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.

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.

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.

Optimizing peripubertal growth in a child with short bowel syndrome on full oral feeding with glucagon-like peptide 2 analog.

Teduglutide is a glucagon-like peptide 2 (GLP-2) analog which acts by increasing intestinal absorption of the remnant bowel for children with short bowel syndrome (SBS) dependent on parenteral nutrition. We present a 13-year-old male patient with type 2 SBS (55 cm of jejunum) from necrotizing enterocolitis on full oral feeding from the age of 12 months. Because of faltering growth from the age of 11 despite oral hyperphagia, he started Teduglutide at the standard dose.

Successful sequential liver and hematopoietic stem cell transplantation in a patient with Fanconi anemia.

Background: In Fanconi anemia bone marrow failure is the major cause of morbidity and mortality and hematopoietic stem cell transplantation represents the only curative treatment. Liver disease, in terms of elevated liver function tests, as well as benign and malignant liver tumors, occurs especially in case of androgen treatment. We report a unique case of a child with Fanconi anemia with FANCD2 mutation who developed neonatal cryptogenic liver cirrhosis and bone marrow failure.

Hepatic hemangioendothelioma of infancy: clinical features of a large cohort of patients and proposed management.

Purpose: The management of hepatic hemangioendothelioma (HHE) may be challenging. We aimed to review a large cohort of children who presented to our centers with symptomatic HHE in the last 16 years.

Methods: We collected age at presentation, clinical features, histology, diagnostic process, management and outcome.

Favorable outcome of primary liver transplantation in children with cirrhosis and hepatocellular carcinoma.

The outcome of HCC after transplantation (OLT) in children is not well known. Unfavorable features based on adult reports may lead to contraindicate OLT even in children. We reviewed a cohort of children with cirrhosis and HCC to evaluate their outcome after primary transplantation.

Late graft dysfunction and autoantibodies after liver transplantation in children: preliminary results of an Italian experience.

Late graft dysfunction (GD) associated with the development of autoantibodies is a common event after pediatric liver transplantation (OLTx) and can present in 2 clinicohistological subsets: de novo autoimmune hepatitis (DNAH) and early chronic rejection (ECR). Sixty out of 247 children developed autoantibodies after OLTx. GD was demonstrated in 22 (37%); based on histology, patients were divided in a DNAH and an ECR group.