New advances in the diagnosis and treatment of biliary atresia

WANG Yan-Fu; FU Jian-Hua; ZHANG Zhi-Bo

doi:10.7499/j.issn.1008-8830.2205180

PDF(608 KB)

Chinese Journal of Contemporary Pediatrics ›› 2022, Vol. 24 ›› Issue (11) : 1269-1274. DOI: 10.7499/j.issn.1008-8830.2205180

REVIEW

New advances in the diagnosis and treatment of biliary atresia

WANG Yan-Fu, FU Jian-Hua, ZHANG Zhi-Bo

Author information +

History +

Abstract

The diagnosis of biliary atresia (BA) is mainly based on clinical manifestations, screening, and related biochemistry tests. In recent years, the development of blood biomarkers and the improvement in ultrasound examination have made it possible for BA to be diagnosed at a younger age. In particular, matrix metalloproteinase-7 shows high sensitivity and specificity and has a higher diagnostic efficiency than existing biochemical parameters, thereby holding a promise for clinical application. Sound touch elastography can increase the diagnostic efficiency for BA in terms of diagnosis and prognostic evaluation. Surgery is still the only method for the treatment of BA at present, with the preferred surgical treatment regimen of Kasai portoenterostomy combined with pharmacotherapies for alleviating infection and inflammation, and the patients who fail Kasai portoenterostomy or have liver dysfunction may require liver transplantation to save their lives. Therefore, the current research on BA should focus on the biomarkers for early diagnosis, specifically targeted drugs, and drugs for preventing progressive liver fibrosis. This article reviews the current diagnosis and treatment methods for BA and discusses the potential research directions.

Key words

Biliary atresia / Diagnosis / Biomarker / Treatment / Neonate

Cite this article

EndNote

Ris (Procite)

Bibtex

Download Citations

WANG Yan-Fu, FU Jian-Hua, ZHANG Zhi-Bo. New advances in the diagnosis and treatment of biliary atresia[J]. Chinese Journal of Contemporary Pediatrics. 2022, 24(11): 1269-1274 https://doi.org/10.7499/j.issn.1008-8830.2205180

