Sara Kathryn Smith

Background and Aims: Management of portal hypertension in children, including primary prophylaxis of variceal bleeding, is controversial due to limited understanding of mortality risk after the first variceal hemorrhage (1VH). This international multicenter study was designed to advance understanding of outcomes after pediatric 1VH. Approach and Results: Center-specific characteristics and approaches to portal hypertension were determined at 44 international sites. K-means clustered the centers based upon volume and complexity of approaches [cluster 1 high (n=5), cluster 2 low (n=21), cluster 3 intermediate (n=18)]. Clinical features and outcomes after 1VH were available from 345 subjects. Factors associated with overall survival (OS) and survival with native liver (SNL) were estimated by Kaplan–Meier survival curves, log-rank tests, univariable and multivariable Cox regression models. OS and SNL were high in portal vein thrombosis and noncirrhotic diseases and significantly higher than biliary atresia and cirrhotic diseases ( p <.001). Diminished SNL was observed in decompensated disease [diuretics (ie, ascites) or hospitalization at 1VH], biliary atresia with poor bile flow, cirrhotic disease, after sclerotherapy, or no endoscopic intervention. Diminished OS was noted in those with cirrhosis or requiring sclerotherapy. Mortality at 6 weeks was determined and compared among clusters using the average treatment effect under the Rubin causal model. Overall, 6-week mortality was 4.1%, 1.4% in nonhospitalized subjects, and was significantly higher in cluster 2 versus 1 centers ( p =0.010). Conclusions: Immediate mortality after 1VH in children is low compared with adults. Outcomes after 1VH are diagnosis-specific and worse in decompensated disease and after sclerotherapy. Center-specific factors may influence immediate outcomes after 1VH.

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.

Pediatric MASLD (previously referred to as NAFLD) incidence has continued to rise along with the obesity pandemic. Pediatric MASLD increases the risk of liver fibrosis and cirrhosis in adulthood. Early detection and intervention can prevent and reduce complications. Liver biopsy remains the gold standard for diagnosis, although imaging modalities are increasingly being used. We performed a retrospective study of 202 children seen in a pediatric gastroenterology clinic with a complaint of abdominal pain, elevated liver enzymes or MASLD, or a combination of the three to evaluate screening methods for MASLD. A total of 134 of the 202 patients included in the study underwent laboratory testing and abdominal ultrasound. Ultrasound images were reviewed with attention to liver size and echotexture by a fellowship-trained pediatric radiologist for liver size and echotexture. Overall, 76.2% of the initial radiology reports correctly identified hepatomegaly based on age and 75.4% of the initial radiology reports correctly described hepatic echogenicity that was consistent with increased hepatic fat deposition. Use of screening ultrasound in concert with other clinical evaluations can be helpful to identify children at risk of MASLD. Utilizing ranges for liver span according to age can help to diagnose hepatomegaly, and understanding how to identify hepatic echogenicity is important for identifying possible hepatic steatosis.

Measles, mumps, rubella (MMR), and varicella incidence rates have increased due to the delayed vaccination schedules of children secondary to the COVID-19 pandemic. Decreased herd immunity creates a risk for immunocompetent children and immunocompromised individuals in the community. Historically, live-attenuated vaccines (MMR and varicella) were recommended before solid organ transplants. The amount of time before transplant when this is appropriate is often debated, as is the utility of vaccine titers. MMR and varicella vaccines previously were not recommended in immunocompromised patients post-solid organ transplant due to the undue risk of transmission and posed infection risk. The new literature on live-attenuated vaccines in post-transplant pediatric patients provides more insight into the vaccines’ safety and efficacy. The present article aims to provide guidance on live-attenuated vaccines (MMR and varicella) in the pre-transplant and post-operative solid organ transplant phases of care in pediatric patients.