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Primary 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 health facilities using a self-administered questionnaire. The data were analyzed using the descriptive method 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%). It was 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 a duration of 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 that need to be ruled out, respectively. These results indicated that primary healthcare providers still had limited knowledge regarding cholestasis. Education on prolonged jaundice in primary health facilities requires improvement to enhance the early detection of biliary atresia. [ABSTRACT FROM AUTHOR]

Progressive familial intrahepatic cholestasis type 4 (PFIC4) is a relatively newly described autosomal recessive disorder caused by biallelic mutations in the gene encoding tight junction protein 2 (TJP2) which is located in chromosome 9q21. PFIC4 is characterised by cholestasis with or without other extrahepatic manifestations. Bleeding tendency due to vitamin k deficiency is a well-known complication of cholestasis. We present a neonate who presented to the Emergency Department at a tertiary care hospital in 2021 with cholestasis and multiple intracranial bleeds. He was found to have severe coagulopathy and his genetic work up revealed a homozygous variant mutation in TJP2 gene causing PFIC4. He had persistent cholestasis that necessitated an internal biliary diversion with some clinical improvement. [ABSTRACT FROM AUTHOR]

Alagille syndrome (AS) commonly presents with cholestasis, much like other liver diseases, making the diagnosis challenging. We report a case of a patient with AS mimicking biliary atresia (BA) with a poor outcome. The infant, a product of a non-consanguineous marriage, presented with jaundice, clay stools, peripheral pulmonary stenosis, atrial septal defect, and butterfly vertebrae. Cholescintigraphy showed an absence of radiotracer excretion. Surgical exploration revealed the presence of a hypoplastic hepatic duct but a normal gallbladder, cystic, and common bile ducts. Intraoperative cholangiogram favored BA, and a Kasai procedure was performed. The liver biopsy demonstrated focal areas of ductular proliferation and periportal ballooning degeneration without bile duct paucity. The patient exhibited worsening cardiac and liver conditions, growth failure, and developmental delay. She died suddenly at home at the age of 34 months. The cholangiographic and histological abnormalities found in our patient were suggestive of BA. At the same time, she displayed four out of five diagnostic criteria for AS. Based on our experience with this case, we suggest expeditious genetic testing should be considered for any case of neonatal cholestasis with diagnostic uncertainty. This may help avoid unwarranted surgical interventions, potentially associated with worse outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Biliary atresia is a rare disease characterized by an obstructive cholangiopathy of intrahepatic and/or extrahepatic bile ducts. Etiology is unknown. The reported two-year survival rate without any intervention is less than 5%. Early intervention is an important predictive factor for successful patient outcome. Aims: The goal of the present study is to examine the clinical use of serum gamma glutamyl transferase (GGT) for early diagnosis and prognostication in biliary atresia following Kasai Hepatoportoenterostomy (KHPE). Materials and methods: This is a retrospective study which was conducted at a large tertiary care pediatric referral center. A total of eighty cases were reviewed from March 2020 to April 2022. The GGT levels were obtained pre-operatively. Baseline parameters like age at surgery, outcome and prognosis were evaluated. Results: 80 infants underwent Kasai Hepatoportoenterostomy. The age of the infants ranged from 31days to 116 days (median age of 62 days). Out of all the cases, 60% (48/80) had GGT value of <400 IU/L, 40% (32/80) had GGT value of >400IU/L. Low GGT(<400IU/L) group had shorter time to liver transplant and a poorer outcome. Conclusions: A low GGT level in biliary atresia might be associated with early onset of severe liver illness, and poor postoperative course after Kasai Hepatoportoenterostomy. Recent advances in non-invasive test like GGT has shown promise as an effective diagnostic and prognostic marker in biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation. [ABSTRACT FROM AUTHOR]

Background: About 3% of all pregnancies are complicated by one of the many types of hepatic diseases. First, there are a variety of liver problems specific to pregnancy that affect people with previously healthy livers. These include intrahepatic cholestasis of pregnancy, which affects 60% of cases, acute fatty liver of pregnancy, and liver dysfunction linked to hyperemesis gravidum, which affects 50% of cases, and preeclampsia, which affects 12% of cases. Objectives: 1. To determine the maternal and fetal outcome in jaundice complicating pregnancy 2. Find out the measures to reduce maternal& fetal mortality & morbidity. 3. To analyse various maternal and fetal complications which can be prevented. Material & Methods: Study Design: Hospital based prospective observational study. Study area:Department of Obstetrics & Gynecology, Mayo institute of Medical Sciences, Barabanki, Uttar Pradesh. Study Period:April 2022 toMarch 2023. Study population:All patients presenting with jaundice to Department of Obstetrics & Gynecologyin antepartum or intrapartum period. Sample size: Study consisted a total of 55 subjects. Sampling Technique: Simple Random technique. Results: In 29% (n=16) of cases, jaundice is due to obstetric cholestasis, who are mostly presented with pruritis and abnormal LFT's in their third trimester, In 27% (n=15)it is due to HELLP syndrome which is associated with preeclampsia in 14%, In 12.7%(n=7) cases jaundice is due to sickle cell anemia and Hepatitis B each, haemolytic anemia due to blood transfusion is seen in 5.45% (3), in 3.6%(n=2) cases cause is AFLP who presented with hepatic encephalopathy and hypoglycemia, cirrhosis is the cause of jaundice in 3.6%(n=2) who presented in their first and second trimester,1.8%(n=1) cases are due to malaria, cholelithiasis and autoimmune hepatitis each. So in nearly half of the cases the cause of jaundice is pregnancy specific causes. Conclusion:Jaundice in pregnancy is a dreadful condition resulting in high maternal and fetal mortality. Even though the blood bank facilities has increased, due to their advanced disease state at admission few patients could not be survived. So early referrals are helpful. Team effort is needed in the management of jaundice, team should constitute obstetrician, physician, gastroenterologist, anaesthetist and neonatologist, so that identification and treatment throughout antepartum, intrapartum and postpartum period prevent and reduce maternal and perinatal morbidity and mortality. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background and Aim: 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. Patients and Methods: 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. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to evaluate the efficacy of adding a spur valve to laparoscopic portoenterostomy for patients with biliary atresia. Methods: We retrospectively reviewed the records of all patients diagnosed with biliary atresia (BA) who underwent laparoscopic portoenterostomy (Lap-PE) between 2013 and 2021. The patients were divided into two groups: Lap-PE with a spur valve (spur group) and without it (control group). Perioperative management was the same in both groups. We compared patient backgrounds and clinical outcomes, including jaundice clearance and the number of postoperative cholangitis episodes. Results: Of 63 patients reviewed, 16 received a spur valve. There were no statistically significant differences in the patient backgrounds between the groups. All patients in the spur group achieved jaundice clearance. The number of postoperative cholangitis episodes one year after surgery was significantly lower in the spur group than in the control group (1 [0–3] vs. 3 [0–9], p = 0.04). The jaundice-free survival rate with the native liver at one year after surgery was significantly higher in the spur group (100% vs. 53%, p = 0.01). Conclusions: Adding a spur valve during Lap-PE significantly lowered the number of cholangitis episodes 1 year after surgery. [ABSTRACT FROM AUTHOR]

