肝门空肠吻合术后早期预测胆道闭锁患儿自体肝存活情况
Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia.
作者信息
Nightingale Scott, Stormon Michael O, O'Loughlin Edward V, Shun Albert, Thomas Gordon, Benchimol Eric I, Day Andrew S, Adams Susan, Shi Edward, Ooi Chee Y, Kamath Binita M, Fecteau Annie, Langer Jacob C, Roberts Eve A, Ling Simon C, Ng Vicky L
机构信息
*Department of Gastroenterology, John Hunter Children's Hospital †Discipline of Paediatrics and Child Health, University of Newcastle, Newcastle, Australia ‡Transplant and Regenerative Medicine Centre §Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children ||Department of Paediatrics, University of Toronto, Toronto, Canada ¶Department of Gastroenterology, The Sydney Children's Hospital Network-Westmead #Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney **Department of Surgery, The Sydney Children's Hospital Network-Westmead, Sydney, Australia ††Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario ‡‡Department of Pediatrics and School of Epidemiology, Public Health and Preventative Medicine, University of Ottawa, Ottawa, Canada §§Department of Gastroenterology, The Sydney Children's Hospital Network-Randwick, Sydney, Australia ||||Department of Paediatrics, University of Otago, Christchurch, New Zealand ¶¶Department of Surgery, The Sydney Children's Hospital Network-Randwick ##School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, Australia ***Division of General Surgery, Hospital for Sick Children †††Department of Surgery, University of Toronto, Toronto, Canada.
出版信息
J Pediatr Gastroenterol Nutr. 2017 Feb;64(2):203-209. doi: 10.1097/MPG.0000000000001289.
OBJECTIVES
Most infants with biliary atresia (BA) require liver transplantation (LT) after hepatoportoenterostomy (HPE), including those who initially clear jaundice. The aim of the present study was to identify clinical and routine laboratory factors in infants with BA post-HPE that predict native liver survival at 2 years.
METHODS
A retrospective cohort study was conducted in 217 patients with BA undergoing HPE in Sydney, Australia and Toronto, Canada between January 1986 and July 2009. Univariate and multivariate logistic regression using backwards-stepwise elimination identified variables at 3 months after HPE most associated with 2-year native liver survival.
RESULTS
Significant variables (P < 0.05) on univariate analysis included serum total bilirubin (TB) and albumin at 3 months post-HPE, bridging fibrosis or cirrhosis on initial liver biopsy, ascites of <3 months post-HPE, type 3 BA anatomy, age at HPE of >45 days, change in length z scores within 3 months of HPE, and center. On multivariate analysis, TB (P < 0.0001) and albumin (P = 0.02) at 3 months post-HPE, and center (P = 0.0003) were independently associated with native liver survival. Receiver operating characteristic analysis revealed an optimal cut-off value of TB <74 μmol/L (4.3 mg/dL; area under the receiver operating characteristic curve 0.8990) and serum albumin level >35 g/L (3.5 mg/dL; area under the receiver operating characteristic curve 0.7633) to predict 2-year native liver survival. TB and albumin levels 3 months post-HPE defined 3 groups (1: TB ≤74 μmol/L, albumin >35 g/L; 2: TB ≤74 μmol/L, albumin ≤35 g/L; 3: TB >74 μmol/L) with distinct short- and long-term native liver survival rates (log-rank P < 0.001). Length z scores 3 months post-HPE were poorer for group 2 than group 1 (-0.91 vs -0.30, P = 0.0217) with similar rates of coagulopathy.
CONCLUSIONS
Serum TB and albumin levels 3 months post-HPE independently predicted native liver survival in BA when controlling for center. Serum albumin level <35 g/L in infants with BA who were no longer jaundiced at 3 months post-HPE was a poor prognostic indicator. Poorer linear growth and absence of significant coagulopathy suggest a role for early aggressive nutritional therapy in this group.
目的
大多数胆道闭锁（BA）婴儿在肝门肠吻合术（HPE）后需要肝移植（LT），包括那些最初黄疸消退的婴儿。本研究的目的是确定HPE后BA婴儿的临床和常规实验室因素，以预测2年时自体肝生存率。
方法
对1986年1月至2009年7月在澳大利亚悉尼和加拿大多伦多接受HPE的217例BA患者进行回顾性队列研究。采用向后逐步排除法进行单因素和多因素逻辑回归，确定HPE后3个月时与2年自体肝生存率最相关的变量。
结果
单因素分析中显著变量（P<0.05）包括HPE后3个月时的血清总胆红素（TB）和白蛋白、初始肝活检时的桥接纤维化或肝硬化、HPE后<3个月的腹水、3型BA解剖结构、HPE时年龄>45天、HPE后3个月内长度z评分的变化以及中心。多因素分析显示，HPE后3个月时的TB（P<0.0001）和白蛋白（P=0.02）以及中心（P=0.0003）与自体肝生存率独立相关。受试者工作特征分析显示，预测2年自体肝生存率的最佳截断值为TB<74μmol/L（4.3mg/dL；受试者工作特征曲线下面积0.8990）和血清白蛋白水平>35g/L（3.5mg/dL；受试者工作特征曲线下面积0.7633）。HPE后3个月时的TB和白蛋白水平定义了3组（1组：TB≤74μmol/L，白蛋白>35g/L；2组：TB≤74μmol/L，白蛋白≤35g/L；3组：TB>74μmol/L），其短期和长期自体肝生存率明显不同（对数秩检验P<0.001）。2组HPE后3个月时的长度z评分比1组差（-0.91对-0.30，P=0.0217），凝血障碍发生率相似。
结论
在控制中心因素的情况下，HPE后3个月时的血清TB和白蛋白水平可独立预测BA患者的自体肝生存率。HPE后3个月时已无黄疸的BA婴儿血清白蛋白水平<35g/L是不良预后指标。线性生长较差且无明显凝血障碍提示该组早期积极营养治疗可能起作用。
Zotero 插件