Zhan Jianghua,Wang Zhiru.Focusing upon hot issues of diagnosing and treating biliary atresia[J].Journal of Clinical Pediatric Surgery,2023,22(03):201-204.[doi:10.3760/cma.j.cn101785-202212026-001]
聚焦胆道闭锁诊疗相关热点问题
- Title:
- Focusing upon hot issues of diagnosing and treating biliary atresia
- Keywords:
- Biliary Atresia; Cholangitis; Laparoscopes; Prognosis
- 摘要:
- 胆道闭锁(biliary atresia,BA)是指以肝内、外胆管进行性炎症和纤维化为特征的新生儿及婴儿期严重肝胆系统疾病,Kasai手术为BA的首选治疗方案,但约60%的患儿手术后仍会进一步发展为肝功能衰竭,最终需通过肝移植挽救生命。目前对于BA病因及发病机制的了解仍不够彻底,对于术后并发症的预防也并无良策,但一直以来争议较大的腔镜下Kasai手术取得了令人满意的效果。本文对当前困扰研究人员的BA诊疗相关热点问题进行评述,为探寻BA未来的研究方向提供思路。
- Abstract:
- As a serious disease of hepatobiliary system in neonates and infants,biliary atresia (BA) is characterized by progressive inflammation and fibrosis of intra-and extrahepatic bile ducts.Kasai operation has remained a preferred treatment option.However,around 60% of children with postoperative liver fibrosis may progress further and eventually liver transplantation is often required for saving lives.Our current understandings of the etiology and pathogenesis of BA are insufficient.And there is no proper way of preventing postoperative complications.Though as a subject of much controversy,Laparoscopic Kasai procedure has achieved satisfactory outcomes.This review focused upon current hot issues of researchers to provide rationales for future researches of BA.
参考文献/References:
[1] Hartley JL,Davenport M,Kelly DA.Biliary atresia[J].Lancet,2009,374(9702):1704-1713.DOI:10.1016/S0140-6736(09)60946-6.
[2] Bezerra JA,Wells RG,Mack CL,et al.Biliary atresia:clinical and research challenges for the twenty-first century[J].Hepatology,2018,68(3):1163-1173.DOI:10.1002/hep.29905.
[3] Pang SY,Dai YM,Zhang RZ,et al.Autoimmune liver disease-related autoantibodies in patients with biliary atresia[J].World J Gastroenterol,2018,24(3):387-396.DOI:10.3748/wjg.v24.i3.387.
[4] Wang J,Xu YH,Chen ZH,et al.Liver immune profiling reveals pathogenesis and therapeutics for biliary atresia[J].Cell,2020,183(7):1867-1883.e26.DOI:10.1016/j.cell.2020.10.048.
[5] Pal N,Joy PS,Sergi CM.Biliary atresia animal models:is the needle in a haystack?[J].Int J Mol Sci,2022,23(14):7838.DOI:10.3390/ijms23147838.
[6] Kretzschmar K,Clevers H.Organoids:modeling development and the stem cell niche in a dish[J].Dev Cell,2016,38(6):590-600.DOI:10.1016/j.devcel.2016.08.014.
[7] Huch M,Gehart H,van Boxtel R,et al.Long-term culture of genome-stable bipotent stem cells from adult human liver[J].Cell,2015,160(1/2):299-312.DOI:10.1016/j.cell.2014.11.050.
[8] Andersson ER,Chivukula IV,Hankeova S,et al.Mouse model of alagille syndrome and mechanisms of jagged1 missense mutations[J].Gastroenterology,2018,154(4):1080-1095.DOI:10.1053/j.gastro.2017.11.002.
[9] Lorent K,Gong WL,Koo KA,et al.Identification of a plant isoflavonoid that causes biliary atresia[J].Sci Transl Med,2015,7(286):286ra67.DOI:10.1126/scitranslmed.aaa1652.
[10] Cui S,Leyva-Vega M,Tsai EA,et al.Evidence from human and zebrafish that GPC1 is a biliary atresia susceptibility gene[J].Gastroenterology,2013,144(5):1107-1115.e3.DOI:10.1053/j.gastro.2013.01.022.
[11] Chen Y,Gilbert MA,Grochowski CM,et al.A genome-wide association study identifies a susceptibility locus for biliary atresia on 2p16.1 within the gene EFEMP1[J].PLoS Genet,2018,14(8):e1007532.DOI:10.1371/journal.pgen.1007532.
[12] Ye YQ,Li ZH,Feng Q,et al.Downregulation of microRNA-145 may contribute to liver fibrosis in biliary atresia by targeting ADD3[J].PLoS One,2017,12(9):e0180896.DOI:10.1371/journal.pone.0180896.
[13] Harpavat S,Garcia-Prats JA,Anaya C,et al.Diagnostic yield of newborn screening for biliary atresia using direct or conjugated bilirubin measurements[J].JAMA,2020,323(12):1141-1150.DOI:10.1001/jama.2020.0837.
