Cases Of Biliary Atresia Research Articles

Study of related factors to vascular complications after pediatric liver transplantation

Objective: To explore the related factors of vascular complications after liver transplantation in children. Methods: This is a retrospective case-series research. The clinical data of 89 pediatric liver transplant patients admitted to the Organ Transplantation Center, the Affiliated Hospital of Qingdao University from January 2016 to March 2024 were collected retrospectively. This study included 44 males and 45 females,aged from 4 months to 17 years. The ratio of graft to recipient weight was 0.6% to 7.7%. The primary diseases included 48 cases of biliary atresia and 41 cases of non-biliary atresia. The t-test, Wilcoxon rank sum test, χ2 test, and Fisher's exact probability method were used for data analysis. Multivariate Logistic regression was used to analyze the related factors of vascular complications. Results: All 89 children with liver transplantation completed surgery successfully. There were 8 cases of arterial complications after surgery, including 6 cases of hepatic artery thrombosis and 2 cases of hepatic artery stenosis. There were 16 cases of portal vein complications after surgery, including 9 cases of portal vein stenosis and 7 cases of portal vein thrombosis. The results of univariate analysis showed that the age of the recipient ≤1 year was the relevant factor for hepatic arterial complications(χ2=4.772,P=0.029). The age of the recipient ≤1 year, the age of the donor, the hepatic phase, and the time of cold ischemia were the relevant factors for the occurrence of portal vein complications(χ2=7.270,Z=388.500,Z=838.000, Z=594.500;all P<0.05). The results of multivariate analysis showed that age(≤1 year vs. >1 year) and duration of cold ischemia(every additional 1 hour) were independent related factors for portal vein complications after liver transplantation in children(both P<0.05). Conclusion: Children aged ≤1 year and with prolonged cold ischemia are more likely to develop portal vein complications after liver transplantation.

The Outcomes and Success Rates of Kasai Operation for Biliary Atresia Cases Using Presurgical Hepatobiliary Scan: Observations at NINMAS

The Outcomes and Success Rates of Kasai Operation for Biliary Atresia Cases Using Presurgical Hepatobiliary Scan: Observations at NINMAS

Primary Healthcare Providers' Knowledge on the Early Detection of Biliary Atresia

Highlights:1. An evaluation is necessary to verify that the knowledge of healthcare providers is sufficient for the early detection of biliary atresia.2. It is crucial to promote education on prolonged jaundice to ensure early detection of biliary atresia in primary health facilities, as some primary healthcare providers have insufficient knowledge on the subject. AbstractPrimary healthcare providers frequently fail to recognize biliary atresia because it closely mimics physiological jaundice. Early detection plays an important role in ensuring the optimal treatment of biliary atresia. Delays in referring to biliary atresia cases remain a problem around the world. The objective of this study was to analyze the knowledge of primary healthcare providers regarding prolonged jaundice as an early sign of biliary atresia. A cross-sectional study was conducted at primary healthcare centers using a self-administered questionnaire. The data were analyzed descriptively with the assistance of IBM SPSS Statistics for Windows, version 21.0 (IBM Corp., Armonk, N.Y., USA). A total of 271 respondents participated in this study, including midwives (63.8%), nurses (24.4%), and medical doctors (8.5%). This study found that 73.8% of the respondents agreed that infants with prolonged jaundice need to undergo a serum bilirubin level assessment. However, 40.2% of the respondents believed that pale stools and dark urine in yellow infants were symptoms of physiological jaundice. Concerning the definition of prolonged jaundice, 209 (77.1%) respondents provided a response indicating more than two weeks. A total of 137 (50.6%) respondents stated that biliary atresia is a disease that must be ruled out first in cases of prolonged jaundice. Nevertheless, 86 (31.7%) and 87 (32.1%) respondents also mentioned that breastfeeding jaundice and breast milk jaundice must be considered as potential causes to be investigated. These results indicated that primary healthcare providers still had limited knowledge regarding cholestasis. Education on prolonged jaundice in primary healthcare centers should be more improved to enhance the early detection of biliary atresia.

