Ye Wen, Narkewicz Michael R, Leung Daniel H, Karnsakul Wikrom, Murray Karen F, Alonso Estella M, Magee John C, Schwarzenberg Sarah Jane, Weymann Alexander, Molleston Jean P
University of Michigan, Ann Arbor, MI.
Digestive Health Institute, Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, CO.
J Pediatr Gastroenterol Nutr. 2018 Jan;66(1):122-127. doi: 10.1097/MPG.0000000000001728.
Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver transplant (LT), and liver-related death (LD), and risk factors for these in CF Foundation Patient Registry subjects with reported cirrhosis.
We determined 10-year incidence rates for VB, LT, LD, and all-cause mortality (ACM), and examined risk factors using competing risk models and Cox-proportional hazard regression.
From 2003 to 2012, 943 participants (41% females, mean age 18.1 years) had newly reported cirrhosis; 24.7% required insulin, 85% had previous pseudomonas. Seventy-three subjects had reported VB: 38 with first VB and new cirrhosis reported simultaneously and 35 with VB after cirrhosis report. Ten-year cumulative VB, LT, and LD rates were 6.6% (95% confidence interval [CI]: 4.0, 9.1%), 9.9% (95% CI: 6.6%, 13.2%), and 6.9% (95% CI: 4.0%, 9.8%), respectively, with an ACM of 39.2% (95% CI: 30.8, 36.6%). ACM was not increased in subjects with VB compared to those without (hazard ratio [HR] 1.10, 95% CI: 0.59, 2.08). CF-related diabetes (HR: 3.141, 95% CI:1.56, 6.34) and VB (HR: 4.837, 95% CI: 2.33, 10.0) were associated with higher LT risk, whereas only worse lung function was associated with increased LD in multivariate analysis. Death rate among subjects with VB was 24% with LT and 20.4% with native liver.
VB is an uncommon complication of CF cirrhosis and can herald the diagnosis, but does not affect ACM. Adverse liver outcomes and ACM are frequent by 10 years after cirrhosis report.
5%至10%的囊性纤维化（CF）患者会发生肝硬化，常伴有门静脉高压。我们分析了3种不良肝脏结局，即静脉曲张出血（VB）、肝移植（LT）和肝脏相关死亡（LD），以及CF基金会患者登记处报告有肝硬化的受试者中这些结局的危险因素。
我们确定了VB、LT、LD和全因死亡率（ACM）的10年发病率，并使用竞争风险模型和Cox比例风险回归分析危险因素。
2003年至2012年期间，943名参与者（41%为女性，平均年龄18.1岁）新报告有肝硬化；24.7%需要胰岛素治疗，85%曾感染过铜绿假单胞菌。73名受试者报告有VB：38名首次发生VB且同时报告有新的肝硬化，35名在报告肝硬化后发生VB。10年累积VB、LT和LD发生率分别为6.6%（95%置信区间[CI]：4.0，9.1%）、9.9%（95%CI：6.6%，13.2%）和6.9%（95%CI：4.0%，9.8%），ACM为39.2%（95%CI：30.8，36.6%）。与未发生VB的受试者相比，发生VB的受试者ACM并未增加（风险比[HR]1.10，95%CI：0.59，2.08）。CF相关糖尿病（HR：3.141，95%CI：1.56，6.34）和VB（HR：4.837，95%CI：2.33，10.0）与更高的LT风险相关，而在多变量分析中，只有较差的肺功能与LD增加相关。发生VB的受试者中，接受LT者的死亡率为24%，未接受LT者为20.4%。
VB是CF肝硬化的一种罕见并发症，可预示诊断，但不影响ACM。肝硬化报告后10年，不良肝脏结局和ACM很常见。
