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Aim Methods Results Conclusion This retrospective study aimed to assess the efficacy of prophylactic antibiotics in preventing ascending cholangitis following Kasai portoenterostomy (KPE). Data from 72 patients treated across four tertiary centres in Israel from 2008 to 2018 were analysed.Clinical and laboratory data were collected from biliary atresia (BA) diagnosis until liver transplantation (LT) or study completion.Median age at KPE was 58.5 days. Successful KPE was achieved in 23 (32%) patients. Ascending cholangitis occurred in 6/23 (26%) successful KPE cases and 15/45 (33%) unsuccessful cases. Primary antibiotic prophylaxis (49% of patients) was associated with earlier onset of cholangitis (median 77 vs 239 days, p = 0.016). During follow‐up, 39% underwent LT, with a 5‐year survival with native liver (SNL) of 54%.Prophylactic antibiotics did not reduce cholangitis rates post‐KPE in our cohort. Further research is essential to optimise management strategies for infants with BA. [ABSTRACT FROM AUTHOR]

Purpose: Bile lake (BL) formation following Kasai portoenterostomy (KPE) can complicate the prognosis of biliary atresia (BA). Percutaneous transhepatic biliary drainage (PTBD) performed under fluoroscopic/ultrasonographic (US) guidance is discussed for the management of BL. Methods: A retrospective review of 64 BA patients treated by KPE (open = 31, laparoscopic = 33) at a single center (2004–2023) identified 9 BL cases (9/64; 14.1%). PTBD was indicated for jaundice or cholangitis refractory to antibiotic therapy. Results: All BL were asymptomatic, diagnosed after an episode of postoperative cholangitis. KPE type was not correlated with BL incidence; 2/31 (6.5%) for open and 7/33 (21.2%) for laparoscopic; p = 0.15. Median onset was postoperative day 273 (IQR: 170–920). One case resolved with antibiotics while another case required early liver transplantation (LTx) due to advanced hepatic dysfunction unrelated to BL. All remaining cases (7/9) had PTBD at a median of 14.3 months (IQR: 7.3–34.7) post-KPE, with successful resolution in 6/7; one case required two PTBD procedures. Post-PTBD biliary peritonitis (n = 3) was resolved by abdominal lavage (laparoscopic = 2; open = 1). Conclusion: Screening for BL is advisable in all postoperative BA patients especially when cholangitis occurs. Although the risk for biliary peritonitis warrants caution, PTBD seems a viable option for managing BL formation after KPE. [ABSTRACT FROM AUTHOR]

Objective Methods Results Conclusion The objective of this study was to investigate the ultrasonographic characteristics and outcomes of fetuses with atypical and non‐visualized fetal gallbladder in our tertiary care hospital.A retrospective analysis was conducted on cases in which the fetal gallbladder was not visualized or exhibited atypical characteristics at our institution over a four‐year period. The patients were divided into two groups: absent gallbladder and atypical gallbladder. The groups with isolated and additional anomalies were analyzed according to their ante‐ and postnatal characteristics.The study comprised 78 patients (37 absent, 41 atypical gallbladder). In the isolated fetal absence of gallbladder group, the gallbladder was visualized in three of 13 patients during antenatal ultrasonographic follow‐up and in half of the remaining 10 patients during postnatal follow‐up. In the postnatal period, five newborns with absent isolated gallbladder are being followed up with suspicion of biliary atresia and isolated gallbladder agenesis. In the absence of a gallbladder with an additional anomaly group, 58% of fetuses died during the intrauterine and neonatal period. Fetuses in the isolated atypical gallbladder group are being followed as healthy after birth. Pregnancies with atypical gallbladder appearance and additional anomalies resulted in 33% neonatal death, 12% intrauterine demise, and 25% termination of pregnancy.In instances where the fetal gallbladder is not consistently discernible, it is imperative to exercise caution with regard to the possibility of biliary atresia. In the event that the fetal gallbladder exhibits unusual characteristics, a meticulous examination for the presence of additional anomalies is recommended. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia (BA) has diverse and unclear pathogenesis, which may be related to immune response in response to a foreign stimulus. T follicular helper (Tfh) cells have been found to play an important role in various immune diseases. Aims: To investigate the expression of Tfh cells in BA and non-BA cholestatic diseases in children. Methods: Transcriptome sequencing and Gene Ontology (GO) enrichment analysis were performed to investigate the differences in gene expression between the BA group and the non-BA cholestasis group. Study the distribution of Tfh cells in liver tissues of the BA and non-BA cholestatic groups through single sample gene set enrichment analysis (ssGSEA). Tfh cells (CD3+Bcl6+) in liver tissues from BA patients were labeled by double immunofluorescent staining to verify their distribution in the liver. Results: Transcriptome sequencing showed differences in gene expression between the BA group and the non-BA cholestasis group. A total of 808 genes were up-regulated and 405 genes were down-regulated in BA, suggesting that there might be a specific immune response in BA. GO enrichment analysis showed that BA group had augmented response to foreign stimulus and increased metabolic process compared to the non-BA cholestatic group. The relative proportion of immune cells was analyzed by ssGSEA method. The proportions of Tfh cells, activated B cells, CD4+ T cells, memory B cells and Th2 cells were higher in the BA group than in the non-BA cholestatic group. Fluorescence immunostaining showed that Tfh cells were significantly increased in liver tissue samples of the BA group compared to the non-BA cholestasis group, which was consistent with the transcriptome sequencing results. Conclusion: Tfh cells share in immune cascade involvement in BA. Our work support immune pathogenesis of the in response to a stimulus that might be foreign in BA. [ABSTRACT FROM AUTHOR]