Analysis of the serum bile acid profile to facilitate diagnosis and differential diagnosis of NA(+)-taurocholate cotransporting polypeptide deficiency

Objective: This study focuses on Na(+)-taurocholate cotransporting polypeptide (NTCP) deficiency to analyze and investigate the value of the serum bile acid profile for facilitating the diagnosis and differential diagnosis. Methods: Clinical data of 66 patients with cholestatic liver diseases (CLDs) diagnosed and treated in the Department of Pediatrics of the First Affiliated Hospital of Jinan University from early April 2015 to the end of December 2021 were collected, including 32 cases of NTCP deficiency (16 adults and 16 children), 16 cases of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), 8 cases of Alagille syndrome, and 10 cases of biliary atresia. At the same time, adult and pediatric healthy control groups (15 cases each) were established. The serum bile acid components of the study subjects were qualitatively and quantitatively analyzed by ultra-high performance liquid chromatography-tandem mass spectrometry. The data were plotted and compared using statistical SPSS 19.0 and GraphPad Prism 5.0 software. The clinical and bile acid profiles of children with NTCP deficiency and corresponding healthy controls, as well as differences between NTCP deficiency and other CLDs, were compared using statistical methods such as t-tests, Wilcoxon rank sum tests, and Kruskal-Wallis H tests. Results: Compared with the healthy control, the levels of total conjugated bile acids, total primary bile acids, total secondary bile acids, glycocholic acid, taurocholic acid, and glycochenodeoxycholic acid were increased in NTCP deficiency patients (P < 0.05). Compared with adults with NTCP deficiency, the levels of total conjugated bile acids and total primary bile acids were significantly increased in children with NTCP deficiency (P < 0.05). The serum levels of taurochenodeoxycholic acid, glycolithocholate, taurohyocholate, and tauro-α-muricholic acid were significantly increased in children with NTCP deficiency, but the bile acid levels such as glycodeoxycholic acid, glycolithocholate, and lithocholic acid were decreased (P < 0.05). The serum levels of secondary bile acids such as lithocholic acid, deoxycholic acid, and hyodeoxycholic acid were significantly higher in children with NTCP deficiency than those in other CLD groups such as NICCD, Alagille syndrome, and biliary atresia (P < 0.05). Total primary bile acids/total secondary bile acids, total conjugated bile acids/total unconjugated bile acids, taurocholic acid, serum taurodeoxycholic acid, and glycodeoxycholic acid effectively distinguished children with NTCP deficiency from other non-NTCP deficiency CLDs. Conclusion: This study confirms that serum bile acid profile analysis has an important reference value for facilitating the diagnosis and differential diagnosis of NTCP deficiency. Furthermore, it deepens the scientific understanding of the changing characteristics of serum bile acid profiles in patients with CLDs such as NTCP deficiency, provides a metabolomic basis for in-depth understanding of its pathogenesis, and provides clues and ideas for subsequent in-depth research.

Agenesis of gallbladder-series of four cases - why it is difficult to diagnose preoperatively?

