结果:共有49例患者进行了肝脏移植。根据诊断HPS时基线PaO2划分的移植后生存率(1,3,5,10年)并无差异。1/1/2002之后进行移植的患者 (n=28)1,3,5年生存率 (92%, 88% 88%)优于之前移植患者(n=21,71%,67% 和67%)。因此研究得出结论,HPS肝移植后长期结局较好,且生存率得到较好改善,但生存率与HPS诊断时的PaO2水平无相关性。
重庆西南医院感染科 朱鹏 摘译
本文来源于[HEPATOLOGY ,2012]
Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era.
Background and Rationale: Hepatopulmonary syndrome (HPS) is a pulmonary vascular disorder occurring as a consequence of advanced liver disease, characterized by hypoxemia due to intrapulmonary vascular dilatations. HPS independently increases mortality, regardless the cause or severity of liver disease. Liver transplantation (LT) improves survival in HPS. We present the largest consecutive series of HPS patients specifically addressing long-term survival relative to the degree of hypoxemia and the era in which LT was conducted. We evaluated 106 HPS patients at the Mayo Clinic from 1986 through 2010. Survival was assessed using Kaplan-Meier methodology. Results: LT was accomplished in 49 HPS patients. Post-LT survival (1, 3, 5 and 10 year) did not differ between groups based on baseline Pa0(2) obtained at the time of HPS diagnosis. Improvements in overall survival at 1, 3 and 5 years post-LT in those HPS patients transplanted after 1/1/2002 (n=28) (92%, 88% and 88%, respectively) as compared to those transplanted prior to that time (n=21) (71%, 67% and 67%, respectively) did not reach statistical significance (5-year p= 0.09). Model for Endstage Liver Disease (MELD) exception to facilitate LT was granted to 21 patients since 1/1/2002 with post-LT survival of 19/21 patients and one wait-list death. Conclusion: Long-term outcome after LT in HPS is favorable with a trend towards improved survival in the MELD exception era since 2002 as compared to earlier HPS transplants. Survival after LT was not associated with PaO(2) levels at the time of HPS diagnosis.
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