IntroductionBiliary 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 regards to the bene t 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. MethodsWe 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 which examined the timing of KP at age 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. ResultsAmong 1,653 potentially eligible studies, 9 articles met the inclusion criteria for the meta-analysis. Meta-analysis for hazard ratios revealed that there was signi cantly 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. ConclusionOur 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. IntroductionBiliary atresia (BA) is a childhood rare disease of liver and bile ducts presenting with biliary obstruction exclusively in the neonatal period (1). BA is the most common cause of neonatal jaundice requiring prompt surgical intervention (the Kasai procedure [KP]) that aims to restore bile ow, and is the most frequent indication for liver transplantation in children. Age at surgery is an important prognostic factor of BA (1) (2). The success of KP, such as the clearance of jaundice and native liver survival (NLS), is associated with infant age at the time of surgery (3). Over the past decade, the average age at KP has remained between 60 to 70 days, and the 10-year NLS is 52.8% in Japan(4) (5). Due to medical advances enabling early diagnosis, the age at BA diagnosis and surgery have decreased in recent years. This has resulted in shifts in surgery being performed at earlier ages at less than 60 days with studies showing better prognosis of KP performed at less than 30 days (6). In contrast, several studies have also observed that the timing of surgery does not impose a signi cant in uence on the clinical outcomes (7) (8).The previous systematic review by Jimenez-Rivera et al. conducted in 2013 (9) investigated the relationship between the timing of KP and patient outcomes. The...
We investigated the age-dependent changes in urinary excretion of glucuronidated bile acids at the C-3 position. Bile acid 3-glucuronides accounted for 0.5% of urinary bile acids in neonates, and the proportion of bile acid 3-glucuronides plateaued at 1–3 years of age. The 3-glucuronides of secondary bile acids were first secreted at 3 months of age, the same time as the establishment of the gut bacterial flora in infants. A considerable portion of bile acid 3-glucuronides were present as non-amidated forms. Our results indicate dynamic hepatic enzyme activity in which the levels of uridine 5′-diphospho-glucuronosyltransferases (UGTs) differ by age group, with higher glucuronidation activity of UGTs towards nonamidated bile acids than amidated bile acids.
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