[14] Chi SQ,Xu PP,Yu P,et al.Dynamic analysis of serum MMP-7 and its relationship with disease progression in biliary atresia:a multicenter prospective study[J].Hepatol Int,2022,16(4):954-963.DOI:10.1007/s12072-022-10322-x.
[15] Zhao JF,Xu XD,Gou QY,et al.TGF-β1-mediated leukocyte cell-derived chemotaxin 2 is associated with liver fibrosis in biliary atresia[J].Front Pediatr,2022,10:901888.DOI:10.3389/fped.2022.901888.
[16] Xu Y,Yu JK,Zhang RZ,et al.The perinatal infection of cytomegalovirus is an important etiology for biliary atresia in China[J].Clin Pediatr (Phila),2012,51(2):109-113.DOI:10.1177/0009922811406264.
[17] Davenport M.Biliary atresia:clinical aspects[J].Semin Pediatr Surg,2012,21(3):175-184.DOI:10.1053/j.sempedsurg.2012.05.010.
[18] Zhao YL,Xu XD,Liu GX,et al.Prognosis of biliary atresia associated with cytomegalovirus:a meta-analysis[J].Front Pediatr,2021,9:710450.DOI:10.3389/fped.2021.710450.
[19] Ginstr?m DA,Hukkinen M,Kivisaari R,et al.Biliary atresia-associated cholangitis:the central role and effective management of bile lakes[J].J Pediatr Gastroenterol Nutr,2019,68(4):488-494.DOI:10.1097/MPG.0000000000002243.
[20] 中华医学会小儿外科学分会肝胆外科学组.胆道闭锁Kasai术后胆管炎诊疗专家共识(2022版)[J].中华小儿外科杂志,2022,43(9):769-774.DOI:10.3760/cma.j.cn421158-20220427-00308. Section of Hepatobiliary Surgery,Branch of Pediatric Surgery,Chinese Medical Association:Expert Consensus on Diagnosing and Treating Cholangitis after Kasai Operation for Biliary Atresia (2022 Edition)[J].Chin J Pediatr Surg,2022,43(9):769-774.DOI:10.3760/cma.j.cn421158-20220427-00308.
[21] Yu P,Li MD,Sun RJ,et al.Primary exploring the value of metagenomic next-generation sequencing in detecting pathogenic bacteria of cholangitis with biliary atresia after Kasai operation[J].Pediatr Surg Int,2022,38(12):1931-1937.DOI:10.1007/s00383-022-05254-4.
[22] 詹江华,王立.胆道闭锁围术期营养状况评价的临床意义[J].天津医药,2019,47(4):342-345.DOI:10.11958/20181411. Zhan JH,Wang L.Clinical significance of evaluating nutrition status in patients with biliary atresia during perioperative period[J].Tianjin Med J,2019,47(4):342-345.DOI:10.11958/20181411.
[23] 詹江华,熊希倩.胆道闭锁Kasai手术加速康复外科理念实施[J].临床小儿外科杂志,2019,18(4):257-260.DOI:10.3969/j.issn.1671-6353.2019.04.002. Zhan JH,Xiong XQ.Conceptual implementation of enhanced recovery after Kasai surgery for biliary atresia[J].J Clin Ped Sur,2019,18(4):257-260.DOI:10.3969/j.issn.1671-6353.2019.04.002.
[24] 张树建,窦然,詹江华.Kasai手术中空肠胆支保留长度与胆管炎发生关系的研究进展[J].临床小儿外科杂志,2020,19(9):851-855.DOI:10.3969/j.issn.1671-6353.2020.09.018. Zhang SJ,Dou R,Zhan JH.Research advances in correlations between Roux-en-Y limb length during Kasai portoenterostomy and cholangitis[J].J Clin Ped Sur,2020,19(9):851-855.DOI:10.3969/j.issn.1671-6353.2020.09.018.
[25] Hinojosa-Gonzalez DE,Bueno LC,Roblesgil-Medrano A,et al.Laparoscopic vs open portoenterostomy in biliary atresia:a systematic review and meta-analysis[J].Pediatr Surg Int,2021,37(11):1477-1487.DOI:10.1007/s00383-021-04964-5.
[26] Dutta S,Woo R,Albanese CT.Minimal access portoenterostomy:advantages and disadvantages of standard laparoscopic and robotic techniques[J].J Laparoendosc Adv Surg Tech A,2007,17(2):258-264.DOI:10.1089/lap.2006.0112.
相似文献/References:
[1]毛永忠,汤绍涛,阮庆兰,等.胆道闭锁肝组织MMP-1、MMP-2、TIMP-1的表达与预后研究[J].临床小儿外科杂志,2007,6(01):5.