Agenesis of gallbladder (AGB) is a rare clinical entity first reported by Bergman in 1702. The incidence is about in 10–65 cases/1,00,000 population. It is seen in 1/6th cases of biliary atresia. It is of unknown etiology and is believed to be due to abnormal embryonic development. Nearly 70% cases are sporadic. AGB may be associated with other congenital abnormalities including the biliary system. The majority (nearly 50%) of the patients are symptomatic with misinterpreted signs and symptoms of cholecystitis and lands up to unnecessary surgery. Our series of four cases have male: female ratio of 3:1. Two of the cases underwent laparoscopic surgery. Among the other two, one had biliary pancreatitis due to primary calculous in the common bile duct (CBD) with upstream dilation. Other cases had chronic pancreatitis with dilated pancreatic duct and a proximal calculous close to ampulla causing proximal dilation of CBD. Two operated cases were postoperatively diagnosed by magnetic resonance cholangiopancreatography (MRCP) and the non-operated patients were also diagnosed by MRCP. Symptomatic AGB is often wrongly interpreted as cholecystitis with cystic duct obstruction or as sclero-atrophic gallbladder, therefore leading to pointless surgical interventions. In case of false-positive ultrasonography (USG) of AGB, it has been suggested that either a loop of gas-containing bowel located in the gallbladder fossa or periportal tissue and subhepatic peritoneal folds impersonate a contracted gallbladder containing gallstones. When USG reveals such reports or in cases of non-visualization in other imaging modalities, the need of additional preoperative imaging investigation must be in the surgeon’s mind such as MRCP to avoid surgery which is not at all required.

Cost-effectiveness analysis of universal screening for biliary atresia in Japan.

To evaluate the cost-effectiveness of universal newborn screening using stool color card or direct bilirubin (DB) testing when comparing with no screening for biliary atresia in Japanese setting. A decision analytic Markov microsimulation model was developed to evaluate the universal screening for biliary atresia. Our screening strategies included stool color card, DB, or no screening. The outcomes of all newborns undergoing 3 strategies were simulated to analyze event-free life-years defined as liver transplant-free survival, costs, and incremental cost-effectiveness ratio (ICER) over a 25-year period with an annual discount rate of 2% applied for both costs and outcomes. A 1-way sensitivity analysis was performed to assess the uncertainty. There were 941 000 newborn infants in our cohort and 114 cases of biliary atresia. The base case analysis showed that the stool color card strategy was $14 927 337 higher than no screening with an increase in 44 more event-free life-years gained, resulting in an ICER of $339 258 per event-free life-year gained. The DB screening strategy compared with stool color card was $138 994 060 higher with an increase in 271 more event-free life-years gained and an ICER of $512 893 per event-free life-year gained. The DB screening strategy compared with no screening resulted in an ICER of $488 639 per event-free life-year gained. The DB screening resulted in 16 fewer liver transplants than stool color card and stool color card had 2 fewer liver transplants than no screening. Universal screening for biliary atresia could be cost-effective depending on the willingness to pay thresholds for health benefits.

Fractionated Bilirubin Among 252 892 Utah Newborns with and Without Biliary Atresia: A 15-year Historical Birth Cohort Study

To determine whether neonatal conjugated or direct bilirubin levels were elevated in infants with biliary atresia, and to estimate the number of newborns that would have positive screens in the nursery necessitating repeat testing following discharge. We used administrative data from a large integrated healthcare network in Utah to identify newborns who had a fractionated bilirubin recorded during birth admission from 2005 through 2019. Elevated conjugated bilirubin was defined as greater than 0.2 mg/dL and direct bilirubin was defined as greater than 0.5 mg/dL (>97.5th percentile for the assays). We performed simulations to estimate the anticipated number of false positive screens. There were 32 cases of biliary atresia and 468,161 live births during the study period (1/14,700). 252,892 newborns had fractionated bilirubin assessed including 26 of those subsequently confirmed to have biliary atresia. Conjugated or direct bilirubin was elevated in all 26 infants with biliary atresia and an additional 3,246 (1.3%) newborns without biliary atresia. Simulated data suggest nine to 21 per 1,000 screened newborns will have an elevated conjugated or direct bilirubin using laboratory-based thresholds for a positive screen. Screening characteristics improved with higher thresholds without increasing false negative tests. This study validates the previous findings that conjugated or direct bilirubin are elevated in the newborn period in patients with biliary atresia. A higher threshold for conjugated bilirubin improved screening performance. Future studies are warranted to determine the optimal screening test for biliary atresia and to assess the effectiveness and cost-effectiveness of implementing such a program.

