应用儿童终末期肝病模型评分预测Kasai术后联合激素治疗胆道闭锁的短期预后

目的 应用儿童终末期肝病模型(pediatric of end-stage liver disease,PELD)评分系统预测Kasai手术联合激素治疗胆道闭锁患儿的预后.方法 80例胆道闭锁患儿随机分为Ksai手术联合激素组及单纯Kasai手术组,应用PELD评分系统对每例患儿进行评分,比较两种方法治疗后生化指标、病死率及PELD分值的不同,探讨PELD评分与预后的关系.结果 PELD分值小于16的手术联合激素组患儿总胆红素、凝血酶原时间的国际标准化比值(INR)及PELD分值分别为(23.3±1.1)mg/L、2.31±0.24和10.6±2.3,明显低于单纯手术组的(28.9±2.1)mg/L、2.63±0.18和13.2±2.7.白蛋白则高于单纯手术组,差异有统计学意义(P＜0.05).16≤PELD＜28的患儿手术联合激素治疗后总胆红素、INR及PELD分值分别为(78.7±1.7)mg/L、3.03±0.24和17.1±1.8.明显低于单纯手术组的(92.6±2.1)mg/L、3.42±0.29和20.8±2.4,差异有统计学意义(P＜0.05).白蛋白的水平比单纯手术组高,差异有统计学意义(P＜0.05).PELD≥28的患儿仅总胆红素两组之间比较有统计学差异.PELD＜28的患儿,手术联合激素组患儿的病死率明显低于单纯手术组,差异有统计学意义.而PELD≥28的患儿的病死率两组之间比较差异无统计学意义.结论 激素通过降低总胆红素、INR及PELD分值,改善肝功能.PELD分值在28以下的患儿,激素辅助治疗可提高这部分胆道闭锁患儿的短期生存率.PELD分值大于28的患儿,即使应用激素治疗也不能提高短期生存率.     

胆道闭锁Kasai术后药物辅助治疗的研究进展

胆道闭锁(biliary atresia, BA)是婴儿特有的肝胆系统疾病, 肝门肠吻合术(Kasai手术)是目前首选的治疗方式。然而, Kasai术后持续进展的肝脏炎症及纤维化导致绝大多数患儿需要肝移植才能长期生存。因此, 如何改善Kasai手术的预后、推迟甚至避免肝移植的发生, 是当前研究的重点和热点。本文回顾并总结Kasai术后辅助性治疗相关文献及最新研究进展, 期望为改善BA患儿预后提供思路。     

Kasai手术前特异性超声征象评估胆道闭锁肝纤维化的价值

目的探讨胆道闭锁(biliary atresia, BA)特异性超声征象与肝纤维化评分的相关性, 为Kasai手术前多模态超声评估肝纤维化程度提供依据。方法回顾性分析2020年2月至2022年9月在南京医科大学附属儿童医院经手术确诊为BA并行Kasai手术67例患儿的临床资料, 其中男26例, 女41例。术前1周内均行BA相关的超声检查, 检查年龄为(50.57±18.53)d, 范围在17～95 d。术后对肝脏病理标本进行Metavir纤维化评分。Pearson分析和Spearman分析法分析超声检查年龄、肝门部三角索带征(triangular cord sign, TC)厚度、胆囊最大截面积(maximum area of the gallbladder, MAG)、肝脏硬度值(liver stiffness measurement, LSM)、肝纤维化评分间的相关性。绘制受试者操作特征(receiver operating characteristic, ROC)曲线评估TC厚度、MAG、LSM诊断BA肝纤维化程度的效能。结果所有患儿都存在一定程度的肝纤维化, 4例(6.0%, ...     

影响胆道闭锁疗效的因素与对策

<正>肝门空肠吻合术(Kasai手术)和肝移植是治疗胆道闭锁(Biliary Atresia,BA)的两种方式。与欧美国家不同,受医疗模式、经济水平、传统观念等因素影响,我国目前对胆道闭锁的治疗以两者结合为主或以Kasai手术为多~([1])。Kasai手术可使部分患儿长期靠自体肝存活,或使肝移植的年龄推后。本文     

胆道闭锁Kasai手术后综合管理

胆道闭锁(biliary atresia,BA)是婴儿期肝内外胆管进行性炎症及肝纤维化的疾病,其病因及疾病进展机制尚不清楚.Kasai手术(Kasai portoenterostomy,KPE)是治疗胆道闭锁的首选术式,成功的Kasai手术能够重建胆汁引流,但Kasai手术后长期自体肝生存(native liver s...     