Purpose: We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA). Methods: LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups. Results: HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018). Conclusions: Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation. [ABSTRACT FROM AUTHOR]

Congenital syphilis (CS) cases in worldwide have reached 700.000 cases until 2023. Especially late or untreated cases can cause up to 80% of poor pregnancy outcomes. This case report discusses the treatment strategy of symptomatic CS case in a newborn with multiple comorbidities. A newborn female infant was admitted with cutaneous lesions comprising white patches with a bluish-red base, as well as jaundice. The patient was born to a single mother with a history of multiple partners and never had an antenatal care. The patient exhibited pale yellow stools with a putty-like consistency and dark-yellow urine. The patient was tachypnea (oxygen saturation of 86% on room air), exhibiting jaundice of the eyes and skin (Cramer IV). Laboratory results were positive for syphilis, severe thrombocytopenia (10×103/µL), hyperbilirubinemia with total bilirubin 18.89 mg/dL, direct bilirubin 11.81 mg/dL, and indirect bilirubin 7.08 mg/dL. Babygram suggested pneumonia, while 2-phase abdominal ultrasound showed impaired gallbladder contractility. The patient was diagnosed with CS, pneumonia neonatal, and cholestasis suspicious for biliary atresia. The patient was stabilized hemodynamically, administered intravenous fluids containing dextrose 10%, benzathine penicillin G 130,000 IU was administered intramuscularly every 24 hours (10 days) according to hospital availability for syphilis treatment, combination of cefoperazone-sulbactam and gentamicin for the treatment of pneumonia, and ursodeoxycholic-acid (UDCA) for the treatment of cholestasis. The patient's condition showed gradual improvement, with the skin lesions also demonstrating improvement, although jaundice persisted. In the last follow-up, due to stable condition, the patient was discharged to outpatient with continued oral UDCA therapy. [ABSTRACT FROM AUTHOR]

Biliary atresia is a progressive cholangiopathy in neonates, which often results in liver failure. In high‐income countries, initial treatment requires prompt diagnosis followed by Kasai portoenterostomy. For those with a late diagnosis, or those in whom Kasai portoenterostomy fails, liver transplantation is the only lifesaving treatment. Unfortunately, in low‐ and middle‐income countries, timely diagnosis is a challenge and liver transplantation is rarely accessible. Here, we discuss the ethical dilemmas surrounding treatment of babies with biliary atresia in Uganda. Issues that require careful consideration include: risk of catastrophic health expenditure to families, ethical dilemmas of transplant tourism, medical risks of maintaining the transplant in a low‐resourced health system, and difficult decisions encountered by the surgeon caring for these patients. Four distinct models of the patient‐physician relationship are applied to biliary atresia in Uganda. These models describe differences in patient and physician roles, and patient values and autonomy. Solid organ transplantation is a rapidly evolving segment of healthcare in Uganda and ongoing policy advancements may shift ethical considerations in the future. [ABSTRACT FROM AUTHOR]

Introduction The native liver survival in biliary atresia (BA) depends on various factors, and one of the crucial factors is the rate of progression of liver fibrosis after portoenterostomy, but there is no reliable investigation to assess it. This study evaluated shear wave elastography (SWE) to detect liver fibrosis in BA patients and assess its utility during follow-up. Materials and Methods This was an observational study; SWE was done preoperatively and postoperatively at 3 and 6 months. The SWE values were analyzed to determine their correlations with preoperative liver histology as well as with postoperative SWE variation between different postoperative outcomes. Results Twenty-one patients were included in the study; the preoperative SWE values were strongly correlated with liver biopsy grading (p < 0.001). At the 3 months postoperatively, SWE was done for 18 children: 12 in group A (patent bilioenteric drainage on hepatobiliary iminodiacetic acid scan) and 6 (nonpatent) in group B; mean SWE value was 12.8 and 17.3 kPa, respectively (p < 0.001). Ten children from group A underwent SWE 6 months postoperatively, and the mean value was 13.23 kPa. Conclusion The SWE values correlate with liver histology grading, suggesting a reliable alternative to biopsy. Additionally, the baseline SWE values and their trend during follow-up can provide information on the disease's progression. [ABSTRACT FROM AUTHOR]

This review article discusses the most common benign biliary disorders and the various radiological findings on multiple modalities. A broad spectrum of diseases including various congenital disorders, infective and parasitic etiologies, immunological pathologies such as primary sclerosing cholangitis, and immunoglobulin G4-related sclerosing cholangitis are discussed along with obstructive diseases and ischemic cholangitis. The article emphasized the imaging differential diagnosis of the above lesions as well as clinical correlates those that are most relevant to radiologists. The article briefly touched upon management and intervention where relevant. [ABSTRACT FROM AUTHOR]

Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant. The incidence rate of BA in the state of Hawai‘i is nearly double the incidence rate in the continental US. International studies have demonstrated that screening programs for BA reduce the age at diagnosis and treatment. However, there has been no statewide analysis on the ages at diagnosis or at Kasai, nor does a statewide screening program for BA exist. The purpose of this study is to review the age of diagnosis and treatment of BA to determine if the current practice in Hawai‘i is in line with the published data. A retrospective chart review of all patients diagnosed with BA at the state’s primary children’s hospital was performed (2009-2023) and 19 patients who underwent the Kasai procedure were identified. The mean age at diagnosis is 71.4 days (n=19) and the mean age at Kasai procedure is 72.0 days (n=19). Both the average age at diagnosis and treatment for BA in Hawai‘i is significantly higher than published data suggesting best outcomes at 30-45 days of life. This review suggests that the implementation of a statewide screening program for BA in Hawai‘i is warranted. [ABSTRACT FROM AUTHOR]

Background: Liver transplantation in pediatric patients is a crucial intervention for treating end-stage hepatic diseases. Despite significant advances in surgical techniques and postoperative care, complications remain a substantial challenge in this population. Biliary stones, an infrequent complication, present challenges in this context. Given the impossibility of endoscopic treatments, different strategies have been explored to address post-liver transplantation gallstones in children by implementing percutaneous treatment with intraductal lithotripsy. Case presentation: A 7-year-old Latin patient, who had a diagnosis of biliary atresia at the age of 2, underwent a liver transplant from a living donor. However, 4 months after the transplant, the patient experienced recurring episodes of cholangitis. Cholangioresonance revealed intrahepatic lithiasis and anastomotic stenosis. Attempted gallstone removal through percutaneous cholangiography proved unsuccessful, as multiple peripheral stones in all ducts remained immobile. Subsequently, a percutaneous endoscopic cholangioscopy using the SpyGlass Discover system for visual examination of the bile ducts + electrohydraulic lithotripsy was performed, effectively removing the stones without any complications. Conclusions: Percutaneous cholangioscopy with intraductal lithotripsy enables accurate identification and extraction of intrahepatic stones without the need for surgical intervention. This method proves to be a valuable alternative in addressing post-transplant biliary stone. In our case, it was performed on a pediatric patient who underwent liver transplantation, which makes it interesting and relevant as there is currently insufficient literature on this approach in such cases in this population. [ABSTRACT FROM AUTHOR]

Background: The study aims to investigate Shear Wave Elastography's accuracy in assessing pediatric cholestasis and differentiating biliary atresia from other causes. The early diagnosis of cholestasis in newborns, when caused by true liver disease and not physiological or transient jaundice is usually eluded. To maximize the diagnostic reliability of ultrasound as an initial imaging modality to diagnose biliary atresia, ultrasound elastography has been introduced as a quantitative non-invasive sonographic technique to measure liver stiffness which is presumed to be different in biliary atresia from other cholestasis as well as follow-up liver stiffness post-Kasai operation. Results: Ten patients (34.48%) were diagnosed with cholestasis with normal/low GGT. Five patients (17.24%) were diagnosed with hepatitis, and 2 patients had syndromes (10.34%). Other causes were found in 11 patients (37.93%). Major bile duct obstruction with fibrosis/cirrhosis was the majority of findings found in biliary atresia patients (100%), most of them with mild fibrosis (40%), followed by moderate (36.67%), then marked fibrosis (13.33%) and secondary cirrhosis (10%). ROC curve analysis shows that the best cut-off value for SWE = 1.97 with a sensitivity of 75.0% and specificity of 83.5%, with positive and negative predictive values were 82.8% and 74.1%, respectively. Mean shear wave elastography was significantly higher among the biliary atresia group (median 2.86) than in non-biliary atresia (median 1.81) and control groups (median 1.64) (P < 0.001) The best cut-off value of shear wear elastography for predicting outcome in pediatric patients post-Kasai procedure was 3.55, with a sensitivity of 66.7%, specificity of 64.7%, positive and negative predictive values were 25% and 91.7% respectively, with accuracy 65%. Conclusion: The results of this study suggest that shear-wave elastography is a useful diagnostic tool for differentiating biliary atresia from other causes of cholestasis in pediatric patients, with a high sensitivity and specificity. Further studies are needed to validate the findings and determine the optimal cut-off value for shear wear elastography in clinical practice. [ABSTRACT FROM AUTHOR]