Effect of Cytomegalovirus Infection on Initial Presentation and Overall Prognosis of Biliary Atresia Patients.

Biliary atresia is known to have a multifactorial etiology and perinatal infection with hepatotropic viruses such as cytomegalovirus (CMV) is a probable trigger in a subset of patients. The aim of the current study is to evaluate the effects of CMV association of biliary atresia on the initial presentation of patients and their response to Kasai portoenterostomy. We conducted a retrospective, single-center study on 20 patients of biliary atresia and classified them into two groups based on their CMV immunoglobulin M (IgM) positivity. We compared the age of initial presentation, the liver biochemistry at presentation, immediate and delayed follow-up, rate of jaundice clearance following Kasai portoenterostomy, and histopathology of liver between the two groups. Data were reported in terms of means, and P < 0.05 was considered significant. Out of 20 cases of biliary atresia, 60% (n = 12) were CMV IgM positive. Infants with CMV-positive status were noted to be older at presentation (88.5 days [65-150 days] vs. 83 days [45-160 days] P < 0.05) were more jaundiced at presentation (total bilirubin - 13.51 mg/dl [9.09-15.99 mg/dl] vs. 11.83 mg/dl [6.5-13.5 mg/dl] P < 0.05), had higher alkaline phosphatase (751.2 IU/L [387-1951 IU/L] vs. 621.75 IU/L [172-857 IU/L] P < 0.05), higher gamma-glutamyl transferase levels (505.58 IU/L [376-1127 IU/L] vs. 376.75 IU/L [186-624 IU/L] P < 0.05), and had higher incidence of splenomegaly. The rate of resolution of jaundice postKasai portoenterostomy was also evidently less in CMV-positive patients. Four out of 12 patients have bilirubin >2 mg/dl at a 6-month follow-up. CMV-associated biliary atresia patients have delayed initial presentation and impaired jaundice clearance postKasai portoenterostomy. The role of antiviral therapy should be studied in this subset of patients.

Comparison of stapler and hand-sewn roux en Y jejunal anastomosis in children

outcomes of stapled and hand-sewn Roux-en-Y intestinal anastomoses in childhood.&#x0D; Materials and Methods: At a university hospital, the records of the children who underwent roux-en-Y anastomosis between December 2007 and December 2014 were reviewed. The data were compared according to the roux-en-Y anastomosis technique used (stapled versus hand-sewn).&#x0D; Results: A total of 52 patients had undergone roux-en-Y anastomosis. All had biliary atresia or choledochal cyst. Thirty-one of the patients were diagnosed with biliary atresia and 21 with choledochal cysts. Staple anastomosis technique was used in 16 of the patients with biliary atresia and 9 of the patients with choledochal cyst. Both in biliary atresia and choledochal cyst cases; operations with stapled anastomosis were significantly shorter than the ones with hand-sewn anastomosis. Among the biliary atresia cases, post-operative oral feeding was initiated significantly earlier in the stapled group, with its lower risks of post-operative cholangitis and longer hospital stay than 7 days.&#x0D; Conclusion: This is the first study in children, confirming the time-saving advantage of stapled anastomosis over hand-sewn, during roux-en-Y anastomosis for biliary atresia and choledochal cyst; along with the safety of stapler use, including the neonates. Stapled anastomosis yields lower complication rates, faster function gain with earlier feeding and hospital discharge.

Treatments and outcomes of intra-operative portal vein thrombosis in living-donor liver transplantation due to biliary atresia

