Cholangioscopy in Children and Adolescents: Utilization, Outcomes, and Safety.

Background: Endoscopic retrograde cholangiopancreatography (ERCP) is increasingly utilized for management of biliary disorders in children and adolescents. Practice patterns surrounding cholangioscopy in pediatric patients, however, are largely uncharacterized.

Methods: We retrospectively analyzed all ERCPs in which cholangioscopy was performed on patients 18 and under at our tertiary care children's hospital from 2015 to 2020 using our institution's paper and electronic medical record system.

Nationwide Evolution of Pediatric Endoscopic Retrograde Cholangiopancreatography Indications, Utilization, and Readmissions over Time.

Objectives: To analyze outcome and utilization trends over time of pediatric endoscopic retrograde cholangiopancreatography (ERCP) in an all-capture US population-level study.

Study Design: Using the National Inpatient Sample (2005-2014) and National Readmission Database (2010-2014), we identified pediatric (age <20 years) hospitalizations during which ERCP was performed and assessed ERCP-associated readmissions. International Classification of Diseases, Ninth Revision, Clinical Modification codes were used to identify hospitalization diagnoses, comorbidities, and patient/hospital characteristics.

Fluoroscopy Time During Endoscopic Retrograde Cholangiopancreatography Performed for Children and Adolescents is Significantly Higher With Low-volume Endoscopists.

Background: Endoscopic retrograde cholangiopancreatography (ERCP) is a fluoroscopy and endoscopy-based procedure important for diagnosis and management of pediatric pancreaticobiliary disorders. Patient, procedure, endoscopist, and facility characteristics have been shown to influence ERCP complexity and procedure outcomes as well as fluoroscopy utilization in adults; however, the extent to which this is true in pediatric patients remains under-studied and there are minimal data regarding fluoroscopy utilization in pediatric ERCP.

Methods: We retrospectively analyzed ERCPs performed on patients <18 years of age at our tertiary care children's hospital from 2002 to 2017 using our institution's paper and electronic medical record system along with a prospectively maintained radiation exposure database.

Open-label prospective therapeutic clinical trials: oral vancomycin in children and adults with primary sclerosing cholangitis.

Background: Oral vancomycin (OV) in primary sclerosing cholangitis (PSC) has been evaluated as a potential therapeutic agent. We report the long-term biochemical course and outcomes of patients with PSC treated with OV.

Methods: Patients were enrolled in 2 open-label clinical trials (ClinicalTrials.

Pediatric Endoscopy Practice Patterns in the United States, Canada, and Mexico.

Background And Aims: Endoscopic procedures are important for diagnosis and management of many gastrointestinal, liver, and biliary conditions in children. Therapeutic endoscopy procedures, including endoscopic retrograde cholangiopancreatography (ERCP), are performed less frequently in children relative to adults. A formal study to evaluate institutional volumes and practice patterns for advanced therapeutic pediatric endoscopy procedures has, however, not been previously undertaken.

Prenatal treatment of ornithine transcarbamylase deficiency.

Purpose Of Study: Patients with neonatal urea cycle defects (UCDs) typically experience severe hyperammonemia during the first days of life, which results in serious neurological injury or death. Long-term prognosis despite optimal pharmacological and dietary therapy is still poor. The combination of intravenous sodium phenylacetate and sodium benzoate (Ammonul®) can eliminate nitrogen waste independent of the urea cycle.

Initial experience with peroral endoscopic myotomy for treatment of achalasia in children.

Background: Achalasia is a primary esophageal motility disorder characterized by aperistalsis of the esophagus and failed relaxation of the lower esophageal sphincter that presents rarely in childhood. The peroral endoscopic myotomy (POEM) procedure is an emerging treatment for achalasia in adults that has recently been introduced into pediatric surgical practice.

Methods: This is a prospective case series of all children referred to Stanford University Lucile Packard Children's Hospital with manometry-confirmed achalasia who underwent a POEM procedure from 2014 to 2016.

Mass cytometry reveals a distinct immunoprofile of operational tolerance in pediatric liver transplantation.

Long-term IS in transplant patients has significant morbidity, poorer quality of life, and substantial economic costs. TOL, defined as graft acceptance without functional impairment in the absence of IS, has been achieved in some pediatric LT recipients. Using mass cytometry, peripheral blood immunotyping was performed to characterize differences between tolerant patients and patients who are stable on single-agent IS.

Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis.

Neonatal cholestasis is a potentially life-threatening condition requiring prompt diagnosis. Mutations in several different genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account for all familial cases. Here we report four individuals from two unrelated families with neonatal cholestasis and mutations in NR1H4, which encodes the farnesoid X receptor (FXR), a bile acid-activated nuclear hormone receptor that regulates bile acid metabolism.

Text Messaging Improves Participation in Laboratory Testing in Adolescent Liver Transplant Patients.

