Liver transplantation (LT) is the standard therapy for end-stage liver disease. allografts are at risk fibrosis, which biopsy gold evaluation but carries risks. There a need noninvasive modalities to track trajectory of fibrosis. We evaluated diagnostic accuracy shear wave elastography (SWE) stiffness (LS) measurements quantify fibrosis in pediatric transplant recipients. Between 2007 and 2024, 93 patients had 106 biopsies performed within 13 months elastography. LS values were significantly...

•The apical glycocalyx is thin and patchy in neonatal compared to adult cholangiocytes.•Neonatal cholangiocytes have immature cell-cell junctions increased permeability.•The submucosal space has minimal collagen I or elastin.•The contains many actively collagen-secreting cells. Background & AimsThe extrahepatic bile duct the primary tissue initially affected by biliary atresia. Biliary atresia a cholangiopathy which exclusively affects neonates. Current animal models suggest that developing...

Eosinophilic esophagitis (EoE) is a chronic antigen-mediated immune disorder of the esophagus. Consensus guidelines recommend obtaining esophageal, gastric, and duodenal biopsies at diagnostic endoscopy when EoE suspected. The utility repeated gastric during follow-up in patients previously diagnosed with not established. aim present study was to explore role children an established diagnosis undergoing repeat assess histological response treatment.Retrospective chart review tertiary care...

Adverse early life events are key factors for development of functional gastrointestinal disorders (FGIDs). Urinary tract infection (UTI) is associated with chronic pelvic pain in adults, a finding that has been recapitulated murine models, but the relation between UTI and abdominal not studied children. We hypothesized infancy increases risk FGIDs (CAP) childhood.The present study included children, ages 4 to 18 years, single first year their siblings no history UTI. Parents completed...

We report the first synthesis of plant isoflavonoid biliatresone. The convergent has been applied to several analogs, which have facilitated structure-activity relationship study for this environmental toxin that, on ingestion, recapitulates phenotype biliary atresia.

The unfolded protein response (UPR) is a coordinated cellular to endoplasmic reticulum (ER) stress that functions maintain homeostasis. When ER unresolved, the UPR can trigger apoptosis. Pathways within influence bile acid metabolism in adult animal models and human liver diseases, however, has not been studied young or pediatric diseases. In this study we sought determine whether weanling age mice had altered activation compared with mice, which could lead increased acid-induced hepatic...

Abstract Macrophages (MΦ) play a role in neonatal etiologies of obstructive cholestasis, however, the for precise MΦ subsets remains poorly defined. We developed murine model bile duct ligation (BDL) to characterize etiology-specific differences cholestatic polarization. Neonatal BDL surgery was performed on female BALB/c mice at 10 days life (DOL) with sham laparotomy as controls. Comparison made Rhesus Rotavirus (RRV)-induced biliary atresia (BA). Evaluation changes day 7 after (BDL and...

There are limited murine models of cholestatic liver diseases characterized by chronic biliary obstruction and resumption bile flow. While duct ligation (BDL) is a well-established model obstructive cholestasis, current BDL reversal (BDLR) alter anatomy. We aimed to develop more physiologic BDLR evaluate the time course mechanism for resolution hepatic injury after obstruction. In present study, we restored flow into duodenum without disruption gall bladder using biocompatible PE-50 tubing....

FXR regulates bile acid metabolism, and null (Fxr-/-) mice have elevated levels progressive liver injury. The inositol-requiring enzyme 1α/X-box binding protein 1 (XBP1) pathway is a protective unfolded response activated in to endoplasmic reticulum stress. Here, we sought determine the role of 1α/XBP1 hepatic toxicity using Fxr-/- mouse model. Western blotting quantitative PCR analysis demonstrated that XBP1 other pathways were 24-week-old compared with 10-week-old but not WT mice. To...

Background: Staged surgical palliation resulting in the Fontan operation increases survival patients born with single ventricle congenital heart disease. survivors are at higher risk for dysfunction multiple organ systems. Recent studies have shown prevalence of elevated hemoglobin A1c (HbA1c) as a marker abnormal glucose metabolism modifiable associated factors such obesity and hypertriglyceridemia. However, other HbA1c this population remain unclear. In adults structurally normal hearts,...

Background Increased intrahepatic bile acids cause endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) is activated to maintain homeostasis. UPR dysregulation, including inositol-requiring enzyme 1α/X-box 1 (IRE1α/XBP1) pathway, associated with adult liver diseases but has not been characterized in pediatric diseases. We evaluated hepatic expression cholestatic disease (CLD) explants hypothesize that an inability appropriately activate IRE1α/XBP1 pathway pathogenesis of...

May 8, 2019April 9, 2019Free AccessAutoimmune hepatitis in Aicardi-Goutières Syndrome (P4.6-045)Zachary Cross, Alyssa Kriegermeier, Joseph McMann, Elizabeth Rand, Asako Takanohashi, Nicole Ulrick, Adeline Vanderver, and Laura AdangAuthors Info & AffiliationsApril 2019 issue92 (15_supplement)https://doi.org/10.1212/WNL.92.15_supplement.P4.6-045 Letters to the Editor

Abstract Background & Aims The extrahepatic bile duct is the primary tissue initially affected by cholangiopathy biliary atresia. Biliary atresia affects neonates exclusively and current animal models suggest that developing uniquely susceptible to damage. In this study, we aimed define anatomical functional differences between neonatal adult mouse ducts. Methods We studied passaged cholangiocytes, BALB/c cholangiocytes isolated ducts, a collagen reporter mouse. included transmission...

Immunosuppression reduction after liver transplant is an important strategy to mitigate long-term medication side effects. We describe our center’s experience with immunosuppression minimization once-daily calcineurin inhibitor dosing. Success was defined as continuing daily monotherapy normal transaminases and no rejection. performed a retrospective review of eligible children who received between 2009 2016, had surveillance biopsy, were on twice-daily monotherapy. Twenty-eight 51 patients...

Abstract Background/Aims Cholestatic liver diseases (CLD) are the leading indication for pediatric transplantation. Increased intrahepatic bile acid concentrations cause endoplasmic reticulum (ER) stress and unfolded protein response (UPR) is activated to maintain homeostasis. UPR dysregulation, including inositol-requiring enzyme 1α/X-box 1 (IRE1α/XBP1) pathway, associated with several adult diseases. We evaluated hepatic expression in patients end-stage CLD hypothesize that an inability...

Abstract Background & Aims Farnesoid X receptor (FXR) regulates bile acid metabolism and FXR null ( Fxr -/- ) mice have elevated levels progressive liver injury. The inositol-requiring enzyme 1α (IRE1α)/X-box binding protein 1 (XBP1) pathway is a protective of the unfolded response (UPR) that activated in to ER stress. In this study we sought determine role UPR mice. Approach Results We examined hepatic gene expression 10- 24-week-old wild type (WT) Hepatic XBP1 other pathways were mice,...

The incidence of syphilis is rising among adolescents necessitating improved provider awareness and screening practices. We present a case an adolescent with acute hepatitis ultimately diagnosed secondary syphilitic hepatitis. Clinical presentation, laboratory abnormalities, histologic features are nonspecific, diagnosis relying on clinical suspicion targeted testing. This highlights the importance for in sexually active adolescents, variety manifestations including those commonly seen by...