Pediatric living-donor liver transplantation (LDLT) has become one of the most effective therapies for pediatric end-stage liver diseases. We aim to investigate the risk factors for intra-operative portal vein thrombosis (PVT) and the short- and long-term outcomes in children post LDLT. This was a retrospective analysis from 584 cases of biliary atresia (BA) patients who had undergone LDLT from January 2014 to December 2019 at our hospital. Patients were divided into PVT and non-PVT groups according to the occurrence of PVT during LDLT. The median age of recipients at transplantation was 7.22 (quartiles, 6.03, 9.50) months, the incidence of intra-operative PVT was 5.31% (31/584). The independent risk factors for intra-operative PVT were the diameter of the recipient's PV not greater than 4mm and a higher ratio of graft-to-recipient PV diameter. The cumulative survival rates of grafts and recipients were 93.5% and 93.5% in the PVT group, and 94.9% and 95.3% in the non-PVT group, respectively, without significant difference. The recovery of graft function was similar in recipients with or without interposed graft vessel (IGV). However, the incidence of PV stenosis was higher in recipients with IGV after LDLT. Intra-operative PVT is a common complication in pediatric LDLT, but an excellent prognosis can be achieved by appropriate and individualized surgical treatment. We noted that intra-operative PVT did not affect the survival rates of grafts and recipients, but there was a higher incidence of PV complications after LDLT. Ⅲ.

The Epidemiology of Biliary Atresia: Exploring the Role of Developmental Factors on Birth Prevalence

To identify key epidemiologic factors relevant to fetal development that are associated with biliary atresia. This population-based registry study examined infants born in Texas between 1999 and 2014. Epidemiologic data relevant to fetal development were compared between cases of biliary atresia identified in the Texas Birth Defects Registry (n=305) vs all live births (n=4 689 920), and Poisson regression was used to calculate prevalence ratios (PRs) and 95% CIs. The prevalence of biliary atresia over the study period was 0.65 per 10 000 live births. Biliary atresia waspositively associated with female sex (adjusted PR, 1.68; 95% CI, 1.33-2.12), delivery before 32-37weeks of gestation (adjusted PR, 1.64; 95% CI, 1.18-2.29), delivery before 32weeks of gestation (adjusted PR, 3.85; 95% CI, 2.38-6.22), and non-Hispanic Black vs non-Hispanic White maternal race/ethnicity (adjusted PR, 1.54, 95% CI, 1.06-2.24), while biliary atresia was inversely associated with season of conception in the fall relative to spring (adjusted PR, 0.62; 95% CI, 0.45-0.86). In addition, biliary atresia was associated with maternal diabetes (adjusted PR, 2.34; 95% CI, 1.57-3.48), with a stronger association with pregestational diabetes compared with gestational diabetes. In subgroup analyses, these associations were present in isolated biliary atresia cases that do not have any additional birth defects. Biliary atresia is associated with multiple factors related to fetal development, including pregestational maternal diabetes, female sex, and preterm birth. These associations also were observed in isolated cases of biliary atresia without other malformations or laterality defects. Our results are consistent with early life events influencing the pathogenesis of biliary atresia, and support further studies investigating in utero events to better understand etiology and time of onset.

Adult Living Donor Liver Transplantation for the Carryover Patients After Kasai Operation Who Have a History of Multiple Laparotomies

Approximately 50 years have passed since the Kasai operation announcement for biliary atresia. In adult liver failure cases, the so-called "the carryover cases after Kasai operation" have increased. These patients often underwent polysurgery. In such cases, adult living-donor liver transplantation (LDLT) is occasionally difficult. Many complications have been reported to be caused by severe cholangitis, hepatic portal regional inflammation, and adhesion. We investigated the complications of adult LDLT in post-Kasai biliary atresia cases with polysurgery. Between 1991 and 2021, we performed 205 LDLT cases. Weinvestigated the outcome of adult LDLT for post-Kasai biliary atresia cases (transplanted over 16 years old) (n=20) and the risk factors for complications after LDLT. On 5 years overall survival, there were no significant differences between "adult LDLT for post-Kasai" group and the others (81.8% vs 81.2%).Adult LDLT for post-Kasai was not found to be a risk factor for complications. However, polysurgery before LDLT was an independent risk factor for biliary stenosis and portal stenosis, as identified in our univariate and multivariate analysis. We analyzed the relationship between biliary stenosis and the frequency of laparotomies using a receiver operating characteristic curve. The analysis showed that the cutoff point (maximum point of sensitivity plus specificity) was more than 3 times that of laparotomies before LDLT. In our study, adult LDLT for post-Kasai cases was not a risk factor for any complications. However, polysurgery before LDLT has been identified as a risk factor for biliary stenosis and portal vein stenosis.