Background: Postoperative outcomes of portoenterostomy (PE) and redo-PE were evaluated using selected biochemical markers (SBM) and biochemical status categories (BSC). Methods: Subjects were 70 consecutive PE performed for biliary atresia. SBM were aspartate aminotransferase (AST)/alanine aminotransferase (ALT), cholinesterase (ChE), and platelet count (PLT) assessed at 1, 2, 3, 6, and 12 months, and thence, annually for a maximum of 10 years. BSC were as follows: all SBM normal (N-SBM), normal AST/ALT (N-SLT), normal ChE (N-ChE), normal PC (N-PLT), all abnormal (A-SBM), abnormal AST/ALT (A-SLT), abnormal ChE (A-ChE), and abnormal PC (A-PLT). Subjects achieving jaundice clearance (JC) and surviving with native livers (SNL) also had gamma glutamyl transpeptidase assessed. Redo-PE indicated for failed PE was assessed postoperatively using the same SBM/BSC protocol. Results: PE were laparoscopic (LPE; n = 40) or open (OPE; n = 30). Mean age/weight at PE and duration of follow-up were similar. For JC, LPE = 34/40 (85.0%) and OPE = 22/30 (73.3%); P = .23. For SNL, LPE = 29/40 (72.5%) and OPE = 16/30 (53.3%); P = .10. LPE and OPE were similar for SBM/BSC, except for a single significant increase in ALT in OPE at 6 months. Redo-PE was performed 17–180 days (mean 67.1 days) after primary PE. AST was significantly increased at the last preredo assessment 3 months after primary PE; P < .05. After redo, AST decreased and SBM/BSC results were equivalent to nonredo subjects. Conclusion: Postoperative biochemical data for all PE cases were comparable; redo-PE would appear to be viable for restoring SBM, and AST could be valuable as a single marker of deterioration in redo cases. [ABSTRACT FROM AUTHOR]

Background: It is impossible to predict which patients with biliary atresia (BA) will fail after Kasai portoenterostomy (KPE). We evaluated the predictive nature of pre-KPE clinical and histological factors on transplant-free survival (TFS) and jaundice clearance. Methods: A retrospective review of patients who received a KPE at our institution (1997–2018) was performed. Primary outcomes were two-year TFS, five-year TFS, and jaundice clearance 3 months after KPE. p < 0.05 was considered significant. Results: Fifty-four patients were included in this study. The two-year TFS was 35.1%, five-year TFS was 24.5%, and 37% patients reached a direct bilirubin (DB) ≤ 2.0 mg/dL 3 months post KPE. The median age at biopsy was younger in the five-year TFS (39.0 (24.5–55.5) vs. 56.0 days (51.0–67.0), p = 0.011). Patients with DB ≤ 1.0 mg/dL 3 months after KPE were statistically younger at biopsy (DB ≤ 1.0 44.0 (26.0–56.0) vs. DB > 1.0 56.0 days (51.0–69.0), p = 0.016). Ductal plate malformation was less frequent in the five-year TFS (16/17, 94.1%, vs. 1/17, 5.9%, p = 0.037). Portal fibrosis (19/23, 82.6%, vs. 4/23, 17.4%, p = 0.028) and acute cholangitis (6/7, 85.7%, vs. 1/7, 14.3%, p = 0.047) occurred less frequently in two-year TFS. Conclusion: Older age at biopsy, acute cholangitis, portal fibrosis, and ductal plate malformation were associated with lower native liver survival. Evaluation in a larger study population is needed to validate these results. [ABSTRACT FROM AUTHOR]

Biliary atresia is one of the commonest causes of prolonged jaundice in neonate, whereas cystic biliary atresia is a rare form of biliary atresia with similar presentation, but a slightly different sonographic finding. Being able to differentiate them radiologically is important as the surgical management is different for these two diagnoses. An ultrasound examination was done for a 2-month-old baby girl with prolonged jaundice with the finding of a cyst along the portal triad, which raised the suspicion for cystic biliary atresia. The diagnosis was confirmed with intraoperative cholangiography (IOC) and Kasai procedure was done. The patient was well after the surgery with resolution of jaundice. [ABSTRACT FROM AUTHOR]

Purpose: Open portoenterostomy (PE) for biliary atresia (BA) is currently more extended (EP) than the original (OP). Typical OP techniques, shallow transection of the biliary remnant and shallow suturing, both lost in EP, were revived as a modified procedure (MP). Postoperative outcomes of EP and MP were compared. Methods: Subjects were 55 consecutive BA patients treated by EP (n = 18) or MP (n = 37) at a single center between 2004 and 2021. Results: Mean follow-up duration was: MP: 15.5 years (range 0.1–12.3 years) and EP: 15.5 years (range 0.38–17.1 years). The ratio of jaundice free (JF; total bilirubin ≤ 1.2 mg/dL) subjects was significantly higher in MP (78.4%) versus EP (50%); p > 0.05, the incidence of bile lakes at the porta hepatis was significantly higher in MP (7/37: 18.9%) versus EP (0/18: 0%); p > 0.05, and Kaplan–Meier analysis showed JF survival with the native liver (JF + SNL) was significantly better in MP (26/37: 70.2%) versus EP (4/18: 22.2%); p > 0.05. All other criteria were similar. Of note, time taken to become JF and the incidence of cholangitis were not significantly different. Conclusions: Shallow transection and shallow suturing would appear to influence postoperative outcome. The etiology of bile lake formation in MP requires urgent confirmation. [ABSTRACT FROM AUTHOR]

To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases. [ABSTRACT FROM AUTHOR]

• Biliary atresia (BA) is a severe obliterative fibrosing cholangiopathy in children. • No studies have investigated circulating IL-34 and liver stiffness in BA children. • Elevated circulating IL-34 levels were positively correlated with severity in BA. • Higher serum IL-34 was significantly associated with decreased survival rate in BA. • Circulating IL-34 could serve as a noninvasive biomarker reflecting severity in BA. Biliary atresia (BA) is a severe congenital disorder with progressive obstructive cholangiopathy in young children. The inflammatory process has been recognized as one of the pathological mechanisms driving bile duct injury. Since interleukin-34 (IL-34) has been reportedly linked to several pathological liver disorders, including inflammation, the current study aimed to analyze circulating IL-34 and the association of circulating IL-34 with hepatic deterioration and clinical outcomes in post-Kasai BA children. Circulating IL-34 levels were analyzed in 89 post-Kasai BA subjects and 45 healthy individuals using an ELISA. Liver stiffness (hardness) was measured by ultrasound elastography. Circulating IL-34 was substantially higher in BA children than in control individuals, particularly those with unfavorable outcomes including hepatic dysfunction, jaundice, and portal hypertension. In BA group, circulating IL-34 was positively correlated with liver stiffness (r = 0.515, p < 0.001), AST (r = 0.403, p < 0.001), ALT (r = 0.279, p = 0.008), total bilirubin (r = 0.224, p = 0.03), ALP (r = 0.255, p = 0.016), and serum IL-6 (r = 0.590, p < 0.001) but inversely correlated with albumin (r = -0.417, p < 0.001). Kaplan-Meier survival analysis showed that higher circulating IL-34 levels were significantly associated with reduced survival rates in BA subjects (p = 0.002). Higher circulating IL-34 values were directly associated with hepatic impairment and the BA severity, implicating that serum IL-34 could be applied as a noninvasive marker for the monitoring of the severity in BA subjects following Kasai portoenterostomy and therapeutic efficacy. [ABSTRACT FROM AUTHOR]

