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Objectives: In 2022, the Biliary Atresia and Related Diseases (BARD) community reached a consensus for the definition of suspected and confirmed cholangitis for biliary atresia (BA) patients after hepatoportoenterostomy (HPE). This study assessed the new standardized BARD definition in a retrospective, multicenter cohort study. Methods: We included BA cases managed between 2010 and 2020 at the Hannover Medical School and Geneva University Hospitals' Swiss Pediatric Liver Center. The standardized BARD cholangitis definition assesses four clinical items and four imaging/laboratory items to define cholangitis. The definition was retrospectively applied to all BA cases having presented, according to their physician, cholangitis within the first year after the HPE. The diagnosis defined by the standardized BARD definition was compared with the final clinical diagnosis made by physicians. The Spearman's correlation coefficient was used to test for correlation between diagnoses made by standardized and clinical appreciation. Results: Of 185 consecutive BA patients, 59 (32%) had at least one episode of cholangitis within the first year after HPE. The correlation between the clinician's impression and the standardized BARD definition was very strong (r = 0.8). Confirmed cholangitis definition coincided with the clinician's impression (2.5 [±0.7]/4 clinical items, 2.6 [±0.5]/4 imaging/laboratory items). For suspected cholangitis, the threshold for diagnosis was lower within the standardized BARD definition (1.1 [±0.3]/4 clinical items, 2.2 [±0.8]/4 laboratory/imaging items). Conclusions: This first retrospective application of the standardized BARD cholangitis definition reveals a very strong correlation with the physician's assessment before standardization. A prospective study is needed to further refine the standardized definition for cholangitis in BA patients.

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OBJECTIVES: To identify and distinguish between racial and socioeconomic disparities in age at hepatology care, diagnosis, access to surgical therapy, and liver transplant-free survival in patients with biliary atresia (BA). METHODS: Single-center retrospective cohort study of 69 BA patients from 2010 to 2021. Patients were grouped into White and non-White cohorts. The socioeconomic milieu was analyzed utilizing neighborhood deprivation index, a census tract-based calculation of six socioeconomic variables. The primary outcomes of this study were timing of the first hepatology encounter, surgical treatment with hepatic portoenterostomy (HPE), and survival with native liver (SNL) at 2 years. RESULTS: Patients were 55% male and 72% White. White patients were referred at a median of 34 days (interquartile range [IQR]: 17-65) vs. 67 days (IQR: 42-133; p = 0.001) in non-White patients. White infants were more likely to undergo HPE (42/50 patients; 84%) compared to non-White (10/19; 53%), odds ratio (OR) 4.73 (95% confidence interval: 1.46-15.31; p = 0.01). Independent of race, patients exposed to increased neighborhood-level deprivation were less likely to receive HPE (OR: 0.49, p = 0.04) and achieve SNL (OR: 0.54, p = 0.02). CONCLUSIONS: Racial and socioeconomic disparities are independently associated with timely BA diagnosis, access to surgical treatment, and transplant-free survival. Public health approaches to improve screening for pathologic jaundice in infants of diverse racial backgrounds and to test and implement interventions for socioeconomically at-risk families are needed.

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Background and Aims: Early identification of prognostic factors to predict transplant/death outcome of biliary atresia (BA) is challenging. We aimed to investigate the longitudinal changes and predictive value of dynamic changes in acoustic radiation force impulse elastography with shear wave speed (SWS) quantification and other parameters within three months after hepatoportoenterostomy (HPE) for 2-year BA outcomes. Methods: Seventy-four patients who underwent HPE between July 2016 and June 2019 were prospectively enrolled. Outcomes were classified into native liver survival and transplant/death groups. Acoustic radiation force impulse elastography was performed sequentially at 3 months intervals post-HPE. Cox regression analysis was used to determine the superior SWS values and other predictors of liver transplantation or death. Results: Among patients 2 years of age, 36 survived with a native liver, nine died, and 29 underwent liver transplantation. The trend in SWS levels in the transplant/death group was significantly different from that in the native liver survival group. ΔSWS at 1-3 months post-HPE and total bilirubin at 1 month post-HPE were selected as superior predictors of liver transplantation or death using multivariate Cox regression models: hazard ratio (HR)=1.927; 95% confidence interval (CI): 1.475-2.661; p<0.001 and HR=1.010; 95% CI: 1.003-1.017; p=0.007, respectively. The combination of the selected ΔSWS and total bilirubin had good predictive power, with an area under the receiver operating characteristics curve of 0.89, specificity 94.44% and sensitivity 73.68%. Conclusions: Our results suggest that early postoperative bilirubin levels and SWS changes were reliable predictors of 2-year BA outcomes.

