Contemporary trends in choledochal cyst excision: An analysis of the pediatric national surgical quality improvement program.

Background: Choledochal cysts are rare congenital anomalies of the biliary tree that may lead to obstruction, chronic inflammation, infection, and malignancy. There is wide variation in the timing of resection, operative approach, and reconstructive techniques. Outcomes have rarely been compared on a national level.

Incidence and Outcomes of Simultaneous Thoracoabdominal Triple Organ Transplantation in the United States.

Background: This study aims to evaluate patient outcomes of simultaneous triple organ transplants, which may provide insight into optimal donor allocation while maximizing recipient benefit.

Methods: Triple organ transplants and their corollary dual organ transplants were identified using the United Network for Organ Sharing database. Triple organ transplants evaluated included heart-lung-kidney (n = 12) and heart-liver-kidney (n = 37).

Safety and Immunogenicity of Live Viral Vaccines in a Multicenter Cohort of Pediatric Transplant Recipients.

Importance: Live vaccines (measles-mumps-rubella [MMR] and varicella-zoster virus [VZV]) have not been recommended after solid organ transplant due to concern for inciting vaccine strain infection in an immunocompromised host. However, the rates of measles, mumps, and varicella are rising nationally and internationally, leaving susceptible immunocompromised children at risk for life-threating conditions.

Objective: To determine the safety and immunogenicity of live vaccines in pediatric liver and kidney transplant recipients.

AHRR Hypomethylation mediates the association between maternal smoking and metabolic profiles in children.

Background: Tobacco smoking during pregnancy is associated with metabolic dysfunction in children, but mechanistic insights remain limited. Hypomethylation of cg05575921 in the aryl hydrocarbon receptor repressor (AHRR) gene is associated with in utero tobacco smoke exposure. In this study, we evaluated whether AHRR hypomethylation mediates the association between maternal smoking and metabolic dysfunction in children.

Development of a national Zoom-based lectureship for pediatric transplant hepatology trainees.

Background: Previous publications identified a gap in standard education on topics related to advanced hepatology and liver transplantation for pediatric transplant hepatology trainees. The Society of Pediatric Liver Transplantation (SPLIT) Education Committee designed a Zoom-based lectureship series for all advanced pediatric transplant hepatology trainees. We aim to describe the educational series and feedback from fellow participants.

Living donor liver transplant candidate and donor selection and engagement: Meeting report from the living donor liver transplant consensus conference.

Introduction: Living donor liver transplantation (LDLT) is a promising option for mitigating the deceased donor organ shortage and reducing waitlist mortality. Despite excellent outcomes and data supporting expanding candidate indications for LDLT, broader uptake throughout the United States has yet to occur.

Methods: In response to this, the American Society of Transplantation hosted a virtual consensus conference (October 18-19, 2021), bringing together relevant experts with the aim of identifying barriers to broader implementation and making recommendations regarding strategies to address these barriers.

Epstein-Barr Virus DNAemia and post-transplant lymphoproliferative disorder in pediatric solid organ transplant recipients.

Background: Pediatric solid organ transplant (SOT) recipients commonly have Epstein-Barr virus (EBV) DNAemia and are at risk of developing post-transplant lymphoproliferative disorder (PTLD). EBV DNAemia has not been analyzed on a continuous scale in this population.

Methods: All children ≤ 18 years of age who underwent SOT at a single center between January 1, 2007 and July 31, 2018 were included in this retrospective study.

Clinical insights from Wolman disease: Evaluating infantile hepatosplenomegaly.

There is a broad differential diagnosis of infantile hepatosplenomegaly, with some etiologies being debilitating and treatable. A structured approach to history, examination, and laboratory and radiographic findings is important in diagnosis. Herein, we present a case of Wolman disease presenting as hepatosplenomegaly in an infant.

Acceptability of an mHealth Family Self-management Intervention (myFAMI) for Pediatric Transplantation Families: Qualitative Focus.

Background: Around 1800 pediatric transplantations were performed in 2021, which is approximately 5% of the annual rate of solid organ transplantations carried out in the United States. Effective family self-management in the transition from hospital to home-based recovery promotes successful outcomes of transplantation. The use of mHealth to deliver self-management interventions is a strategy that can be used to support family self-management for transplantation recipients and their families.

Alterations in DNA methylation associate with fatty liver and metabolic abnormalities in a multi-ethnic cohort of pre-teenage children.

Non-Alcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Epigenetic alterations, such as through DNA methylation (DNAm), may link adverse childhood exposures and fatty liver and provide non-invasive methods for identifying children at high risk for NAFLD and associated metabolic dysfunction. We investigated the association between differential DNAm and liver fat content (LFC) and liver injury in pre-adolescent children.

Feasibility and Acceptability of a mHealth Self-Management Intervention for Pediatric Transplant Families.

Families of pediatric solid organ transplant recipients need ongoing education and support in the first 30 days following hospital discharge for the transplantation. The purpose of this report is to describe the feasibility, acceptability, and preliminary efficacy of a mHealth family-self management intervention, (), designed to improve post-discharge outcomes of coping, family quality of life, self-efficacy, family self-management, and utilization of health care resources. We enrolled 46 primary family members.