Background: In biliary atresia (BA), the ultrasonic triangular cord (TC) sign is positive at ≥ 3 mm, but sometimes there is BA even if it is ≤ 3 mm. For improving the ultrasonographic diagnosis, we have established a new evaluation, adding the ratio of the anterior/posterior thickness (TC ratio) in the hyperechoic area and the presence of a cystic lesion in the triangular cord (TCC). Methods: We examined 24 cases of suspected BA who demonstrated acholic stools from 2006 to 2020. We retrospectively reviewed the timing of ultrasonographic diagnosis, the gallbladder diameter, gallbladder mucosal irregularity, the TC sign, TCC, and the TC ratio. Results: In the BA group (n = 10) vs the Non-BA group (n = 14), the age at ultrasonography was 75 ± 41.7 vs. 81 ± 39.1 days (p = 0.72), the gallbladder diameter was 12.1 ± 9.7 vs. 24.2 ± 6.96 mm (p = 0.02), irregularity of gallbladder mucosa was 7 cases vs. 1 case (p < 0.01), and TC sign was 3.9 ± 1.3 vs. 2.0 ± 0.49 mm (p = 0.01), respectively. TCC was observed in 8/10 cases in the BA group and none in the Non-BA group (p < 0.01). TC ratio was 3.40 ± 0.68 (BA group) and 1.59 ± 0.41 (Non-BA group) (p < 0.01). Conclusion: The ultrasonic TC ratio improves the diagnostic accuracy of BA. TCC is a specific finding in the BA group. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the clinical characteristics of cystic biliary atresia (CBA) and evaluate the midterm follow-up outcomes after laparoscopic treatment. Methods: We analyzed and compared data retrospectively on CBA patients (group A) and nonsyndromic type III biliary atresia (BA) patients (group B), who underwent laparoscopic Kasai portoenterostomy (LKPE) during the same period. Results: There were no significant differences in operative time, conversion rate, or the incidence of any postoperative complications between groups A and B (P > 0.05). The mean age at surgery (P < 0.01), rates of clearance of jaundice (CJ), cholangitis (P < 0.05), and 5-year survival with a native liver (SNL) were significantly lower in group A than in group B. Among the 35 patients with CBA, the CJ and 5-year SNL rates were significantly better in those with type I (n = 27) than in those with type IIId (n = 8) (P < 0.05). Conclusions: LKPE is a feasible and safe procedure for CBA. The 5-year SNL after LKPE was better in patients with CBA than in those with nonsyndromic type III BA. The 5-year SNL after LKPE for type I CBA was better than that for type IIId CBA. [ABSTRACT FROM AUTHOR]

Spontaneous extrahepatic bile duct perforation presents in infants with minimal or no jaundice, abdominal distension, pale stools and biliary ascites and requires prompt diagnosis and surgical intervention. The association of epidermolysis bullosa simplex, congenital adrenal hyperplasia and spontaneous perforation of extrahepatic bile duct has not been reported previously. The diagnosis of spontaneous extrahepatic bile duct perforation should be suspected when there is bilious ascites; the majority of the patients need surgical intervention, which is beneficial for both diagnosis and treatment. [Extracted from the article]

Purpose: The key characteristic of biliary atresia (BA) is obliteration of the extrahepatic bile ducts at the level of the porta hepatis. We aimed to relate the immunohistochemical features of remnant biliary ductules at the porta hepatis with clinical features and outcomes.Methods: Samples were immunostained with anti-cytokeratin 20 (CK20), vimentin and alpha-smooth muscle actin (aSMA). Primary outcome was set as clearance of jaundice (bilirubin ≤ 20 μmol/L) following Kasai portoenterostomy (KPE).Results: Eighty-two cases were classified into syndromic BA (n = 10), cystic BA (n = 7), CMV IgM+ BA (n = 9) and isolated BA (n = 56). CK20 expression was confirmed in 40/82 (49%), and vimentin expression in 19/82 (23%). aSMA was negative in all cases studied. CK20 expression was less common in isolated BA (n = 20/56, 36%) compared to CMV IgM+ BA (n = 8/9, 89%), cystic BA (n = 7/7, 100%) (isolated BA vs non-isolated BA, P = 0.0008). There was no difference in vimentin expression among the sub-groups (isolated BA vs. non-isolated BA; P = 0.39). CoJ was achieved in 52/82 (63%) overall with significant difference depending simply on sub-group [e.g. syndromic BA 9/10 (90%)]. CK20 expression was associated with a diminished rate of CoJ in the entire cohort [CK20+ 32/56 (57%) vs. CK20- 20/26 (77%); P = 0.04]. By contrast no correlation was observed between vimentin expression and CoJ (P = 0.13).Conclusion: CK20+ expression was associated with reduced clearance of jaundice in BA and a trend towards reduced native liver survival. [ABSTRACT FROM AUTHOR]

Purpose: Portoenterostomy (PE) is the standard treatment for biliary atresia (BA). However, micro-bile ducts are difficult to identify with surgical loupes and dissect systematically. We report the effects of our attempts to dissect hilar tissue using a surgical microscope. Methods: Microscopy-assisted portoenterostomy (MAPE) was initiated in 2014. Patients born between 2000 and 2013 who underwent PE until day 70 without a surgical microscope for BA were gathered as historical control. MAPE in re-do PE cases (Re-MAPE) was evaluated in the same manner. Results: Ten patients underwent MAPE for BA during the study period. 17 patients in the conventional PE group were gathered. In the MAPE group, the jaundice clearance rate was 80%, compared with 53% in the conventional PE group. Re-MAPE was performed in four patients, who had a jaundice clearance rate of 75%, essentially identical to the rate with initial MAPE. At age 4 years, the native liver survival rate was 58% in the MAPE group and 38% in the conventional PE group. The native liver survival rate in the Re-MAPE group was 75%. Conclusion: MAPE is useful for sharing the surgical field during open PE in patients with BA. It may improve the rate of jaundice clearance. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aim: To (i) review the aetiologies of neonatal cholestasis among term and preterm neonates at a single tertiary centre in Australia; (ii) identify clinical variables associated with biliary atresia (BA) and non‐BA aetiology of neonatal cholestasis; (iii) investigate the utility of hepatobiliary scintigraphy in predicting BA among term and preterm neonates. Methods: A retrospective cohort study of neonates born and investigated for cholestasis at two co‐located neonatal and children facilities from January 2013 to December 2017. Results: Of the 139 neonates with cholestasis, BA and intestinal‐failure‐associated liver‐disease was the most common cause of neonatal cholestasis in term (18%) and preterm (66%) cohorts, respectively. Incidence of BA was higher in term (1:6) than preterm (1:50) neonates (OR 10.29; 95% CI 2.06–49.97, P = 0.0024). Higher birthweight, acholic stool, absent or abnormal gallbladder on ultrasound was significantly associated with BA while gestational age ≤32 weeks, total parenteral nutrition ≥14 days and low albumin were associated with non‐BA aetiology of cholestasis. In diagnosing BA, non‐draining hepatobiliary scintigraphy demonstrated a lower specificity (73% vs. 90%) and lower positive predictive value (25% vs. 78%) in preterm compared to term neonates. Conclusion: Aetiology of cholestasis among preterm neonates differs from those in term neonates and currently existing diagnostic algorithm for neonatal cholestasis may need to be modified for preterm cohort, taking into account the prevalence for each aetiology, potential predictors and cost‐efficiency. [ABSTRACT FROM AUTHOR]

