先天性胆道闭锁行与未行葛西术患儿临床病理特点——附58例对比研究

肝脏 ›› 2019, Vol. 24 ›› Issue (2): 126-129.

先天性胆道闭锁行与未行葛西术患儿临床病理特点——附58例对比研究

赵新颜, 罗文萍, 刘立伟, 魏琳, 何恩辉, 贾继东, 孙丽莹, 朱志军

100050 首都医科大学附属北京友谊医院肝病研究中心(赵新颜,罗文萍,刘立伟,贾继东);肝移植中心,国家消化系统疾病临床医学研究中心(赵新颜,罗文萍,刘立伟,魏琳,何恩辉,贾继东,孙丽莹,朱志军);首都医科大学儿童肝脏移植临床诊疗中心(魏琳,何恩辉,孙丽莹,朱志军)

收稿日期:2018-12-09 发布日期:2020-04-10
通讯作者: 朱志军,Email: zhu-zhijun@outlook.com
基金资助:
国家自然科学基金(81670545)

Difference in clinicopathological features of congenital biliary atresia children with and without Kasai procedure: a comparative study in 58 cases

ZHAO Xin-yan, LUO Wen-ping, LIU Li-wei, WEI Lin, HE En-hui , JIA Ji-dong, SUN Li-ying, ZHU Zhi-jun

Liver Research Center, Beijing Friendship Hospital, Capital Medical University; Liver Transplant Center, Beijing Friendship Hospital, Capital Medical University; National Clinical Research Center for Digestive Disease, Beijing 100050, China(ZHAO Xin-yan, LUO Wen-ping, LIU Li-wei, WEI Lin, HE En-hui, JIA Ji-dong , SUN Li-ying, ZHU Zhi-jun); Pediatric liver transplantation research center, Capital Medical University, Beijing 100050, China (SUN Li-ying, ZHU Zhi-jun)

Received:2018-12-09 Published:2020-04-10

摘要/Abstract

摘要： 目的比较先天性胆道闭锁行与未行葛西术的肝脏临床、病理特点变化,阐述该病进展规律。方法回顾性分析2017年1月至2018年10月首都医科大学附属北京友谊医院肝移植中心行肝移植术的58例胆道闭锁患儿总结临床、腹部超声及手术切除病肝病理形态特点。结果胆道闭锁患儿58例,未行葛西术者14例,行葛西术者44例。中位发病年龄未行葛西术组6.5(6.0,8.0)月,葛西术组12.0(8.0,36.0)月。男女比例为1∶1.2。与未行葛西术组患儿比较,葛西术后组患儿总胆红素显著减低[334.90(274.89,506.52)比51.89(17.40,222.13) μmol/L,P<0.01],终末期肝病模型评分(MELD)显著改善[15.44±8.31比 21.24±6.02,P=0.019]。葛西术后急性胆汁淤积病理特征如肝细胞淤胆、毛细胆管胆栓及Kupffer细胞淤胆显著改善;细胆管反应及细胆管胆栓显著减轻;纤维间隔显著变窄。结论本研究通过对比分析胆道闭锁行与未行葛西术的临床病理学特点,阐明了两者的主要区别,有助于提高对本病的认识及诊断准确率。

关键词: 先天性胆道闭锁, 葛西术, 病理特点, 肝移植术

Abstract: Objective To investigate clinicopathological features of congenital biliary atresia (BA) children with or without Kasai procedure for improving the understanding and diagnostic accuracy of BA.Methods The clinical data of BA patients who were hospitalized in Beijing Friendship Hospital from January 2017 to October 2018 were retrospectively analyzed.Results Fifty-eight qualified cases were enrolled in this study. Among all the patients, 14 cases (24.1%) didn’t have Kasai procedure (non-Kasai group), and 44 cases (75.9%) underwent Kasai procedure (Kasai group) previously. Medium age was 6.5 (6.0, 8.0) months in non-Kasai group and 12.0 (8.0, 36.0) months in Kasai group, which showed significantly difference (P<0.001). Ratio of male to female was 1∶1.2. Total bilirubin level and model for end-stage liver disease score in Kasai group were significantly lower than those in non-Kasai group, respectively [51.89 (17.40, 222.13) vs. 334.90 (274.89, 506.52), P<0.001; 15.44 ± 8.31 vs. 21.24 ± 6.02, P=0.019]. In Kasai group, pathological features of acute cholestasis such as hepatocellular cholestasis, bile plugs in bile capillary and cholestasis in Kupffer cells, were significantly improved. Moreover, ductular reactions and bile plugs in bile ductules were reduced, and widths of fibrous septa were narrower.Conclusion The main differences of clinicopathological features in BA patients pre- and post- Kasai would contribute to a better understanding and improvement in diagnostic accuracy of this disease.

Key words: Congenital biliary atresia, Kasai procedure, Histological features, Liver transplantation

赵新颜, 罗文萍, 刘立伟, 魏琳, 何恩辉, 贾继东, 孙丽莹, 朱志军. 先天性胆道闭锁行与未行葛西术患儿临床病理特点——附58例对比研究[J]. 肝脏, 2019, 24(2): 126-129.