Efficacy and safety of mammalian target of rapamycin inhibitors following intestinal and multivisceral transplantation.

This is a descriptive study reviewing the outcomes of mammalian target of rapamycin inhibitors (mTORs) in intestinal (IT) and multivisceral transplantation (MVT). This study included 22 patients, 20 adults, and two children, and an overall mean age of 46 years old at the time of transplantation. Twelve patients (54.

Liver fibrosis during clinical ascertainment of glycogen storage disease type III: a need for improved and systematic monitoring.

Purpose: In glycogen storage disease type III (GSD III), liver aminotransferases tend to normalize with age giving an impression that hepatic manifestations improve with age. However, despite dietary treatment, long-term liver complications emerge. We present a GSD III liver natural history study in children to better understand changes in hepatic parameters with age.

MR Imaging Evaluation of Inflammatory Bowel Disease in Children:: Where Are We Now in 2019.

Inflammatory bowel disease (IBD) is an important cause of abdominal pain in the pediatric population. Magnetic resonance enterography (MRE) plays a crucial role in assessment of disease severity, location, extent of disease, and assessment for associated complications. As MR imaging technology has advanced, new techniques have been brought into clinical practice.

Defining Pediatric Failure to Thrive in the Developed World: Validation of a Semi-Objective Diagnosis Tool.

There is no concordance between current diagnostic criteria for failure to thrive (FTT). We analyzed validity of the Semi-Objective Failure to Thrive (SOFTT) diagnosis tool, which uses a combination of subjective and objective components to make the diagnosis of FTT. The tool was used to diagnose FTT in 94 patients who met 1 of 7 accepted criteria for FTT.

Improved contemporary outcomes of liver transplantation for pediatric hepatoblastoma and hepatocellular carcinoma.

Purpose: Improvement in outcomes of LT for pediatric HB and HCC has been reported in small series. We analyzed national outcomes and changes in donor, recipient, and perioperative factors over time that may contribute to survival differences.

Methods: The UNOS database was queried for patients age <21 years that underwent LT for a primary diagnosis of HB or HCC (1987-2017).

Cystic biliary atresia: A distinct clinical entity that may mimic choledochal cyst.

Cystic biliary atresia (CBA) is a relatively uncommon but clinically significant variant of biliary atresia. The presence of a cyst in the hepatic hilum on imaging in an infant with cholestasis supports the diagnosis of CBA, but can also be seen in patients with a choledochal cyst-the main differential diagnosis in patients with CBA. The reported case outlines the clinical presentation and imaging findings in a patient with surgically confirmed and treated CBA and emphasizes the importance of distinguishing CBA from choledochal cyst at diagnostic imaging given the disparate timing and type of surgical treatment necessary for successful management of these distinct entities.

Vulnerability and chronic illness management in pediatric kidney and liver transplant recipients.

Context: Solid-organ transplant is the treatment of choice for end-stage organ failure and requires a transition from management of a life-threatening condition to a chronic illness. Despite research focusing on quality of life after transplant, there is a gap addressing the role of managing a chronic illness focusing on vulnerability and impact on family.

Objective: Identify patient and family patterns of adaptation among kidney and liver transplant recipients in regard to (1) vulnerability, (2) impact of illness on the family, (3) family functioning, and (4) quality of life (parent and child report).

Kinetics of phytosterol metabolism in neonates receiving parenteral nutrition.

Background: Phytosterols in soybean oil (SO) lipids likely contribute to parenteral nutrition-associated liver disease (PNALD) in infants. No characterization of phytosterol metabolism has been done in infants receiving SO lipids.

Methods: In a prospective cohort study, 45 neonates (36 SO lipid vs.

Adherence to Infliximab Treatment in a Pediatric Inflammatory Bowel Disease Cohort.

The aims of the study were to describe infliximab adherence in a pediatric inflammatory bowel disease cohort, to identify demographic and disease factors associated with adherence, and to examine differences in acute care use among adherent and nonadherent patients. Charts of patients who received infliximab at the Children's Hospital of Wisconsin (CHW) between October 2010 and October 2012 were retrospectively reviewed. A total of 151 patients met the inclusion criteria; 91.

Liver disease in the adolescent.

This article discusses common liver diseases in the adolescent. Briefly reviewed is the evaluation of the adolescent with new-onset liver enzyme elevation. Then the article discusses common liver diseases, such as nonalcoholic fatty liver disease, hepatitis, metabolic disease, biliary atresia, cystic fibrosis, and inherited disorders of cholestasis.

Respiratory syncytial virus and pediatric liver transplant: one center's experience.

Respiratory syncytial virus (RSV) is a ubiquitous virus responsible for acute infections of the respiratory tract in patients of all ages. RSV presents significant health risks to immunocompromised patients. Two patients, 1 before a liver transplant and 1 after a liver transplant, died of a severe RSV infection.