Objective: To determine the frequencies of various etiologies of neonatal cholestasis diagnosed by clinical findings and laboratory investigations at the Pak Emirates Military Hospital, Rawalpindi, Pakistan. Study Design: Cross-sectional study. Place and Duration of Study: Pediatric Department of Pak Emirates Military Hospital, Rawalpindi Pakistan, from Jan 2021 to Apr 2022. Methodology: Infants of either gender aged 14 days to six months admitted to Inpatient facility who had jaundice with direct bilirubin and more than 20% of total bilirubin were included in the study. The proforma was formulated to record the clinical features, laboratory investigations, weight, level of activity and consanguinity among the parents. Results: A total of 146 infants were included in the study. Jaundice was seen in 100% of infants, hepatomegaly in 66.4%, splenomegaly in 38.4%, followed by ascites in 25%. The most common aetiology of neonatal cholestasis was Biliary Atresia 26.7% in the extrahepatic Group, Idiopathic Neonatal Hepatitis 25.3% in the intrahepatic Group. Consanguinity was present in parents of 65% of infants. Conclusion: The most common aetiology of extrahepatic Neonatal Cholestasis was Biliary Atresia, while Idiopathic Neonatal Hepatitis and Progressive Familial Intrahepatic Cholestasis were the most common causes of intrahepatic cholestasis. [ABSTRACT FROM AUTHOR]

• This study clearly showed the usefulness of living donor liver transplantation after the Kasai operation. • Active introduction of living donor liver transplantation for patients with poor clearance of jaundice after the Kasai operation improved overall survival. Living donor liver transplantation (LDLT) has been actively performed for patients with poor clearance of jaundice after the Kasai operation for biliary atresia (BA). The present study clarified the usefulness of LDLT for BA. Between 2000 and 2020, 24 patients (late group) underwent radical surgery for BA in our institute. The overall survival rate, native liver survival rate, and proportion of LDLT in the late group were retrospectively compared with those of 47 patients treated before 1999 (early group). P values <.05 was considered statistically significant. The overall survival rates at 5, 10, and 15 years were 57%, 54%, and 49%, respectively, in the early group and 100%, 100%, and 100% in the late group (P <.001). The native liver survival rates at 5, 10, and 15 years were 57%, 52%, and 39%, respectively, in the early group and 57%, 49%, and 42% (P =.993) in the late group. In the early group, LDLT was performed in 7 of 47 patients (15%), and the overall survival rate after LDLT was 71%. In the late group, LDLT was performed in 11 of 24 patients (46%), and the overall survival rate after LDLT was 100%. The long-term outcomes after the Kasai operation for BA have improved in recent years. There were no marked differences in long-term native liver survival before and after 2000. LDLT was actively introduced for patients with poor clearance of jaundice after the Kasai operation, and the survival rate significantly improved. [ABSTRACT FROM AUTHOR]

Aim: This study compared the outcomes of patients with biliary atresia (BA) treated according to a standardised protocol with historical patients.Methods: This is a single-centred retrospective study of BA patients treated from 1980 to 2016. A standardised treatment protocol was established since 2008 regarding peri-operative management. The outcomes being compared between the two groups (Groups I and II = before and after 2008, respectively) were jaundice clearance (JC), incidence of recurrent cholangitis, hospital admission and native liver survival (NLS).Results: A total of 128 patients were included (Group I = 100, Group II = 28). The overall median follow-up period was 15.3 years (I vs. II = 20.6 years vs. 5.1 years, respectively). There was no significant difference in the JC at the sixth month between the two groups (I vs. II = 60.0 vs. 82.1%, respectively, P = 0.07). The incidence of recurrent cholangitis was similar between the two groups (I vs. II = 39 vs. 35.7%, respectively, P = 0.45), but the median hospital admission episode per patient was non-significantly higher in Group I (I vs. II = 4.2 vs. 2.7, respectively, P = 0.08). There was an improvement in the 1-year NLS rate in Group II (I vs. II = 69.0 vs. 85.7%, respectively, P = 0.05).Conclusions: The introduction of a standardised management protocol has improved the short-term outcome of BA patients, with a better 1-year NLS observed. [ABSTRACT FROM AUTHOR]

Infants with persistent conjugated jaundice, lasting longer than 14 days of age in a term infant and 21 days in a preterm infant, should be referred to a national paediatric liver unit for investigation of the cause. This paper reviews the ultrasound findings in such cases with a particular emphasis upon the diagnosis of biliary atresia. [ABSTRACT FROM AUTHOR]

Aim of the study: Biliary atresia is an idiopathic, destructive disease that affects both extrahepatic and intrahepatic bile ducts with severe inflammation and manifests as progressive jaundice within the first few months of life. In this study, we aimed to investigate the significance of genetic mutations in the onset of biliary atresia disease. Material and methods: With the approval of the ethics committee and parental consent, blood was taken from patients to obtain their DNA, and the study commenced. In this prospective study, we examined the DNA of 10 patients with no disease other than biliary atresia, and an exome sequence analysis was performed with the new-generation DNA sequencing method. The genetic structure of biliary atresia disease was examined by statistical analysis of the mutations, which were determined according to the reference DNA sequencing. Results: In the exome sequence analysis, the number of mutations detected among the patients changed significantly; the lowest number was 12,591, and the maximum was 19,863. By examining these mutations, we identified the mutated genes that were common to all patients. Conclusions: In this study, the highest mutation rates were detected in the PRIM2 and MAP2K3 genes. These genes have not previously been associated with biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Native liver survival after laparoscopic Kasai portoenterostomy (Lap-PE) for biliary atresia (BA) is controversial. We examined whether a jaundice-free native liver survival rate is comparable between conventional Kasai portoenterostomy (Open-PE) and Lap-PE. Then, the impact of the two types of PE on subsequent living-donor liver transplantation (LTx) was addressed in this study.Methods: The jaundice-free rate in 1- and 2-year-old patients who underwent Open-PE and Lap-PE from January 2006 to December 2017 was investigated. Additionally, perioperative data (duration from the start of surgery to the completion of hepatectomy and others) of patients aged 2 years or younger who underwent LTx after either Open-PE or Lap-PE from 2006 to 2017 were evaluated.Results: Thirty-one (67%) out of 46 Open-PE patients and 23 (77%) out of 30 Lap-PE patients showed native liver survival with jaundice-free status at 1 year of age (p = 0.384); 29 (63%) out of 46 Open-PE patients and 19 (70%) out of 27 Lap-PE patients showed native liver survival with jaundice-free status at 2 years of age (p = 0.524); there were no significant differences. Additionally, there were 37 LTx cases after PE within 2 years of birth, including 29 Open-PE and 8 Lap-PE cases. The patients in the Lap-PE group had fewer adhesions and significantly shorter durations of surgery up to the completion of the recipient's hepatectomy and durations of post-LTx hospital stay compared to the Open-PE group. There were no differences in blood loss or duration of stay in intensive care unit between the Lap-PE and Open-PE groups.Conclusions: Jaundice-free native liver survival rate has been comparable between Open-PE and Lap-PE. Lap-PE resulted in fewer adhesions, contributing to better outcomes of subsequent LTx compared to Open-PE. [ABSTRACT FROM AUTHOR]