Ultrasound shear wave elastography: does it add value to gray-scale ultrasound imaging in differentiating biliary atresia from other causes of neonatal jaundice?

Neonatal/infantile jaundice is relatively common, and most cases resolve spontaneously. However, in the setting of unresolved neonatal cholestasis, a prompt and accurate assessment for biliary atresia is vital to prevent poor outcomes. To determine whether shear wave elastography (SWE) alone or combined with gray-scale imaging improves the diagnostic performance of US in discriminating biliary atresia from other causes of neonatal jaundice over that of gray-scale imaging alone. Infants referred for cholestatic jaundice were assessed with SWE and gray-scale US. On gray-scale US, two radiology readers assessed liver heterogeneity, presence of the triangular cord sign, hepatic artery size, presence/absence of common bile duct and gallbladder, and gallbladder shape; associated interobserver correlation coefficients (ICC) were calculated. SWE speeds were performed on a Siemens S3000 using 6C2 and 9L4 transducers with both point and two-dimensional (2-D) SWE US. Both univariable and multivariable analyses were performed, as were receiver operating characteristic curves (ROC) and statistical significance tests (chi-squared, analysis of variance, t-test and Wilcoxon rank sum) when appropriate. There were 212 infants with biliary atresia and 106 without biliary atresia. The median shear wave speed (SWS) for biliary atresia cases was significantly higher (P<0.001) than for non-biliary-atresia cases for all acquisition modes. For reference, the median L9 point SWS was 2.1m/s (interquartile range [IQR] 1.7-2.4m/s) in infants with biliary atresia and 1.5m/s (IQR 1.3-1.9m/s) in infants without biliary atresia (P<0.001). All gray-scale US findings were significantly different between biliary-atresia and non-biliary-atresia cohorts (P<0.001), intraclass correlation coefficient (ICC) range 0.7-1.0. Triangular cord sign was most predictive of biliary atresia independent of other gray-scale findings or SWS - 96% specific and 88% sensitive. Multistep univariable/multivariable analysis of both gray-scale findings and SWE resulted in three groups being predictive of biliary atresia likelihood. Abnormal common bile duct/gallbladder and enlarged hepatic artery were highly predictive of biliary atresia independent of SWS (100% for girls and 95-100% for boys). Presence of both the common bile duct and the gallbladder along with a normal hepatic artery usually excluded biliary atresia independent of SWS. Other gray-scale combinations were equivocal, and including SWE improved discrimination between biliary-atresia and non-biliary-atresia cases. Shear wave elastography independent of gray-scale US significantly differentiated biliary-atresia from non-biliary-atresia cases. However, gray-scale findings were more predictive of biliary atresia than elastography. SWE was useful for differentiating biliary-atresia from non-biliary-atresia cases in the setting of equivocal gray-scale findings.

A Research Study on the Current Status of Biliary Atresia Cases in Adults and Status of Public Subsidies

A Research Study on the Current Status of Biliary Atresia Cases in Adults and Status of Public Subsidies

Gastrointestinal perforations and associated risk factors in children after liver transplantation.