References

1 Hsiao CH, Chang MH, Chen HL, et al. Universal screening for biliary atresia using an infant stool color card in Taiwan[J]. Hepatology, 2008, 47(4): 1233-1240. PMID: 18306391. DOI: 10.1002/hep.22182.
2 Wada H, Muraji T, Yokoi A, et al. Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: a regional survey of over 20 years[J]. J Pediatr Surg, 2007, 42(12): 2090-2092. PMID: 18082714. DOI: 10.1016/j.jpedsurg.2007.08.035.
3 Schreiber RA, Barker CC, Roberts EA, et al. Biliary atresia: the Canadian experience[J]. J Pediatr, 2007, 151(6): 659-665.e1. PMID: 18035148. DOI: 10.1016/j.jpeds.2007.05.051.
4 Livesey E, Cortina Borja M, Sharif K, et al. Epidemiology of biliary atresia in England and Wales (1999-2006)[J]. Arch Dis Child Fetal Neonatal Ed, 2009, 94(6): F451-F455. PMID: 19457876. DOI: 10.1136/adc.2009.159780.
5 Chardot C, Buet C, Serinet MO, et al. Improving outcomes of biliary atresia: French national series 1986-2009[J]. J Hepatol, 2013, 58(6): 1209-1217. PMID: 23402746. DOI: 10.1016/j.jhep.2013.01.040.
6 Ortiz-Perez A, Donnelly B, Temple H, et al. Innate immunity and pathogenesis of biliary atresia[J]. Front Immunol, 2020, 11: 329. PMID: 32161597. PMCID: PMC7052372. DOI: 10.3389/fimmu.2020.00329.
7 He L, Chung PHY, Lui VCH, et al. Current understanding in the clinical characteristics and molecular mechanisms in different subtypes of biliary atresia[J]. Int J Mol Sci, 2022, 23(9): 4841. PMID: 35563229. PMCID: PMC9103665. DOI: 10.3390/ijms23094841.
8 沈淳, 郑珊, 王玮, 等. 手术年龄对胆道闭锁Kasai手术后预后影响的研究[J]. 临床小儿外科杂志, 2007, 6(3): 10-12. DOI: 10.3969/j.issn.1671-6353.2007.03.004.
9 中华医学会小儿外科学分会肝胆外科学组, 中国医师协会器官移植医师分会儿童器官移植学组. 胆道闭锁诊断及治疗指南(2018版)[J]. 中华小儿外科杂志, 2019, 40(5): 392-398. DOI: 10.3760/cma.j.issn.0253-3006.2019.05.003.
10 Nizery L, Chardot C, Sissaoui S, et al. Biliary atresia: clinical advances and perspectives[J]. Clin Res Hepatol Gastroenterol, 2016, 40(3): 281-287. PMID: 26775892. DOI: 10.1016/j.clinre.2015.11.010.
11 钟志海, 潘静, 蒋宏, 等. 胆道闭锁患儿早期胆红素变化[J]. 临床小儿外科杂志, 2015, 14(2): 106-109. DOI: 10.3969/j.issn.1671-6353.2015.02.007.
12 Zheng JC, Ye YQ, Wang B, et al. Biliary atresia screening in Shenzhen: implementation and achievements[J]. Arch Dis Child, 2020, 105(8): 720-723. PMID: 32518136. DOI: 10.1136/archdischild-2019-317787.
13 余莉, 黄维勇, 郭勇, 等. 比色卡提高医生对陶土色大便识别的调查研究[J]. 临床小儿外科杂志, 2019, 18(8): 692-698. DOI: 10.3969/j.issn.1671-6353.2019.08.015.
14 汤悦, 朱叶, 姜璟, 等. 新生儿胆道闭锁筛查和诊断系统评价和Meta分析[J]. 中国循证儿科杂志, 2020, 15(6): 411-418. DOI: 10.3969/j.issn.1673-5501.2020.06.003.
15 He L, Ip DKM, Tam G, et al. Biomarkers for the diagnosis and post-Kasai portoenterostomy prognosis of biliary atresia: a systematic review and meta-analysis[J]. Sci Rep, 2021, 11(1): 11692. PMID: 34083585. PMCID: PMC8175424. DOI: 10.1038/s41598-021-91072-y.
16 付海燕, 赵瑞芹, 白革兰, 等. 血清γ-谷氨酰转肽酶联合直接胆红素诊断婴儿胆道闭锁的价值[J]. 中国当代儿科杂志, 2019, 21(12): 1198-1202. PMID: 31874659. PMCID: PMC7389014. DOI: 10.7499/j.issn.1008-8830.2019.12.009.
17 Tang KS, Huang LT, Huang YH, et al. Gamma-glutamyl transferase in the diagnosis of biliary atresia[J]. Acta Paediatr Taiwan, 2007, 48(4): 196-200. PMID: 18265540.
18 姜璟?, 董瑞, 张志波, 等. 胆道闭锁Nomogram诊断模型多中心验证及优化[J]. 中华小儿外科杂志, 2020, 41(7): 628-632. DOI: 10.3760/cma.j.cn421158-20190412-00298.
19 Nomden M, Beljaars L, Verkade HJ, et al. Current concepts of biliary atresia and matrix metalloproteinase-7: a review of literature[J]. Front Med (Lausanne), 2020, 7: 617261. PMID: 33409288. PMCID: PMC7779410. DOI: 10.3389/fmed.2020.617261.
20 谌亚星, 邓蕾, 李燕翔, 等. 血清基质金属蛋白酶-7在胆道闭锁患儿诊断中的临床价值评价[J]. 中华小儿外科杂志, 2021, 42(9): 781-787. DOI: 10.3760/cma.j.cn421158-20200804-00533.
21 裴家好, 陈功, 郑珊, 等. PIVKA-Ⅱ对胆道闭锁的诊断价值及其与肝功能的相关性研究[J]. 临床小儿外科杂志, 2018, 17(11): 809-813. DOI: 10.3969/j.issn.1671-6353.2018.11.003.
22 宫颖新, 王萍, 白玉坤, 等. 胆道闭锁患儿血清GPC3、TGF-β1和VEGF水平与肝硬度值和肝功能的关系研究[J]. 现代生物医学进展, 2021, 21(1): 154-157. DOI: 10.13241/j.cnki.pmb.2021.01.034.
