Rodrigo Vázquez Frias

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.

Postbiotics, defined by the ISAPP as preparations of inanimate microorganisms and/or their components that confer health benefits, represent a promising category of microbiome-derived solutions. This position paper highlights their clinical relevance, particularly in pediatrics, while addressing key aspects of definition, safety, quality, and strain-level specificity. Evidence supports the use of Lactobacillus LB –including L. fermentum CNCM I-2998 and L. delbrueckii subsp. lactis CNCM I-4831– in reducing the duration and severity of acute diarrhea in children. Other strains, such as Bifidobacterium breve C50, Streptococcus thermophilus 065, Lacticaseibacillus paracasei CBA L74, Lactiplantibacillus plantarum LPL28, and Ligilactobacillus salivarius AP-32, show promise in preventing infections, supporting oral health, and modulating immune responses. Additional postbiotics, including Limosilactobacillus reuteri DSM 17648, expand their potential into metabolic and gastrointestinal disorders. Collectively, postbiotics emerge as clinically valuable interventions, bridging science and medical practice.

Alagille syndrome (ALGS) is a multisystem disorder characterized by a paucity of intrahepatic bile ducts and cholestasis, often requiring liver transplantation before adulthood. Due to the lack of genotype–phenotype correlation, case series are essential to understand disease presentation and prognosis. Data on Mexican ALGS patients are limited. Therefore, we aimed to characterize a large series of Mexican patients by consolidating cases from major institutions and independent geneticists, with the goal of generating one of the most comprehensive cohorts in Latin America. We retrospectively analyzed clinical records of pediatric ALGS patients, focusing on demographics, clinical features, laboratory and imaging results, biopsy findings, and transplant status. Genetic testing was performed for all cases without prior molecular confirmation. We identified 52 ALGS cases over 13 years; 22 had available clinical records. Of these, only 6 had molecular confirmation at study onset, prompting genetic testing in the remaining 16. We identified six novel JAG1 variants and several previously unreported phenotypic features. A liver transplantation rate of 13% was observed in the cohort. This study represents the largest molecularly confirmed ALGS cohort in Mexico to date. Novel genetic and clinical findings expand the known spectrum of ALGS and emphasize the need for improved therapies, such as IBAT inhibitors, which may alleviate symptoms and reduce the need for transplantation.