Colorado School of Public Health at University of Colorado Denver, Aurora, CO, USA.
Liver Transpl. 2012 May;18(5):585-93. doi: 10.1002/lt.23389.
Severe liver disease affects 4.5% to 10% of individuals with cystic fibrosis (CF) and is the third-leading cause of death. Liver transplantation (LT) is an accepted therapy, but the effects of liver disease and LT on pulmonary function in patients with CF are controversial. Our aim was to characterize changes in pulmonary function in LT patients with CF. Using mixed effect models, we analyzed pulmonary function before and after transplantation in 168 LT patients and 840 non-LT patients with CF who were matched by age, sex, pancreatic status, infections with US CF Foundation Patient Registry data (1989-2007). The primary outcome was the change in the forced expiratory volume in 1 second (FEV(1); percent predicted) in LT and non-LT in the 3-years periods before or after transplantation; second we compared FEV(1) changes. In the 3 years before transplantation, LT had lower initial FEV(1) values (71.5% ± 1.9%, P < 0.001) and a slower decline (+0.1% ± 0.4%/year, P < 0.001) than non-LT (79.6% ± 1.3% and -1.3% ± 0.2%/year, respectively). There was no difference in the FEV(1) decline after transplantation (-1.4% ± 0.4%/year for LT versus -2.1% ± 0.2%/year for non-LT, P = 0.14). Both the (P = 0.003) and (P = 0.001) had a slower FEV(1) decline in the period before transplantation versus after transplantation. In conclusion, pulmonary function is lower and declines more slowly in patients with CF before LT versus, but parallels the decline in non-LT after transplantation. LT is neither beneficial nor detrimental to pulmonary function in CF but returns FEV(1) decline to the same trajectory found for matched non-LT individuals with CF.
严重的肝脏疾病影响 4.5%至 10%的囊性纤维化（CF）患者，是导致死亡的第三大原因。肝移植（LT）是一种公认的治疗方法，但 CF 患者的肝脏疾病和 LT 对肺功能的影响存在争议。我们的目的是描述 CF 患者 LT 后肺功能的变化。使用混合效应模型，我们分析了 168 例 CF 患者 LT 前后和 840 例 CF 患者非 LT 匹配（根据年龄、性别、胰腺状态、美国 CF 基金会患者登记处数据中感染情况匹配）的肺功能，时间为 1989 年至 2007 年。主要结局是 LT 和非 LT 患者在移植前 3 年和移植后 3 年的第 1 秒用力呼气量（FEV1；预测百分比）的变化；其次比较 FEV1 的变化。在移植前 3 年内，LT 的初始 FEV1 值较低（71.5%±1.9%，P<0.001），下降速度较慢（0.1%±0.4%/年，P<0.001），而非 LT 的初始 FEV1 值较高（79.6%±1.3%和-1.3%±0.2%/年，分别）。移植后 FEV1 的下降没有差异（LT 为-1.4%±0.4%/年，而非 LT 为-2.1%±0.2%/年，P=0.14）。（P=0.003）和（P=0.001）的 FEV1 下降在移植前和移植后都较慢。总之，CF 患者在 LT 前的肺功能较低，下降速度较慢，但与移植后非 LT 的下降速度一致。LT 对 CF 患者的肺功能既无益处也无损害，而是使 FEV1 下降恢复到与匹配的非 LT 个体相同的轨迹。
