Phenotypic Divergence of JAG1- and NOTCH2-Associated Alagille Syndrome & Disease-Specific NOTCH2 Variant Classification Guidelines.

Background & Aims: Alagille syndrome (ALGS) is a rare, autosomal dominant disorder with high phenotypic heterogeneity. Disease-causing variants are primarily identified in Jagged1 (JAG1), with fewer reported in NOTCH2. JAG1 variants cause disease through a mechanism of haploinsufficiency, but the mechanism for NOTCH2 variants is not completely understood, making classification of variants more challenging.

A Home-Like Setting and a Multidisciplinary Team in the Care of Kidnapped Children: A Retrospective Descriptive Study.

Aim: In October 2023, Hamas kidnapped 257 Israelis, including 38 children. Most of the children and some of the women were released after 49-54 days. Data or protocols for managing children returning from captivity are scarce.

Fluid Overload and Graft Injury Following Pediatric Liver Transplantation: A Single-Center Analysis.

We aimed to compare graft injury and complications after liver transplantation in children with higher versus lower fluid balance. In a cohort of 79 pediatric liver transplant recipients, we analyzed the associations of decreases in alanine aminotransferase (ALT) and bilirubin (delta ALT, delta bilirubin) with fluid balance in the first six postoperative days and associations of fluid balance with vascular complications and mean ALT one year after the transplantation. Patients who developed vascular complications had significantly higher mean cumulative fluid balance during the first three postoperative days, as well as higher mean fluid balance on postoperative days 0 (POD0) and 2 (POD2) ( < 0.

Finger thoracostomy: Significant risks and unproven benefits in prehospital settings.

Background: Trauma is a leading cause of preventable death, with a significant portion of trauma deaths occurring in the prehospital setting. Interventions such as chest drainage may play a critical role in managing life-threatening conditions but face challenges due to poorly defined indications and reliance on anecdotal evidence rather than rigorous studies. Among chest drainage techniques, finger thoracostomy (FT) is a well-described, but controversial, method for decompressing the pleural cavity in emergencies like tension pneumothorax or hemothorax.

Long-term laboratory follow-up is essential in pediatric patients with celiac.

Objectives: Celiac disease (CeD) requires long-term follow. The role of laboratory testing other than celiac serology during follow up is unclear. We aimed to determine which laboratory tests are required during follow up based on the prevalence of abnormal tests and timing of abnormalities appearance.

Primary antibiotic prophylaxis in biliary atresia did not demonstrate decreased infection rate: Multi-centre retrospective study.

Aim: This retrospective study aimed to assess the efficacy of prophylactic antibiotics in preventing ascending cholangitis following Kasai portoenterostomy (KPE). Data from 72 patients treated across four tertiary centres in Israel from 2008 to 2018 were analysed.

Methods: Clinical and laboratory data were collected from biliary atresia (BA) diagnosis until liver transplantation (LT) or study completion.

An innocent bystander or a predisposing culprit? Kidney injury following pediatric liver transplantation.

Background: Survival after pediatric liver transplantation has increased dramatically over the years, revealing extra-hepatic complications including impaired kidney function. We conducted a large single-center retrospective study to evaluate kidney outcomes after pediatric liver transplantation.

Methods: From electronic charts of 121 children who underwent liver transplantation during 2007-2020, we collected pre- and post-transplant data.

Medical perspectives on Israeli children after their release from captivity - A retrospective study.

Aim: Following the Hamas terror attack on Israeli towns on October 2023, 250 individuals were taken into captivity. On November-December 2023, during the cease-fire deal, 26 women and children were released. This study is the first to describe the physical and behavioural findings in children and their mothers in the immediate phase of returning home from captivity.

Biliary strictures post pediatric liver transplantation-incidence and risk factors in a single tertiary referral transplant center.

Background: Biliary strictures are a significant cause of morbidity and graft loss in pediatric liver transplant recipients. Risk factors for the development of biliary strictures are not fully established. We aimed to evaluate the incidence of biliary strictures and treatment modalities outcomes and to identify potential risk factors for occurrence.

A significant increase in anthropometric indices during long-term follow-up of pediatric patients with celiac disease, with no endocrine disorders.

