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World Journal of Pediatric Surgery 2022Previous studies have suggested an association between vascular endothelial growth factor A () rs3025039 polymorphism and biliary atresia (BA). However, this conclusion... (Review)
Review
BACKGROUND
Previous studies have suggested an association between vascular endothelial growth factor A () rs3025039 polymorphism and biliary atresia (BA). However, this conclusion is controversial and there is no published pooled evidence of this association.
METHODS
This study was conducted and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses. The protocol was registered with PROSPERO (International Prospective Register of Systematic Reviews). A thorough search was performed on databases including PubMed, Embase, and Chinese Biomedical Database up to August 2020. This study included 846 cases of BA and 2821 controls concerning rs3025039 polymorphism. We selected relevant studies based on the following inclusion criteria: (1) the study design was case-control and cohort and (2) the patients carried standard clinical diagnoses of BA, etc. The exclusion criteria were as follows: (1) patients with other related diseases, (2) lack of requisite information and (3) duplicate data. The OR (odd ratio) and the corresponding 95% CI (confidence interval) were calculated to estimate the association.
RESULTS
This study on rs3025039 polymorphism in the Chinese population included 846 cases and 2821 controls. The results showed that there was no significant association between rs3025039 and susceptibility to BA under four genetic models. The results of the subgroup analysis were similar to the overall results.
CONCLUSIONS
This meta-analysis shows that rs3025039 was not associated with susceptibility to BA in the Chinese population. Further validation may entail additional research.
PROSPERO REGISTRATION NUMBER
CRD42020203812.
PubMed: 36474631
DOI: 10.1136/wjps-2021-000344 -
Pediatric Gastroenterology, Hepatology... Sep 2022No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary... (Review)
Review
No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08-0.33; =46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00-63.63; =96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01-0.73; =80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00-0.62; =69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00-3.22; =86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00-175.21; =94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.
PubMed: 36148293
DOI: 10.5223/pghn.2022.25.5.353 -
Frontiers in Surgery 2022Good outcomes of biliary atresia (BA) are conventionally achieved after early Kasai portoenterostomy (KP). However, in some recent pieces of literature, there are... (Review)
Review
BACKGROUND
Good outcomes of biliary atresia (BA) are conventionally achieved after early Kasai portoenterostomy (KP). However, in some recent pieces of literature, there are discrepancies in the influence of age in Kasai procedure on postoperative short-term prognosis. This meta-analysis aims to evaluate the effects of earlier KP on short-term surgical prognosis of BA and clarify these discrepancies in recent studies.
METHODS
To identify related studies, PubMed, Embase, Web of Science, Cochrane, and the Chinese National Knowledge Infrastructure database were searched up to March 2022. Data for the impact of age at KP on clinical prognosis were extracted, including jaundice clearance rate (JCR) and native liver survival rate (NLSR).
RESULTS
A total of 14 articles were included in the present study, which involve a total of 3,276 patients with BA who underwent Kasai procedure. Compared with patients older than 91 days of age, patients 90 days of age or younger exhibited significantly better JCR [odds ratio (OR), 3.05; 95% confidence interval (CI), 2.23-4.17; < .001] and a more favorable NLSR (OR, 1.72; 95% CI, 1.37-2.15; < .001). The NLSR of patients younger than 60 days of age was significantly higher than those of patients from 61 to 90 days of age (OR, 1.41; 95% CI, 1.18-1.68; < .001). There was no significant difference in JCRs between patients aged 60 days of age or younger and those aged 61-90 days of age (OR, 1.31; 95% CI, 0.95-1.81; = 0.10). Among patients 30 days of age or younger, 31-45 days of age, and 46-60 days of age, there were also no significant differences in JCR.
CONCLUSION
A significantly better short-term JCR and NLSRs were achieved among patients with BA treated using a KP procedure at ≤90 days of age compared with those treated at >90 days of age. There was no further improvement in the short-term JCR when the procedure was performed at ≤60 days compared with those treated at 61-90 days of age. However, treatment at ≤60 days of age was associated with a significant improvement in NLSR. Therefore, the timing of KP does exert an important effect on short-term clinical outcomes of patients with BA.
PubMed: 36117834
DOI: 10.3389/fsurg.2022.924506 -
Frontiers in Pharmacology 2022Postoperative adjuvant steroid therapy is regarded as the conventional treatment for patients with biliary atresia (BA) who have undergone Kasai portoenterostomy (KP)....