To explore the distribution of biliary ductules in biliary remnants of patients with biliary atresia and to investigate the relationship between the ductules and the prognosis after Kasai portoenterostomy. From October 01, 2015 to September 30, 2017, 46 patients who were diagnosed as type 3 biliary atresia were enrolled in this study. Continuous sections of biliary remnants were stained with cytokerantin 19 antibody. The number, area, and distribution of micro-biliary ductules of each section were recorded. According to the number of ductules in the most proximal section (n ≥ 20 or n < 20), patients were divided into two groups (A or B) and followed up for 1-3 y, including cholangitis, jaundice clearance, and survival with native liver. Four patients had no micro-biliary ductules. In 17 patients with ductules, the numbers at bilateral parts were similar (P > 0.05), while the ductules in the middle part were significantly less than bilateral parts (P < 0.05). Starting from 2 mm from the proximal end of remnants, the number of ductules significantly and gradually decreased (P < 0.05). The total area of ductules in Group A was significantly increased compared to that in Group B (P < 0.05). Patients in Group A had significantly higher jaundice clearance rate and better survival rate with native liver than patients in Group B (both, P < 0.05). Patients had significantly higher incidence of cholangitis in Group A compared to Group B (P < 0.05). The number/area of ductules yielded by technical precision is closely related to effective bile drainage, jaundice clearance, and first onset of cholangitis in patients after Kasai procedure. [ABSTRACT FROM AUTHOR]

Objective: To determine the etiology and clinical profile of chronic liver disease in children presenting at Pak Emirates Military Hospital Rawalpindi. Study Design: Cross-sectional study. Place and Duration of Study: This study was conducted at the Department of Pediatrics, Pak Emirates Military Hospital Rawalpindi, from Sep 2015 to Sep 2016. Methodology: After informed written consent, 84 consecutive children diagnosed of chronic liver disease were included in the study and demographic features, presentation and underlying etiology. Results: There were 43 (51.2%) male and 41 (48.8%) female children with a mean age of 46.62 ± 46.89 months. Frequent presenting complaints were jaundice (32.1%) persistent neonatal jaundice (29.8%), abdominal distension (27.4%). Hepatosplenomegaly was the frequent presentation of children with glycogen storage disease while persistent neonatal jaundice was associated with biliary atresia and neonatal hepatitis. Wilson disease presented with neurological symptoms. Glycogen storage disease and biliary atresia were the most frequent underlying etiologies recorded in 15 (17.9%) children. The frequency of Wilsons disease was significantly higher among children aged between 5-10 years. Conclusion: The frequent clinical presentations of children with chronic liver disease were jaundice, abdominal distension and hepatosplenomegaly. Glycogen Storage Disorders, Biliary atresia and Wilsons Disease and were the most frequently encountered etiologies. [ABSTRACT FROM AUTHOR]

Purpose: High-dose postoperative steroid therapy after Kasai portoenterostomy is reported to improve jaundice clearance and a strong anti-inflammatory activity might prevent fibrous tissue formation which is often observed at the porta hepatis in revision surgery. We started steroid pulse therapy for the patients with cessation of decrease in jaundice and aimed to evaluate the efficacy in this study.Methods: The demographics and outcomes of patients who underwent laparoscopic Kasai portoenterostomy and received steroid pulse therapy within 2 months postoperatively between September 2014 and December 2018 were retrospectively reviewed; the therapy was determined successful when the serum total bilirubin level decreased to or below two-thirds of the pre-therapy level after 2 weeks. Patient data in the successful group were compared with those in the unsuccessful group.Results: Steroid pulse therapy was successful in seven of 16 patients (43.8%). The percentage of patients whose serum total bilirubin level decreased to normal was significantly higher in the successful group at 3 months (85.7% vs. 11.1%, P = 0.0028) and after all (100% vs. 33.3%, P = 0.011).Conclusions: Steroid pulse therapy was effective for some patients. Unsuccessful cases may have little chances of jaundice clearance; revision Kasai portoenterostomy would be a good option. [ABSTRACT FROM AUTHOR]

Background: Although open portoenterostomy (OPE) is considered the standard treatment for biliary atresia (BA), laparoscopic portoenterostomy (LPE) is conducted and reported by many investigators. Data on the safety and efficacy of LPE remain controversial. The aim of this meta-analysis is to compare the safety and efficacy of LPE and OPE for the treatment of BA.Methods: Three electronic databases were searched: PubMed, Embase, and the Cochrane Library. The eligible studies were limited to those published in English. The following keywords were used: "biliary atresia," "laparoscopic portoenterostomy," "Kasai portoenterostomy," "open portoenterostomy," "surgery," and "treatment."Results: Nine studies, including 434 patients, were analyzed. The operative time of LPE was significantly longer than that of OPE (MD = 40.55 min, 95% CI 4.83-76.27 min, P = 0.03). There was no significant difference between the two groups in terms of the time of hospital stay, the volume of intraoperative blood loss, or the rates of cholangitis, early clearance of jaundice or two-year survival with the native liver. The subgroup analyses revealed that the rate of early clearance of jaundice in the LPE group was significantly higher than that in the OPE group in studies published after 2016 (95% CI 1.04-1.75; P = 0.02).Conclusions: The present meta-analysis provides evidence that LPE is a feasible option for patients with BA. LPE should be revaluated by further studies and longer follow-up. [ABSTRACT FROM AUTHOR]

Background/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population. Patients and Methods: We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases. Data on clinical, biochemical, imaging, and histopathological characteristics were collected by chart review. The two primary study outcomes were (1) successful Kasai portoenterostomy (KPE) defined as resolution ofjaundice (total serum bilirubin <20 µmol/L) and (2) survival with native liver. Results: Over the study period, we evaluated 450 cases of infantile cholestasis. In all, 21 cases (11 males) were diagnosed with BA (4.7%). BA cases were first seen by pediatric gastroenterologists at a median age of 65 days. KPE was performed in 12 cases at a median age of 73 days. Successful KPE was achieved in four cases (33%). Five of the remaining eight cases had LT and three died before LT. Nine of the 21 BA cases were denied KPE and had primary LT at median 8 months of age. The native liver 4-year survival rate was 14.3% and the overall survival rate was 81%. Conclusion: BA is an uncommon cause of infantile cholestasis in Saudi Arabia. Our study provides a snapshot of the epidemiology of BA in Saudi Arabia that is characterized by late referral to pediatric gastroenterologists and poor outcome without LT. [ABSTRACT FROM AUTHOR]

