Primary and Secondary Prophylaxis of Gastrointestinal Bleeding in Children with Portal Hypertension: A Multicenter National Study by SIGENP.

: Portal hypertension (PH) is a common complication in children with chronic liver diseases. Primary and secondary prophylaxis of variceal bleeding in these patients remains controversial. Our study aims to evaluate the management of gastrointestinal (GI) varices in children with PH in Italy.

Foreign Body Ingestion in Children: A 16-year Experience.

Purpose: Foreign body (FB) ingestion is a common health problem that affects children. It requires endoscopic removal in up to 20% of cases. In this study, we reviewed all our pediatric cases of FB ingestion requiring endoscopy and compared their management to the published guidelines.

Odevixibat for Episodic Intrahepatic Cholestasis due to Biallelic Mutations in ATP8B1: A Case Series.

Background & Aims: In episodic intrahepatic cholestasis (IC; known historically as benign recurrent intrahepatic cholestasis), intermittent cholestasis is typically followed by periods of remission. During cholestatic episodes, symptoms can include jaundice, fatigue, abdominal pain, diarrhoea, and severe pruritus that can interfere with patients' lives. Here, we report clinical features and response to odevixibat, an ileal bile acid transporter inhibitor, in patients with episodic IC associated with biallelic mutations in ATP8B1.

Variability in the management of portal hypertension across European countries: A survey-study by ESPGHAN Portal Hypertension Special Interest Group.

Portal hypertension (PHT) remains a significant burden in children with chronic liver disease (CLD) or portal vein thrombosis (PVT). Management strategies vary depending on the underlying cause and clinical presentation. There is a lack of consensus on various aspects of PHT, including the optimal approach to screening, prophylactic endoscopy and treatment, and pharmacological interventions.

Cytomegalovirus-RNA Accurately Identifies Clinically Significant Infection Needing Preemptive Therapy in Liver Transplanted Children: A Proof-of-Concept Study.

Preemptive therapy (PET) is safe and effective in controlling Cytomegalovirus (CMV) infection after pediatric liver transplantation (LT) and allows to observe the kinetics of quantitative CMV-DNA viral load till it reaches the treatment thresholds. While early detection of low-to-moderate CMV-DNA levels may not indicate active viral replication, awaiting the viral load to exceed the treatment threshold may lead to viremic breakthroughs and CMV disease. We assessed the capacity of quantitative CMV-RNA (UL21.

Real-world experience with odevixibat in children with progressive familial intrahepatic cholestasis.

Background & Aims: A previously published trial demonstrated that odevixibat is effective in the treatment of cholestatic pruritus of children with progressive familial intrahepatic cholestasis (PFIC). Real-world experience is necessary to confirm the results of registration trials with selective eligibility criteria. We present our 'real-life experience' of the effectiveness and safety of odevixibat in patients with different PFIC subtypes.

Genotypes and different clinical variants between children and adults in progressive familial intrahepatic cholestasis: a state-of-the-art review.

Introduction: Progressive Familial intrahepatic cholestasis (PFIC) are rare disorders of bile acid (BAs) secretion and transport with a genetic background. PFIC are paediatric manifestations, but the same variants causing PFIC can also cause cholestasis with a later paediatric onset or adult-onset cholestatic disease (AOCD). Pruritus is a symptom of cholestasis that can be so devastating that it requires a liver transplant (LT) in children; some PFIC types have been described as at risk of liver cancer development.

Acute-on-chronic liver failure (ACLF): the 'Kyoto Consensus'-steps from Asia.

Acute-on-chronic liver failure (ACLF) is a condition associated with high mortality in the absence of liver transplantation. There have been various definitions proposed worldwide. The first consensus report of the working party of the Asian Pacific Association for the Study of the Liver (APASL) set in 2004 on ACLF was published in 2009, and the "APASL ACLF Research Consortium (AARC)" was formed in 2012.

Percutaneous management of chronic total occlusion of the portal vein: a retrospective analysis of technical aspects and outcomes.

Background: Chronic total occlusion (CTO) of the portal vein is one of the main causes of portal hypertension, which may result in life-threatening complications often managed by interventional radiology (IR). The aim of this study is to report the innovative experience with percutaneous revascularization therapy in the management of portal vein CTO in paediatric and adult patients.

Materials And Methods: From January 2020 to December 2023 consecutive paediatric and adult patients with severe portal hypertension resulting from portal vein CTO who underwent attempts at percutaneous recanalization were retrospectively reviewed.

Early steroids after pediatric liver transplantation protect against T-cell-mediated rejection: Results from the ChilSFree study.

Steroid-free immunosuppression protocols gained popularity in pediatric liver transplantation (pLT) after the introduction of IL-2-receptor blockade for induction therapy. We analyzed the clinical and immunologic outcome data of the multicenter prospective observational ChilSFree study to compare the impact of steroid-free versus steroid-containing immunosuppressive therapy following pLT in a real-life scenario. Two hundred forty-six children [55.

