Severity assessment to guide empiric antibiotic therapy for cholangitis in children after Kasai portoenterostomy: a multicenter prospective randomized control trial in China.

BACKGROUND: Cholangitis is common in patients with biliary atresia following Kasai portoenterostomy (KPE). The prompt use of empiric antibiotics is essential due to the lack of identified microorganisms. The authors aimed to validate a severity grading system to guide empiric antibiotic therapy in the management of post-KPE cholangitis. MATERIALS AND METHODS: This multicenter, prospective, randomized, open-label study recruited patients with post-KPE cholangitis and was conducted from January 2018 to December 2019. On admission, patients were categorized into mild, moderate, and severe cholangitis according to the severity grading system. Patients in the mild cholangitis group were randomized to receive cefoperazone sodium tazobactam sodium (CSTS) or meropenem (MEPM). Patients with severe cholangitis were randomized to treatment with MEPM or a combination of MEPM plus immunoglobulin (MEPM+IVIG). Patients with moderate cholangitis received MEPM. RESULTS: The primary endpoint was duration of fever (DOF). Secondary outcomes included blood culture, length of hospital stay, incidence of recurrent cholangitis, jaundice clearance rate, and native liver survival (NLS). For mild cholangitis, DOF, and length of hospital stay were similar between those treated with CSTS or MEPM (all P >0.05). In addition, no significant difference in recurrence rate, jaundice clearance rate, and NLS was observed between patients treated with CSTS and MEPM at 1-month, 3-month, and 6-month follow-up. In patients with moderate cholangitis, the DOF was 36.00 (interquartile range: 24.00-48.00) h. In severe cholangitis, compared with MEPM, MEPM+IVIG decreased DOF and improved liver function by reducing alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, and direct bilirubin at 1-month follow-up. However, recurrence rate, jaundice clearance rate, and NLS did not differ significantly between MEPM+IVIG and MEPM at 1-month, 3-month, and 6-month follow-up. CONCLUSIONS: In patients with post-KPE cholangitis, MEPM is not superior to CSTS for the treatment of mild cholangitis. However, MEPM+IVIG treatment was associated with better short-term clinical outcomes in patients with severe cholangitis.

Prophylactic Antibiotics to Prevent Cholangitis in Children with Biliary Atresia After Kasai Portoenterostomy: A Meta-Analysis.

OBJECTIVES: A connection between the bowel and bile ducts after the Kasai hepatoportoenterostomy (HPE) procedure poses a risk of ascending cholangitis. There were only a few evidence-based consensuses on the benefits of prophylactic antibiotics. This study aims to assess the value of prophylactic antibiotics in reducing the risk of cholangitis following the Kasai HPE procedure. METHODS: Meta-analysis is performed using random-effects model from the search result of 5 online databases (PubMed, Google Scholar, EBSCO MEDLINE, ClinicalTrials.gov , and EuropePMC) from inception to October 27, 2021. The keywords used were "antibiotic," "antimicrobial," "Kasai," "portoenterostomy," "biliary atresia," and "bile duct atresia." Cochrane Risk of Bias tool and Newcastle-Ottawa Scale is used to assess the risk of bias. The outcomes are incidence of cholangitis and native liver survival. RESULTS: Six studies consisting of 4 cohorts and 2 cross-sectional studies were extracted. A total of 714 patients reported different cholangitis incidence after prophylactic antibiotics administration post-Kasai HPE. The incidence of cholangitis following Kasai HPE was not statistically significant among participants. There is conflicting evidence on the efficacy of antibiotics in prolonging native liver survival. CONCLUSIONS: The existing evidence does not support the administration of prophylactic antibiotics in preventing cholangitis after Kasai HPE among biliary atresia patients. Additionally, their roles in native liver survival are still inconclusive. The fact that there were heterogeneous method and antibiotic usage between existing studies must also be highlighted for better design in future studies.

Laparoscopic Kasai Procedure of Treating Biliary Atresia with an Aberrant Right Hepatic Artery.