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BACKGROUND: Children with biliary atresia (BA) may experience various infections (e.g., cholangitis, bacteremia, and viral respiratory infections (VRI)) throughout their disease course. This study aimed to identify and describe these infections and their risk factors for development in children with BA. METHODS: This retrospective observational study identified infections in children with BA using predefined criteria, including VRI, bacteremia with and without central line (CL), bacterial peritonitis, positive stool pathogens, urinary tract infections, and cholangitis. Infections were identified until liver transplant, death or last follow-up with native liver. Infection-free survival was estimated by Kaplan-Meier analysis. Logistic regression was used to estimate odds of infection per clinical characteristics. Cluster analysis was performed to identify patterns of infection development. RESULTS: 48 of 65 (73.8%) children had ≥1 infection during their disease course (mean length of follow up: 40.2 months). Cholangitis (n = 30) and VRI (n = 21) were most common. Nearly half (45%) of all infections developed within 3-months of Kasai hepatoportoenterostomy. Kasai performed ≥45 days of life was associated with 3.5-fold increased risk of any infection (95% CI 1.2-11.4). Risk of VRI was inversely related to platelet count at 1-month post-Kasai (OR 0.5, 0.19-0.99). Cluster analysis of infectious patterns identified three unique cohorts of patients based on their infection history: no/few infections (n = 18), mostly cholangitis (n = 20) or mixed infections (n = 27). CONCLUSION: Variability of infection risk exists amongst children with BA. Age at Kasai and platelet count are risk factors for future infections, suggesting that patients with more severe disease are at greater risk. Cirrhosis associated immune deficiency may exist in chronic pediatric liver disease and should be the subject of future investigations in order to optimize outcomes.

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BACKGROUND: Controversies in management of biliary atresia (BA) after hepatoportoenterostomy (HPE) lead to variable treatment protocols. We implemented standardized medical management after HPE, customizing the use of antibiotics and corticosteroids based on patient-specific factors. METHODS: In this retrospective analysis, 20 consecutive infants underwent HPE for BA and were compared to a historical cohort. Analysis of successful biliary drainage 3 months after HPE (defined as serum total bilirubin <2 mg/dL) was the primary endpoint; survival with native liver at 2 years was the secondary endpoint. RESULTS: Sixteen of 20 (80%) infants had successful bile drainage, compared to 8 of 20 (40%) infants in the historical cohort (P = 0.0225). Sixteen of 20 patients in the new protocol have reached 2 years of age or required liver transplantation. Among the sixteen, 11 (68.8%) are alive with native livers versus 10 of 20 (50%) in the historical cohort (P = 0.0970). CONCLUSION: This preliminary report suggests the potential benefit of tailored use of postoperative antibiotics and corticosteroids in improving biliary drainage after HPE. LEVEL OF EVIDENCE: III.

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BACKGROUND: The quality of life of patients with Biliary Atresia (BA) have not been systematically examined. The goal of this meta-analysis is to determine patients' postoperative health-related Quality of life (HrQoL) with native or transplanted livers. METHODS: From 2000 to August 2021, a literature-based search for relevant cohorts was conducted using Pubmed/Medline, the Cochrane Library, and Embase. Original research on BA, Hepatoportoenterostomy (HPE), portoenterostomy, Kasai, Liver transplantation and HrQoL was included. Using RevMan, a forest plot analysis of HrQoL after surgical treatment after BA was calculated (version 5.4). Using MetaXL, a pooled prevalence for cholangitis, secondary liver transplantation, or related malformations was computed (version 5.3). RESULTS: Nine studies compared individuals with BA to an age-matched healthy control group. 4/9 (n = 352) of these studies found poorer scores for BA patients, while 5/9 (n = 81) found equivalent health status. Factors associated with HrQoL: older age at the time of the survey was linked to greater HrQoL; whereas females, higher total bilirubin and the amount of immunosuppressive medicines were associated with lower HrQoL in BA patients. CONCLUSION: The current study emphasises the critical need to improve the many parameters influencing HrQoL in BA patients, as well as the methods utilized to assess those factors. This includes immunosuppression, withdrawal from polydrug regimes and recognizing the differences in disease burden between males and females. TYPE OF STUDY: Systematic review. LEVEL OF EVIDENCE: Level III.