Postoperative adjuvant steroid therapy is regarded as the conventional treatment for patients with biliary atresia (BA) who have undergone Kasai portoenterostomy (KP). However, whether the steroid therapy can improve BA outcomes is controversial. This meta-analysis aimed to evaluate the effects of adjuvant steroid therapy on the surgical prognosis of BA. We searched related studies published in PubMed, Embase, Web of Science, Cochrane, and the Chinese National Knowledge Infrastructure database up to May 2022. Data on the effect of steroid use on the clinical prognosis of the patients, including the jaundice clearance rate (JCR), native liver survival rate (NLSR) at 6, 12, and 24 months after KP, and the incidence of cholangitis, were extracted. Subgroup analyses based on age at KP, administration method, initial dosage, and steroid type were conducted. Statistical analysis was conducted using Stata/SE 12.0. Eleven articles (a total of 1,032 patients) were included in the present meta-analysis. The results demonstrated that postoperative adjuvant steroid therapy improved JCR at the 6/12/24-month follow-up (RR: 1.35, 95% CI: 1.18-1.55, < 0.001; RR:1.49, 95% CI, 1.12-1.99, = 0.006; RR: 1.41, 95% CI: 1.14-1.75, = 0.002) and improved NLSR at the 24-month follow-up (RR: 1.31, 95% CI: 1.03-1.68, = 0.028). However, steroids could not significantly improve NLSR at the 6/12-month follow-up (RR: 1.06; 95% CI: 0.98-1.15; = 0.17; RR: 1.22; 95% CI: 0.97-1.54; = 0.095), and might not decrease the incidence of postoperative cholangitis (RR: 0.78, 95% CI: 0.60-1.01, = 0.058). Furthermore, subgroup analyses confirmed that three variables (age at KP, administration method, and initial dosage) could affect the efficacy of steroids in BA patients. Postoperative adjuvant steroid therapy can significantly improve bile flow. The superiority of steroid therapy was more remarkable in patients aged ≤70 days at KP than in those aged >70 days. Additionally, intravenous followed by oral steroid administration method and medium initial dosage seemed to have the more reliable efficiency on bile flow. And patients treated by steroid had better long-term (24-month) native liver survival, but there is no significant effect on short-term native liver survival and postoperative cholangitis. Further studies are warranted.
PubMed: 36188593
DOI: 10.3389/fphar.2022.956093 -
Scientific Reports Jun 2021To evaluate the accuracy of biomarkers for the early diagnosis of biliary atresia (BA) and prognostic stratification after Kasai portoenterostomy (KPE). We conducted a... (Meta-Analysis)
Meta-Analysis
To evaluate the accuracy of biomarkers for the early diagnosis of biliary atresia (BA) and prognostic stratification after Kasai portoenterostomy (KPE). We conducted a systematic review of PubMed, Web of Science, Embase, Scopus and OVID for English literature reporting BA biomarkers published before August 2020. Screening, data extraction, and quality assessment were performed in duplicate. A total of 51 eligible studies were included in the systematic review, and data from 12 (4182 subjects) were extracted for meta-analysis regarding the following 2 domains: (1) serum matrix metallopeptidase-7 (MMP-7), interleukin33 (IL-33) and γ-glutamyl transferase (GGT) to differentiate BA from non-BA; (2) the aspartate aminotransferase to platelet ratio index (APRi) to predict post-KPE liver fibrosis/cirrhosis. The summary sensitivity, specificity and area under the curve (AUC) of MMP-7 for diagnosing BA were 96%, 91% and 0.9847, respectively, and those of GGT were 80%, 79% and 0.9645, respectively. The summary sensitivity and specificity of IL-33 for diagnosing BA were 77% and 85%, respectively. The summary sensitivity and specificity of APRi for predicting post-KPE liver fibrosis were 61% and 80%, respectively, and the summary sensitivity, specificity and AUC of APRi for predicting post-KPE cirrhosis were 78%, 83% and 0.8729, respectively. Moreover, good evidence was shown in investigations of serum IL-18 and IL-33 in distinguishing BA from healthy controls, serum IL-18 for prognosis of post-KPE persistent jaundice, and serum hyaluronic acid and MMP-7 for prognosis of post-KPE significant liver fibrosis. MMP-7, IL-33 and GGT are useful biomarkers to assist in the diagnosis of BA. APRi might be used to predict post-KPE significant liver fibrosis and cirrhosis. These noninvasive biomarkers can be integrated into the management protocol of BA.
Topics: Biliary Atresia; Biomarkers; Humans; Matrix Metalloproteinase 7; Portoenterostomy, Hepatic; Prognosis
PubMed: 34083585
DOI: 10.1038/s41598-021-91072-y -
Translational Pediatrics May 2022Biliary atresia (BA) is one of the most fatal hepatobiliary diseases in infants and young children, and is easy to cause obstructive jaundice in infants and children,...