Background: An aberrant vascular anatomy might present a technical pitfall for biliary atresia (BA) surgery. The purpose of this study was to report the rare cases and discuss the significance and management strategy for BA with an aberrant right hepatic artery (ARHA) by laparoscopic Kasai procedure in children. Methods: The subjects for this study were 10 consecutive type III BA patients with an ARHA who had laparoscopic Kasai procedure at our institute between January 2012 and August 2021. The common bile duct was mobilized between the right hepatic artery and the right branch of portal vein, and then lifted to the liver hilum. The fibrous cord was transected and then the laparoscopic Kasai was carried out. Results: All patients survived the laparoscopic Kasai without any intraoperative complications. The mean operative time was 235 minutes for each laparoscopic Kasai. The mean follow-up time was 32.6 months. The total and direct bilirubin dropped to normal within 4 months of surgery in 7 patients. One patient died of repeated cholangitis and liver failure 1 year after surgery. In the additional 2 patients the bilirubin levels dropped significantly after the surgery but elevated again because of repeated cholangitis and requiring ongoing observation and intermittent treatment. Conclusions: With the perfect laparoscopic skills, the common bile duct could be safely mobilized between the right hepatic artery and right branch of portal vein for the infants with type III BA associated with an ARHA, and laparoscopic Kasai could be carried out safely and successfully.

Comparison of Liver Transplant Outcomes in Biliary Atresia Patients, Considering Whether They Underwent the Kasai Procedure Beforehand: A Single Center Analysis of 72 Patients.

BACKGROUND: This study examines the results of liver transplantation (LT) in patients with biliary atresia, considering whether they underwent the Kasai procedure beforehand. LT and determine postoperative and long-term graft outcomes. METHODS: This single-center, retrospective study included 72 pediatric patients diagnosed with postpartum biliary atresia who underwent LT between 2010 and 2022. We included patients who underwent LT either after or without the Kasai procedure and compared the demographic data of the patients with various factors, such as the Pediatric End-Stage Liver Disease scores and laboratory values. RESULTS: The study included 72 patients, with 39 of them being female (54.2%) and 33 of them being male (45.8%). Of the 72 patients in the study, 47 (65.3%) had undergone the Kasai procedure, and 25 (34.7%) had not. The preoperative and postoperative month 1 bilirubin values were lower in patients who underwent the Kasai procedure and were higher in postoperative months 3 and 6. Preoperative bilirubin values, postoperative month 3 bilirubin values, and preoperative albumin values were higher in patients who developed mortality (P < .05). Cold ischemia time was longer in patients who developed mortality (P < .05). CONCLUSIONS: Our study showed a higher mortality rate in patients who underwent the Kasai procedure. The results also showed that LT was more effective in children, as patients with Kasai had higher mean bilirubin values and higher preoperative albumin values than patients without Kasai.

Risk Assessment of Liver Transplantation After Kasai Portoenterostomy in Children and Adults.

INTRODUCTION: Reports of liver transplantation (LT) after Kasai portoenterostomy (KPE) in adult patients with biliary atresia are scarce. The aim of this study was to evaluate the outcomes and investigate the risk factors of LT after KPE in both pediatric and adult patients. METHODS: We retrospectively reviewed a prospective database of patients with biliary atresia who underwent LT after KPE. Eighty-nine consecutive patients were included, and risk factors for in-hospital mortality after LT were assessed. RESULTS: The median age of the patients was 2 y (range, 0-45 y). Forty-six patients (51.7%) had a history of upper abdominal surgery after KPE. The in-hospital mortality rate was 5.6% (5 patients). Of these, 80% of patients with mortality were aged ≥17 y, and all patients with mortality had a history of two or more upper abdominal surgeries. In the univariate and receiver operating characteristic curve analyses, age ≥17 y and the number of previous upper abdominal surgeries ≥2 were identified as possible risk factors. CONCLUSIONS: Our study suggests that older age and multiple previous upper abdominal surgeries are important risk factors for mortality after LT following KPE. We believe that these findings will serve as indications for safe LT in future patients.

Treatment of intractable cholangitis in children with biliary atresia: Impact on outcome.

