Comprehensive Unit for Health Economic Evidence Review and Decision Support (CHEERS), Ritsumeikan University, Kyoto, Japan.
Graduate School of Public Health, St. Luke's International University, Tokyo, Japan.
Eur J Pediatr. 2023 Jun;182(6):2693-2704. doi: 10.1007/s00431-023-04925-1. Epub 2023 Mar 31.
Biliary atresia (BA) is a childhood rare disease of the liver and bile ducts that requires prompt surgical intervention. Age at surgery is an important prognostic factor; however, controversy exists with regard to the benefit of early Kasai procedure (KP). We aimed to conduct a systematic review and meta-analysis to examine the relationship between the age at KP and native liver survival (NLS) of BA patients. We performed the electronic database search using Pubmed, EMBASE, Cochrane, and Ichushi Web and included all relevant studies published from 1968 up to May 3, 2022. Studies that examined the timing of KP at ages 30, 45, 60, 75, 90, 120, and/or 150 days were included. The outcome measures of interest were NLS rates at 5, 10, 15, 20, and 30 years post-KP and the hazard ratio or risk ratio for NLS. The quality assessment was used using the ROBINS-I tool. Among 1653 potentially eligible studies, nine articles met the inclusion criteria for the meta-analysis. Meta-analysis for hazard ratios revealed that there was a significantly faster time to liver transplantation in the group of patients who had KP at later timing as compared with earlier KP (HR = 2.12, 95% CI 1.51-2.97). The risk ratio comparing KP ≤ 30 days and KP ≥ 31 days on native liver survival was 1.22 (95% CI 1.13-1.31). The sensitivity analysis showed that comparing KP ≤ 30 days and KP 31-60 days, the risk ratio was 1.13, 95% CI 1.04-1.22. Conclusion: Our meta-analysis showed the importance of early diagnosis and surgical interventions ideally before 30 days of life in infants with BA on native liver survival on 5, 10, and 20 years. Therefore, effective newborn screening of BA targeting KP ≤ 30 days is needed to ensure prompt diagnosis of affected infants. What is Known: • Age at surgery is an important prognostic factor. What is New: • Our study performed an updated systematic review and meta-analysis to examine the relationship between age at Kasai procedure and native liver survival in patients with BA.
先天性胆道闭锁（BA）是一种儿童期罕见的肝脏和胆管疾病，需要及时进行手术干预。手术时的年龄是一个重要的预后因素；然而，对于早期开腹手术（KP）的益处存在争议。我们旨在进行系统评价和荟萃分析，以检查 KP 年龄与 BA 患者的肝固有生存（NLS）之间的关系。我们使用 Pubmed、EMBASE、Cochrane 和 Ichushi Web 进行了电子数据库搜索，并纳入了截至 2022 年 5 月 3 日发表的所有相关研究。纳入了研究 KP 在 30、45、60、75、90、120 和/或 150 天时的时机的研究。感兴趣的结局测量指标为 KP 后 5、10、15、20 和 30 年的 NLS 率以及 NLS 的风险比或危险比。使用 ROBINS-I 工具进行质量评估。在 1653 项潜在合格研究中，有 9 项文章符合荟萃分析的纳入标准。荟萃分析危险比表明，与早期 KP 相比，KP 较晚时机的患者肝移植时间明显更快（HR=2.12，95%CI 1.51-2.97）。比较 KP≤30 天和 KP≥31 天的 NLS 的风险比为 1.22（95%CI 1.13-1.31）。敏感性分析表明，比较 KP≤30 天和 KP 31-60 天，风险比为 1.13，95%CI 1.04-1.22。结论：我们的荟萃分析表明，对于 BA 婴儿，在 5、10 和 20 年内，早期诊断和手术干预非常重要，理想情况下应在出生后 30 天内进行。因此，需要针对 KP≤30 天的 BA 进行有效的新生儿筛查，以确保及时诊断出受影响的婴儿。已知：•手术时的年龄是一个重要的预后因素。新内容：•我们的研究进行了更新的系统评价和荟萃分析，以检查 KP 年龄与 BA 患者的肝固有生存之间的关系。