Aim of the study: Biliary atresia (BA) is an uncommon disorder of the liver and bile ducts affecting infants and is characterized by progressive fibrosclerosing obstruction of the extrahepatic biliary tree leading to end-stage liver failure. The purpose of this study was to determine serum glypican-3 (GPC3) levels and liver stiffness in children with BA and the correlation of glypican-3 with clinical parameters. Material and methods: Seventy-five post-Kasai BA patients and 28 healthy age-matched controls were registered. Serum GPC3 levels were examined by enzyme-linked immunosorbent assay. Liver stiffness measurement was analyzed by transient elastography. Results: BA patients had significantly greater serum GPC3 and liver stiffness values than controls (p < 0.001). Serum GPC3 and liver stiffness values were significantly higher in jaundiced BA patients than in non-jaundiced BA patients (p < 0.001). Additionally, serum glypican-3 was associated with liver stiffness and serum total bilirubin (p < 0.001, respectively). Conclusions: Elevated serum GPC3 levels were associated with hepatic dysfunction and the severity of BA. As a result, serum GPC3 and liver stiffness might serve as biomarkers reflecting the deterioration of hepatic function and the outcome in post-Kasai BA. [ABSTRACT FROM AUTHOR]

Purpose: Laparoscopic Kasai portoenterostomy (LKP) remains controversial in the management of infants with biliary atresia (BA). There are no data reporting the 10-year native liver survival rate after LKP. The study aims to present the 10-year native liver survival rate after LKP and complications in native liver survivors after Kasai portoenterostomy (KP).Methods: A retrospective review was conducted for 31 consecutive infants with BA who underwent KP by day 75 of life in our institute from January 1993 to December 2007. The demographics and outcomes of patients after LKP and open KP (OKP) were compared.Results: Eleven patients underwent LKP and 20 patients underwent OKP. No statistical difference was observed in the age at operation and the preoperative bilirubin level. The operative time for LKP was significantly longer than that for OKP (mean 314.5 minutes versus 271.5 minutes, P = .03). The 10-year native liver survival rate was 45% (5/11) after LKP and was 85% (17/20) after OKP (P = .03). Forty percent (2/5) of the native liver survivors in the LKP and 71% (12/17) in the OKP developed complications within 10 years after KP (P = .23).Conclusions: The 10-year native liver survival rate in patients who underwent LKP by 75 days of life was 45%. With superior 10-year liver survival rate and comparable complication rate after OKP, OKP is still the treatment of choice for BA in our institute. [ABSTRACT FROM AUTHOR]

Objectives: This study reports frequency of different causes of infant cholestasis diagnosed by clinical and biochemical parameters as well as liver biopsy. Methods: Cholestasis persisting beyond two-weeks of age in 46 infants less than 6 months old was evaluated. A checklist including signs and symptoms, as well as laboratory, imaging and liver biopsy results was prepared. According to diagnosis, patients were classified into two groups: biliary atresia (BA) and non-biliary atresia (Non-BA) and results were compared between two groups. P value < 0.05 was considered significant. Results: Forty six infants (25 girls, 21 boys) with cholestasis and mean age of 65 ± 99 days were included in the study. Most of the infants (42%) were referred at the age of > 91 days. The most common symptoms and signs were jaundice in 100% followed by hepatomegaly (78%), splenomegaly (52%), acholic stool (35%) and dark urine (37%). There were no significant differences between the two groups for age, gender, onset of jaundice and organomegaly. Patients in BA group experienced more acholic stool, abnormal AST, ALT and bilirubin (P < 0.05). The most common underlying causes of liver disease in study group were BA (30.4%) and idiopathic neonatal hepatitis (INH) in 30.4% followed by metabolic disorders. Conclusions: Sixty one percent of infants with cholestasis in present study had BA and INH. A systemic approach is the key for rapid diagnosis to rule out BA and other treatable disorders for a better outcome. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice.Methods: We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB). Hepatic subcapsular flow was reviewed on Doppler US. We performed conditional inference tree analysis using MRI findings to generate a decision tree model.Results: A total of 208 infants were included, 112 in the BA group and 96 in the non-BA group. Mean age at the time of MRI was 58.7 ± 36.6 days. Visibility of CBD, abnormality of GB and MR-TCT were good discriminators for the diagnosis of BA and the MRI-based decision tree using these findings with MR-TCT cut-off 5.1 mm showed 97.3 % sensitivity, 94.8 % specificity and 96.2 % accuracy.Conclusions: MRI-based decision tree model reliably differentiates BA in infants with jaundice. MRI can be an objective imaging modality for the diagnosis of BA.Key Points: • MRI-based decision tree model reliably differentiates biliary atresia in neonatal cholestasis. • Common bile duct, gallbladder and periportal signal changes are the discriminators. • MRI has comparable performance to ultrasonography for diagnosis of biliary atresia. [ABSTRACT FROM AUTHOR]

Aim: This study investigates the fall in total serum bilirubin levels within 1 week after surgery, as a marker to predict early outcome in biliary atresia (BA) patients post-Kasai portoenterostomy (KP). Methods: The ratio of total serum bilirubin levels at the 7th postoperative day to the preoperative level (TB7/TB0) in patients undergoing KP was calculated (January 2011-July 2015). Patients were stratified after 3-months follow-up into outcome groups depending on the clinical clearance of jaundice and TB7/TB0 ratio was correlated to outcome and liver histopathological changes in these groups. Results: Sixty-one patients (M:F = 44:17), median age 75 days were included. At the end of 3 months, 27 (44.39%) were anicteric while 26 (42.6%) were still clinically jaundiced. Patients with a higher median value of TB7/TB0, that is, 0.856 were more likely to have jaundice at the end of 3 months as compared to patients with a lower median value of 0.615 (P < 0.0001). A cutoff TB7/TB0 ratio >0.723 predicted the KP outcome with 84.6% sensitivity and 81.5% specificity. The difference in TB7/TB0 ratio between patients with varying severity of liver histopathological changes was also significant, namely, cholestasis (P = 0.01), hepatocellular damage (P = 0.03), portal inflammation (P = 0.04), and portal fibrosis (P = 0.02). Conclusions: The rapidity of fall in the total serum bilirubin levels within 1 week post-KP was able to predict the likely outcome in BA patients. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy. These morbidities impose a huge impact on the quality of life of the patients and their families. Herein, we performed a comprehensive review on the clinical status and quality of life of long-term survivors of biliary atresia with their native livers, to facilitate meticulous longitudinal follow-up of these patients, and alert caregivers the probable complications to be aware of. [ABSTRACT FROM AUTHOR]

