Management of hepatic artery thrombosis and stenosis after pediatric liver transplantation: Variability and agreement in management practices.

Guidelines for managing hepatic artery thrombosis (HAT) and stenosis (HAS) after pediatric liver transplantation (pLT) are lacking, with heterogeneous local practices. This study aims to evaluate management practices for HAT and HAS after pLT. An online and paper-based survey was sent to 36 international pLT centers.

The association between childhood obesity and major adverse liver outcomes in adolescence and young adulthood.

Background & Aims: Paediatric obesity is associated with liver steatosis and injury. We aimed to investigate the association between paediatric obesity and the risk of major adverse liver outcomes (MALOs) during adolescence and adulthood.

Methods: A cohort study of children with overweight or obesity enrolled in the Swedish Childhood Obesity Treatment Register (1997-2020) was performed (n = 29,321).

Portal Vein Obstruction in Pediatric Liver Transplant Patients: An Evaluation of Self-Reported Management Practices.

Background And Aims: Portal vein obstruction (PVO) is a known complication after pediatric liver transplantation (pLT). Effective management strategies are crucial in improving patient outcomes. This study investigated the various practice patterns related to PVO management to clarify the degree of consensus on the diverse facets of care involved in addressing PVO after pLT.

Various Regimens for Autoimmune Hepatitis in Northern European Children Show Equivalent Outcomes at 1 Year: A Retrospective Study.

Objective: To compare the remission rates after 1 year of treatment between two regimens: tacrolimus (with or without prednisolone) or prednisolone (with or without azathioprine) in children with autoimmune liver disease.

Study Design: Retrospective data from children (n = 157) with biopsy-proven autoimmune liver disease in five tertiary northern European centers were included. Data were collected at presentation and after 3, 6, and 12 months.

Pediatric Autoimmune Pancreatitis: Clinical Findings and Outcomes in Sweden.

Introduction: Pediatric autoimmune pancreatitis (AIP) is a rare form of pancreatitis in children with poorly understood pathophysiology. It is a recognized risk factor for chronic pancreatitis in approximately 4% of pediatric cases. This study aims to describe the demographics, clinical characteristics, and outcomes of a large Swedish cohort of pediatric AIP patients.

Steatotic Liver Disease in Pediatric Obesity and Increased Risk for Youth-Onset Type 2 Diabetes.

Objective: To assess 1) the association between metabolic dysfunction-associated steatotic liver disease (MASLD) in pediatric obesity and youth-onset type 2 diabetes, 2) the joint effect of MASLD and intermediate hyperglycemia on type 2 diabetes risk, and 3) the effect of obesity treatment on type 2 diabetes risk.

Research Design And Methods: A cohort study using the Swedish Childhood Obesity Treatment Register (Barnobesitas Registret i Sverige [BORIS]) (1999-2020) linked with national registers was conducted. We included 10,346 children with overweight or obesity and 59,336 matched control individuals.

Good long-term outcomes of primary sclerosing cholangitis in childhood.

Background & Aims: Primary sclerosing cholangitis (PSC) is a rare progressive liver disease associated with inflammatory bowel disease (IBD). It is usually diagnosed in adults but can also present in children. Data on long-term outcomes of pediatric PSC are limited.

Empagliflozin for treating neutropenia and neutrophil dysfunction in 21 infants with glycogen storage disease 1b.

Empagliflozin has been successfully repurposed for treating neutropenia and neutrophil dysfunction in patients with glycogen storage disease type 1b (GSD 1b), however, data in infants are missing. We report on efficacy and safety of empagliflozin in infants with GSD 1b. This is an international retrospective case series on 21 GSD 1b infants treated with empagliflozin (total treatment time 20.

∆-3-oxo-5β-reductase deficiency: favorable outcome in 16 patients treated with cholic acid.

Background: Oral cholic acid therapy is an effective therapy in children with primary bile acid synthesis deficiencies. Most reported patients with this treatment have 3β-hydroxy-Δ-C-steroid oxidoreductase deficiency. The aim of the study was the evaluation of cholic acid therapy in a cohort of patients with the rarer Δ-3-oxosteroid 5β-reductase (Δ-3-oxo-R) deficiency.

A retrospective case-control study of gastrostomy use in children undergoing hematopoietic cell transplantation.

Background: Maintaining a good nutritional status during the hematopoietic cell transplantation (HCT) procedure is challenging in the pediatric population.

Methods: In a multicentric retrospective study, we compared the outcome of nutritional status and HCT-related parameters in 227 pediatric patients during and after HCT between 2005 and 2015. 112 patients received a gastrostomy before the start of HCT (GS group), and 115 did not receive a gastrostomy (NGS).

