This Obesity Medicine Association (OMA) Expert Joint Perspective examines steatotic liver disease (SLD), which is composed of metabolic dysfunction-associated steatotic liver disease (MASLD), and metabolic dysfunction-associated steatohepatitis (MASH) in children with obesity. The prevalence of obesity is increasing, rates have tripled since 1963 from 5 % to now 19 % of US children affected in 2018. MASLD, is the most common liver disease seen in children, can be a precursor to the development of Type 2 Diabetes (T2DM) and is the primary reason for liver transplant listing in young adults. We must be vigilant in prevention and treatment of MASLD in childhood to prevent further progression. This joint clinical perspective is based upon scientific evidence, peer and clinical expertise. The medical literature was reviewed via PubMed search and appropriate articles were included in this review. This work was formulated from the collaboration of eight hepatologists/gastroenterologists with MASLD expertise and two physicians from the OMA. The authors who are experts in the field, determined sentinel questions often asked by clinicians regarding MASLD in children with obesity. They created a consensus and clinical guideline for clinicians on the screening, diagnosis, and treatment of MASLD associated with obesity in children. Obesity and the comorbidity of MASLD is increasing in children, and this is a medical problem that needs to be addressed urgently. It is well known that children with metabolic associated chronic disease often continue to have these chronic diseases as adults, which leads to reduced life expectancy, quality of life, and increasing healthcare needs and financial burden. The authors of this paper recommend healthy weight reduction not only through lifestyle modification but through obesity pharmacotherapy and bariatric surgery. Therefore, this guidance reviews available therapies to achieve healthy weight reduction and reverse MASLD to prevent progressive liver fibrosis, and metabolic disease.
11 November 2024 in Journal of Pediatric Gastroenterology and Nutrition
Epstein–Barr virus (EBV) is strongly associated with the development of post-transplant lymphoproliferative disorder (PTLD) in pediatric liver transplant recipients. PTLD is one of the most common malignancies following liver transplantation and is associated with significant morbidity and mortality. Factors such as EBV–serostatus mismatch and prolonged or high levels of immunosuppression impact a patient’s risk of developing PTLD. While pre-transplant EBV serological screening and post-transplant monitoring of EBV-DNA levels are strongly recommended, universal guidelines for its prevention and management are lacking. Due to a lack of robust prospective studies, current clinical practices vary widely. The treatment of PTLD typically involves reducing immunosuppression and using targeted therapies such as rituximab, or chemotherapy for refractory cases. This review aims to address our current understanding of EBV’s relationship with PTLD, evaluate the available treatment modalities, and highlight evolving strategies for using EBV as a biomarker for PTLD screening and prevention.
5 July 2025 in American Journal of Transplantation
Simultaneous heart liver transplantation (SHLT) has expanded in recent years, bolstered by increases in congenital heart disease (CHD) indications and improvements in organ preservation. We performed a contemporary analysis of patients undergoing SHLT for CHD vs non-CHD, further exploring the impact of machine perfusion (MP). Patients undergoing SHLT between 2010-2024 were retrospectively identified using the UNOS database, assessing a primary outcome of patient survival and secondary outcome of MP utilization. 535 patients underwent SHLT, 224(41.9%) with CHD indication and 311(58.1%) non-CHD. CHD recipients had significantly lower one-(72.9% vs. 89.4%,p<0.01) and five-year survival (66.1% vs. 81.4%,p<0.01) compared to non-CHD recipients. CHD indication was independently associated with a two-fold increased mortality risk, compared to a non-CHD diagnosis (HR:2.31, 95%CI:[1.53,3.47]). Decision tree boosting demonstrated a 2.9% higher relative mortality likelihood after SHLT for recipients with CHD. From 2022-2024, SHLT MP utilization for CHD increased; among 111 patients, 18.0% received a MP liver, 8.1% a MP heart, and 10.8% MP heart and MP liver. In this analysis of modern SHLT cases, patients with CHD had suboptimal outcomes; several donor and recipient factors also impacted mortality. Awareness of these factors can guide pre-transplant optimization, donor/recipient matching, and strategic MP use to improve survival.
Pediatric genetic and metabolic liver diseases comprise a broad spectrum of conditions and represent the second most common indication for liver transplantation following biliary atresia. The decision to transplant can be challenging and requires consideration of several factors including hepatic involvement, extra-hepatic manifestations, and anticipated post-transplant outcomes. This review examines pediatric genetic and metabolic liver diseases, their pathophysiology, clinical presentation, and the role of liver transplantation. Export