Kasai手术临床应用和研究进展

Kasai手术(Kasai procedure,KP)是治疗小儿肝胆疾病重建胆道最常用的手术方法之一,但目前关于KP临床应用性研究论述较少,作者现就KP临床应用性研究进行综述. 一、KP临床研究概述 二十世纪KP手术问世,并成为有效的外科治疗先天性胆道闭锁(bilary atresia,BA)方法.目前KP是绝大多数BA的首选治疗;术后50％～60％可获得正常胆汁引流;术后50％患儿依赖自体肝脏生存(survival with a native liver,SNL)超过20年[1].伴随肝移植的出现,外科医生用肝移植来治疗BA.但目前小儿肝移植相匹配的供体稀少;减体积、劈离式肝移植技术要求高;婴幼儿胆道、门静脉和胆管较细吻合难度高术后并发症易导致肝移植失败[2].     

胆道闭锁诊治新进展

胆道闭锁(Biliary Atresia,BA)是以肝内外胆管闭锁和梗阻性黄疸为特点的小儿外科常见畸形,其发病率为1/5 000～1/18 000.Kasai手术是该病一线治疗方法,但经Kasai手术治疗的BA患儿仍有约70%最终发展为肝硬化,需要接受肝移植治疗.早期诊断和治疗是提高BA预后的重要途径,因此本文对BA诊断和治疗方面的最新研究进展进行综述,以期提高BA诊断水平和改善BA治疗效果.     

胆道闭锁诊治新进展

胆道闭锁(Biliary Atresia,BA)是以肝内外胆管闭锁和梗阻性黄疸为特点的小儿外科常见畸形,其发病率为1/5 000～1/18 000.Kasai手术是该病一线治疗方法,但经Kasai手术治疗的BA患儿仍有约70%最终发展为肝硬化,需要接受肝移植治疗.早期诊断和治疗是提高BA预后的重要途径,因此本文对BA诊断和治疗方面的最新研究进展进行综述,以期提高BA诊断水平和改善BA治疗效果.     

HDAC2的表达水平评分与胆道闭锁肝纤维化进展及预后的关系

目的探究组蛋白脱乙酰酶(histone deacetylase, HDAC)2在胆道闭锁(biliary atresia, BA)患儿肝组织中表达水平、诊断价值及其与BA肝纤维化进展和预后的关系。方法回顾性分析2019年1月至2021年12月因黄疸在天津市儿童医院普外科行手术治疗的患儿44例, 包括BA患儿34例(BA组)和其他胆汁淤积性肝脏疾病10例(DC组)。使用生物信息学分析HDAC中HDAC1～HDAC11在BA中的表达情况。取BA组和DC组患儿肝组织样本, 通过免疫组织化学染色和实时荧光定量PCR检测HDAC2在两组患儿肝组织中表达水平, 对HDAC2的表达进行评分。使用受试者操作特征(receiver operating characteristic, ROC)曲线评估HDAC2评分对BA的诊断价值。分析BA患儿HDAC2表达与一般临床资料的关系。所有BA患儿在Kasai术后均进行了随访, 记录术后6个月时的黄疸清除率(jaundice clearance rate, JCR)和自体肝生存率(survival with native liver rate, SNL)。采用C...     

活体肝移植治疗胆道闭锁:22例单中心诊治经验

目的 总结活体肝移植治疗儿童终末期肝病胆道闭锁(BA)的外科治疗经验.方法 2006年6月至2009年7月,共完成亲属活体部分肝移植治疗胆道闭锁22例.术后平均随访18.5(1至36个月)个月,回顾总结肝移植患儿临床病理资料.结果 22例胆道闭锁患儿中已行Kasai手术9例,20例低于1岁,21例低于10kg.其手术平均时间、平均出血均无差别.供肝受体质量(GR/WR)比3.5(2.1～5.7)%.有27例次术后并发症,包括3例术后出血再手术止血,1例门静脉并发症,肝静脉并发症1例,3例肝动脉并发症,胆道并发症1例.有3例围手术死亡,1例再次移植,急性排斥5例(22.7%).6个月、1年和3年受体生存率分别为86.4%、82.2%和82.2%.结论 受体体重,移植物受体质量比,是否行Kasai手术对肝移植手术效果无影响;适当的供肝质量,细致的手术技巧可增加手术成功率.     

Impaired intention‐to‐treat survival after listing for liver transplantation in children with biliary atresia compared to other chronic liver diseases: 20 years’ experience from the Nordic countries

Biliary atresia (BA) is the most common indication for LT in children. We investigated whether this diagnosis per se, compared to other chronic liver diseases (OCLD), had an influence on patient survival. Data from 421 Scandinavian children, 194 with BA and 227 with OCLD, listed for LT between 1990 and 2010 were analyzed. The intention‐to‐treat survival and influencing risk factors were studied. Patients with BA had higher risk of death after listing than patients with OCLD. The youngest (<1 year) and smallest (<10 kg) children with the highest bilirubin (>510 μmol/L), highest INR (>1.6), and highest PELD score (>20) listed during 1990s had the worst outcome. Given the same PELD score, patients with BA had higher risk of death than patients with OCLD. For adolescents, low weight/BMI was the only prognostic marker. Impaired intention‐to‐treat survival in patients with BA was mainly explained by more advanced liver disease in younger ages and higher proportion of young children in the BA group rather than diagnosis per se. PELD score predicted death, but seemed to underestimate the severity of liver disease in patients with BA. Poor nutritional status and severe cholestasis had negative impact on survival, supporting the “sickest children first” allocation policy and correction of malnutrition before surgery.     