Background: In solid organ transplant patients, non-participation in all aspects of the medical regimen is a prevalent problem associated with adverse consequences particularly in the adolescent and young adult (AYA) age group. This study is the first to evaluate the feasibility, utility and impact of a text messaging (TM) intervention to improve participation in laboratory testing in adolescent liver transplant patients.

Methods: AYA patients, aged 12 to 21 years, were recruited for a prospective pilot trial evaluating a TM intervention delivered over a 1-year period.

Novel protocol including liver biopsy to identify and treat CD8+ T-cell predominant acute hepatitis and liver failure.

In the majority of children with ALF, the etiology is unknown and liver transplantation is often needed for survival. A patient case prompted us to consider that immune dysregulation may be the cause of indeterminate acute hepatitis and liver failure in children. Our study includes nine pediatric patients treated under a multidisciplinary clinical protocol to identify and treat immune-mediated acute liver injury.

Evaluation of ethanol lock therapy in pediatric patients on long-term parenteral nutrition.

Background: Pediatric home parenteral nutrition (PN) patients present a unique challenge with risks of catheter-associated bloodstream infections (CABSIs), sometimes requiring subsequent catheter removal. Recurrent infections can lead to line removal and potential loss of venous access in the future.

Objective: Demonstrate that weekly ethanol lock therapy decreases CABSIs in long-term home PN patients and decreases line removals due to infections.

Echinocandin and ethanol lock therapy treatment of fungal catheter infections.

Ethanol lock therapy has been implemented to prevent infections of central venous catheters as well as to treat infections. Fungal catheter-associated blood stream infections are historically more difficult to treat and have required removal of central venous catheters. We report the largest case series to date, successfully treating 5 of 7 fungal catheter-associated blood stream infections with ethanol lock therapy and systemic echinocandin administration.

Immunophenotyping of peripheral eosinophils demonstrates activation in eosinophilic esophagitis.

Background And Aim: Eosinophilic esophagitis (EoE) is a chronic inflammatory disorder characterized by upper gastrointestinal symptoms and the presence of high numbers of eosinophils in the esophagus. Although eosinophils in the esophagus have been found to be activated in subjects with EoE, detailed studies of intracellular signaling pathways involved in the mechanism of activation of eosinophils in EoE have heretofore been limited. The aim of the study was to assess whether any surface molecules or transcription factors are activated in peripheral eosinophils in subjects with EoE.

Long-term outcome following pediatric liver transplantation for metabolic disorders.

In order to determine long-term outcome, including survival, growth and development, following liver transplantation in children with metabolic disorders, we retrospectively reviewed charts of 54 children with metabolic disorders evaluated from 1989-2005 for presenting symptoms, transplantation timing and indications, survival, metabolic parameters, growth, and development. Thirty-three patients underwent liver transplantation (12 received combined liver-kidney transplants) at a median age of 21 months. At a median follow-up of 3.

Combined liver-kidney transplantation in children: indications and outcome.

Although it remains a relatively infrequent procedure in children, CLKT has become a viable option for a select group of pediatric patients with severe liver and kidney disease. Most are performed for rare primary diseases such as PH1, but a selected few are performed in the setting of concomitant hepatic and renal failure of uncertain etiology and prognosis. This article reviews the indications for and outcomes following CLKT in children.

Non-adherence to post-transplant care: prevalence, risk factors and outcomes in adolescent liver transplant recipients.

This study examined the prevalence, demographic variables and adverse outcomes associated with non-adherence to post-transplant care in adolescent liver transplant recipients. We conducted a retrospective chart review of 111 adolescent patients (age 12-21 yr) greater than six months post-transplantation and defined non-adherence as not taking the immunosuppressive(s) or not attending any clinic visit in 2005. Fifty subjects (45.

Outcomes of transplantation in children with primary hepatic malignancy.

HBL and HCC are the most common hepatic malignancies in children. The role of OLT in children with HCC is still a matter of debate. The aim of this study was to review our experience of OLT for HCC.

Parenteral nutrition-associated cholestasis related to parental care.

Parenteral nutrition-associated cholestasis (PNAC) is a complication not uncommon in the pediatric population. In severe cases, patients require a liver transplant. To our knowledge, we report the only case of PNAC with end-stage liver failure in a child with short bowel syndrome that resolved with a change in caretaker.

Complete immunosuppressive withdrawal as a uniform approach to post-transplant lymphoproliferative disease in pediatric liver transplantation.

Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disease (PTLD) in pediatric liver transplant recipients is associated with a high mortality (up to 60%) and the younger age groups, who are predominantly EBV-naïve, are at highest risk for development of this disease. The aim of this study is to assess, in this high-risk group, patient outcome and graft loss to rejection when complete withdrawal of immunosuppressive agents (IMS) is instituted as the mainstay of treatment in addition to the use of standard therapy. A retrospective analysis of 335 pediatric patients whose liver transplants were performed by our team between September 1988 and September 2002, was carried out through review of computer records, database and patient charts.