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(1) Background: Acute cholangitis during the first year after Kasai hepatoportoenterostomy (HPE) has a negative impact on patient and native liver survival. There are no consistent guidelines for the definition, treatment, and prophylaxis of cholangitis after HPE. The aim of this study was to develop definition, treatment, and prophylaxis guidelines to allow for expeditious management and for standardization in reporting. (2) Methods: the Delphi method, an extensive literature review, iterative rounds of surveys, and expert panel discussions were used to establish definition, treatment, and prophylaxis guidelines for cholangitis in the first year after HPE. (3) Results: Eight elements (pooled into two groups: clinical and laboratory/imaging) were identified to define cholangitis after HPE. The final proposed definitions for suspected and confirmed cholangitis are a combination of one element, respectively, two elements from each group; furthermore, the finding of a positive blood culture was added to the definition of confirmed cholangitis. The durations for prophylaxis and treatment of suspected and confirmed cholangitis were uniformly agreed upon by the experts. (4) Conclusions: for the first time, an international consensus was found for guidelines for definition, treatment, and prophylaxis for cholangitis during the first year after Kasai HPE. Applicability will need further prospective multicentered studies.

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Introduction: Surgical treatment of biliary atresia (BA) is still based on sequential strategy with Kasai hepatoportoenterostomy (KP) followed by liver transplantation (LT), in case of complicated secondary biliary cirrhosis. Concerns have been expressed regarding the risks of LT related to previous KP, suggesting primary LT as an exclusive treatment of BA. Methods: Single-center retrospective analysis including 393 pediatric patients who underwent LT for BA from 1993 to 2018, categorized into two groups: with (KP) or without (NoKP) previous KP. Pre-LT clinical condition was estimated considering age at LT, time on waiting list, pediatric end-stage liver disease score (PELD), and presence of portal vein hypoplasia. Post-LT outcome was evaluated considering patient and graft survival rates, and need for early reoperation due to abdominal or graft-related complications (<45 days after LT). Results: Two-hundred ninety-six patients (75.3%) were categorized in the KP group, and 97 (24.7%) in the NoKP group. Median age at LT was 1.14 years in the KP group and 0.85 years in the NoKP group (p < 0.0001). PELD score was significantly less severe in KP patients (p < 0.05). One-year patient survival rates were 96.9 and 96.8% in the KP and NoKP groups, respectively (p = 0.43), and the corresponding graft survival was 92.5 and 94.8% (p = 0.97). The need for early reoperation was more frequent in the KP group (29.8%) vs. NoKP group (12.4%, p = 0.01). The rate of bowel perforation was non-significantly higher in the KP group (8.1%) vs. NoKP group (3.1%, p = 0.11). Conclusions: The sequential strategy including KP and LT allowed performing LT in patients with significant older age and better clinical conditions, when compared to those transplanted without previous KP. Patient and graft survivals were not impacted by previous KP. Although previous KP was associated with an increased rate of post-LT surgical complications, bowel perforation and bleeding did not occur significantly more frequently. Such results support the current strategy based on sequential treatment.

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BACKGROUND: The role of microbiota in biliary atresia (BA) remains unclear. The aim of our study was to assess efficacy and safety of LGG treatment in children with BA after HPE with special focus on bacterial cholangitis (BCH) and quantitative assessment of the gut microbiota composition and metabolism. METHODS: We performed double-blind placebo controlled trial with patients randomized into treatment group who received LGG (n = 14) and placebo (n = 16). The gut microbiota and short-chain fatty acids (SCFA) were assessed at baseline and after 6 months of treatment. Clinical and laboratory parameters including episodes of bacterial cholangitis (BCH) were collected during the study period and after 2-year follow-up. Additionally, stool composition of BA patients was compared with healthy age-matched control group. RESULTS: There were lower concentration of SCFA in children with BA compared to control group and significant increase in the number of Enterococcus bacteria. After 6 months of treatment, neither laboratory parameters nor gut microbiota composition differed between LGG group and placebo. PP analysis results were similar to ITT analysis, no significant differences between study and control group. Overall, there were 11 (36%) patients who developed at least one episode of bacterial cholangitis; 3 (21%) in the LGG group compared to 8 (50%) placebo group (p = 0.14). Bacterial cultures were positive in 22% of cases and recurrence after the first episode was observed in 27% of patients. The level of total bilirubin decreased below 2 mg/dl after 6 months of the study in 6 (42.8%) patients in the LGG group and in 8 (50%) patients in the placebo group (p = 0.73). During 2-year follow-up 6 out of 14 patients (42.8%) in the LGG group and 11 out of 16 placebo patients (68.7%) underwent liver transplantation (p = 0.27). CONCLUSIONS: Patients with BA present with specific microbiota profiles and decreased SCFA what gives opportunities to implement novel therapeutic options based on modulation of  microbiota. Whether LGG is an effective therapy needs to be studied in a larger group with similar outcome parameters.