Transient hyperphosphatasemia (TH) is a benign condition observed among healthy infants and children < 5 years old. It is characterized by an elevation in serum alkaline phosphatase (ALP) in the absence of other signs of organ disease. Prognosis is excellent, and ALP levels stabalize within 4 months. The aim of this case report is to promote broader awareness of TH so further unnecessary workup is avoided. The patient was a 12-month-old girl who presented with pale stools, a single episode of bloody stool, and elevation (incidentally found) in her ALP of 2379 IU/L. A small anal fissure was present, and the remainder of her physical examination was typical. The differential diagnosis included biliary atresia, liver disease, bone disease, and TH. Further testing was typical and included complete blood count (CBC, consisting of hemoglobin, hematocrit, white blood cell count, and platelet count), comprehensive metabolic panel (CMP, consisting of glucose, creatinine, BUN, electrolytes, and liver function markers), calcium, phosphate, parathyroid hormone, gamma-glutamyl transferase, and 25-hydroxy vitamin D. Liver ultrasound was also typical without evidence of biliary atresia. The diagnosis of TH was made. The patient was monitored clinically. Repeat blood work was completed 2 months later, with ALP levels returning to the typical range. Overall, TH is a benign self-limiting condition that can be managed by observation and serial measurement of ALP without further unnecessary investigations. [ABSTRACT FROM AUTHOR]

Background: The effects of anesthetics on liver and kidney functions after infantile living-related liver transplantation (LRLT) are unclear. This study aimed to investigate the effects of propofol-based total intravenous anesthesia (TIVA) or desflurane-based inhalation anesthesia on postoperative liver and kidney functions in infant recipients after LRLT and to evaluate hepatic ischemia–reperfusion injury (HIRI). Methods: Seventy-six infants with congenital biliary atresia scheduled for LRLT were randomly divided into two anesthesia maintenance groups: group D with continuous inhalation of desflurane and group P with an infusion of propofol. The primary focus was to assess alterations of liver transaminase and serum creatinine (Scr) levels within the first 7 days after surgery. And the peak aminotransferase level within 72 h post-surgery was used as a surrogate marker for HIRI. Results: There were no differences in preoperative hepatic and renal functions between the two groups. Upon the intensive care unit (ICU) arrival, the levels of aspartate aminotransferase (AST, P = 0.001) and alanine aminotransferase (ALT, P = 0.005) in group P were significantly lower than those in group D. These changes persisted until the fourth and sixth days after surgery. The peak AST and ALT levels within 72 h after surgery were also lower in group P than in group D (856 (552, 1221) vs. 1468 (732, 1969) U/L, P = 0.001 (95% CI: 161–777) and 517 (428, 704) vs. 730 (541, 1100) U/L, P = 0.006, (95% CI: 58–366), respectively). Patients in group P had lower levels of Scr upon the ICU arrival and on the first day after surgery, compared to group D (17.8 (15.2, 22.0) vs. 23.0 (20.8, 30.8) μmol/L, P < 0.001 (95% CI: 3.0–8.7) and 17.1 (14.9, 21.0) vs. 20.5 (16.5, 25.3) μmol/L, P = 0.02 (95% CI: 0.0–5.0) respectively). Moreover, the incidence of severe acute kidney injury was significantly lower in group P compared to that in group D (15.8% vs. 39.5%, P = 0.038). Conclusions: Propofol-based TIVA might improve liver and kidney functions after LRLT in infants and reduce the incidence of serious complications, which may be related to the reduction of HIRI. However, further biomarkers will be necessary to prove these associations. [ABSTRACT FROM AUTHOR]

Aim Methods Results Conclusions Inchinkoto (ICKT) is administered to patients with biliary atresia (BA) after surgery. Gardeniae fructus which is contained in ICKT is reported to be associated with idiopathic mesenteric phlebosclerosis (IMP). We investigated the influence of ICKT on calcification of the mesenteric veins suspected of IMP in BA patients.All BA patients with native liver survival who received ICKT after Kasai portoenterostomy were reviewed. All patients underwent abdominal computed tomography (CT) during treatment. Calcification of the mesenteric veins on the most recent CT scan after the initiation of ICKT was assessed.Twenty patients (6 males and 14 females) were enrolled in this study. Of these patients, 17 (85.0%) and 10 (50%) patients had used ICKT for ≥5 years and >10 years, respectively. Calcification of the mesenteric veins was detected in one patient, who was a 14 year old girl. The total dosage of Gardeniae fructus was 21 800 g.The incidence of calcification of the mesenteric veins in BA patients receiving ICKT was low. However, it is necessary to be aware that ICKT containing Gardeniae fructus is associated with the risk of developing calcification of the mesenteric veins suspected of IMP. [ABSTRACT FROM AUTHOR]

Background: Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed "hepatic hilar cysts". Case presentation: A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm. Conclusions: The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types. [ABSTRACT FROM AUTHOR]

Purpose: This study assessed the benefits of quality threshold (QT) implementation for liver shear wave elastography (SWE) in children during free breathing. Methods: The QT, which adjusts the SWE map display based on shear wave quality, was set at 55%. Phantom measurements (PMs) were taken with a fixed probe using QT (termed PM-1); a moving probe without QT (PM-2); and a moving probe with QT (PM-3). Each measurement was subjected to random samplings of various sizes. Clinical measurements (CMs) were obtained from children with biliary atresia using following protocols: CM-1, manually defined regions of interest (ROIs); CM-2, default ROIs without QT; and CM-3, default ROIs with QT. Elasticity measurements were compared across fibrosis grades, and color patterns on the SWE maps were analyzed. Results: In the phantom experiments, the moving probe produced lower elasticity measurements; this difference decreased upon QT application. With the moving probe, random sampling indicated fewer interquartile range-to-median ratios exceeding 30% upon QT application (4% vs. 14% when five values were sampled, P=0.004). In clinical experiments, QT improved the differentiation of fibrosis grade in patients over 5 years old, with a significant difference between moderate and severe fibrosis (P=0.004). Elasticity variability was positively correlated with fibrosis grade (τ=0.376, P<0.001). Certain apparent errors, termed artificial stripe patterns, were not eliminated by QT. Conclusion: Applying QT to exclude low-quality pixels can minimize measurement error and improve differentiation of liver fibrosis grades. The presence of an artificial stripe pattern on the SWE map may indicate images requiring exclusion. [ABSTRACT FROM AUTHOR]