Aim Of Study: The diagnosis of biliary atresia (BA) remains challenging and delay can lead to significant morbidity with time to surgery a key factor in determining outcome. Prematurity may impact on outcome potentially delaying diagnosis. We sought to assess whether the premature BA infants (PBA) have a delayed time to surgery and as such, worse outcomes?Methods: Review of a single-centre prospectively maintained database. Prematurity was defined as delivery < 37/40 gestation. PBA was compared with date-matched term biliary atresia controls on a 2:1 basis. Primary outcomes were clearance of jaundice (< 20 μmol/L) and native liver survival. A retrospective assessment of liver fibrosis was made on biopsies at diagnosis and at Kasai portoenterostomy (KPE) in both premature and term cohorts. Data are quoted as median (range) unless indicated. A P value of ≤ 0.05 was considered statistically significant.Results: 21 (female n = 14, 67%) premature infants with BA were treated in the period Jan. 1988-Dec. 2016 and compared with 41 contemporaneous term BA controls. Median gestation was 33 (29-36) weeks and birth weight 1930 (948-4230)g. Twin pregnancy (n = 10) was the leading cause for prematurity and significantly higher than the controls (48 vs. 0%; P < 0.0001). Maternal co-morbidity was high (n = 10, 48%) including pre-eclampsia (19%) and diabetes (14%). Liver biopsy was performed in 19 (90%) patients (all diagnostic) at a median of 57 (4-266) days. Delayed diagnosis (> 50 days) was seen in n = 13 but not associated with parenteral nutrition use (46 vs. 33%, P = 0.59) or phototherapy (50 vs. 83%, P = 0.19). Both BASM (33 vs. 7.5%; P = 0.01) and duodenal atresia (19 vs. 0%; P = 0.01) were seen more frequently in the PBA cohort. Mean fibrosis scores (Ishak) from diagnostic biopsies were lower in the premature group than the control group (2.71 vs. 3.53, P = 0.043) indicating less fibrosis but this equalized by time of subsequent KPE (P = 0.17). Primary surgery was Kasai portoenterostomy (n = 20) at an older median age than controls (65 vs. 56 days; P = 0.06). Liver transplantation was the primary procedure in one late-presenting child. There was an increased but non-significant clearance of jaundice in the PBA group [n = 12/20 (60%) vs 20/41 (48%); P = 0.23] post-KPE. Native liver survival and true survival were not different (P = 0.58 and 0.23).Conclusions: PBA infants have similar outcomes to term infants, despite delayed diagnosis and higher frequency of the syndromic form. The high incidence of discordant twins supports the theory that epigenetic modifications could contribute to the pathogenesis of BA.Level Of Evidence: IIIc Retrospective Matched Cohort Study. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study is to evaluate hepatic artery resistive index (HARI) as a noninvasive prognostic predictor by correlating it with peripheral blood nitric oxide (NO) levels, portal pressure (PP) and histopathological changes in the liver in patients of biliary atresia (BA). Materials and Methods: Twenty-five patients were included in the study prospectively from November 2012 to June 2014. All patients underwent Doppler sonography to calculate the HARI preoperatively. Peripheral blood NO was also measured preoperatively. Biochemical liver function tests (LFTs) were measured preoperatively and at 1, 3, and 6 months postoperatively. The PP was measured intraoperatively, and a liver biopsy was taken in all patients. Disappearance of jaundice defined successful surgical treatment. Postoperatively, a hepatobiliary IminoDiacetic Acid scan (HIDA) was done to demonstrate a patent bilio-enteric pathway. Results: The mean preoperative HARI was 0.78 ± 0.105, and the median was 0.80 (range 0.60-1.0). The median HARI was used to correlate the other parameters; 13 (52%) patients had HARI ≥0.8. The mean PP was 24.96 ± 6.54 mmHg. The HARI had a strong correlation with PP (P = 0.0001) and (NO) (P = 0.0001); with every 0.1 increase in HARI, there was 5.2 mmHg increase in PP and 3.8 µmol/L increase in NO. The histological parameters which reached significance in relation to HARI were hepatocellular damage, bile duct inflammation, portal inflammation, and portal fibrosis. The postoperative improvement in LFT was significantly better in patients with HARI <0.8. All four patients who died during or after the study period had HARI >0.8, elevated PP, and NO levels. Conclusions: Preoperative HARI was found to have a direct correlation with PP and peripheral blood NO as a measure of portal hypertension. A preoperative HARI ≥0.8 should be considered as a risk factor for poor outcomes in BA. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia (BA) is a severe chronic liver disease characterized by progressive obstructive cholangiopathy of biliary tract. Heat shock protein 70 (HSP70) is involved in protecting cells against a wide variety of stress and plays a protective role in tissue damage. The purpose of this study was to investigate serum HSP70 and liver stiffness in BA and determine the association of serum HSP70, liver stiffness, and outcome parameters in post-Kasai BA patients.Methods: One hundred post-Kasai BA patients and 40 controls were enrolled. Serum HSP70 levels were analyzed using enzyme-linked immunosorbent assay. Liver stiffness values were assessed by transient elastography.Results: BA patients had significantly higher serum HSP70 and liver stiffness values than controls. Serum HSP70 and liver stiffness values were markedly elevated in BA patients with jaundice compared to those without jaundice (P < 0.001). Furthermore, serum HSP70 was more elevated in BA children with portal hypertension than those without portal hypertension (35.1 ± 2.1 vs. 27.9 ± 2.5 ng/mL, P < 0.001). Moreover, serum HSP70 was positively correlated with serum aspartate aminotransferase (r = 0.491, P < 0.001), alanine aminotransferase (r = 0.448, P < 0.001), total bilirubin (r = 0.303, P = 0.002), alkaline phosphatase (r = 0.414, P < 0.001), and liver stiffness values (r = 0.455, P < 0.001). There was a negative correlation between serum HSP70 and serum albumin (r = -0.434, P = 0.001).Conclusion: Serum HSP70 and liver stiffness values were higher in BA patients than controls. The increased serum HSP70 was correlated with hepatic dysfunction in BA. Consequently, serum HSP70 and liver stiffness could serve as non-invasive parameters reflecting the severity in post-Kasai BA. [ABSTRACT FROM AUTHOR]

Cholestatic jaundice in new born and infants results from biliary obstruction or hepatocellular dysfunction. Biliary atresia (BA) and Idiopathic neonatal hepatitis comprises the major aetiology. Cholestasis due to toxins is rare in infants. Indian childhood cirrhosis (ICC) and ICC like diseases have been described in infants. Herein, authors are describing a case of infantile cholestasis presenting at 4 months of age who was diagnosed to have copper related hepatotoxicosis on liver biopsy. Copper tumblers were used for preparation of formula milk that likely was the source of exogenous copper and the child improved well after removing the source of exogenous copper. [ABSTRACT FROM AUTHOR]