OBJECTIVES: To evaluate the response and outcome with prolonged intravenous antibiotics including home-based intravenous antibiotics in children with intractable cholangitis (IC) after Kasai portoenterostomy (KPE) for biliary atresia (BA). METHODS: A retrospective review of treatment and outcome of children with IC post KPE (no resolution after four weeks of antibiotics) was done between 2014 and 2020. A protocol-based antibiotic regimen was used based on sensitivity and hospital antibiogram. Children afebrile for more than three days were discharged on home intravenous antibiotics (HIVA). RESULTS: Twenty children with IC were managed with prolonged antibiotic regimen, including HIVA. All patients were initially listed for liver transplantation (LT) with indication being IC (n = 20) with portal hypertension (n = 12). Seven patients had bile lakes of which four underwent percutaneous transhepatic biliary drainage. Bile culture grew Klebsiella in four and Escherichia coli and Pseudomonas one each. There were eight children with IC who had positive blood culture with most of these organisms being gram-negative (Escherichia coli: 5, Klebsiella pneumoniae: 2, Enterococcus: 1). Median duration of antibiotics was 58 days (interquartile range [IQR] 56-84). Median follow-up period post cholangitis was three years (IQR 2-4). Following treatment, 14 patients were successfully delisted from LT waitlist and are presently jaundice-free. Two of the five patients undergoing LT died of sepsis. One patient died awaiting LT. CONCLUSION: Timely and aggressive step-up antibiotic regimen may successfully treat IC and prevent/delay LT. HIVA provides a cost-effective and comfortable environment for a child which might improve compliance with intravenous antibiotics.

Advances in understanding of biliary atresia pathogenesis and progression - a riddle wrapped in a mystery inside an enigma.

INTRODUCTION: Biliary atresia is a potentially fatal condition of the bile ducts - both intra- and extrahepatic, for which we have no cure. Though principally a cholestatic condition, much of its pathology stems from its tendency to aggressively induce liver fibrosis and ultimately cirrhosis, only partially restrained by the portoenterostomy. AREAS COVERED: This review is based on the current literature exploring the heterogeneous nature of biliary atresia. Thus, there are various phenotypes or variants of biliary atresia, each potentially with different etiological backgrounds caused by a number of hypothetical pathological mechanisms thought to be important in the genesis of the condition. Search methodology: the review (Oct. - Nov. 2022) is based on a search of PubMed (NLM) using main keyword 'biliary atresia' with supplementary searches using 'fibrosis'; 'inflammation'; 'BASM'; 'genetics'; 'surgery'; 'experimental'; 'etiology'; 'virology'; 'cases'; and 'syndromes.' EXPERT OPINION: Future developments will be made on matching clinical variants with a more distinct pathophysiological discrimination and those pathways linking the initial cholestatic phase of biliary atresia to the early stages of fibrosis.

In vitro fertilization: an unexpected finding in a cohort of patients with biliary atresia.

In biliary atresia (BA), efforts to prevent premature liver transplantation (LT) are aimed at early diagnosis, timing of Kasai-portoenterostomy (KPE), and centralization of care. This report presents the clinical picture, treatment strategies, and outcomes of BA patients with no previous treatment. A retrospective cohort study (Jan/2001 to Jan/2021) was conducted to evaluate the outcome of patients with BA referred to a single team. Study groups were: 1) Kasai-only group (K-only) n=9), 2) LT-only group (n=7), and 3) Kasai+LT group (K+LT) (n=23). Survival with native liver and overall survival were 22.9 and 94.8%, respectively, at 120 months of follow-up. There was no difference in age at KPE in the K-only group (46.8±21.8 days) vs K+LT (52.1±22 days), P=0.4. Ten (25.6%) patients were babies conceived through in vitro fertilization (IVF). Four IVF patients (40%) presented associated congenital heart disease vs 5 patients (17%) in the remaining group (P=0.14). Two of the IVF patients were premature (<37 weeks). Median maternal age at birth was 35 years (33 to 41 years). Excellent patient survival is expected for patients with BA with the available treatment strategies. IVF+BA was an unexpected prevalent association in this cohort, and further studies are required to better understand these findings.