Objective: Biliary atresia (BA) is an obstructive cholangiopathy that involves the intrahepatic and extrahepatic bile ducts. Ultrasound (US) can aid in evaluation of the biliary system and be efficiently used in daily practice. However, most studies on US for diagnosing BA have been conducted in developed countries. Therefore, we have aimed to evaluate the diagnostic accuracy of US in BA in infants with cholestasis from a developing country. Materials and Methods: This retrospective study used data collected from our hospital medical records. The US findings were compared with the gold standard intraoperative or cholangiography findings. Results: Thirty-five BA patients (19 males and 16 females) and 36 controls (20 males and 16 females) were included in the study. Most of the patients (85.7%) were ≤ 6 months old. The absence of a gallbladder demonstrated 71.42% sensitivity (Sn), 91.67% specificity (Sp), 89.29% positive predictive value (PPV), 76.74% negative predictive value (NPV), 8.57 positive likelihood ratio (LR+), and 0.31 negative likelihood ratio (LR−) for diagnosing BA. The triangular cord sign demonstrated 14.28% Sn, 100% Sp, 100% PPV, 76.74% NPV, ∞ LR+, and 0.86 LR- for diagnosing BA. The combination of gallbladder absence and a positive triangular cord sign demonstrated 82.85% Sn, 91.67% Sp, 90.63% PPV, 84.61% NPV, 9.95 LR+, and 0.19 LR− for diagnosing BA. Conclusion: The diagnostic accuracy of US in BA is high, indicating that it can be the imaging tool of choice in infants with cholestasis. Ultrasound is safe and can be easily used in daily practice without the risk of radiation exposure. [ABSTRACT FROM AUTHOR]

Biliary atresia is a rare liver disease of unknown etiology affecting approximately 1 in 10,000 children. This disease initially presents as inflammatory obstruction of bile ducts leading to cholestasis and eventually fibrosis of hepatic tissue. Affected patients are ideally treated early with portoenterostomy (Kasai procedure) as age at surgery is an important prognostic factor for native liver survival and need for liver transplant. This study aimed to evaluate the age at which patients in the United States are receiving this procedure. The American College of Surgeons National Surgical Quality Improvement Program Pediatric database was used to identify patients between 2012 and 2021 who underwent a primary procedure of portoenterostomy. The age at time of surgery and perioperative analysis was performed. The data underwent simple descriptive statistics. Eight hundred twenty four patients were identified who underwent Kasai procedure. Four hundred seventy four (58.2%) were female with the predominant race being White (49.5%). The median age at surgery was 57 d old (interquartile range 41-71). Readmission and reoperation rates within 30 d were 30% and 15.2%, respectively. There were no deaths within 30 d. Within the National Surgical Quality Improvement Program database, the median age of pediatric patients undergoing Kasai procedure for biliary atresia in the United States exceeds the goal of 45 d. Further studies are needed to investigate factors that may affect time to diagnosis and time to Kasai procedure. [ABSTRACT FROM AUTHOR]

Background and aims: Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy. The histopathological diagnosis is often challenging and an immunohistochemical marker is often sought as an adjunct. We evaluated MMP7 immunohistochemistry in BA and other non-BA pediatric cholestatic liver diseases. Materials and methods: MMP7 immunohistochemistry was applied in 5 age-matched normal control, 23 cases of BA and 43 cases of non-BA pediatric cholestasis including 16 cases of choledochal cyst (CC), and a multiplication score was obtained by multiplying the intensity and percentage positivity in the cholangiocytes. Results: BA showed a high mean MMP7 multiplication score which was significantly different from the normal control and other non-BA pediatric cholestatic diseases including CC (p value < 0.001). The sensitivity, specificity, positive, and negative predictive values of MMP7 immunohistochemistry were 91.3%, 93.02%, 87.5%, and 95.2% respectively. Conclusion: MMP7 immunohistochemistry may be an adjunct to histomorphology in BA. [ABSTRACT FROM AUTHOR]