23 Kanegawa K, Akasaka Y, Kitamura E, et al. Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction[J]. AJR Am J Roentgenol, 2003, 181(5): 1387-1390. PMID: 14573442. DOI: 10.2214/ajr.181.5.1811387.
24 Zhou W, Zhou L. Ultrasound for the diagnosis of biliary atresia: from conventional ultrasound to artificial intelligence[J]. Diagnostics (Basel), 2021, 12(1): 51. PMID: 35054217. PMCID: PMC8775261. DOI: 10.3390/diagnostics12010051.
25 Zhou W, Yang Y, Yu C, et al. Ensembled deep learning model outperforms human experts in diagnosing biliary atresia from sonographic gallbladder images[J]. Nat Commun, 2021, 12(1): 1259. PMID: 33627641. PMCID: PMC7904842. DOI: 10.1038/s41467-021-21466-z.
26 张金山, 李龙. 胆道闭锁诊治新进展[J]. 中华小儿外科杂志, 2011, 32(5): 387-391. DOI: 10.3760/cma.j.issn.0253-3006.2011.05.017.
27 Zhou W, Li X, Zhang N, et al. The combination of conventional ultrasound and shear-wave elastography in evaluating the segmental heterogeneity of liver fibrosis in biliary atresia patients after Kasai portoenterostomy[J]. Pediatr Surg Int, 2021, 37(8): 1099-1108. PMID: 33966098. DOI: 10.1007/s00383-021-04920-3.
28 段星星, 杨柳, 文欢, 等. 声触诊弹性成像联合谷氨酰转肽酶检测在胆道闭锁诊断中的应用研究[J]. 临床小儿外科杂志, 2022, 21(1): 46-50. DOI: 10.3760/cma.j.cn.101785-202105063-009.
29 姜璟?, 汤悦, 朱叶, 等. 基于超声、肝胆核素显像和磁共振胆胰管成像影像学检查诊断胆道闭锁准确性研究的系统评价和Meta分析[J]. 中国循证儿科杂志, 2020, 15(3): 166-176. DOI: 10.3969/j.issn.1673-5501.2020.03.002.
30 赵阳, 王建华, 米荣, 等. 3个月龄内胆汁淤积症与胆道闭锁患儿胆道造影术的指征分析[J]. 中华妇幼临床医学杂志(电子版), 2019, 15(2): 205-210. DOI: 10.3877/cma.j.issn.1673-5250.2019.02.013.
31 Hartley JL, Davenport M, Kelly DA. Biliary atresia[J]. Lancet, 2009, 374(9702): 1704-1713. PMID: 19914515. DOI: 10.1016/S0140-6736(09)60946-6.
32 Zhang Y, Wang Q, Pu S, et al. A novel model for predicting the clearance of jaundice in patients with biliary atresia after Kasai procedure[J]. Front Pediatr, 2022, 10: 837247. PMID: 35174110. PMCID: PMC8841871. DOI: 10.3389/fped.2022.837247.
33 董淳强, 杨体泉, 董昆, 等. 肝门肠吻合术治疗胆道闭锁112例报告[J]. 中华小儿外科杂志, 2013, 34(3): 180-184. DOI: 10.3760/cma.j.issn.0253-3006.2013.03.006.
34 熊晓峰, 冯杰雄. 胆道闭锁Kasai手术效果影响因素的研究进展[J]. 中华小儿外科杂志, 2016, 37(5): 382-386. DOI: 10.3760/cma.j.issn.0253-3006.2016.05.014.
35 Ayuso L, Vila-Carbó JJ, Lluna J, et al. Laparoscopic Kasai portoenterostom: present and future of biliary atresia treatment[J]. Cir Pediatr, 2008, 21(1): 23-26. PMID: 18444386.
36 孙旭, 李龙, 叶茂, 等. 腹腔镜与开放Kasai手术治疗 Ⅲ 型胆道闭锁疗效的前瞻性对比研究[J]. 中华小儿外科杂志, 2013, 34(1): 22-25. DOI: 10.3760/cma.j.issn.0253-3006.2013.01.006.
37 Tiao MM, Yang CY, Tsai SS, et al. Liver transplantation for biliary atresia in Taiwan[J]. Transplant Proc, 2008, 40(10): 3569-3570. PMID: 19100440. DOI: 10.1016/j.transproceed.2008.07.136.
38 张聪, 赵升桥, 詹江华. 胆道闭锁Kasai术与肝移植的研究进展[J]. 中华器官移植杂志, 2020, 41(11): 701-704. DOI: 10.3760/cma.j.cn421203-20200523-00161.
39 Sundaram SS, Mack CL, Feldman AG, et al. Biliary atresia: indications and timing of liver transplantation and optimization of pretransplant care[J]. Liver Transpl, 2017, 23(1): 96-109. PMID: 27650268. PMCID: PMC5177506. DOI: 10.1002/lt.24640.
40 朱叶, 陈功. 胆道闭锁潜在治疗药物的研究进展[J]. 中华小儿外科杂志, 2021, 42(6): 572-576. DOI: 10.3760/cma.j.cn421158-20200103-00005.
41 葛军涛, 李龙. 胆道闭锁Kasai术后胆管炎的病因及治疗[J]. 中华小儿外科杂志, 2013, 34(5): 387-390. DOI: 10.3760/cma.j.issn.0253-3006.2013.05.017.
42 杨梦, 胡艳. 胆道闭锁术后中药干预治疗的研究进展[J]. 中国中医急症, 2014, 23(7): 1312-1314. DOI: 10.3969/j.issn.1004-745X.2014.07.041.
43 赵翊杉, 李玉兰, 韩娟, 等. 中西医结合治疗胆管闭锁Kasai术后胆管炎的临床研究[J]. 发育医学电子杂志, 2019, 7(1): 38-42. DOI: 10.3969/j.issn.2095-5340.2019.01.009.
44 Lampela H, Kosola S, Heikkil? P, et al. Native liver histology after successful portoenterostomy in biliary atresia[J]. J Clin Gastroenterol, 2014, 48(8): 721-728. PMID: 24275708. DOI: 10.1097/MCG.0000000000000013.
45 Kasahara M, Umeshita K, Sakamoto S, et al. Liver transplantation for biliary atresia: a systematic review[J]. Pediatr Surg Int, 2017, 33(12): 1289-1295. PMID: 28983725. DOI: 10.1007/s00383-017-4173-5.