Cholestasis always indicates a pathological process that can result in chronic liver dysfunction, the necessity of liver transplantation and even death. (1) Cholestasis is a process in which there is a decrease in biliary flow, histological evidence of deposition of bile pigments in hepatocytes and bile ducts, and an increase in the serum concentrations of products excreted in bile. Cholestasis can occur at any age. It is caused by alteration of the formation of bile by the hepatocytes or by obstruction of the flow in the intrahepatic or extrahepatic biliary tracts. Neonatal cholestasis occurs in the first (3) months of life with elevated serum levels of direct bilirubin, cholesterol and bile acids. (2) In our environment, the most frequent cause is idiopathic neonatal hepatitis, followed by infectious causes. Obstructive causes have the worst prognoses. Among them, the most common is biliary atresia in which progressive obliteration of the extra hepatic biliary tract, parenchymal damage and intrahepatic biliary tract cause cirrhosis and death before the patient reaches three years of age. (1-3) The prognosis improves with surgical management if it is performed within the first 45 to 60 days of life. (3) The most common presentation is a healthy full-term newborn who develops jaundice, hypocolony, firm hepatomegaly and splenomegaly between the 2nd and 6th weeks of life. The principal serum marker that supports diagnosis is elevation of gamma glutamyltransferase while ultrasound of the liver following fasting has the greatest sensitivity and specificity. The only method for confirming the diagnosis is exploratory laparotomy with direct vision of the bile duct by intraoperative cholangiography. (4) In addition to correcting the cause and treating cholestasis, nutritional management with an adequate diet with high levels of medium chain triglycerides and fat-soluble vitamins is important for management of all patients with cholestasis. (5) [ABSTRACT FROM AUTHOR]

Purpose: Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure.Methods: This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis.Results: The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors.Conclusions: The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed. [ABSTRACT FROM AUTHOR]

Objective To evaluate the effects of portoenterostomy (Kasai surgery) on living donor liver transplantation (LDLT) for children with biliary atresia (BA). Methods A total of 150 children with BA, who were treated with LDLT in our center from September 2006 to September 2014, were retrospectively analysed. The children were categorized into Kasai group (90 cases, 60%) and non-Kasai (60 cases, 40%) group, based on whether they had previously undergone Kasai procedure pre-LDLT. Clinical data, incidence of complications and accumulated survival rates were compared between two groups. Results The ages of pediatric patients were 4.9-87.0 months. The patient age and height were significantly higher in Kasai group than those of non-Kasai group (P < 0.05). The serum bilirubin level was lower before surgery in Kasai group than that of non-Kasai group (P < 0.05). There were no significant differences in body weight, pediatric end stage of liver disease (PELD) score, graft to recipient body weight ratio (GRWR), operation time and blood loss between two groups (P > 0.05). Similarly, there were no significant differences in pulmonary infection, acute rejection, portal vein thrombosis, hepatic artery occlusion and biliary complications between the two groups (P > 0.05). The overall complication rate of post-LDLT was 61.1% in Kasai group, which was higher than that in non-Kasai group (43.3%,χ2=4.580, P=0.032). Totally, there were 7 cases (4.7%) died on post-LDLT, in which there were 6 cases (4.0%) in Kasai group including 5 cases of multiple organ failure and 1 case of severe pulmonary infection, and 1 case (0.7%) in non-Kasai group, who died of multiple organ failure due to preoperative gastrointestinal bleeding for emergency surgery. There were no serious complications and death in donors. The overall cumulative survival rates were 98.6%, 96.6%, 94.9% and 92.7% in 1 month, 1 year, 3 years and 5 years after LDLT, respectively. And there were no significant differences in survival rates in 1 month, 1 year, 3 years and 5 years between two groups (χ2=1.490, P=0.222) with the rates of 98.9%, 96.5%, 93.8%, 91.3% in Kasai group and 98.3%, 96.6%, 96.4%, 95.5% in non-Kasai group. Conclusion Performing Kasai procedure can acquire satisfied results to pediatric patients with BA pre-LDLT, without increasing the incidence of major complications and mortality post-LDLT. And the accumulated survival rate is not different in pediatric patients received Kasai surgery compared with that in non-Kasai patient. Besides that, Kasai surgery might postpone the time of receiving LDLT, benefit to the growth of children and reduce the jaundice of pre-LDLT. [ABSTRACT FROM AUTHOR]

Aim: Children with biliary atresia (BA) born in countries with temperate climate showed month-of-birth (MoB) predilection during cooler months. To date, no study on the MoB-BA association has been performed in a tropical country. Our aim was to define MoB variation in children with BA in a tropical country.Methods: We studied 150 children diagnosed with BA between January 1996 and April 2015 at a teaching hospital. MoB was defined by two categories based on the precipitation: rain and dry, and three categories based on the air temperature: high, average and low. We applied the country's population data on the number of births in each period as the expected proportions of birth.Results: A slightly higher proportion of BA children was born in the rainy months (52.7%); however, the difference was not significant compared to the general population's birth (P = 0.87). For the MoB based on the air temperature, no statistically significant difference was noted. Males with BA seemed to have a greater MoB variation compared to females, but this did not reach statistical significance.Conclusion: We could not find an association between MoB and BA in a tropical country. Multinational studies may aid in understanding the MoB-BA association in the tropical countries. [ABSTRACT FROM AUTHOR]

Aims: In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing “extended” Kasai portoenterostomy (KPE), a modification of the original procedure. Materials and Methods: Since 2013, we have used the technique of “extended KPE” and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015). Results: Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ± 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months. Conclusion: In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the incidence of portal hypertension (PHT) in biliary atresia (BA) patients and to monitor its progress after Kasai portoenterostomy (KP) by measuring nitric oxide (NO) levels in peripheral blood. Materials and Methods: A prospective cross-sectional study conducted over a period of 2 years. Intraoperative portal pressure (PP) and blood NO levels at presentation, 1-month, 3-month, and 6-month follow-up, were correlated with clinical and biochemical parameters in BA patients. The mean NO level in age-matched control group was 4.64 ± 2.32 μmol/L. Results: Thirty-four BA patients underwent KP over a period of 2 years. The mean age of presentation was 2.7 months (range 1-4 months). The mean intraoperative PP was 21.3 ± 5.4 mmHg. The mean PP in patients aged <60 days, 61-90 days, and >90 days was 18.53 ± 4.45 mmHg, 20.33 ± 3.07 mmHg, and 26.5 ± 5.01 mmHg, respectively. The mean PP in the patients who underwent successful KP was 16.75 ± 3.54 mmHg while for those who continued to have jaundice it was 23.94 ± 4.63 mmHg (P < 0.001). NO levels closely followed the PP as shown by the regression equation NO = 4.79 + 0.64 PP mmHg, R2 = 0.69. The mean NO level at presentation was 18.48 ± 4.17 μmol/L and at 1-month, 3-month, and 6-month follow-up was 11.94 ± 5.62 μmol/L, 10.79 ± 6.02 μmol/L, and 9.93 ± 6.53 μmol/L, respectively (P < 0.001). The difference in NO levels was also statistically significant between the patients who cleared jaundice and those with persisting jaundice. Conclusion: All BA patients had PHT at presentation. PHT worsens with age and has an adverse effect on outcome of KP. NO levels in blood closely follow PP and higher levels are associated with poor outcome. [ABSTRACT FROM AUTHOR]