Background: The aim of the present study was to compare the outcomes of pediatric LT with left liver grafts, obtained either from a living donor (LD) or a split deceased donor (sDD). Methods: Retrospective single‐center study from 2002 to 2022. All pediatric LT with left liver grafts (not including middle hepatic vein) from LD or sDD were included. Reduced grafts were not included. Results: A total of 112 pediatric LT were performed: 58 with LD grafts and 54 with sDD grafts (17 split ex situ and 37 in situ). Donor characteristics were similar, apart from donor age (33 years in LD vs. 30 years in sDD, p = 0.03). Indications were similar with 55% biliary atresia in each group. Retransplantation was more frequently performed in the sDD group (2% vs. 15%, p = 0.01). Recipient age, weight, and PELD score at transplant were not significantly different between groups. Cold ischemia time was longer for sDD (158 min in LD vs. 390 min in sDD; p < 0.0001). Posttransplant peak ALT was higher with sDD grafts (1470 vs. 1063, p = 0.018), and hospital stay was longer with sDD grafts (27 vs. 21 days, p = 0.005). However, there was no difference between groups in terms of major morbidity (Dindo‐Clavien grade ≥3), vascular and biliary complications, and 90‐day mortality. Patient survival at 10 years was 93.1% for LD and 92.8% for sDD (p = 0.807). Graft survival at 10 years was 89.7% for LD and 83.1% for sDD (p = 0.813). Conclusions: Technically similar LD and sDD grafts achieve very similar postoperative and long‐term outcomes with excellent patient and graft survival. [ABSTRACT FROM AUTHOR]

Biliary atresia (BA) is a congenital disease that occurs when extrahepatic bile ducts are either absent or deficient, resulting in liver fibrosis, portal hypertension, and eventually cirrhosis. It is the most common cause of persistent obstructive jaundice in newborns lasting more than two weeks is this condition. Abdominal ultrasound (US) is the primary imaging technique used to diagnose BA, while computed tomography (CT) is reserved for more complex cases. The gold standard for diagnosing BA is still intraoperative cholangiogram with liver biopsy. Treatment for BA usually involves Kasai hepatoportoenterostomy, but some patients still require liver transplantation due to diagnostic delays and advanced disease. In this study, the authors present the case of a 3-month-old infant with biliary atresia and its ultrasound characteristics, who underwent liver transplantation due to advanced disease. The primary objective of imaging is to provide a prompt diagnosis, given the crucial significance of timely surgical intervention. [ABSTRACT FROM AUTHOR]

Objective: Tc-99m N-pyridoxyl-5-methyl-tryptophan (PMT) hepatobiliary scintigraphy has high diagnostic performance for biliary atresia. Our hospital implements standard Tc-99m PMT administration followed by a 6 h static imaging review; booster doses are given in cases requiring 24 h delayed scans. This study aimed to evaluate the diagnostic performance of this method. Methods: A total of 37 pediatric patients who underwent Tc-99m PMT biliary scintigraphy were classified into the surgically-diagnosed biliary atresia or non-biliary atresia groups. The absence of tracer accumulation in the small bowel was considered a hepatobiliary scintigraphic diagnosis of biliary atresia. The Clopper–Pearson method was used to calculate the 95% confidence intervals (CIs) for determining the diagnostic accuracy, negative predictive value, positive predictive value, sensitivity, and specificity of Tc-99m PMT biliary scintigraphy. Results: Among the 37 patients, 12 were classified into the diagnosis of biliary atresia group. Regarding biliary scintigraphy findings, 16 of 37 patients demonstrated tracer accumulation in the small bowel within 6 h of testing. These cases were diagnosed as non-biliary atresia, requiring no further testing or booster administration. In contrast, 21 patients underwent delayed testing requiring booster administration, which revealed 13 without tracer excretion and 11 who were diagnosed with biliary atresia. Among the eight patients with tracer accumulation, only one was diagnosed with biliary atresia. Furthermore, two cases without tracer excretion and seven cases with tracer excretion were clinically diagnosed as non-biliary atresia. The diagnostic performance of our examination was as follows: a diagnostic accuracy of 91.9% (34/37; 95% CIs 78.0–98.3%), sensitivity of 91.6% (11/12; 95% CIs 61.5–99.8%), specificity of 92.0% (23/25; 95% CIs 74.0–99.0%), a positive predictive value of 84.6% (11/13; 95% CIs 54.6–98.0%), and a negative predictive value of 95.8% (23/24; 95% CIs 78.9–99.9%). Conclusions: Our protocol for Tc-99m PMT biliary scintigraphy using tracer booster administration demonstrated reliable diagnostic performance for biliary atresia. Notably, 43% of cases did not require booster administration, indicating that lesser radiation exposure may still yield comparable diagnostic accuracy. [ABSTRACT FROM AUTHOR]