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Background and objectives: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management. This study aimed to analyze the evolution and survival of patients with BA referred for HPE (Kasai operation) in our department. Materials and Methods: A retrospective analysis was performed on fourteen patients with BA, diagnosed in the pediatric department and further referred for HPE in our surgical department between 2010 and 2016. After HPE, the need for transplantation was assessed according to patients cytomegalovirus (CMV) status, and histological and biochemical analysis. Follow-up results at 1-4 years and long term survival were assessed. Results: Mean age at surgery was 70 days. Surgery in patients younger than 60 days was correlated with survival. Jaundice's clearance rate at three months was 36%. Total and direct bilirubin values had a significant variation between patients with liver transplants and native liver (p = 0.02). CMV was positive in eight patients, half with transplant need and half with native liver survival. Smooth muscle actin (SMA) positivity was proof of advanced fibrosis. The overall survival rate was 79%, with 75% for native liver patients and an 83% survival rate for those with liver transplantation. Transplantation was performed in six patients (43%), with a mean of 10 months between HPE and transplantation. Transplanted patients had better survival. Complications were diagnosed in 63% of patients. The mean follow-up period was six years. Conclusions: HPE, even performed in advanced cirrhosis, allows a significant survival, and ensures an essential time gain for patients requiring liver transplantation. A younger age at surgery is correlated with a better outcome, despite early CMV infection.

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AIM OF THE STUDY: Biliary atresia (BA) is a fibro-inflammatory cholangiopathy of intra- and extrahepatic biliary radicles. The standard-of-care treatment is surgical restoration of bile flow by Kasai hepatoportoenterostomy (HPE). We aimed to identify the predictors of short-term outcome of the Kasai operation three months postoperatively. MATERIAL AND METHODS: This retrospective study included 107 infants diagnosed with BA by intraoperative cholangiography. All underwent a Kasai operation. The surgical outcome was classified after 3 months post-operatively as successful (bilirubin ≤ 2 mg/dl) or failed (bilirubin > 2 mg/dl). The two groups were compared according to basic clinical, ultrasonographic and histopathological characteristics. RESULTS: Of the studied patients 29 (27.1%) had a successful outcome while 78 (72.9%) had failed Kasai HPE. Of the preoperative characteristics, lower age and lower serum alkaline phosphatase (ALP) were significantly associated with successful surgical outcome (p = 0.009 and < 0.0001, respectively). In addition, surgical type of BA affected the short-term outcome (p = 0.017), while there was no statistically significant difference regarding the other studied parameters between groups. Age of 69.5 days or less was predictive of successful outcome with 74.4% specificity but with low sensitivity (58.6%), and ALP at a cutoff level of 532.5 U/l or less was predictive of successful outcome with 75.9% sensitivity and 74.4% specificity. CONCLUSIONS: Younger age at the time of surgery and lower ALP are good predictors for the short-term outcome of Kasai HPE with better performance of ALP. This may help to anticipate those who can benefit from surgical correction and those who should be given high priority for transplant referral.

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Biliary atresia is a progressive inflammatory sclerosing disease of the bile ducts in the neonatal liver. Without surgical intervention these patients are destined to succumb to the disease. The development of the hepatoportoenterostomy in 1959 and liver transplantation in 1963 ushered a new era of success treating these patients. While many surgical modifications and adjuncts to treatment have been attempted over the last 50 years, the mainstay of treatment to give the child the best chance at prolonged survival with the native liver is a properly performed operation, in a timely fashion, with minimal post-operative complications. This review presents the authors current practice guideline to achieve these goals.