BACKGROUND
Biliary atresia (BA) is one of the most fatal hepatobiliary diseases in infants and young children, and is easy to cause obstructive jaundice in infants and children, and liver transplantation in children. BA and infantile hepatitis syndrome are the main causes of cholestatic jaundice. The early clinical manifestations are extremely similar, and timely identification is difficult. Therefore, early and differential diagnosis of BA is crucial. At present, there are many imaging diagnosis methods for BA, such as ultrasound examination and nuclear magnetic resonance. Ultrasound elastography (USE) is the most popular method. However, the application of USE in BA is still in the exploratory stage.
METHODS
PubMed, Web of Science, Embase, Cochrane Library, and other databases were searched for articles on USE diagnosis of pediatric surgical BA. Later, the articles were screened, and the included articles should meet the following requirements: the research objects were children with BA; the intervention measures were USE diagnosis; the control group was diagnosed by other methods than USE. RevMan5.3 was adopted for quality assessment, and forest maps and summary receiver operating characteristic (SROC) curves were constructed.
RESULTS
Finally, seven articles covering 490 patients were included. There were 120 patients in the control group. The included articles showed good quality and there was no obvious bias. The area under the SROC curve (AUC) for USE diagnosis of BA was 0.93, the sensitivity was 0.93 (95% CI: 0.72-1.00), and the specificity was 0.95 (95% CI: 0.74-1.00).
DISCUSSION
USE showed high diagnostic value for pediatric surgical biliary tract closure.
PubMed: 35685066
DOI: 10.21037/tp-22-159 -
Children (Basel, Switzerland) Oct 2022The early diagnosis of biliary atresia (BA) in cholestatic infants is critical to the success of the treatment. Intraoperative cholangiography (IOC), an invasive imaging... (Review)
Review
The early diagnosis of biliary atresia (BA) in cholestatic infants is critical to the success of the treatment. Intraoperative cholangiography (IOC), an invasive imaging technique, is the current strategy for the diagnosis of BA. Ultrasonography has advanced over recent years and emerging techniques such as shear wave elastography (SWE) have the potential to improve BA diagnosis. This review sought to evaluate the diagnostic efficacy of advanced ultrasonography techniques in the diagnosis of BA. Six databases (CINAHL, Medline, PubMed, Google Scholar, Web of Science (core collection), and Embase) were searched for studies assessing the diagnostic performance of advanced ultrasonography techniques in differentiating BA from non-BA causes of infantile cholestasis. The meta-analysis was performed using Meta-DiSc 1.4 and Comprehensive Meta-analysis v3 software. Quality Assessment of Diagnostic Accuracy Studies tool version 2 (QUADAS-2) assessed the risk of bias. Fifteen studies consisting of 2185 patients (BA = 1105; non-BA = 1080) met the inclusion criteria. SWE was the only advanced ultrasonography technique reported and had a good pooled diagnostic performance (sensitivity = 83%; specificity = 77%; AUC = 0.896). Liver stiffness indicators were significantly higher in BA compared to non-BA patients (p < 0.000). SWE could be a useful tool in differentiating BA from non-BA causes of infantile cholestasis. Future studies to assess the utility of other advanced ultrasonography techniques are recommended.
PubMed: 36360404
DOI: 10.3390/children9111676 -
Canadian Journal of Gastroenterology =... Aug 2011The role of adjuvant steroid therapy in the postoperative management of patients with biliary atresia (BA) is unclear. (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The role of adjuvant steroid therapy in the postoperative management of patients with biliary atresia (BA) is unclear.
OBJECTIVE
To systematically review the literature and perform a meta-analysis to determine the efficacy of adjuvant steroid therapy post-Kasai portoenterostomy (KP) on BA outcome.
METHODS
A systematic review and meta-analysis of randomized trials and⁄or observational studies that examined the role of steroids on BA outcomes published between January 1969 and June 2010 was conducted. Studies were identified using the Medline, PubMed, EMBASE and Cochrane databases.
RESULTS
Sixteen observational studies and one randomized controlled trial (RCT) were found. Four of the 16 observational studies (160 participants) and the RCT (73 participants) met the entry criteria and were eligible to be included in the analysis. There was no statistically significant difference in the effect of steroids either on normalizing serum bilirubin levels at six months (pooled OR 1.48 [95% CI 0.67 to 3.28]) or in delaying the need for early liver transplantation (within the first year post-KP (pooled OR 0.59 [95% CI 0.21 to 1.72]).
CONCLUSION
The present meta-analysis did not find a significant effect of steroid over standard therapy, either in normalizing serum bilirubin levels at six months or at delaying the need for early liver transplantation post-KP. RCT studies of sufficient size and comprehensive design using high-dose steroids are needed to determine the effectiveness of steroids on the short and intermediate post-KP outcomes for BA patients.