The efficacy of measurement of the serum beta-d glucan in the patients with biliary atresia

Background

The pediatric end-stage liver disease (PELD) score is not a direct index that reflects the degree of hepatocellular injury. Beta-d glucan (BDG) in the portal vein blood is processed by the hepatic reticuloendothelial system. It is possible that the hepatic clearance of BDG may be used as a biological index to assess the liver function. In this study, the relationship between PELD score and hepatic clearance of BDG was made clear in order to study the efficacy of measurement of the serum BDG.

Methods

This study including 21 patients with biliary atresia (BA) who underwent liver transplantation (LT) was performed. The BDG was measured in the preoperative peripheral vein blood and the portal vein blood at the time of LT.

Results

The portal vein blood showed a significantly high level of BDG than the peripheral vein blood (p?<?0.01). There was a significant negative correlation between the PELD score and the hepatic clearance of BDG in the 10 patients who were indicated for LT due to liver failure (p?<?0.01).

Conclusion

The serum BDG can be used as a biological index in place of liver metabolism and should be measured in BA patients as a non-invasive indicator of the degree of progression of liver failure.     

Hyponatremia increases mortality in pediatric patients listed for liver transplantation

Carey RG, Bucuvalas JC, Balistreri WF, Nick TG, Ryckman FR, Yazigi N. Hyponatremia increases mortality in pediatric patients listed for liver transplantation.
Pediatr Transplantation 2010: 14: 115–120. © 2009 John Wiley & Sons A/S.
Abstract:  To evaluate hyponatremia as an independent predictor of mortality in pediatric patients with end-stage liver disease listed for transplantation. We performed a single-center retrospective study of children listed for liver transplantation. We defined hyponatremia as a serum sodium concentration <130 mEq/L that persisted for at least seven days. The primary outcome was death on the waiting list. Ninety-four patients were eligible for the study. The prevalence of hyponatremia was 26%. Kaplan–Meier survival analysis demonstrated that patients with hyponatremia had decreased pretransplant survival compared with patients who maintained a serum sodium >130 mEq/L (p < 0.001). Univariable association analyses demonstrated death on the waiting list was also associated with higher median PELD scores at listing (p = 0.01), non-white race (p = 0.02), and age <1 yr (p = 0.001). Logistic regression analysis identified hyponatremia and non-white race as independently associated with pretransplant mortality [OR = 8.0 (95% CI: 1.4–45.7), p = 0.02 and OR = 6.3 (95% CI: 1.25–33.3), p = 0.03]. When hyponatremia was added to the PELD score, it was significantly better in predicting mortality than the PELD score alone ( c -statistic = 0.79, p = 0.03). Hyponatremia identifies a subset of pediatric patients with increased risk of pretransplant mortality and improves the predictive ability of the current PELD score.     

The efficacy of serum brain natriuretic peptide for the early detection of portopulmonary hypertension in biliary atresia patients before liver transplantation

Severe portopulmonary hypertension (POPH) is a contraindication for liver transplantation (LT) because of the high risk of postoperative heart failure. The early detection of POPH is important for patients with biliary atresia (BA). Brain natriuretic peptide (BNP) is known to be correlated with liver fibrosis in patients with liver cirrhosis. The aim of this study was to elucidate the efficacy of BNP measurement for the follow‐up of patients with BA. Thirty‐two patients with BA were identified from September 2011 to December 2016. As indices of liver fibrosis/cirrhosis, APRI (P < .0001), FIB‐4 (P < .0001), Child‐Pugh score (P < .0001), IV collagen (P = .0005), and hyaluronic acid (P = .0291) had high or moderate correlations with BNP. Patients with splenomegaly, esophageal varices, liver fibrosis, and collateral veins had significantly higher BNP levels than those without. Patients diagnosed with POPH had significantly higher BNP levels in comparison with those patients without (P = .0068). In contrast, PELD/MELD scores showed an almost negligible correlation with the BNP level. LT was successful in 3 asymptomatic BA patients with POPH who had high BNP levels despite the low PELD/MELD scores. In conclusion, routine serum BNP surveillance can be easy to predict asymptomatic POPH. This may help to identify POPH before it reaches a stage that would contraindicate LT.     

