Hepatic Venous Pressure Gradient Measurements in Children: Correlation With Hepatic Histology and Clinical Indicators of Portal Hypertension.

OBJECTIVES: In adults, elevated hepatic venous pressure gradients (HVPGs) are correlated with the degree of liver fibrosis on histopathology and predict worse outcomes including variceal bleeding and death. We aimed to examine the association between HVPG measurements, histopathologic findings, and clinical indicators of portal hypertension in children. METHODS: Utilizing retrospective data from 2 pediatric centers between 2006 and 2015, we identified children who underwent simultaneous HVPG measurement and transjugular liver biopsy. Medical charts were reviewed for histopathology, imaging, endoscopic, and clinical data. RESULTS: Forty-one children (median age 11 years) were included in the analysis with diagnoses of acute hepatitis (n = 15), chronic liver disease (n = 12), hepatic noncirrhotic portal hypertension (n = 4), acute liver failure (n = 3), and nonhepatic causes of portal hypertension (n = 7). Elevated mean HVPG measurements were found in children with acute liver failure (10 mmHg, range 4-12) and chronic liver disease (7 mmHg, range 1-12). HVPG measurements did not correlate with the histological severity of fibrosis (ρ = 0.23, P = 0.14) or portal inflammation (ρ = 0.24, P = 0.29), and no difference was found in HVPG when comparing children with and without a history of variceal bleeding (P = 0.43). CONCLUSIONS: HVPG measurements do not correlate significantly with the degree of hepatic fibrosis on biopsy. Furthermore, HVPG measurements are not associated with the presence of varices or history of variceal bleeding, suggesting the possibility of intrahepatic shunting in children with advanced liver disease. Therefore, unlike in adults, HVPG measurements may not accurately predict children who are at risk of complications from portal hypertension.

The microbiota in inflammatory bowel disease: current and therapeutic insights.

Inflammatory bowel disease is a heterogeneous group of chronic disorders that result from the interaction of the intestinal immune system with the gut microbiome. Until recently, most investigative efforts and therapeutic breakthroughs were centered on understanding and manipulating the altered mucosal immune response that characterizes these diseases. However, more recent studies have highlighted the important role of environmental factors, and in particular the microbiota, in disease onset and disease exacerbation. Advances in genomic sequencing technology and bioinformatics have facilitated an explosion of investigative inquiries into the composition and function of the intestinal microbiome in health and disease and have advanced our understanding of the interplay between the gut microbiota and the host immune system. The gut microbiome is dynamic and changes with age and in response to diet, antibiotics and other environmental factors, and these alterations in the microbiome contribute to disease onset and exacerbation. Strategies to manipulate the microbiome through diet, probiotics, antibiotics or fecal microbiota transplantation may potentially be used therapeutically to influence modulate disease activity. This review will characterize the factors involved in the development of the intestinal microbiome and will describe the typical alterations in the microbiota that are characteristic of inflammatory bowel disease. Additionally, this manuscript will summarize the early but promising literature on the role of the gut microbiota in the pathogenesis of inflammatory bowel disease with implications for utilizing this data for diagnostic or therapeutic application in the clinical management of patients with these diseases.

Dietary Therapies in Pediatric Inflammatory Bowel Disease: An Evolving Inflammatory Bowel Disease Paradigm.

Nutrition has long been recognized as a critical component in the treatment of pediatric inflammatory bowel disease (IBD). Formerly, nutritional interventions have focused on targeting improved weight gain and linear growth, as well as correction of micronutrient deficiencies. Recently, there has been growing interest and study of dietary interventions for induction and maintenance of remission. In addition to exclusive enteral nutrition, successes have been achieved with specific exclusion diets. This article evaluates current literature regarding the role of diet and nutrition in pathogenesis of disease, as well as the role of diet as primary therapy for pediatric IBD.

Management of ascites in children.

Ascites is the pathologic accumulation of fluid within the peritoneal cavity. There are many causes of fetal, neonatal and pediatric ascites; however, chronic liver disease and subsequent cirrhosis remain the most common. The medical and surgical management of ascites in children is dependent on targeting the underlying etiology. Broad categories of management strategies include: sodium restriction, diuresis, paracentesis, intravenous albumin, prevention and treatment of infection, surgical and endovascular shunts and liver transplantation. This review updates and expands the discussion of the unique considerations regarding the management of cirrhotic and non-cirrhotic ascites in the pediatric patient.