Comparison of Long-Term Outcomes Achieved With Live Donor and Split Deceased Donor Liver Grafts in Pediatric Liver Transplantation.

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.

Ethnicity and socio-economic deprivation in children with intestinal failure in New Zealand: Disparities in incidence, but not in outcomes.

Aims: The New Zealand National Intestinal Failure and Rehabilitation Service (NZ-NIFRS) was established in October 2015 to gather longitudinal data on the aetiology, clinical course and outcomes of children with intestinal failure (IF). One main objective is to achieve health equity for patients with IF in NZ.

Methods: Clinical outcomes (enteral autonomy, parenteral nutrition (PN) dependence, death or intestinal transplantation) for IF patients diagnosed from October 2015 to 2018 were analysed; comparisons were made by ethnicity and socio-economic status (SES) using published 'prioritised-ethnicity' health data and the NZ index of deprivation, respectively.

Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA.

Background And Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the G lobal AL agille A lliance (GALA) study.

Small and large bowel anatomy is associated with enteral autonomy in infants with short bowel syndrome: A retrospective cohort study.

Background: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA.

Methods: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015.

Long-term outcomes of liver transplantation for homozygous familial hypercholesterolaemia in Australia and New Zealand.

Background And Aims: Homozygous familial hypercholesterolaemia (FH) causes severe cardiovascular disease from childhood. Conventional drug therapy is usually ineffective; lipoprotein apheresis (LA) is often required. Liver transplantation (LT) can correct the metabolic defect but is considered a treatment of last resort.

Long-term Outcome of Asymptomatic Patients With Graft Fibrosis in Protocol Biopsies After Pediatric Liver Transplantation.

Background: The histological prevalence of allograft fibrosis in asymptomatic children after liver transplantation (LT) is well documented. However, long-term graft and patient survival remain unclear. This retrospective multicenter study aims to determine the prevalence of allograft fibrosis and analyze the long-term outcome for patients transplanted in childhood.

Rising Incidence of Inflammatory Bowel Disease in South Asian Children in New Zealand-A Retrospective Population-Based Study.

Objectives: High rates of inflammatory bowel disease (IBD) are reported in children of South Asian (SA) descent in some western countries. This population-based study describes the incidence and clinical course of IBD in SA children compared to non-South Asian (NSA) children in New Zealand (NZ).

Methods: Children (≤15 years) with new-onset IBD presenting to a centralized tertiary referral center in Auckland, NZ from 2010 to 2020 were identified.

Atopy and allergy following solid organ transplantation: A 15-year experience.

Aim: There are increasing reports of atopy/allergy following solid organ transplantation, especially paediatric liver transplantation (LT) with minimal New Zealand (NZ) data. We describe the prevalence of transplant-acquired atopy and allergy (TAA) in NZ paediatric liver transplant recipients, compared to paediatric kidney and adult liver transplants.

Methods: TAA focussed health questionnaires were sent to patients selected from the NZ transplant registry (transplanted between January 2003 and December 2017).

Australasian paediatric gastroenterologist practices of coeliac disease diagnosis before and during the COVID-19 pandemic.

Aim: To explore the perceptions and practices of Australasian paediatric gastroenterologists in diagnosing coeliac disease (CD) before and during the COVID-19 pandemic.

Methods: Paediatric gastroenterologists in Australasia were invited via email to complete an anonymous online questionnaire over a 2-week period in 2021.

Results: The questionnaire was completed by 39 respondents: 33 from Australia and six from New Zealand (NZ) equating to a 66% response rate.

An international multicenter validation study of the Toronto listing criteria for pediatric intestinal transplantation.

Deciding which patients would benefit from intestinal transplantation (IT) remains an ethical/clinical dilemma. New criteria* were proposed in 2015: ≥2 intensive care unit (ICU) admissions, loss of ≥3 central venous catheter (CVC) sites, and persistently elevated conjugated bilirubin (CB ≥ 75 μmol/L) despite 6 weeks of lipid modification strategies. We performed a retrospective, international, multicenter validation study of 443 children (61% male, median gestational age 34 weeks [IQR 29-37]), diagnosed with IF between 2010 and 2015.

An Atypical Case of Very Early-onset Familial Adenomatous Polyposis Associated With Focal Cortical Dysplasia.

We describe a female toddler with rectal bleeding from extensive colonic polyposis, and diagnosed with familial adenomatous polyposis. She has epilepsy from infancy attributed to focal cortical dysplasia. Hepatoblastoma was diagnosed at 13 months of age.

Assessment of Disease-related Knowledge Among Children With Inflammatory Bowel Disease and their Family Using IBD-KID2: Evaluating Tool Generalizability.

Unlabelled: Children with inflammatory bowel disease (IBD) and their families benefit from improved knowledge of their disease and treatment. Knowledge levels of individual family members are infrequently studied but may identify where education is best directed. We aimed to assess disease-specific knowledge among children with IBD, parents, and siblings, using a validated assessment tool (IBD-KID2), and to establish generalizability of IBD-KID2.

Diagnostic utility of upper and lower gastrointestinal endoscopy for the diagnosis of acute graft-versus-host disease in children following stem cell transplantation: A 12-year experience.

Background: Endoscopically obtained mucosal biopsies are the gold standard for diagnosing acute graft-versus-host disease of the gastrointestinal tract (GI-GVHD). There is no consensus on the ideal endoscopic approach in children. We aimed to ascertain which gastrointestinal sites and endoscopic approaches were most helpful for diagnosing acute GVHD and whether clinical symptoms can guide the endoscopic approach.

Neonatal liver disease.

Neonatal liver disease encompasses many diagnoses, including structural and genetic aetiologies. Many have significant health implications requiring long-term specialist treatment including liver transplantation. Jaundice is a common presenting feature.

Paediatric eosinophilic oesophagitis in New Zealand: A 3-year prospective study.

Aim: Eosinophilic oesophagitis (EoE) is a rare, chronic, relapsing immune/antigen-mediated disease characterised by symptoms of oesophageal dysfunction, with a paucity of data among New Zealand (NZ) children. This 3-year prospective study aimed to characterise EoE diagnosed nationally and to describe initial treatment strategies adopted.

Methods: Information on new diagnoses of paediatric EoE was obtained via the New Zealand Paediatric Surveillance Unit, through monthly questionnaires.

Three-year outcomes of childhood inflammatory bowel disease in New Zealand: A population-based cohort study.

Background And Aim: High rates of inflammatory bowel disease (IBD) have been documented in New Zealand (NZ) children. The objectives of this study were to describe the outcomes and disease course of childhood IBD in the first 3 years following diagnosis.

Methods: All children diagnosed with IBD in 2015 in NZ were included.

Paediatric liver transplantation in Australia and New Zealand: 1985-2018.

Liver transplantation has become the standard of care for children with end-stage liver disease. In Australia and New Zealand, there are four paediatric liver transplant units, in Sydney, Melbourne, Brisbane and Auckland. Over the past 30 years, there have been significant changes to indications for transplant, as well as medical and surgical advances.

Infections among pediatric transplant candidates: An approach to decision-making.

Introduction: The presence of infections in the immediate pretransplant period poses challenges in decision-making. Delaying transplantation because of these infections may be required, but is associated with a risk to the potential recipient. The aim of this project was to develop a structured framework based on expert opinion to guide decision-making regarding the safety of transplantation for candidates with infection immediately before transplant, and to show how this framework can be applied to clinical scenarios.