ZHAO Xin-yan, LUO Wen-ping, LIU Li-wei, WEI Lin, HE En-hui , JIA Ji-dong, SUN Li-ying, ZHU Zhi-jun. Difference in clinicopathological features of congenital biliary atresia children with and without Kasai procedure: a comparative study in 58 cases[J]. Chinese Hepatolgy, 2019, 24(2): 126-129.

参考文献

[1] Hartley J L, Davenport M, Kelly D A. Biliary atresia. Lancet (London, England), 2009,374:1704-1713.
[2] 李英存, 张明满, 蒲从伦, 等. 胆道闭锁与婴儿肝炎综合征肝活检病理对比分析. 临床小儿外科杂志, 2017,16:142-145.
[3] Kilgore A, Mack C L. Update on investigations pertaining to the pathogenesis of biliary atresia. Pediatr Surg Int, 2017,33:1233-1241.
[4] Li Q, Wan P, Zhang J, et al. Liver transplantation for biliary atresia: A single-center study from mainland China. World J Gastroenterol, 2015,21:9638-9647.
[5] Kasahara M, Umeshita K, Sakamoto S, et al. Living donor liver transplantation for biliary atresia: An analysis of 2085 cases in the registry of the Japanese Liver Transplantation Society. Am J Transplant, 2018,18:659-668.
[6] 高伟. 小儿肝移植的适应证. 临床小儿外科杂志, 2017,16:121-126.
[7] Wan P, Xu D, Zhang J, et al. Liver transplantation for biliary atresia: A nationwide investigation from 1996 to 2013 in mainland China. Pediatr Transplant, 2016,20:1051-1059.
[8] Brittain JM, Kvist N, Johansen LS, et al. Hepatobiliary scintigraphy for early diagnosis of biliary atresia. Dan Med J, 2016,63.pii:A5253.
[9] Wildhaber BE. Biliary atresia: 50 years after the first kasai. ISRN Surg, 2012,2012:132089.
[10] Sundaram SS, Mack CL, Feldman AG, et al. Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl, 2017,23:96-109.
[11] 闫非易, 朱志军, 孙丽莹, 等. 胆道闭锁患儿肝移植术后生长发育的情况分析. 临床和实验医学杂志, 2017,16:1881-1884.
[12] Bezerra JA, Wells RG, Mack CL, et al. Biliary atresia: clinical and research challenges for the 21(st) century. Hepatology (Baltimore, Md.), 2018.
[13] 王琨, 王翠秀, 王秀. 新生儿肝内胆汁淤积症与胆道闭锁的临床特征比较. 肝脏, 2017,22:545-548.
[14] Zhang S, Wu Y, Liu Z, et al. Hepatic pathology of biliary atresia: A new comprehensive evaluation method using liver biopsy. Turk J Gastroenterol, 2016,27:257-263.
[15] Dani C, Pratesi S, Raimondi F, et al. Italian guidelines for the management and treatment of neonatal cholestasis. Ital J Pediatr, 2015,41:69.
[16] Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: joint recommen-dations of the North American Society for Pediatric Gastroen-terology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr, 2017,64:154-168.
[17] Chen G, Xue P, Zheng S, et al. A pathological scoring system in the diagnosis and judgment of prognosis of biliary atresia. J Pediatr Surg, 2015,50:2119-2123.
[18] Lee WS, Looi LM. Usefulness of a scoring system in the interpretation of histology in neonatal cholestasis. World J Gastroenterol, 2009,15:5326-5333.
[19] Russo P, Magee JC, Anders RA, et al. Key histopathologic features of liver biopsies that distinguish biliary atresia from other causes of infantile cholestasis and their correlation with outcome: a multicenter study. Am J Surg Pathol, 2016,40:1601-1615.
[20] 杨文萍, 吴艳, 张守华, 等. 胆道闭锁患儿肝脏组织病理改变的综合评价. 临床小儿外科杂志, 2015,14:10-15.
[21] Sharma S, Das P, Dattagupta S, et al. Liver and portal histopathological correlation with age and survival in extra hepatic biliary atresia. Pediatr Surg Int, 2011,27:451-461.
[22] Burt AD, Portmann BC, Ferrell LD. MacSween's pathology of the liver. Fifth edition, Elsevier, 2007,564-569.
[23] Saxena R. Practical hepatic pathology: A diagnostic approach. Second edition, Elsevier, 2018,71-76.
[24] Matsuura T, Kohashi K, Yanagi Y, et al. A morphological study of the removed livers from patients receiving living donor liver transplantation for adult biliary atresia. Pediatr Surg Int, 2012,28:1171-1175.
[25] 刘丹丹, 詹江华, 高伟, 等. 胆道闭锁Kasai术后胆管病理改变的研究. 中华小儿外科杂志, 2014,35:248-253.
[26] Webb NL, Jiwane A, Ooi CY, et al. Clinical significance of liver histology on outcomes in biliary atresia. J Paediatr Child Health, 2017,53:252-256.