In this study, possible risk factors of gastrointestinal perforations (GIP) that increase mortality after liver transplantation in children were investigated. One hundred and thirty-one pediatric patients who underwent 139 liver transplants between January 2016 and February 2020 were evaluated retrospectively based on preoperative and surgical data. Furthermore, cases with biliary atresia, which constitute 26.7% (35) of the patients, were compared within themselves and with other groups. It was found that the cases that developed perforations were younger, lower in weight, and had higher number of surgeries than those who did not, while the mortality and morbidity rates were higher in these patients. When cases with biliary atresia were analyzed within themselves, no significant difference was found between perforated biliary atresia and non-perforated cases in terms of age, weight, and previous surgery. When biliary atresia and other etiologies were compared, biliary atresia cases were found to be transplanted at a younger age, at a lower weight, and this group had a higher risk for perforation. Early laparotomy should be performed in order to reduce mortality in GIPs. Patients that are younger, underweight, previously operated, and using mesh must be closely monitored.

Value of the International Classification of Diseases code for identifying children with biliary atresia.

Background Although identifying cases in large administrative databases may aid future research studies, previous reports demonstrated that the use of the International Classification of Diseases, Tenth Revision (ICD-10) code alone for diagnosis leads to disease misclassification.Purpose We aimed to assess the value of the ICD-10 diagnostic code for identifying potential children with biliary atresia.Methods Patients aged <18 years assigned the ICD-10 code of biliary atresia (Q44.2) between January 1996 and December 2016 at a quaternary care teaching hospital were identified. We also reviewed patients with other diagnoses of code-defined cirrhosis to identify more potential cases of biliary atresia. A proposed diagnostic algorithm was used to define ICD-10 code accuracy, sensitivity, and specificity.Results We reviewed the medical records of 155 patients with ICD-10 code Q44.2 and 69 patients with other codes for biliary cirrhosis (K74.4, K74.5, K74.6). The accuracy for identifying definite/probable/possible biliary atresia cases was 80%, while the sensitivity was 88% (95% confidence interval [CI], 82%–93%). Three independent predictors were associated with algorithm-defined definite/probable/possible cases of biliary atresia: ICD-10 code Q44.2 (odds ratio [OR], 2.90; 95% CI, 1.09–7.71), history of pale stool (OR, 2.78; 95% CI, 1.18–6.60), and a presumed diagnosis of biliary atresia prior to referral to our hospital (OR, 17.49; 95% CI, 7.01–43.64). A significant interaction was noted between ICD-10 code Q44.2 and a history of pale stool (P<0.05). The area under the curve was 0.87 (95% CI, 0.84–0.89).Conclusion ICD-10 code Q44.2 has an acceptable value for diagnosing biliary atresia. Incorporating clinical data improves the case identification. The use of this proposed diagnostic algorithm to examine data from administrative databases may facilitate appropriate health care allocation and aid future research investigations.

Living donor liver transplantation for more than 30-year survived patients with native liver after Kasai operation for biliary atresia

ObjectivesA number of patients have developed liver failure after the Kasai operation for biliary atresia (BA), even after long-term postoperative course. We report our experience regarding four cases of biliary atresia required and treated with living donor liver transplantation (LDLT) during adulthood. MethodsAll four patients underwent Kasai operation for BA from 1974 to 1983 and had been followed-up for more than 30 years in our institute. Then, they developed liver failure and treated with LDLT. These four patients’ records were reviewed retrospectively. All data are described using the median value. ResultsThree female and one male were included. Kasai operation was performed at the age of 61 days. The type of biliary obstruction was type III-b1-ν in all. The survival period of the native liver was 440 months. The age at LDLT was 37 years. The reasons of deterioration of native liver were recurrent cholangitis in two and pregnancy and delivery in other two. The age of living donor was 40 years. Right liver grafts were used in all. Percentage of the real graft volume per standard liver volume was 47%. All patients were alive and overall graft survival rate was 100% at a follow-up period of 65 months after LDLT. ConclusionEven after more than 30 year-survival with native liver after Kasai operation, liver failure may be caused by recurrent cholangitis or pregnancy and delivery. LDLT may be effective treatment for those adult patients if an adequate living donor is available.

