Utilization of Segmental Grafts Is Associated With Higher Transplant Rates in Pediatric Patients.

Introduction: In July 2017, a policy to increase the use of segmental grafts (SGs) was implemented at our institution. The aim was to compare changes in waitlist activity after implementation of this policy.

Methods: A single-center, retrospective study.

Fractionated Bilirubin Among 252 892 Utah Newborns with and Without Biliary Atresia: A 15-year Historical Birth Cohort Study.

Objectives: To determine whether neonatal conjugated or direct bilirubin levels were elevated in infants with biliary atresia (BA) and to estimate the number of newborns who would have positive screens in the nursery necessitating repeat testing after discharge.

Study Design: We used administrative data from a large integrated healthcare network in Utah to identify newborns who had a fractionated bilirubin recorded during birth admission from 2005 through 2019. Elevated conjugated bilirubin was defined as greater than 0.

Serum biomarkers correlated with liver stiffness assessed in a multicenter study of pediatric cholestatic liver disease.

Background And Aims: Detailed investigation of the biological pathways leading to hepatic fibrosis and identification of liver fibrosis biomarkers may facilitate early interventions for pediatric cholestasis.

Approach And Results: A targeted enzyme-linked immunosorbent assay-based panel of nine biomarkers (lysyl oxidase, tissue inhibitor matrix metalloproteinase (MMP) 1, connective tissue growth factor [CTGF], IL-8, endoglin, periostin, Mac-2-binding protein, MMP-3, and MMP-7) was examined in children with biliary atresia (BA; n = 187), alpha-1 antitrypsin deficiency (A1AT; n = 78), and Alagille syndrome (ALGS; n = 65) and correlated with liver stiffness (LSM) and biochemical measures of liver disease. Median age and LSM were 9 years and 9.

Revision of meso-Rex bypass utilizing a collateral vein in a patient with portal steal phenomenon after liver transplant: A case report.

Introduction: In children with extrahepatic portal vein obstruction or those who develop portal vein thrombosis after liver transplant, the use of Meso-Rex Bypass (MRB) creates a more physiological state by redirecting mesenteric blood flow back into the intrahepatic portal system via a venous conduit.

Presentation Of Case: A 3-year-old female with biliary atresia associated with polysplenia syndrome and a surgical history of Kasai portoenterostomy procedure, and an ABO incompatible whole liver transplant. Within a year after transplant she presented with prehepatic portal hypertension, that was treated with MRB using a deceased donor ABO compatible iliac vein as conduit.

The Impact of Increased Allocation Priority for Children Awaiting Liver Transplant: A Liver Simulated Allocation Model (LSAM) Analysis.

Objective: The aim of the study was to investigate the impact of prioritizing infants, children, adolescents, and the sickest adults (Status 1) for deceased donor livers. We compared outcomes under two "SharePeds" allocation schema, which prioritize children and Status 1 adults for national sharing and enhanced access to pediatric donors or all donors younger than 35 years, to outcomes under the allocation plan approved by the Organ Procurement and Transplant Network in December 2017 (Organ Procurement and Transplantation Network [OPTN] 12-2017).

Methods: The 2017 Liver Simulated Allocation Model and Scientific Registry of Transplant Recipients data on all US liver transplant candidates and liver offers 7/2013 to 6/2016 were used to predict waitlist deaths, transplants, and post-transplant deaths under the OPTN 12-2017 and SharePeds schema.

Frailty in Children with Liver Disease: A Prospective Multicenter Study.

Objective: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool.

Study Design: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage.

Identification of Pediatric Patients With Celiac Disease Based on Serology and a Classification and Regression Tree Analysis.

Background & Aims: Celiac disease is detected using serology and endoscopy analyses. We used multiple statistical analyses of a geographically isolated population in the United States to determine whether a single serum screening can identify individuals with celiac disease.

Methods: We performed a retrospective study of 3555 pediatric patients (18 years old or younger) in the intermountain West region of the United States from January 1, 2008, through September 30, 2013.

Neonatal Cholestasis Caused by Undiagnosed Maternal Graves' Disease.

Neonatal cholestasis results from a variety of etiologies, including anatomic, infectious, and metabolic abnormalities. Hyperthyroidism, in contrast to hypothyroidism, is infrequently associated with neonatal cholestasis. Newborn screening is an important tool to detect newborn metabolic disorders, including thyroid dysfunction.

Perceptions of gender equality in work-life balance, salary, promotion, and harassment: results of the NASPGHAN task force survey.

Objectives: Gender equality in the workplace has not been described in pediatric gastroenterology.

Methods: An electronic survey that explored perceptions of career parity, work-life balance, and workplace harassment was sent to all members of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Reponses were anonymous.

