Preservation of fat mass at the expense of lean mass in children with end-stage chronic liver disease.

Background: Sarcopenia predicts morbidity and mortality in end-stage chronic liver disease (ESCLD). Here, we describe changes in body composition in children with ESCLD before and after liver transplantation (LT).

Methods: Retrospective analysis of whole body DXA scans performed before and after LT over 4 years.

Clinical outcomes of ABCB4 heterozygosity in infants and children with cholestatic liver disease.

Objectives: Biallelic variants in the adenosine triphosphate binding cassette subfamily B member 4 (ABCB4) gene which encodes the multidrug resistance 3 protein (MDR3) leads to progressive familiar intrahepatic cholestasis type 3. However, monoallelic variants are increasingly recognized as contributing to liver disease in adults. Our aim was to describe the clinical characteristics of MDR3 heterozygous variants in a large cohort of infants and children with cholestatic liver disease.

Transient elastography and von Willebrand factor as predictors of portal hypertension and decompensation in children.

Background & Aims: Von Willebrand factor antigen (vWFAg), a protein measured to test the level of vWF released from the vascular endothelium has gained much attention as a marker for portal hypertension (PHT) severity. The objectives of this study were to investigate the use of vWFAg as a biomarker along with liver and spleen stiffness measurements by transient elastography as potential predictors of clinically significant varices (CSV), variceal bleeding (VB) and decompensation in children with PHT.

Methods: This observational prospective cohort study included 117 children (median age 10 [IQR 6-14] years) who underwent oesophagogastroduodenoscopy between January'2012 to November'2021 and a validation group of 33 children who underwent the same procedure between December'2021 to March'2023.

Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges.

In children, fatty liver disease is a group of disorders that often overlaps with inherited metabolic disorders (IMDs), which requires prompt diagnosis and specific management. Metabolic dysfunction-associated fatty liver disease (MAFLD) or, formerly, non-alcoholic fatty liver disease (NAFLD) is the hepatic component of a multisystemic disease that requires a positive criteria in metabolic dysfunction for diagnosis. However, in children, the diagnosis of MAFLD is one of the exclusions of an IMD [paediatric fatty liver disease (PeFLD) type 1] including the possibility that an IMD can be identified in the future following investigations that may be negative at the time.

Long-term outcomes of pediatric liver transplantation in acute liver failure end-stage chronic liver disease: A retrospective observational study.

Background: Children with acute liver failure (ALF) who meet the criteria are eligible for super-urgent transplantation, whereas children with end-stage chronic liver disease (ESCLD) are usually transplanted electively. Pediatric liver trans plantation (PLT) in ALF and ESCLD settings has been well described in the literature, but there are no studies comparing the outcomes in these two groups.

Aim: To determine if there is a difference in post-operative complications and survival outcomes between ALF and ESCLD in PLT.

Sarcopenia and Fat Mass in Children With Chronic Liver Disease and Its Impact on Liver Transplantation.

Unlabelled: In adults, weight loss and sarcopenia are prognostic indicators of poor outcomes for patients awaiting liver transplant (LT). We tested the hypothesis that sarcopenia in children awaiting LT was related to poor outcomes.

Methods: Children with end-stage chronic liver disease undergoing assessment for LT were recruited into an observational longitudinal study.

Sclerosing Cholangitis in Pediatric Inflammatory Bowel Disease: Early Diagnosis and Management Affect Clinical Outcome.

Objective: To describe the characteristics and clinical course of children and young persons with inflammatory bowel disease (IBD) and sclerosing cholangitis (SC).

Study Design: Retrospective analysis of clinical characteristics, management, and outcome of two separate cohorts of children and young persons with IBD-SC managed in a tertiary pediatric gastroenterology center and in a tertiary pediatric hepatology center in the UK.

Results: Eighty-two pediatric patients (31% female) with IBD-SC and a mean age at diagnosis of 11.

Clinical management of sickle cell liver disease in children and young adults.

Liver involvement in sickle cell disease (SCD) is often referred to as sickle cell hepatopathy (SCH) and is a complication of SCD which may be associated with significant mortality. This review is based on a round-table workshop between paediatric and adult hepatologists and haematologists and review of the literature. The discussion was prompted by the lack of substantial data and guidance in managing these sometimes very challenging cases.

Value of Serum Zinc in Diagnosing and Assessing Severity of Liver Disease in Children With Wilson Disease.

Objectives: Wilson disease (WD) is a rare inborn error of copper metabolism with diverse manifestations. There has been no study of zinc (Zn), the copper's antagonist, in WD diagnosis and severity so far. Our aims were to evaluate serum Zn in WD and its correlation with the disease severity score (revised WD index).

The International Liver Transplantation Society Living Donor Liver Transplant Recipient Guideline.

Living donor liver transplantation (LDLT) has been increasingly embraced around the world as an important strategy to address the shortage of deceased donor livers. The aim of this guideline, approved by the International Liver Transplantation Society (ILTS), is to provide a collection of expert opinions, consensus, and best practices surrounding LDLT. Recommendations were developed from an analysis of the National Library of Medicine living donor transplantation indexed literature using the Grading of Recommendations Assessment, Development and Evaluation methodology.

Primary Budd-Chiari Syndrome in Children: King's College Hospital Experience.

Primary Budd-Chiari syndrome is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary Budd-Chiari syndrome presenting from January 2001 to November 2015 to our hospital. Seven children were identified.

Biomarkers in Pediatric Liver Disease.

The chronic nature of liver diseases in children and adults merits close follow-up for disease progression and/or treatment evaluation. Disease progression involves injury to liver cells resulting in cell death, varying degrees of inflammation, steatosis depending on the insult, oxidative stress, and eventually fibrosis and cirrhosis unless the process is modified with treatment or spontaneous recovery. Inflammation, cell death, and fibrosis are the three major processes that determine the outcome of liver disease irrespective of the etiology.

Omega-3 Fatty Acid-Rich Parenteral Nutrition: Is It a Double-Edged Sword?

Parenteral nutrition (PN) has been strongly associated with intestinal failure-associated liver disease. Cholestasis, liver steatosis, and liver fibrosis are features of this liver injury, which can progress to end stage liver disease. Omega-3 fatty acid rich PN has been shown to alleviate cholestasis and steatosis.

Molecular mechanisms of cachexia in chronic disease.

Cachexia is a metabolic syndrome that manifests with excessive weight loss and disproportionate muscle wasting. It is related to many different chronic diseases, such as cancer, infections, liver disease, inflammatory bowel disease, cardiac disease, chronic obstructive pulmonary disease, chronic renal failure and rheumatoid arthritis. Cachexia is linked with poor outcome for the patients.