Celiac disease (CeD) is likely to be associated with growth impairment and poor weight gain. However, long-term growth patterns following diagnosis are poorly characterized. We evaluated long-term anthropometric changes in a large cohort of pediatric patients with CeD.

Clinical profile of re-hospitalizations in pediatric kidney and liver transplant recipients.

Background: Solid organ transplantation has evolved in recent decades, resulting in a rise in patient and graft survival. Frequent hospitalizations affect graft function, patients' health, and quality of life. This study characterizes the frequency and causes of post-transplant hospitalizations among pediatric recipients.

Cell-based BSEP trans-inhibition: A novel, non-invasive test for diagnosis of antibody-induced BSEP deficiency.

Background & Aims: Antibody-induced bile salt export pump deficiency (AIBD) is an acquired form of intrahepatic cholestasis, which may develop following orthotopic liver transplantation (OLT) for progressive familial intrahepatic cholestasis type 2 (PFIC-2). Approximately 8-33% of patients with PFIC-2 who underwent a transplant develop bile salt export pump (BSEP) antibodies, which trans-inhibit this bile salt transporter from the extracellular, biliary side. AIBD is diagnosed by demonstration of BSEP-reactive and BSEP-inhibitory antibodies in patient serum.

Adaptive immune response to BNT162b2 mRNA vaccine in immunocompromised adolescent patients.

Protective immunity against COVID-19 is orchestrated by an intricate network of innate and adaptive anti-viral immune responses. Several vaccines have been rapidly developed to combat the destructive effects of COVID-19, which initiate an immunological cascade that results in the generation of neutralizing antibodies and effector T cells towards the SARS-CoV-2 spike protein. Developing optimal vaccine-induced anti-SARS- CoV-2 protective immunity depends on a fully competent immune response.

Prehospital Tranexamic Acid Administration in Pediatric Trauma Patients: A Propensity-Matched Analysis of the Israeli Defense Forces Registry.

Objectives: Tranexamic acid (TXA) administration confers a survival benefit in bleeding trauma patients; however, data regarding its use in pediatric patients are limited. This study evaluates the prehospital treatment with TXA in pediatric trauma patients treated by the Israel Defense Forces Medical Corps (IDF-MC).

Design: Retrospective, cohort study using the Israel Defense Forces registry, 2011-2021.

Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency.

Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.

Acute Hepatitis of Unknown Etiology Among Young Children: Research Agenda by the ESPGHAN Hepatology Committee.

In April 2022, an increased incidence of acute hepatitis cases of unknown etiology among previously healthy children across the United Kingdom was described. Since, more than 270 cases from the United Kingdom and hundreds more from all across the world have been reported. The majority of affected children were younger than 6 years of age.

Long COVID-19 Liver Manifestation in Children.

Objectives: Severe acute respiratory syndrome coronavirus 2, the novel coronavirus responsible for coronavirus disease (COVID-19), has been a major cause of morbidity and mortality worldwide. Gastrointestinal and hepatic manifestations during acute disease have been reported extensively in the literature. Post-COVID-19 cholangiopathy has been increasingly reported in adults.

Portal plate bile duct diameter in biliary atresia is associated with long-term outcome.

Purpose: Kasai portoenterostomy (KPE) is the only treatment currently available for biliary atresia (BA). Age at KPE and surgical experience are prognostic factors for a successful KPE. Here, we aimed to assess whether the size of bile ductules at the porta hepatis during KPE correlates with KPE success and transplant-free survival (TFS).

Treatment adherence and behavior of pediatric liver transplant recipients during the COVID-19 pandemic.

Background: The COVID-19 pandemic has affected medical care worldwide. Thus, we aimed to assess the impact of the COVID-19 pandemic on pediatric LT recipients.

Methods: A cross-sectional study based on a structured internet or telephone survey was conducted among pediatric LT recipients.

Body composition correlates with laboratory parameters and disease severity in infants with biliary atresia.

Aim: Infants with biliary atresia (BA) generally have chronic malnutrition. However, the best anthropometric measure to assess malnutrition and its correlation with disease severity is unknown. We aimed to assess correlations of various anthropometric measurements, including air displacement plethysmography (ADP), with laboratory parameters and with the pediatric end-stage liver disease (PELD) score in infants with BA.