Renal replacement therapy in infants and children with hepatorenal syndrome awaiting liver transplantation: a case-control study.

Limited data on short- and long-term outcomes of renal replacement therapy (RRT) in pediatric liver transplantation (LT) patients exist. We evaluated risk factors for RRT in pediatric LT recipients with hepatorenal syndrome (HRS) and described the outcomes. We performed a single-center, case-control study of LT recipients who required RRT for HRS from 1999 to 2011.

Cholangiocarcinoma and high-grade dysplasia in young patients with primary sclerosing cholangitis.

Introduction: Cholangiocarcinoma (CCA) is very often an adulthood disease with primary sclerosing cholangitis (PSC) as one of the risk factors. It is rarely seen in the pediatric population, and when it is diagnosed before adulthood, it can be associated with PSC as well as HIV infection, biliary atresia, radiation therapy, and choledochal cyst. Although there have been some case reports of pediatric CCA, cases of childhood CCA associated with PSC are still relatively rare.

Outcome after discontinuation of immunosuppression in children with autoimmune hepatitis: a population-based study.

Objective: To assess sustained immunosuppression-free remission (SIFR) in children with autoimmune hepatitis (AIH).

Study Design: We retrospectively reviewed all children with AIH in the region between 1986 and 2011 using a population-based methodology.

Results: We identified 56 children with AIH (62.

Effect of decreased parenteral soybean lipid emulsion on hepatic function in infants at risk for parenteral nutrition-associated liver disease: a pilot study.

Purpose: We performed a pilot trial to compare reduced dose versus standard soybean lipid emulsion in neonates at risk for parenteral nutrition-associated liver disease.

Methods: A prospective randomized controlled trial was performed (2009-2011) enrolling surgical patients ≥ 26 weeks' gestation anticipated to require >50% of daily caloric intake from parenteral nutrition (PN) for at least 4 weeks. Randomization occurred into either reduced (1.

Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history.

Unlabelled: The epidemiology and natural history of pediatric primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH) are not well characterized. Using multiple, overlapping search strategies followed by a detailed records review, we identified all cases of pediatric PSC, ASC, AIH, and inflammatory bowel disease (IBD) in a geographically isolated region of the United States. We identified 607 cases of IBD, 29 cases of PSC, 12 cases of ASC, and 44 cases of AIH.

Liver transplantation in a 7-month-old girl with Caroli's disease.

Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension.

Clinical variables as prognostic tools in pediatric-onset ulcerative colitis: a retrospective cohort study.

Background: Clinical variables may identify a subset of patients with pediatric-onset ulcerative colitis (UC) (≤18 years at diagnosis) at risk for adverse outcomes. We postulated that routinely measured clinical variables measured at diagnosis would predict colectomy in patients with pediatric-onset UC.

Methods: We conducted a chart review of patients with pediatric-onset UC at a single center over a 10-year period.

Novel screening assay performance in pediatric celiac disease and adult dermatitis herpetiformis.

Objectives: : Several serologic assays are commercially available to aid in the diagnosis of gluten-sensitive enteropathy (GSE). Our objective in this study was to assess the performance of a novel combined antigen-screening assay for GSE.

Patients And Methods: : Deidentified sera from 111 pediatric patients suspected of having celiac disease (CD), 130 adults diagnosed with dermatitis herpetiformis (DH), and 77 pediatric and 49 adult normal controls were included in the study.

Elimination of soybean lipid emulsion in parenteral nutrition and supplementation with enteral fish oil improve cholestasis in infants with short bowel syndrome.

Background: Parenteral nutrition-associated liver disease (PNALD) is a potentially fatal complication for children with intestinal failure. Fish oil-based lipid emulsions have shown promise for the treatment of PNALD but are not readily available. Six cases are presented in which cholestasis resolved after soybean lipid emulsion (SLE) was removed from parenteral nutrition (PN) and enteral fish oil was given.

Closed gastroschisis: total parenteral nutrition-free survival with aggressive attempts at bowel preservation and intestinal adaptation.

Background: In infants with gastroschisis antenatal closure of the umbilical defect results in a proximal atresia with ischemia and/or volvulus of the extracorporeal midgut. It has been described as "closed gastroschisis" or "vanishing midgut."

Methods: A 10-year review of 219 gastroschisis patients identified 10 infants with this rare complication.

Strongly positive tissue transglutaminase antibodies are associated with Marsh 3 histopathology in adult and pediatric celiac disease.

Goals: Our objective was to determine whether high serologic IgA tissue transglutaminase antibodies (TTGA) are exclusively associated with celiac disease (CD).

Background: IgA TTGA are found in the serum of most individuals with CD. This serologic marker is used to screen individuals with suspected CD for duodenal biopsy, the gold standard of CD diagnosis.