The Natural History of Gastroesophageal Varices in Children With Portal Hypertension.

Background & Aims: In children with portal hypertension, the natural history of gastroesophageal varices remains unclear. We report our experience.

Methods: From 1990 to 2021, 1586 children with portal hypertension underwent ≥1 diagnostic upper gastrointestinal endoscopies before any treatment, including 590 with ≥2 endoscopies.

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.

Craniosynostosis as a cause of intracranial hypertension in Alagille syndrome: a case series of 6 consecutive pediatric patients.

Objective: Craniosynostoses are an underrecognized cause of intracranial hypertension (ICH), especially when associated with congenital syndromes. Alagille syndrome (ALGS) is a multisystem disorder with typical facial features and hepatobiliary, cardiac, vascular, skeletal, and ocular manifestations. The occurrence of craniosynostosis in ALGS is rare and can be associated with chronic ICH, requiring craniofacial surgery to increase the intracranial volume.

Pediatric hepatic hemangiomas: spectrum and prognostic significance of initial ultrasound findings.

Background: Hepatic hemangiomas may be associated with serious complications; however, it is unknown whether ultrasound (US) features can predict complications.

Objective: To analyze initial US features of hepatic hemangiomas predictive of complications.

Materials And Methods: This is a single-center retrospective cohort study of clinical, biological, and imaging data of infants with hepatic hemangioma between 2000 and 2018.

Outcomes of 38 patients with PFIC3: Impact of genotype and of response to ursodeoxycholic acid therapy.

Background & Aims: Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare liver disease caused by biallelic variations in . Data reporting on the impact of genotype and of response to ursodeoxycholic acid (UDCA) therapy on long-term outcomes are scarce.

Methods: We retrospectively describe a cohort of 38 patients with PFIC3 with a median age at last follow-up of 19.

Vaccination practices in pediatric transplantation: A survey among member centers of the European reference network TransplantChild.

Background: There is considerable variation in vaccination practices between pediatric transplant centers. This study aims to evaluate active immunization attitudes and practices among ERN-TransplantChild centers and identify potential areas of improvement that could be addressed by shared evidence-based protocols.

Methods: A cross-sectional questionnaire of attitudes and practices toward immunization of pediatric SOT and HSCT candidates and recipients was sent to a representative member of multidisciplinary teams from 27 European centers belonging to the ERN-TransplantChild.

Efficacy and Safety of Endoscopic Primary Prophylaxis of Bleeding in Children With High-Risk Gastroesophageal Varices.

Objectives: Primary prophylaxis of bleeding is debated in children with gastroesophageal varices; one of the reasons is the limited number of studies concerning its efficacy and safety. We report our experience with endoscopic primary prophylaxis.

Methods: From 2006 to 2019, 145 children (median age, 3.

Spontaneous evolution patterns of focal congenital hepatic hemangiomas: a case series of 25 patients.

Background: Hepatic hemangiomas are the most common benign liver tumors of infancy. They are termed congenital if fully developed at birth or infantile if they appear in the first weeks of life. Previous studies suggested that most focal hepatic hemangiomas are congenital in nature, exhibit no postnatal growth and have an evolution that parallels their cutaneous counterparts.

A Model for Early Endoscopic Detection of High-Risk Gastroesophageal Varices in Children With Biliary Atresia.

Objective: In children with biliary atresia and portal hypertension, progression to gastroesophageal varices carrying a risk of bleeding depends on age, total serum bilirubin concentration and initial endoscopic features. We report an attempt to use these factors for early detection of high-risk varices (HRVs).

Methods: Based on different combinations of these factors, a model was set to estimate the probabilities of emergence of HRVs at various time intervals.

Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey.

(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for comparison between international centers and between various organ transplant systems and modalities; (2) Methods: A cross-sectional questionnaire of relevant PTLD practices in pediatric transplantation was sent to multidisciplinary teams from 17 European center members of ERN TransplantChild to evaluate the centers' approach strategies for diagnosis and treatment and how current practices impact a cross-sectional series of PTLD cases; (3) Results: A total of 34 SOT programs from 13 European centers participated.

Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre.

Background: Congenital portosystemic shunts are rare vascular malformations that may have an impact on the heart-lung system. Associated congenital and/or acquired heart diseases are poorly reported.

Aims: To analyse cardiovascular disorders within a large congenital portosystemic shunt population, and develop a diagnostic strategy.

Long term results of liver transplantation for alpha-1 antitrypsin deficiency.

Introduction: Liver transplantation (LT) is the therapeutic option for end-stage liver disease associated with alpha1 antitrypsin (A1AT) deficiency. The aim of the present retrospective study was to report on long-term outcomes following LT for A1AT deficiency.

Methods: The medical records of 90 pediatric and adult patients transplanted between 1982 and 2017 in France and Geneva (Switzerland) were reviewed.

Long-term results of pediatric liver transplantation for autoimmune liver disease.

Background: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are rare indications for liver transplantation (LT) in children. The aim of the present retrospective multicenter study was to evaluate long-term outcome after LT for autoimmune liver disease in childhood.

Methods: Retrospective data from 30 children who underwent a first LT from 1988 to 2018 were collected.

Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction.

Chronic non cirrhotic extrahepatic portal vein obstruction (EHPVO) refers to the cavernomatous transformation of the portal vein (the so-called "portal cavernoma") which occurs following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic [1].

The French paediatric cohort of Castleman disease: a retrospective report of 23 patients.

Background: Castleman disease (CD) is a rare non-malignant lymphoproliferation of undetermined origin. Two major disease phenotypes can be distinguished: unicentric CD (UCD) and multicentric CD (MCD). Diagnosis confirmation is based on histopathological findings in a lymph node.

mTOR inhibitors in pediatric liver transplant recipients.

Background: During the past decade, mTOR inhibitors (mTORi), everolimus and sirolimus, have been increasingly used after adult liver transplantation (LT). The aim of the present study was to describe the use of mTORi in pediatric LT recipients.

Methods: All pediatric LT recipients who received mTORi before December 2017 from 4 European pediatric LT centers were included and analyzed.

Congenital portosystemic shunts: diagnosis and treatment.

Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia.

Prenatal diagnosis of intra hepatic arterio venous fistula: case report and review of the literature.

Aim: To describe the prenatal features and management of a congenital intra hepatic fistula.

Material And Methods: Case report Results: Congenital intra hepatic fistula are extremely rare. The prenatal ultrasound seiology is described.

Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding.

Background & Aims: Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues.

Methods: From 1989 to 2014, we managed 1300 children with portal hypertension.

Progression to high-risk gastroesophageal varices in children with biliary atresia with low-risk signs at first endoscopy.

Objectives: Biliary atresia carries a risk of bleeding because of portal hypertension. Our goal was to define the factors associated with the emergence of endoscopic signs carrying a high risk of bleeding in children who did not display these signs at the first upper gastrointestinal endoscopy.

Methods: From 1989 to 2013, a total of 225 children with low-risk signs at the first endoscopic examination underwent ≥2 upper gastrointestinal endoscopic examinations.

[Viral hepatitis in children and adolescents].

Vaccines have helped to reduce the transmission of hepatitis A and B. Mother-to-baby transmission during pregnancy is a frequent method of contamination.Viral hepatitis in an infant is a medical emergency: it is essential to be able to recognise a serious acute form quickly.

Experience with endoscopic management of high-risk gastroesophageal varices, with and without bleeding, in children with biliary atresia.

Background & Aims: Biliary atresia, the most common cause of childhood cirrhosis, increases the risks for portal hypertension and gastrointestinal bleeding. We report the results from a single-center study of primary and secondary prophylaxis of bleeding in children with portal hypertension and high-risk varices.

Methods: We collected data from 66 children with major endoscopic signs of portal hypertension, including grade 3 esophageal varices or grade 2 varices with red wale markings and/or gastric varices, treated consecutively from February 2001 through May 2011.