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.     

Indications for liver transplantation

Patients should be considered for liver transplantation if they have evidence of fulminant hepatic failure, a life-threatening systemic complication of liver disease, or a liver-based metabolic defect or, more commonly, cirrhosis with complications such as hepatic encephalopathy, ascites, hepatocellular carcinoma, hepatorenal syndrome, or bleeding caused by portal hypertension. While the complications of cirrhosis can often be managed relatively effectively, they indicate a change in the natural history of the disease that should lead to consideration of liver transplantation. Referral to a liver transplant center is followed by a detailed medical evaluation to ensure that transplantation is technically feasible, medically appropriate, and in the best interest of both the patient and society. Patients approved for transplantation are placed on a national transplant list, although donor organs are allocated locally and regionally. Since 2002, priority for transplantation has been determined by the Model of End-Stage Liver Disease (MELD) score, which provides donor organs to listed patients with the highest estimated short-term mortality.     

神经电生理监测在经皮内窥镜下腰椎间盘切除术中的应用

目的:探讨神经电生理监测在经皮内窥镜下腰椎间盘切除术中的应用。方法:回顾性分析2014年4月~2014年7月收治的37例单节段腰椎间盘突出症患者的临床资料,男16例,女21例;平均年龄48.4±17.5岁。其中单纯腰椎间盘突出症35例,腰椎间盘突出合并椎管狭窄症2例。所有患者均在局部麻醉下行微创经侧路经皮内窥镜下腰椎间盘摘除术(percutaneous endoscope lumbar discectomy,PELD)。术中运用自发性肌电图(spontaneous electromyography,SEMG)监测神经根功能,同时纪录患者的主观感受(疼痛),分析两者之间的关系。结果:在手术通道放置过程中,所有患者在出现腰部疼痛时,神经电生理无明显改变。2例患者无明显腿痛,电生理却出现明显的成串、波幅较大的肌电反应,调整通道后电生理改变趋于正常。35例患者有明显腿痛且神经电生理出现明显的成串、波幅较大的肌电反应,立即提醒术者,查找原因,调整手术通道后,患者腿痛消失,且电生理发生明显改变,异常肌电反应减轻,趋于正常。所有患者在腰椎间盘髓核摘除时,无明显疼痛且神经电生理无明显变化。在剥离、解压粘连神经根时,均出现明显连串的电位波形改变和明显的腿痛,停止手术操作后,腿痛消失,神经电生理恢复正常。在直视下刺激神经根时,患者出现明显的腿痛,同时神经电生理出现连串的电位。所有患者健侧的监测结果均无明显电生理改变。术后所有患者均无神经并发症。以神经电生理监测作为安全指标,其真阳性率为100%,而以疼痛作为安全指标,其真阳性率为94.6%;假阴性率为5.4%。结论:术中应用自发性肌电图监测可提供更为客观的安全指标,从而提高手术安全性。     

Galactose half-life is a useful tool in assessing prognosis of chronic liver disease in children

In children, optimal timing of liver transplantation (LT) is crucial, but reliable prognostic tools for chronic liver diseases are scarce. We assessed the predictive value of galactose half‐life (Gal½) for LT or death. A retrospective search of hospital database 2003–2010 revealed 92 consecutive children with chronic liver disease (36 biliary atresia) whose liver function was assessed with Gal½ measurement. Gal½, follow‐up data, and liver biochemistry were recorded and pediatric/model for end‐stage liver disease (P/MELD) scores calculated. Patients listed for LT or those who died within 1 year of the Gal½ measurement (Group 1) were compared to those surviving without listing (Group 2). Predictive value of Gal½ and P/MELD for listing for LT was assessed with area under the receiver operating characteristic curve (AUROC) analysis. Group 1 had markedly increased median Gal½ [17.0 (interquartile range 12.5–28.5) min] and higher P/MELD [13 (?1–23)] compared with group 2, [10.5 (9.5–12.5) min and ?1 (?8–8); P < 0.001 for both]. Both Gal½ and P/MELD (P < 0.001) predicted listing or death with respective AUROCs of 0.808 (95% CI 0.704–0.913) and 0.780 (0.676–0.890), and 85% sensitivity and 69% specificity for Gal½≥12.0 min. Gal½ is a useful tool when evaluating 1‐year prognosis in children with chronic liver disease.     

Liver and Intestine Transplantation in the United States, 1995–2004

Three years of survival data are now available and the impact of the model for end-stage liver disease (MELD) allocation system is becoming clear. After a decline in new registrants to the waiting list in 2002, the number increased to 10 856 new patients in 2004. Since the implementation of MELD, the percentage of patients who have been on the list for 1–2 years has declined from 24% to 19%. There has been a shift upward in the percentage of patients with higher MELD scores on the waiting list.
An increasing percentage of adult living donor liver recipients are over the age of 50 years; from 1% in 1997 to 51% in 2004. Parents donating to children (93% of living donors in 1995), represented only 14% in 2004. Long-term adjusted patient survival declined with increasing recipient age in adults following either DDLT or LDLT.
Cirrhosis caused by chronic hepatitis C virus (HCV) is the leading indication for liver transplantation and is associated with reduced long-term survival in recipients with HCV compared to those without HCV, 68% at 5 years compared to 76%.
Although the intestine waiting list has more than doubled over the last decade, an increasing number of centers now perform intestinal transplantation with greater success.     

Evaluation of the PELD risk score as a severity index of biliary atresia

Purpose

The authors evaluated the validity of the Pediatric End-Stage Liver Disease (PELD) Risk Scoring System as a severity index for patients with biliary atresia.

Methods

Individual hospital records of 104 patients with biliary atresia were reviewed at our institution and divided into 3 groups: nontransplant survivors (n = 61), nontransplant deaths (n = 17), and transplant patients (n = 26). PELD risk scores were calculated according to Wiesner et al, multiplied by 10, and rounded to the nearest integer, as is done in determining model of end-stage liver disease (MELD) scores.

Results

The PELD scores showed a significant difference between nontransplant survivors (range, −21 to 15) and dying nontransplant patients during their last few months of life (range, 2 to 40). No survivors except those below the age of one year recorded scores above 10. Transplant patients had higher scores (range, −5 to 37) before transplantation than nontransplant survivors. However, the scores were not elevated in elderly patients with intractable cholangitis, fulminant variceal rupture, and hepatopulmonary syndrome.

Conclusions

PELD profiling is a useful scoring system for selecting patients with the most severe liver dysfunction caused by biliary atresia. However, we advise caution in using this system for patients under the age of 1 year and for older patients with long-term complications.     

局部麻醉行PELD时颈痛与颈椎硬膜外压力变化的相关性研究

目的 探讨局麻下椎板间入路行经皮内窥镜下腰椎髓核摘除术(PELD)术中颈痛与颈椎硬膜外压力(EP)变化的关系.方法 收集该院骨科2013～2015年28例因腰椎间盘突出症行经椎板间入路PELD的患者,术前于C5～C6节段安置颈椎硬膜外导管并连接压力传感器,整个手术过程持续监测颈椎EP.结果 28例患者中有6例发生了颈痛,术中颈痛患者开始诉颈痛时的颈椎EP值(51.5±10.0)mm Hg和最大颈椎EP值(67.8±12.4)mm Hg都明显高于未发生颈痛患者的最大颈椎EP值(29.6±12.6)mm Hg,差异有统计学意义(P＜0.05).颈椎EP值越高的患者发生颈痛的概率越高(P＜0.05).结论 椎板间入路行PELD时颈痛的发生与颈椎EP增高有关.