Topics: Biliary Atresia; Bilirubin; Chemotherapy, Adjuvant; Glucocorticoids; Humans; Liver Transplantation; Portoenterostomy, Hepatic; Treatment Outcome
PubMed: 21912769
DOI: 10.1155/2011/125610 -
Translational Pediatrics Jan 2024Kasai procedure and liver transplantation are effective ways to save the life of children with biliary atresia (BA). However, with the gradual development of liver...
BACKGROUND
Kasai procedure and liver transplantation are effective ways to save the life of children with biliary atresia (BA). However, with the gradual development of liver transplantation technology, scholars have questioned the necessity of the Kasai procedure. Therefore, we conducted a meta-analysis to evaluate the effect of previous Kasai procedures on liver transplantation in children with BA.
METHODS
Seven databases were searched and screened from the establishment of the database to May 3, 2023. The data in the included literature were extracted for meta-analysis to compare the differences between the Kasai group and the non-Kasai group. Finally, a publication bias test, sensitivity analysis, subgroup analysis, and systematic review were performed.
RESULTS
A total of 26 studies were included in which 6,522 children with BA underwent liver transplantation, including 4,989 in the Kasai group. Compared with the non-Kasai group, the Kasai group had older age [standardized mean difference (SMD) =0.64; 95% confidence interval (CI): 0.46, 0.82; P<0.001] (I=78.6%), heavier weight (SMD =0.41; 95% CI: 0.33, 0.48; P<0.001) (after sensitivity analysis, I=0.0%), lower pediatric end-stage liver disease (PELD) (SMD =-0.41; 95% CI: -0.48, -0.35; P<0.001) (I=20.1%), longer operation time (SMD =0.33; 95% CI: 0.01, 0.65; P<0.001) (I=83.2%), more intraoperative blood loss (SMD =0.26; 95% CI: 0.06, 0.46; P=0.012) (I=19.1%), shorter intensive care unit (ICU) stay (SMD =-0.09; 95% CI: -0.34, 0.15; P=0.027) (I=68.6%) and higher incidence of intestinal perforation [odds ratio (OR) =1.96; 95% CI: 1.20, 3.18; P=0.007] (I=7.4%) and biliary complications (OR =1.41; 95% CI: 1.05, 1.89; P=0.024) (I=31.4%). In the "Asia" subgroup, the Kasai group was older (SMD =0.68; 95% CI: 0.52, 0.84; P<0.001) (I=28.2%). In the "Cases since 2000" subgroup, there was no significant difference in operation time between the two groups (I=28.5%). In the "Other" and the "non-Asia" subgroup, there was no significant difference in length of intensive care unit (ICU) stay between the two groups (I=0.0%). However, there were no significant differences in other postoperative complications and prognostic indicators between the two groups.
CONCLUSIONS
For children with BA undergoing liver transplantation, although previous Kasai procedure may increase the risk of intraoperative bleeding, biliary complications, and intestinal perforation, it does not affect the main clinical outcomes, and can even delay the timing of liver transplantation and improve the preoperative status of children. Therefore, when BA children have no obvious contraindications to Kasai procedure, the sequential treatment of Kasai procedure-liver transplantation should be supported first.
PubMed: 38323176
DOI: 10.21037/tp-23-504 -
Frontiers in Pediatrics 2021The etiology of biliary atresia is unclear, but viral infection has been implicated. The aim of the current meta-analysis was to investigate relationships between...
The etiology of biliary atresia is unclear, but viral infection has been implicated. The aim of the current meta-analysis was to investigate relationships between cytomegalovirus (CMV) and the prognosis of biliary atresia. PubMed, Embase, the Cochrane Library, the China National Knowledge Infrastructure database, and Wanfang Data electronic databases were searched for eligible studies. Each relevant text was thoroughly reviewed and examined, including related papers in their reference lists. A total of nine studies including 784 patients were included in the analysis. Biliary atresia patients with CMV exhibited significantly lower jaundice clearance (odds ratio: 0.46, < 0.0001; = 15%, = 0.31). There were no significant differences in the rates of cholangitis or native liver survival. CMV-pp65-positive biliary atresia patients had a significantly lower rate of jaundice clearance (odds ratio: 5.87, = 0.003; = 0%, = 0.71) and a significantly higher rate of cholangitis (odds ratio: 0.21, = 0.01; = 0%, = 0.43) than CMV antibody-positive biliary atresia patients. Biliary atresia patients who were also infected with CMV had a poorer prognosis, particularly with respect to jaundice clearance. CMV status may influence the prognosis of biliary atresia. Clinicians should be able to routinely identify the subset of biliary atresia patients who are also CMV-positive, in order to improve native liver survival.
PubMed: 34490166
DOI: 10.3389/fped.2021.710450