Feto-maternal microchimerism is the bidirectional transfer of cells through the placenta during pregnancy that can affect the health of both the mother and the offspring, even in childhood or adulthood. However, microchimerism seems to have different consequences in the mother, who already has a developed immune system, than in the fetus, which is vulnerable with immature defense mechanisms. Studies have shown that the presence of fetal microchimeric cells in the mother can be associated with reduced fetal growth, pre-eclampsia, miscarriage, premature birth, and the risk of autoimmune disease development in the future. However, some studies report that they may also play a positive role in the healing of maternal tissue, in cancer and cardiovascular disease. There are few studies in the literature regarding the role of maternal microchimeric cells in fetal autoimmunity. Even fewer have examined their association with the potential triggering of autoimmune diseases later in the offspring's life. The objectives of this review were to elucidate the mechanisms underlying the potential association between maternal cells and autoimmune conditions in offspring. Based on our findings, several hypotheses have been proposed regarding possible mechanisms by which maternal cells may trigger autoimmunity. In Type 1 diabetes, maternal cells have been implicated in either attacking the offspring's pancreatic β-cells, producing insulin, differentiating into endocrine and exocrine cells, or serving as markers of tissue damage. Additionally, several potential mechanisms have been suggested for the onset of neonatal lupus erythematosus. In this context, maternal cells may induce a graft-versus-host or host-versus-graft reaction in the offspring, function as effectors within tissues, or contribute to tissue healing. These cells have also been found to participate in inflammation and fibrosis processes, as well as differentiate into myocardial cells, potentially triggering an immune response. Moreover, the involvement of maternal microchimeric cells has been supported in conditions such as juvenile idiopathic inflammatory myopathies, Sjögren's syndrome, systemic sclerosis, biliary atresia, and rheumatoid arthritis. Conversely, no association has been found between maternal cells and celiac disease in offspring. These findings suggest that the role of maternal cells in autoimmunity remains a controversial topic that warrants further investigation. [ABSTRACT FROM AUTHOR]

Background & aimsBiliary atresia (BA) is poorly understood and leads to liver transplantation (LT), with the requirement for and associated risks of lifelong immunosuppression, in most children. We performed a genome-wide association study (GWAS) to determine the genetic basis of BA.MethodsWe performed a GWAS in 811 European BA cases treated with LT in US, Canadian and UK centers, and 4,654 genetically matched controls. Whole-genome sequencing of 100 cases evaluated synthetic association with rare variants. Functional studies included whole liver transcriptome analysis of 64 BA cases and perturbations in experimental models.ResultsA GWAS of common single nucleotide polymorphisms (SNPs), i.e. allele frequencies >1%, identified intronic SNPs rs6446628 in AFAP1 with genome-wide significance (p = 3.93E-8) and rs34599046 in TUSC3 at sub-threshold genome-wide significance (p = 1.34E-7), both supported by credible peaks of neighboring SNPs. Like other previously reported BA-associated genes, AFAP1 and TUSC3 are ciliogenesis and planar polarity effectors (CPLANE). In gene-set-based GWAS, BA was associated with 6,005 SNPs in 102 CPLANE genes (p = 5.84E-15). Compared with non-CPLANE genes, more CPLANE genes harbored rare variants (allele frequency

Background and purpose: Children with cholestasis experience a wide spectrum of disease, ranging from transient liver function abnormalities to life-threatening conditions. These children may require hospitalization or specialized care at various times due to liver enzyme disorders and associated clinical issues. In severe cases, liver damage can lead to acute liver failure, necessitating liver transplantation or resulting in death. Early diagnosis of the etiology of cholestasis can be crucial for improving prognosis. This study aimed to determine the frequency, causes, and outcomes of cholestasis in infants referred to Amir Kola Children's Hospital. Materials and methods: This retrospective descriptive study included all infants with cholestasis admitted to AmirKola Children's Hospital between 2012 and 2022. Inclusion criteria were infants under 2 years of age diagnosed with cholestasis. Infants with incomplete medical records were excluded from the study. Hepatic cholestasis was diagnosed based on the infant's medical history and confirmed by a pediatric gastroenterologist. All infants underwent diagnostic evaluation tests and abdominal ultrasound. Liver biopsy was performed when necessary to assess liver pathology. Short-term outcomes of hepatic cholestasis, including length of hospitalization, frequency of hospitalization, need for special care, liver transplantation, and mortality, were evaluated. Demographic information, along with the causes and outcomes of cholestasis, were reported using measures of central tendency (mean) and dispersion (standard deviation), or as frequencies and percentages. Results: During the study period (2012-2022), 3,650 infants under 2 years of age were admitted to Amir Kola Children's Hospital. The frequency of cholestasis among hospitalized children was 54 (1.47%). The most common symptom of cholestasis was hepatomegaly, observed in 35 (64%) infants. The leading causes of cholestasis included biliary atresia in 21 (38.8%) infants, idiopathic hepatitis in 17 (31.4%) infants, and congenital metabolic disorders in 7 (12.96%) infants. Of the children in the study, 23 (42%) were hospitalized at least once, and 10 (18.5%) were hospitalized twice. Liver biopsy was performed in 32 (60.4%) of the 54 children; 11 had neonatal cholestasis, 7 had normal ultrasound results, and 13 had giant cell hepatitis and neonatal hepatitis (10 with normal ultrasound results), with 2 showing normal pathology reports. Additionally, 20 (37%) of these children died, 24 (44%) recovered, and 9 required liver transplantation. Conclusion: According to the results of this study, more than one-third of children with cholestasis died, with biliary atresia and idiopathic hepatitis identified as the most common causes. Biliary atresia was also the most frequent indication for liver transplantation. These findings highlight the need for timely diagnostic examinations, testing, and specialized evaluations to support early diagnosis and treatment in infancy.