Outcome of Children with Transjugular Intrahepatic Portosystemic Shunt: A Meta-Analysis of Individual Patient Data.

Purpose: The purpose of the study was to investigate outcome after pediatric transjugular intrahepatic portosystemic shunt (TIPS) with respect to survival MATERIAL AND METHODS: After searching for studies on TIPS in children in Ovid, Medline, Embase, Scopus and Cochrane published between 2000 and 2022, individual patient data were retrieved from five retrospective cohorts. Overall survival (OS) and transplant-free survival (TFS) were calculated using Kaplan-Meier analysis and log-rank test and compared to the indication (ascites vs. variceal bleeding) as well as to the level of obstruction (pre-hepatic vs.

Treatment with an ileal bile acid transporter inhibitor in patients with TJP2 deficiency.

There are no published data on the use of odevixibat, a selective ileal bile acid transporter (IBAT) inhibitor, in children with tight junction protein 2 (TJP2) deficiency (also named as PFIC-4). We describe a case series of five children treated with odevixibat. After treatment, serum bile acids (sBA) decreased compared to baseline [mean value: 244 (±125), vs 38 (±34) µmol/L; p = 0.

Interim results from an ongoing, open-label, single-arm trial of odevixibat in progressive familial intrahepatic cholestasis.

Background & Aims: PEDFIC 2, an ongoing, open-label, 72-week study, evaluates odevixibat, an ileal bile acid transporter inhibitor, in patients with progressive familial intrahepatic cholestasis.

Methods: PEDFIC 2 enrolled and dosed 69 patients across two cohorts; all received odevixibat 120 μg/kg per day. Cohort 1 comprised children from PEDFIC 1, and cohort 2 comprised new patients (any age).

Identifying the Aetiology of Acute Liver Failure Is Crucial to Impact Positively on Outcome.

Management of children with acute liver failure is challenging. In this retrospective study, paediatric patients diagnosed with ALF at our centre, in the last 26 years, were divided into two groups (G1 = diagnosed from 1997 to 2009; G2 = from 2010 to 2022) and compared to see whether they differed with regard to aetiologies, need for liver transplantation (LT), and outcome. A total of 90 children (median age 4.

Paediatric porto-sinusoidal vascular disease: Two different clinical phenotypes with subtle histological differences.

Background And Aims: In paediatrics, porto-sinusoidal vascular disease (PSVD) is relatively unknown and probably underdiagnosed. We aimed to describe clinical phenotypes, histology and outcome of children diagnosed with PSVD.

Methods: Retrospective multicentre study of children diagnosed with PSVD.

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.

Case Report: Add-on treatment with odevixibat in a new subtype of progressive familial intrahepatic cholestasis broadens the therapeutic horizon of genetic cholestasis.

Odevixibat, an ileal bile acid transporter (IBAT) inhibitor, is effective for the treatment of pruritus in children diagnosed with progressive familial intrahepatic cholestasis (PFIC) type 1 and 2. There are no studies showing the efficacy of Odevixibat in children with different subtypes of PFIC. We describe the case of a 6-year-old girl with chronic cholestatic jaundice.

Pediatric Gastroenterology and Hepatology in Italy before and after the COVID-19: Lessons learned and management changes by SIGENP.

Around the world, the 2019 Coronavirus disease (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has raised serious public health problems and major medical challenges. The Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) published several papers on the impact of COVID-19 on the current management, diagnosis, and treatment of acute and chronic gastrointestinal, hepatic, immune-mediated, and functional disorders. The present article summarizes the most relevant SIGENP reports and consensus during and after the peak of the COVID-19 outbreak, including the diagnosis and treatment of inflammatory bowel disease (IBD), indications and timing of digestive endoscopy, and insights into the novel hepatitis.

Diagnosing Acute Cellular Rejection after Paediatric Liver Transplantation-Is There Room for Interleukin Profiles?

Background: The current gold standard to diagnose T-cell-mediated acute rejection (TCMR) requires liver histology. Using data from the ChilSFree study on immune response after paediatric liver transplantation (pLT), we aimed to assess whether soluble cytokines can serve as an alternative diagnostic tool in children suspected to have TCMR.

Methods: A total of n = 53 blood samples obtained on the day of or up to 3 days before liver biopsy performed for suspected TCMR at median 18 days (range 7-427) after pLT in n = 50 children (38% female, age at pLT 1.

Cold Ischemia Time and Graft Fibrosis Are Associated with Autoantibodies after Pediatric Liver Transplantation: A Retrospective Cohort Study of the European Reference Network .

The reported prevalence of autoantibodies (AAB) (ANA, SMA, LKM, SLA) after pediatric liver transplantation (pLTX) varies considerably from 26-75%, but their clinical impact on outcome is uncertain. We aimed to study the prevalence of AAB after pLTX, their association with donor-, transplant-, and recipient-characteristics, and their relation to outcome. In our multicenter retrospective study, we aimed to clarify conflicting results from earlier studies.