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OBJECTIVE: To assess the diagnostic and prognostic usefulness of the serum matrix metallopeptidase-7 (MMP-7) level for biliary atresia in infants with cholestasis after hepatoportoenterostomy. STUDY DESIGN: We enrolled 100 infants with cholestasis (age, 43.56 ± 1.97 days; 62 males) with a direct bilirubin level of >1 mg/dL, of whom 36 (36%) were diagnosed with biliary atresisa. The MMP-7 levels in serum samples collected during the cholestasis workup and 6 months after hepatoportoenterostomy were assessed by enzyme-linked immunosorbent assay. We quantified liver fibrosis by Picro Sirius red staining of collagen in specimens from the 81 infants with cholestasis. RESULTS: Infants with biliary atresisa had a significantly higher serum MMP-7 level than that of non-biliary atresisa infants with cholestasis of equivalent age (P < .0001). Receiver operating characteristic analysis showed that a serum MMP-7 level of >1.43 ng/mL was predictive of biliary atresisa in infants with cholestasis (diagnostic accuracy, 88%). There was a positive correlation between the serum MMP-7 level and the severity of liver fibrosis (P = .0002). Survival analysis showed that the frequency of liver transplantation was significantly higher in infants with biliary atresisa with a serum MMP-7 level of >10.30 ng/mL compared with a serum MMP-7 level of ≤10.30 ng/mL after hepatoportoenterostomy (hazard ratio, 4.22; P = .02). CONCLUSIONS: The serum MMP-7 level, which reflects the severity of liver fibrosis and can be determined noninvasively, may facilitate the diagnosis of biliary atresisa among infants with cholestasis. Moreover, the serum MMP-7 level after hepatoportoenterostomy is associated with a need for liver transplantation in infants with biliary atresisa.

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Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.

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PURPOSE: The purpose of this study was to determine perioperative risk factors for need of liver transplantation following hepatoportoenterostomy. METHODS: A retrospective review of patients undergoing hepatoportoenterostomy for biliary atresia at our institution from 1990 to 2016 was completed. RESULTS: A total of 81 patients were identified with a median age of 51 days (IQR: 33-68) at hepatoportoenterostomy and a median follow-up time of 5.7 years (IQR: 1-11.6). Ten-year overall survival was 93% (95% CI: 84-97). Thirty-six patients (44%) ultimately required transplantation at a median time from hepatoportoenterostomy of 8.9 months (IQR: 5.2-19). The 10-year transplant-free survival was 36% (95%CI: 24-49). Steroid use (N=42) was not associated with improved 10-yr transplant-free survival (33% vs. 38%, p=0.690). Age at hepatoportoenterostomy was not significantly associated with the need for transplantation. Multivariable logistic regression analysis demonstrated that total bilirubin >2mg/dL (OR: 97, p<0.001) and albumin < 3.5g/dL (OR: 24, p=0.027) at 3 months after surgery were independent predictors of the need for transplantation, while adjusting for age, sex, prematurity, and steroid use. CONCLUSION: Overall survival for children with biliary atresia is excellent, although most patients will ultimately require liver transplantation. Total bilirubin and albumin level at 3 months following hepatoportoenterostomy are predictive of the need for transplantation. Steroid use is not associated with improved outcomes.

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Biliary atresia (BA) is a pathology that causes jaundice in children because of an obstruction of the obliterated biliary tract. Despite being considered a rare disease, the incidence can reach 1/2400 live births in the Pacific region. The etiology is unknown. If untreated, it will lead to cirrhosis and death. Early operation (within 60 days after birth) is the most important prognostic factor. The objective of this article is to present the technical description step by step of a hepatoportoenterostomy operation in a female 61 days of age with BA.

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BACKGROUND: Biliary atresia (BA) causes progressive fibrosis and obliteration of the biliary tree, resulting in bile stasis and eventual liver dysfunction. It affects 1 in 10,000-18,000 infants, and if left untreated, universally leads to liver failure. The Kasai hepatoportoenterostomy (KPE) was developed as an effective surgical therapy for BA and can restore bile drainage into the intestine. Traditionally, the KPE procedure extra-corporealizes the liver to expose the portal plate. Here, we describe modifications to the procedure via a smaller incision in which the liver remains within the abdominal cavity and we compare the outcomes of this technique to previous institutional outcomes and to contemporary international series. MATERIALS AND METHODS: We identified all patients who underwent KPE for BA at a single institution between 1994 and 2012. Patient outcomes after the modified KPE performed from 2004 to 2012 were compared to data from infants who underwent the traditional KPE from 1994 to 2003. RESULTS: Ninety-nine patients were identified. Fifty-two were in the traditional KPE group and 47 in the modified KPE group. There was no difference in mean age at surgery. Median follow-up was 64 months (traditional KPE) and 46 months (modified KPE). The rate of native liver survival (39.1% vs 48.5%), overall survival (89.2% vs 97.8%), liver transplant occurring under one year of age (36.5% vs 40.4%) and median time to liver transplant (188 vs 172 days) were not statistically different between groups (p > 0.05 for all comparisons). The results of the modified KPE compared favorably to published outcomes. CONCLUSION: The described modifications to the KPE appear to yield equivalent outcomes when compared to the traditional KPE procedure and compare well with published outcomes in the literature. It is possible that the procedure described here results in less scarring and technically easier liver transplant procedures. LEVEL OF EVIDENCE: Level III.