Peri-Kasai portoenterostomy sutures anchoring the jejunal loop to Glisson capsule: A novel modification to reduce the incidence of cholangitis.

BACKGROUND: Kasai portoenterostomy (KPE) is the standard surgical management for biliary atresia (BA). To improve the outcome these infants were operated on within the first two months of life. The success of the procedure is reflected by clearance of jaundice and either absence or occurrence of fewer attacks of cholangitis. The failure of the procedure indicates liver transplantation (LT). OBJECTIVE: to reduce the incidence of the recurrent attacks of cholangitis by peri-KPE sutures anchoring the jejunal loop to the Glisson capsule. METHODS: It is a retrospective study that included 45 infants diagnosed with BA and who were operated on at an age younger than 60 days. They were categorized into two groups, Group A (n = 23) included infants treated with the classic KPE, and Group B (n = 22) included infants treated in the same way plus peri KPE sutures anchoring the jejunal loop to the Glisson capsule. RESULTS: The mean operative time in Group A was 149.3 min versus 164.8 min in Group B (p-value 0.039). The mean level of bilirubin was 2.2 versus 2.1 in Group A and Group B respectively at two years follow up. The total attacks of cholangitis per patient were significantly lower in Group B than in Group A (cutoff value = 3), which was reflected by the significant reduction of the incidence of LT in Group B. CONCLUSION: peri KPE sutures anchoring the jejunal loop to the Glisson capsule significantly reduced the incidence of recurrent attacks of cholangitis and subsequently decreased the requirement of LT on the short-term follow-up.

Biliary atresia-An experience from the first pediatric liver transplant center in Pakistan.

BACKGROUND: Biliary atresia (BA) is the most common cause of neonatal cholestatic syndrome. The true incidence of BA in Pakistan is largely unknown. AIM: This study aimed to report the clinical features, age at diagnosis and outcomes of biliary atresia from the first pediatric liver transplant center in Pakistan. METHODS: The study was done in Shifa International hospital from 2013 to 2020. All babies who had biliary atresia confirmed by laboratory investigation were included. Demographic data, age of presentation, clinical presentation, supporting investigations like liver function tests, ultrasound abdomen, HIDA scan and liver biopsy were noted. Outcome related to Kasai portoenterostomy, liver transplant, complications and immunosuppressant agents were noted. RESULT: A total of 42 children were included, 23 (54.7%) males and 19 (45.2%) were females. Jaundice was seen in all patients (100%) followed by acholic stools (81%). Associated malformations were noted in 6 (14.2%) patients. Liver function tests confirmed obstructive cholestasis (p 0.04). Kasai was done in 19 (45%) patients only, living donor liver transplant was performed in 6 (14%) patients. Age range of transplant patients was from 3 months to 1 year. Indication for liver transplant was failed Kasai in 1(16.7%) patient and chronic liver disease in 5 (83.3%) patients. LDLT survivors were 10 months to 1 year of age at the time of transplant, mean age was 10.6 months. Maximum survival noted so far is 7 years. Acute complications seen post-transplant were sepsis (three patients), surgical site infections (two patients), biliary leaks and acute cellular rejection in one patient each. Chronic graft rejection, portal vein stricture needing stenting was done in one patient. DISCUSSION: All patients underwent LDLT from related donors wih no donor related mortality. All are deceased patients were yonger and had advanced disease. BA remains third most commo indication of transplant in our center. CONCLUSION: Liver transplant is the only lifesaving procedure after failed Kasai or as primary liver transplant due to advance liver disease. The advent of liver transplantation services offers survival and improving outlook of the disease.

A Comparison of Laparoscopic-Modified Kasai Versus Conventional Open Kasai for Biliary Atresia in Infants: A Single-Center Experience.