[2]毛永忠,汤绍涛,阮庆兰,等.胆道闭锁肝脏组织MMP-2、TGF-β_1的表达及意义[J].临床小儿外科杂志,2007,6(03):5.
[3]董淳强 杨体泉 董昆. 胆道闭锁术后早期胆管炎风险因素分析[J].临床小儿外科杂志,2013,12(05):348.
[4]陈功 郑珊 孙松. 不同年龄胆道闭锁患儿手术效果分析[J].临床小儿外科杂志,2014,13(01):22.
[5]谭梅军 陶强 黄金狮. IL-17在胆道闭锁患儿肝组织中的表达及意义[J].临床小儿外科杂志,2014,13(04):302.
[6]杨文萍 吴艳 张守华. 胆道闭锁患者肝脏组织病理改变的综合评价[J].临床小儿外科杂志,2015,14(01):10.
[7]李康 阳历 汤绍涛. 胆道闭锁患者肝内胆管γδT 细胞和调节性T细胞浸润及意义[J].临床小儿外科杂志,2015,14(01):16.
[8]刘丹丹 詹江华 高伟. 胆道闭锁患者肝门的病理解剖学研究[J].临床小儿外科杂志,2015,14(01):20.
[9]张震 李龙 乔国梁. 胆道闭锁Kasai术后生存率及条件生存率分析[J].临床小儿外科杂志,2015,14(01):25.
[10]庞文博 张廷冲 彭春辉. 强力抗感染和免疫调节预防Kasai术后近期胆管炎[J].临床小儿外科杂志,2015,14(01):29.
[11]余梦楠 刘钢 黄柳明 王伟. 胆道闭锁术后反复发作性胆管炎的细菌谱分析及抗生素选择[J].临床小儿外科杂志,2012,11(03):187.
[J].Journal of Clinical Pediatric Surgery,2012,11(03):187.
[12]王培,冯杰雄.胆道闭锁术后胆管炎抗生素分级治疗的多中心研究方案[J].临床小儿外科杂志,2019,18(11):922.[doi:10.3969/j.issn.1671—6353.2019.11.006]
Wang Pei,Feng Jiexiong.The antibiotics treatment of cholangitis Post-Kasai Portoenterostomy: A multi-center study protocol[J].Journal of Clinical Pediatric Surgery,2019,18(03):922.[doi:10.3969/j.issn.1671—6353.2019.11.006]
[13]詹江华,孙溶涓,王智茹,等.日本《胆道闭锁临床实践指南》解读[J].临床小儿外科杂志,2023,22(03):205.[doi:10.3760/cma.j.cn101785-202212025-002]
Zhan Jianghua,Sun Rongjuan,Wang Zhiru,et al.Interpretations of Clinical Practice Guidelines for Biliary Atresia in Japan[J].Journal of Clinical Pediatric Surgery,2023,22(03):205.[doi:10.3760/cma.j.cn101785-202212025-002]
[14]赵一霖,吴晓霞,徐晓丹,等.巨细胞病毒感染对胆道闭锁Kasai手术预后影响的研究[J].临床小儿外科杂志,2023,22(03):232.[doi:10.3760/cma.j.cn101785-202212017-006]
Zhao Yilin,Wu Xiaoxia,Xu Xiaodan,et al.Prognosis of biliary atresia associated with cytomegalovirus[J].Journal of Clinical Pediatric Surgery,2023,22(03):232.[doi:10.3760/cma.j.cn101785-202212017-006]
[15]吴晓霞,刘文跃,孙雪,等.胆道闭锁患儿Kasai手术后频发胆管炎的危险因素及预后分析[J].临床小儿外科杂志,2023,22(03):244.[doi:10.3760/cma.j.cn101785-202212005-008]
Wu Xiaoxia,Liu Wenyue,Sun Xue,et al.Risk factors and prognosis of recurrent cholangitis after Kasai in biliary atresia patients[J].Journal of Clinical Pediatric Surgery,2023,22(03):244.[doi:10.3760/cma.j.cn101785-202212005-008]
[16]王磊,兰照平,高琪,等.胆道闭锁患儿Kasai手术后胆管炎的危险因素分析[J].临床小儿外科杂志,2023,22(11):1039.[doi:10.3760/cma.j.cn101785-202211061-007]
Wang Lei,Lan Zhaoping,Gao Qi,et al.Analysis of risk factors for cholangitis after Kasai procedure in biliary atresia children[J].Journal of Clinical Pediatric Surgery,2023,22(03):1039.[doi:10.3760/cma.j.cn101785-202211061-007]
备注/Memo
收稿日期:2022-12-9。
基金项目:天津市科技局重大专项(21ZXGWSY00070);新疆维吾尔自治区自然科学基金(2022D01A27、2021D01A38);天津大学儿童医院面上项目(Y2020002)
通讯作者:詹江华,Email:zhanjianghuatj@163.com