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Neonatal cholestasis (NC) is a diagnostic dilemma frequently countered in a neonatal care unit. Early diagnosis is vital for achieving an optimal patient outcome as many causes of cholestasis such as biliary atresia are time-sensitive and amenable to treatment if analyzed and treated early. Nonetheless, it is not generally simple to analyze these cases right on time as some of them are regularly missed due to the presence of pigmented stools, lack of newborn metabolic screening, and named as instances of prolonged jaundice. In this manner, we prescribe to explore all reasons for prolonged jaundice stretching out past 14 days in neonates. Besides, we suggest that stool card ought to be a piece of release rundown for all newborn children being released from the nursery. This is of most extreme significance in the nation like India where guaranteeing customary follow-up is as yet a tough assignment. These stool cards will help in the early determination of patients with NC particularly biliary atresia and guarantee their auspicious cure. Another reason which needs exceptional say is parenteral nutrition-associated liver illness, as the proportion of preterm babies is getting greater and greater with better neonatal care. These extreme preterm infants are in the requirement for prolonged (>14 days) total parenteral nourishment because of which they are at high hazard for NC contrasted with their more developed peers. In this survey, we will give an understanding of clinical approach, differential diagnosis, and clinical review of NC.

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OBJECTIVE: To assess factors associated with timing of hepatoportoenterostomy (HPE) and adverse perioperative outcomes in patients with biliary atresia in the US. STUDY DESIGN: We examined hospitalizations in infants aged <1 year using the National Inpatient Sample database for 2000-2011. We identified cases using the International Classification of Diseases, Ninth Revision, Clinical Modification codes for biliary atresia and HPE. Multivariable logistic regression models were used to examine association between select factors and age at HPE, as well as adverse perioperative outcomes. RESULTS: Our analysis of 1243 biliary atresia hospitalizations showed that only 37.7% of patients had HPE in the first 60 days of life. Patients who underwent HPE after 60 days of age were uninsured, were more likely to be black (aOR, 4.22; 95% CI, 1.49-11.95), less likely to be admitted at a teaching hospital (aOR, 0.27; 95% CI 0.10-0.79), and less likely to have a concomitant congenital malformation (aOR, 0.49; 95% CI 0.25-0.98). Patients with delayed age at HPE incurred significantly higher hospital costs ($57 914 vs $34 074; P = .026). Delayed age at HPE and weekend admission were independently associated with increased odds of adverse perioperative outcome (aOR, 1.09; 95% CI, 1.01-3.02 and 3.98; 95% CI, 1.67-9.46, respectively). CONCLUSION: Current outcomes in patients with biliary atresia in the United States are suboptimal and result in higher costs. The specific factors associated with delayed care are further evidence that universal health care and screening are needed for all infants, along with systematic referral of potential patients with biliary atresia to specialized health centers.

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Kawasaki's disease (KD) is a systemic vasculitis often seen with viral and bacterial infections. Cholangitis is a known complication in biliary atresia patients post Kasai Portoenterostomy (KP). However KD, in a biliary atresia patient post KP has not been previously reported. A 1 years old girl who had previously undergone a KP for BA, presented with cholangitis which was presumed to be caused by a previous enterobacter infection that she had 2 months ago. However, on treating the cholangitis, the patient developed fever again after ten days which persisted even after changing the antibiotics. By this time she also displayed three of five characteristic features of KD in form of fever, strawberry tongue and cervical adenopathy. Investigations showed high ESR, high CRP, thrombocythemia and dilated coronary vessels on echocardiography. Treatment with intravenous immunoglobulin (IVIG) and steroids caused the symptoms to subside.

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