Background: The purpose of this study was to evaluate the efficacy of our modified laparoscopic Kasai portoenterostomy for type III biliary atresia (BA) in children. Methods: A total of 56 type III BA patients underwent laparoscopic-modified Kasai procedure from January 2015 to July 2021. Conventionally control group of 45 BA patients from January 2012 to January 2016 were operated by open Kasai. The clinical data between the two groups were retrospectively compared. Results: The mean operating time was 235.5 ± 20.5 minutes (range, 210-275 minutes) in Group I, whereas 180.5 ± 25.5 minutes (range, 155-210 minutes) in Group II. The oral intake resumption was faster in Group I than that in Group II (mean 3.5 days versus 4.5 days). The follow-up time was mean 18.5 months in Group I and 24 months in Group II. The clearance of jaundice (total bilirubin <20 µmol/L) was significant different in Group I (78.6%) (44/56) versus that of Group II (74.3%) (33/45). The native liver survival rate was 71.4% (40/56) in Group I and was 66.7% (30/45) in Group II at the follow-up time, respectively. Conclusion: The laparoscopic-modified Kasai would improve the outcome of BA patients.

Laparoscopic internal intestinal drainage of bile lakes in a patient with recurrence of jaundice after laparoscopic revision of Kasai portoenterostomy for biliary atresia: A case report.

Bile lake, of the postoperative complications after Kasai portoenterostomy (PE) for biliary atresia, causes cholangitis that may induce progressive fibrosis of the liver. Standard treatment for bile lakes has not yet been established, but there are reports that surgical internal intestinal drainage for bile lakes effectively prevents cholangitis and maintains jaundice-free status. In this case, insertion of the percutaneous transhepatic biliary drainage into the bile lake allowed continuous drainage of large volumes of bile juice. However, reoperation following laparotomy increases the surgical risk of subsequent liver transplantation due to postoperative adhesion. Laparoscopic surgery was selected for the patient who was likely to require liver transplantation in the future. In this case, laparoscopic internal intestinal drainage of bile lakes was performed safely by a Cavitron ultrasonic surgical aspirator for the recurrence of jaundice after laparoscopic revision of PE. Cholangitis and jaundice were rapidly resolved after this surgical procedure.

Liver Explants of Biliary Atresia Patients Transplanted in Adulthood Show Features of Obliterative Portal Venopathy: Case Series and Guidelines for Pathologic Reporting of Adult Explants.

CONTEXT.­: Biliary atresia (BA) patients can have portal vein (PV) abnormalities. OBJECTIVE.­: To investigate the explant pathology of BA patients transplanted in adulthood with a focus on portal venous abnormalities. DESIGN.­: Adult BA liver explants were reviewed, along with prior biopsies, Kasai portoenterostomy (KP), and relevant medical records. RESULTS.­: Three explants were identified; all patients were female, with age at diagnosis, KP, and liver transplantation (LT) as follows: (1) less than 1 week, 8 days, and 25 years; (2) 15 weeks, 16 weeks, and 32 years; and (3) 7 weeks, 8 weeks, and 33 years, respectively, with normalization of conjugated bilirubin within 6 months of KP and development of portal hypertension (PHTN) within 3 years of KP for all 3. The first 2 had recurrent cholangitis. Duration of pre-LT PHTN was 22, 29, and 30 years, and that of pre-LT cholangitis was 9, 3, and 0 years, respectively. All 3 explants showed hilar and extrahepatic fibromyxoid intimal hyperplasia of the PV with parenchymal hepatoportal sclerosis. Cholestasis was limited to those with a history of cholangitis. Patient 3, without cholangitis, showed delicate septal fibrosis with peripheral accentuation without biliary cirrhosis. CONCLUSIONS.­: In the context of a functioning KP, cholestasis and biliary cirrhosis are likely related to recurrent cholangitis, which may or may not occur after KP. In the absence of biliary cirrhosis, PHTN may be secondary to obliterative venopathy. Adult BA explants should be sampled thoroughly, with a focus on hilar/perihilar connective tissue to include PV branches. Explants may not show biliary cirrhosis and should be reported with appropriate clinicopathologic correlation.

Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa.

