BACKGROUND Very few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis. AIMS To evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia. METHODS All children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA = no ascites; A1 = grade 1 ascites; A2 = grade 2 ascites; and A3 = grade 3 ascites. The primary endpoint of the study was mortality within the first year after patient inclusion. Ninety-day mortality was also evaluated. Prognostic factors related to both endpoints also were studied. RESULTS One-year patient survival for NA was 97.1%, versus 80.8% for A1, versus 52% for A2, versus 13.6 for A3 (p < 0.001). The presence of ascites increased mortality by 17 times. In the multivariate analysis, clinically detectable ascites (HR 3.14, 95% CI 1.14-8.60, p = 0.026), lower sodium (HR 1.15, 95% CI 1.04-1.27, p = 0.006), higher bilirubin (HR 1.06, 95% CI 1.00-1.12, p = 0.023), and higher PELD score (HR 1.05, 95% CI 1.02-1.08, p = 0.001) were all associated with decreased survival. Lower serum sodium (HR 1.20, 95% CI 1.09-1.32, p < 0.001) and higher PELD score (HR 1.03, 95% CI 1.001-1.063, p = 0.043) were associated with increased 90-day mortality. CONCLUSIONS Clinically detectable ascites is associated with decreased 1-year survival of children with biliary atresia. These patients should be treated with caution and prioritized for liver transplantation.

中文翻译：

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胆源性闭锁继发性肝硬化患者的腹水严重程度与死亡率增加有关。

背景技术很少有先前的研究调查肝硬化患儿腹水作为预后因素的存在。据我们所知，尚无以前的研究评估胆道闭锁和肝硬化患儿腹水严重程度与患者生存率之间的关系。目的评估腹水严重程度与肝硬化和胆道闭锁患儿生存之间的关系。方法将2000年至2014年在我院评估的所有继发于胆道闭锁的肝硬化患儿纳入本研究。患者分为四组：NA =无腹水；A1 = 1级腹水；A2 = 2级腹水；和A3 = 3级腹水。研究的主要终点是患者入院后第一年内的死亡率。还评估了90天的死亡率。还研究了与两个终点相关的预后因素。结果NA的一年患者生存率为97.1％，而A1为80.8％，A2为52％，A3为13.6（p <0.001）。腹水的存在使死亡率增加了17倍。在多变量分析中，临床上可检测到的腹水（HR 3.14，95％CI 1.14-8.60，p = 0.026），较低的钠盐（HR 1.15，95％CI 1.04-1.27，p = 0.006），较高的胆红素（HR 1.06，95％ CI 1.00-1.12，p = 0.023）和较高的PELD评分（HR 1.05，95％CI 1.02-1.08，p = 0.001）均与存活率降低相关。较低的血清钠（HR 1.20，95％CI 1.09-1.32，p <0.001）和较高的PELD评分（HR 1.03，95％CI 1.001-1.063，p = 0.043）与90天死亡率增加相关。结论临床可检测到的腹水与胆道闭锁患儿的1年生存期降低有关。