Does the pediatric end-stage liver disease score or hepatic artery resistance index predict outcome after liver transplantation for biliary atresia?

The pediatric end-stage liver disease score (PELD) was devised and validated as a tool for predicting mortality and morbidity in children with chronic liver disease waiting for a liver transplant (LT). It has become a useful guide for prioritizing organ allocation in the United States. The hepatic artery resistance index (HARI) also predicts waiting list mortality in children with biliary atresia. Does the PELD score or HARI predict outcome after LT for biliary atresia? Twenty consecutive children who underwent LT for biliary atresia between 2001 and 2005 were reviewed. Their PELD score was calculated periodically between listing and transplantation and HARI was measured at listing. Outcome variables were operative blood transfusion requirements, ICU stay and postoperative stay. Median age at LT was 8 (2–204) months. After allowing for the type of graft, the PELD score and the change in PELD score between listing and LT (ΔPELD) showed no significant correlation with blood transfusion requirements, but both the PELD score at listing and ΔPELD showed a trend toward a statistically significant positive correlation with overall hospital stay. Pre-transplant HARI showed a statistically significant positive correlation with the PELD score at listing (r = 0.46, p = 0.05) but did not correlate significantly with hospital stay. In this relatively small but homogeneous group of children undergoing LT for biliary atresia, PELD, and ΔPELD scores showed a trend toward a statistically significant positive correlation with overall hospital stay. However, neither PELD scores nor the pre-transplant HARI showed a definite correlation with outcome. Post-transplant complications are probably more important factors determining ICU and hospital stay in children currently transplanted for biliary atresia.     

Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation

OBJECTIVES: To test the hypothesis that risk analysis from the time of listing for liver transplantation (LT) focuses attention on areas where outcomes can be improved. STUDY DESIGN: Competing outcomes and multivariate models were used to determine significant risk factors for pretransplantation and posttransplantation mortality and graft failure in patients with biliary atresia (BA) listed for LT and enrolled in the Studies of Pediatric Liver Transplantation (SPLIT) registry. RESULTS: Of 755 patients, most were infants (age < 1 year). Significant waiting list mortality risk factors included infancy and pediatric end-stage liver disease (PELD) score > or = 20, whose components were also continuous risk factors. Survival posttransplantation (n=567) was 88% at 3 years. Most deaths were from infection (37%). Posttransplantation mortality risk factors included infant recipients, height/weight < -2 standard deviations (SD), use of cyclosporine versus tacrolimus and retransplantation. Graft failure risks included height/weight < -2 SD, cadaveric partial donors, donor age < or = 5 months, use of cyclosporine versus tacrolimus, and rejection. CONCLUSIONS: Referral for LT should be anticipatory for infants with BA with failed portoenterostomies. Failing nutrition should prompt aggressive support. Post-LT risk factors are mainly nonsurgical, including nutrition, the relative risk of infection over rejection, and the choice of immunosuppression.     

儿童DCD供肝肝移植术后胆道并发症高危因素研究

目的 分析儿童心脏死亡器官捐献(DCD)肝移植受者术后胆道并发症发生的高危因素.方法 收集天津市第一中心医院2013年3月至2015年3月施行的48例儿童DCD肝移植手术的临床资料,回顾性分析临床因素对受者胆道并发症的影响.结果 48例儿童DCD肝移植受者术后共11例发生胆道并发症,发生率为22.9％.单因素分析显示胆道并发症组与对照组间的热缺血时间(P=0.003)差异有统计学意义(P＜0.05),受体年龄(P--0.998)、受体性别(P=0.094)、MELD评分(P=0.159)、PELD评分(P=0.740)、Child-Pugh评分(P=0.159)、冷缺血时间(P=0.990)、受体ICU逗留时间(P=0.105)、是否发生感染(P=0.930)、有无其他并发症(门静脉狭窄/血栓形成、肝动脉栓塞、DGF)(P=0.268)以及A3O血型是否相容(P=1.106)差异无统计学意义(P＞0.05).多因素分析显示热缺血时间(P=0.020,OR=10.367,95％可信区间为1.451～74.089)是术后胆道并发症的独立危险因素.结论 胆道并发症仍然是儿童DCD肝移植术后的重要难题,热缺血时间是受者术后胆道并发症的独立危险因素.因此选择更短热缺血时间的CDC供肝可以降低儿童肝移植受者胆道并发症的发生率.     

Use of a pediatric end-stage liver disease score for deceased donor allocation: The United States experience

The Pediatric end-stage liver disease (PELD) score was developed as a measure of the severity of chronic liver disease that would predict mortality or children awaiting liver transplant. From multivariate analyses a model was derived that included five objective factors which together comprise the PELD score. The factors are growth failure, age less than 1 year, international normalized ratio (INR), serum albumin and total bilirubin.