BACKGROUND: Biliary atresia (BA) is an obstructive inflammatory disease of the bile ducts. Without intervention, the disease rapidly progresses to liver cirrhosis and fibrosis, with end-stage liver failure and death occurring within the first 3 years of life. It is the most common indication for liver transplantation (LT) in the paediatric population. The management of BA in South Africa (SA) faces multiple challenges, such as late referrals and socioeconomic burdens, with suboptimal outcomes. OBJECTIVES: To determine risk factors and shortcomings that are detrimental to the outcome of the paediatric patient population by reviewing the profile, management and outcome of patients with BA treated at Universitas Academic Hospital Complex (UAHC), Bloemfontein, SA. METHODS: This was a retrospective analytical record review of all patients diagnosed with BA and treated at UAHC from 1 January 2009 to 31 December 2019. RESULTS: In total, 67 patients were included; 74.6% were female, and 86.6% were black Africans. Most (62.7%) had isolated BA. A Kasai portoenterostomy (KPE) was performed in 32 patients (47.8%). Of 5 patients referred for LT evaluation, 2 received a transplant. Of 55 patients with known outcomes, 5.5% (n=3) survived and 94.5% (n=52) died after receiving a KPE or palliative treatment. Of the 3 patients who were alive at the end of the study period, 1 had a KPE and 2 had LTs. CONCLUSION: Late presentation, cholangitis and cessation of bile flow after an initial successful KPE, and socioeconomic challenges are issues of concern and had a detrimental influence on the outcome of BA in our study population. Implementing screening measures and education programmes at the primary healthcare level is essential to diagnose and refer BA patients timeously. Establishing support systems to assist socioeconomically disadvantaged patients will enable them to qualify for LT.

Relationship between the expression levels of CD4+ T cells, IL-6, IL-8 and IL-33 in the liver of biliary atresia and postoperative cholangitis, operative age and early jaundice clearance.

OBJECTIVE: To investigate the expression levels of CD4+ T cells, IL-6, IL-8 and IL-33 in liver tissue of BA, and the relationship with postoperative cholangitis, operative age and early jaundice clearance. METHODS: 45 cases of jaundice treated in the hospital from June 2018 to May 2020 were analyzed retrospectively. The expression and distribution of these factors were detected by HE staining and immunohistochemistry, the total bilirubin level and the incidence of cholangitis were recorded, and the relationship between liver inflammation level and the postoperative incidence of cholangitis, age of operation and early jaundice clearance were compared. RESULTS: Immunohistochemistry showed that the expression of CD4+ T cells, IL-6, IL-8 and IL-33 in the BA group were higher than those in the CBD group. ROC curve analysis showed the AUC of CD4+ T cells, IL-6 and IL-8 were 0.869, 0.886 and 0.838, respectively. The expression level of CD4+ T cells was negatively correlated with the decline rate of TBIL 3 months after operation, and the expressions of IL-8 and IL-33 were negatively correlated with the decline rate of TBIL 1 week after operation. CONCLUSION: The high expression of CD4+ T cells, IL-6, IL-8 and IL-33 in the BA liver tissue may lead to cholangitis and can be used as a predictor of early jaundice clearance. The degree of liver inflammation infiltration had nothing to do with the age of operation and is not a risk factor for postoperative cholangitis.

Laparoscopic revision of Kasai portoenterostomy after initial laparoscopic portoenterostomy in patients with biliary atresia: a limited but positive effect on native liver survival.

PURPOSE: It is important for patients with biliary atresia (BA) to retain the native liver after Kasai portoenterostomy (PE). Laparoscopic PE (Lap-PE) is standard, whereas laparoscopic revision of PE (Lap-revision) is performed as needed. We report the medium-term outcomes of Lap-revision. METHODS: We retrospectively reviewed the demographics and outcomes of 63 patients who underwent Lap-PE between 2013 and 2021. Indications for revision included recurrent jaundice, repeat cholangitis, and persistent jaundice with temporary biliary excretion following the initial PE. We compared liver transplantation (LT) data of patients who underwent Lap-revision with those of patients who did not. RESULTS: Lap-revision was performed in 20 patients. Of those 17 (excluding the two who were jaundice-free before the age of 1, and the one who underwent open conversion for bleeding), 11 (65%) were jaundice-free 1 year after Lap-revision. The surgical parameters of LT did not differ between patients who underwent Lap-revision and those who did not. CONCLUSION: Lap-revision for patients with BA had a limited but positive effect on native liver survival and did not adversely affect subsequent LT. Therefore, Lap-revision could be second-line standard therapy in patients with BA who have demonstrated biliary drainage at least once after initial PE.