Risk and protective factors of non-alcoholic fatty liver disease in paediatric obesity: A nationwide nested case-control study.

Not all children with obesity carry a similar risk of non-alcoholic fatty liver disease (NAFLD). We investigated the effect of obesity severity, metabolic risk parameters, and obesity treatment outcome on later risk of NAFLD in paediatric obesity. We conducted a nested case-control study of children and adolescents enrolled in the Swedish Childhood Obesity Treatment Register (BORIS) (2001-2016).

[Microwave ablation of the spleen: An easy and gentle method for treatment of secondary splenomegaly in children. A case report].

Portal hypertension (PHT) in children may be caused by many different liver and vascular diseases and can lead to life threatening complications such as splenomegaly with thrombocytopenia. The treatment of choice for symptomatic splenomegaly is Partial Splenic Embolization (PSE) which is effective but painful, and therefore not offered to children younger than 10 years of age at the Karolinska University Hospital in Stockholm.  Percutaneous microwave ablation (MWA) is a well-established method in adults for the treatment of tumors in the liver and other organs.

Systematic Review and Meta-analysis of Histological Gastric Biopsy Aspects According to the Updated Sydney System in Children.

Objectives: A descriptive and comparative study of gastric histological aspects according to the updated Sydney classification (USC), obtained from Helicobacter pylori-positive versus H pylori-negative children referred for upper gastrointestinal endoscopy.

Methods: The Prisma method was used to perform a systematic review and meta-analysis. Selection criteria were based on following key words USC, H pylori, children, endoscopy, or biopsy.

Prevalence of increased transaminases and its association with sex, age, and metabolic parameters in children and adolescents with obesity - a nationwide cross-sectional cohort study.

Background: Childhood obesity increases the risk of non-alcoholic fatty liver disease marked by elevated alanine aminotransferase (ALT). This study investigated the prevalence of increased ALT in children and adolescents with obesity, and its associations with sex, age, degree of obesity, and metabolic parameters.

Methods: Individuals between 5 and 17.

Diagnostic disagreement between clinical standard histopathological- and retrospective assessment of histopathology-based gastrointestinal graft-versus-host disease in children.

Background: No previous paediatric study has evaluated the frequency of diagnostic disagreement between clinical standard histopathological assessment (CSHA) and retrospective, independent, histopathological assessment (RIHA) of gastrointestinal Graft-Versus-Host Disease (GI-GVHD) METHODS: In a retrospective cohort study, based on gastrointestinal biopsies collected from allogeneic HSCT-treated children (<18 years) with symptom-based GI-GVHD, we evaluated; disagreement of histopathology-based GI-GVHD diagnosis in CSHA vs RIHA, and potential clinical consequences of differences between the assessments. The CSHA-based diagnoses were retrieved from histopathology reports. The RIHA was performed by one pathologist, blinded to the CSHA outcomes and based on the minimal criteria for histopathology-based GI-GVHD diagnosis by the NIH 2014.

Helicobacter pylori Infection in Pediatric Patients Living in Europe: Results of the EuroPedHP Registry 2013 to 2016.

Objectives: The aim of the study was to assess clinical presentation, endoscopic findings, antibiotic susceptibility and treatment success of Helicobacter pylori (H. pylori) infected pediatric patients.

Methods: Between 2013 and 2016, 23 pediatric hospitals from 17 countries prospectively submitted data on consecutive H.

Clinical relevance of endoscopy with histopathological assessment in children with suspected gastrointestinal graft-versus-host disease.

Endoscopy with histopathological assessment is an established practice to confirm gastrointestinal graft-versus-host disease (GI-GVHD). However, the clinical relevance of this approach in children is incompletely evaluated. In a retrospective cohort study, we investigated the frequency of treatment changes in response to histopathological findings in all children (<18 years) in Sweden who underwent endoscopy for suspected GI-GVHD (2000-2013) after receiving hematopoietic stem cell transplantation.

Research priorities for liver glycogen storage disease: An international priority setting partnership with the James Lind Alliance.

The international liver glycogen storage disease (GSD) priority setting partnership (IGSDPSP) was established to identify the top research priorities in this area. The multiphase methodology followed the principles of the James Lind Alliance (JLA) guidebook. An international scoping survey in seven languages was distributed to patients, carers, and healthcare professionals to gather uncertainties, which were consolidated into summary questions.

Choice of Endoscopic Procedure in Children With Clinically Suspected Gastrointestinal Graft-versus-host Disease.

Objectives: Gastrointestinal graft-versus-host disease (GI-GVHD) is a potentially life-threatening complication after hematopoietic stem cell transplantation. Symptoms indicating GI-GVHD motivates endoscopy with biopsy sampling and histopathological confirmation. Optimal extent of endoscopy in children is, however, presently unknown.