Intrahepatic cystic lesions in children with biliary atresia after Kasai procedure

BackgroundIntrahepatic cystic lesion (ICL) is a common complication for biliary atresia post-Kasai portoenterostomy. The purpose of this study was to review the cases in our hospital and assess the correlation between characteristics of ICL and clinical outcomes. MethodsWe retrospectively analyzed 787 cases of biliary atresia from 2012 to 2016. Demographics, clinical details, and postoperative outcomes were reviewed. ResultsA total of 76 patients were diagnosed with ICLs using ultrasound post-Kasai procedure, and the incidence was 9.7%. Preoperative characteristics showed no significant differences between ICL (+) and ICL (−) groups. Nearly 70% (53/76) of the patients with ICLs had a history of cholangitis. The 2-year native liver survival rate was 60.4% for those with a history of cholangitis and 87% for those without (P = 0.017). Further analysis showed that the 2-year native liver survival rate was 42.9% for those diagnosed within 3 months post-Kasai procedure, 54.2% for those diagnosed between 3 and 6 months, and 80.0% for those diagnosed beyond 6 months (P = 0.002), while no significance was observed for type (P = 0.094) or site (P = 0.406) of ICL. ConclusionPatients with ICLs had a high incidence of cholangitis. The prognosis was closely related with the history of cholangitis and the onset time of ICLs. Level of evidenceLevel II.

Nationwide population-based study showed that the rotavirus vaccination had no impact on the incidence of biliary atresia in Korea.

Rotaviruses have been associated with biliary atresia. This study investigated whether the rotavirus vaccine, which was introduced to Korea in 2008, had an impact on the incidence of biliary atresia. We identified all rotavirus infections (n=436826) and biliary atresia cases (n=528) diagnosed from 2006 to 2015 from insurance and health databases. The annual and seasonal incidence of biliary atresia and rotavirus infection rates in neonates and children were calculated. The difference in the risk of biliary atresia between rotavirus-infected and non-infected neonates was analysed. The incidence of rotavirus infections was 20.6 versus 13.4 per 1000 cases before (2006-2008) and after (2009-2015) vaccine implementation. However, neonatal rotavirus infection rates did not decrease, with an incidence of 14.5 versus 14.8 per 1000 cases before and after vaccination. The biliary atresia incidence remained constant at 12.0 per 100000 cases. Rotavirus infections in neonates were a risk factor for biliary atresia (odds ratio 3.14, 95% confidence interval 1.87-5.26). Rotavirus vaccination had no impact on the incidence of biliary atresia, possibly because the vaccination did not change the neonatal rotavirus infection rate through herd immunity. However, rotavirus infections in neonates were significantly associated with biliary atresia.

Clinical study and management of surgical problem of hepatobiliary and pancreatic disease in children

Background: Conditions which can be treated by surgery like biliary atresia etc., can lead to infantile jaundice. For better outcome it is important that the diagnosis be made quickly and precisely. There is danger of irreversible damage to the liver if prompt action is not taken. Here comes the role of diagnostic imaging which can help for prompt and precise diagnosis. This helps to act rapidly for the surgeon. The objective was to study the surgical problem of hepatobiliary and pancreatic disease in children.Methods: Hospital-based follow up study was carried out among 10 pediatric patients with surgical problems hepatobiliary and pancreatic diseases. After admission detailed history of all the cases were taken according to the proforma approved by the guide information regarding the age at presentation to hospital, gestational age, birth weight, age at onset of jaundice, nature of symptoms, duration of symptoms was obtained.Results: Out of the total cases, 50% constituted the biliary atresia cases followed by choledochal cyst in 30% of the cases. Chronic pancreatitis constituted 20% of the cases. In 20% of the patients the birth weight was less than two kg. All 5 patients had icterus. 3 patients had icterus. Ultrasound showed that the most common finding was hepatomegaly in 60% of the cases. All 5 patients had good uptake of radiotracer and excretion of radiotracer in intestine was poor in all 5 patients. The mean value of total bilirubin is 13.9.Conclusions: Early and rapid intervention in biliary atresia cases reduces morbidity and mortality.