Biochemical markers to predict the development of gastrointestinal bleeding and esophageal varices after portoenterostomy in biliary atresia.

PURPOSE: Gastrointestinal bleeding (GIB) due to esophageal varices (EV) is one of the factors that negatively impact native liver survival of patients with biliary atresia (BA). Gastrointestinal fibroscopy (GIF) is usually used to determine the presence of EVs; however, it requires general anesthesia. The aim of this study is to search for markers in blood tests obtained during routine check-ups that can predict the development of GIB. METHODS: Data of patients with BA who underwent portoenterostomy at our hospital from 2014 to 2020 were retrospectively reviewed. The patients' data were assigned to three groups according to specific time points: Group B, which included data at GIB; Group NB-T, which included data at GIF and EV treatment; and Group NB-NT, which included data at GIF without treatment. The data in Group B were compared to those of other groups. RESULTS: In our study, GIB occurred in 11 patients, and 12 cases and 8 cases were classified into Groups NB-NT and NB-T, respectively. Compared with the other groups, only ChE and M2BPGi in Group B showed statistically significant differences. CONCLUSIONS: ChE and M2BPGi are useful for predicting GIB.

The preoperative lymphocyte ratio and postoperative C-reactive protein are related to the surgical outcome in biliary atresia: an analysis of serial ubiquitous markers of inflammation.

PURPOSE: Various prognostic predictors for biliary atresia (BA) have been identified. This study aimed to evaluate the serial changes in the preoperative and postoperative ubiquitous inflammatory biomarkers and their relationship with the outcomes in patients with BA. PATIENTS AND METHODS: Forty-three BA patients were retrospectively reviewed to investigate serial levels of ubiquitous inflammatory biomarkers, including C-reactive protein (CRP) and lymphocyte ratio, and outcomes. The patients with BA were divided based on their outcomes into two prognostic groups: the native liver survivor group (n = 30) and the survivors with living-donor liver transplant group (n = 13). RESULTS: The area under the receiver operating characteristic (ROC) curve analysis showed that a preoperative lymphocyte ratio of < 61% and CRP value > 0.1 mg/dl predicted a poor outcome. In the ROC curve analysis, the timing of reaching the cut-off value of CRP after Kasai portoenterostomy was postoperative day (POD) 57. The third postoperative week, which was the timing of the discontinuation of steroid therapy, was the branchpoint of inflammatory markers between the two prognostic groups. CONCLUSION: The POD 57 CRP level predicts the surgical outcome of Kasai portoenterostomy. The postoperative anti-inflammatory management of BA can be monitored by the ubiquitous inflammatory biomarkers CRP and the preoperative lymphocyte ratio.

Current Understanding in the Clinical Characteristics and Molecular Mechanisms in Different Subtypes of Biliary Atresia.

Biliary atresia is a severe obliterative cholangiopathy in early infancy that is by far the most common cause of surgical jaundice and the most common indicator for liver transplantation in children. With the advanced knowledge gained from different clinical trials and the development of research models, a more precise clinical classification of BA (i.e., isolated BA (IBA), cystic BA (CBA), syndromic BA (SBA), and cytomegalovirus-associated BA (CMVBA)) is proposed. Different BA subtypes have similar yet distinguishable clinical manifestations. The clinical and etiological heterogeneity leads to dramatically different prognoses; hence, treatment needs to be specific. In this study, we reviewed the clinical characteristics of different BA subtypes and revealed the molecular mechanisms of their developmental contributors. We aimed to highlight the differences among these various subtypes of BA which ultimately contribute to the development of a specific management protocol for each subtype.