Abstract Background Biliary atresia (BA) has diverse and unclear pathogenesis, which may be related to immune response in response to a foreign stimulus. T follicular helper (Tfh) cells have been found to play an important role in various immune diseases. Aims To investigate the expression of Tfh cells in BA and non-BA cholestatic diseases in children. Methods Transcriptome sequencing and Gene Ontology (GO) enrichment analysis were performed to investigate the differences in gene expression between the BA group and the non-BA cholestasis group. Study the distribution of Tfh cells in liver tissues of the BA and non-BA cholestatic groups through single sample gene set enrichment analysis (ssGSEA). Tfh cells (CD3+Bcl6+) in liver tissues from BA patients were labeled by double immunofluorescent staining to verify their distribution in the liver. Results Transcriptome sequencing showed differences in gene expression between the BA group and the non-BA cholestasis group. A total of 808 genes were up-regulated and 405 genes were down-regulated in BA, suggesting that there might be a specific immune response in BA. GO enrichment analysis showed that BA group had augmented response to foreign stimulus and increased metabolic process compared to the non-BA cholestatic group. The relative proportion of immune cells was analyzed by ssGSEA method. The proportions of Tfh cells, activated B cells, CD4+ T cells, memory B cells and Th2 cells were higher in the BA group than in the non-BA cholestatic group. Fluorescence immunostaining showed that Tfh cells were significantly increased in liver tissue samples of the BA group compared to the non-BA cholestasis group, which was consistent with the transcriptome sequencing results. Conclusion Tfh cells share in immune cascade involvement in BA. Our work support immune pathogenesis of the in response to a stimulus that might be foreign in BA.

Introduction The native liver survival in biliary atresia (BA) depends on various factors, and one of the crucial factors is the rate of progression of liver fibrosis after portoenterostomy, but there is no reliable investigation to assess it. This study evaluated shear wave elastography (SWE) to detect liver fibrosis in BA patients and assess its utility during follow-up.

Background: To predict native liver survival (NLS) after Kasai portoenterostomy (KP) for biliary atresia (BA) using pre-operative clinical data. Materials and Methods: Pre-operative data were collected from 29 patients with BA who underwent KP at our department between 1989 and 2017 and were analysed including serum albumin, bilirubin, prothrombin time-international normalised ratio, body height, body weight, age at KP, paediatric end-stage liver disease score calculated using the pre-operative data and the period of NLS. Results: The 10-year NLS rate of all patients was 51%. A multivariate analysis revealed that among all factors, the pre-KP serum albumin level was the only independent predictor of NLS (P = 0.04, hazard ratio = 0.269, 95% confidence interval = 0.077–0.934). The area under the receiver operating characteristic curve for NLS, determined using pre-KP serum albumin was 0.760 and 3.75 mg/dl was selected as the cut-off value. There was a significant difference in NLS between patients with high (≥3.8 mg/dl) and low (≤3.7 mg/dl) pre-KP serum albumin (90.0% vs. 31.5%, P < 0.01). Conclusions: Decreased pre-KP serum albumin may reflect not only functional impairment of the liver, but also the inflammatory process, which is hypothesized to occur during its advancement. The pre-KP serum albumin level may be a good prognostic factor for NLS in post-KP BA patients.

Abstract Background The study aims to investigate Shear Wave Elastography's accuracy in assessing pediatric cholestasis and differentiating biliary atresia from other causes. The early diagnosis of cholestasis in newborns, when caused by true liver disease and not physiological or transient jaundice is usually eluded. To maximize the diagnostic reliability of ultrasound as an initial imaging modality to diagnose biliary atresia, ultrasound elastography has been introduced as a quantitative non-invasive sonographic technique to measure liver stiffness which is presumed to be different in biliary atresia from other cholestasis as well as follow-up liver stiffness post-Kasai operation. Results Ten patients (34.48%) were diagnosed with cholestasis with normal/low GGT. Five patients (17.24%) were diagnosed with hepatitis, and 2 patients had syndromes (10.34%). Other causes were found in 11 patients (37.93%). Major bile duct obstruction with fibrosis/cirrhosis was the majority of findings found in biliary atresia patients (100%), most of them with mild fibrosis (40%), followed by moderate (36.67%), then marked fibrosis (13.33%) and secondary cirrhosis (10%). ROC curve analysis shows that the best cut-off value for SWE = 1.97 with a sensitivity of 75.0% and specificity of 83.5%, with positive and negative predictive values were 82.8% and 74.1%, respectively. Mean shear wave elastography was significantly higher among the biliary atresia group (median 2.86) than in non-biliary atresia (median 1.81) and control groups (median 1.64) (P < 0.001) The best cut-off value of shear wear elastography for predicting outcome in pediatric patients post-Kasai procedure was 3.55, with a sensitivity of 66.7%, specificity of 64.7%, positive and negative predictive values were 25% and 91.7% respectively, with accuracy 65%. Conclusion The results of this study suggest that shear-wave elastography is a useful diagnostic tool for differentiating biliary atresia from other causes of cholestasis in pediatric patients, with a high sensitivity and specificity. Further studies are needed to validate the findings and determine the optimal cut-off value for shear wear elastography in clinical practice.