One of the most important causes of portal hypertension among children is extrahepatic portal vein thrombosis (EHPVT). The most common risk factors for EHPVT are neonatal umbilical vein catheterization, transfusions, bacterial infections, dehydration, and thrombophilia. Our study aimed to describe the clinical manifestations, treatment, evolution, and risk factors of children with EHPVT. Methods: We analyzed retrospectively all children admitted and followed in our hospital with EHPVT between January 2011–December 2020. The diagnosis was made by ultrasound or contrast magnetic resonance imaging. We evaluated the onset symptoms, complications, therapeutic methods, and risk factors. Results: A total of 63 children, mean age 5.14 ± 4.90 (33 boys, 52.38%), were evaluated for EHPVT during the study period. The first symptoms were upper gastrointestinal bleeding (31 children, 49.21%) and splenomegaly (22 children, 34.92%). Thrombocytopenia was present in 44 children (69.84%). The most frequent risk factors were umbilical vein catheterization (46 children, 73.02%) and bacterial infections during the neonatal period (30 children, 47.62%). Protein C, protein S, antithrombin III levels were decreased in 44 of the 48 patients tested. In 42 of these cases, mutations for thrombophilia were tested, and 37 were positive. Upper digestive endoscopy was performed in all cases, revealing esophageal varices in 56 children (88.89%). All children with gastrointestinal bleeding received an octreotide infusion. In 26 children (41.27%), variceal ligation was performed, and in 5 children (7.94%), sclerotherapy. Porto-systemic shunt was performed in 11 children (17.46%), and Meso-Rex shunt was done in 4 children (6.35%). The evolution was favorable in 62 cases (98.41%). Only one child died secondary to severe sepsis. Conclusions: EHPVT is frequently diagnosed in the last period in our region due to the increased use of umbilical vein catheterization. Furthermore, genetic predisposition, neonatal bacterial infections, and prematurity certainly play an important role in this condition. A proactive ultrasound assessment of children with risk factors for EHPVT should be encouraged for early diagnosis and treatment.

Background: Acute liver failure (ALF) is a rare disease, associated with high mortality, despite optimal medical therapy without emergency liver transplantation. Knowing the possible cause of ALF plays a vital role in the management, as the child could benefit from effective specific therapies in emergencies. Methods: We have analyzed the etiology and outcome of ALF in children followed-up in a tertiary care hospital between January 2012–December 2018. The patients were grouped into different age categories: neonates (0–1 month), infants (1–12 months), children (1–14 years), and teenagers (14–18 years). Results: 97 children (46 males, 47.42%, the mean age of 7.66 ± 8.18 years) were admitted with ALF. The most important causes of ALF were in neonates and infants, infections (72.72%), and metabolic disorders (43.47%), in children and adolescents were the toxic causes (60% and 79.41%). The mortality rate was 31.95% (31 patients), mainly in ALF due to infections or metabolic disorders. Conclusions: In neonates and infants, the main causes of ALF were infections and metabolic diseases, while in older children and teenagers, were toxin-induced liver injuries. The mortality among neonates and infants was significantly higher than in other ages. Early recognition and immediate therapeutic intervention could improve the outcome of these patients.

Results NAFLD was diagnosed in 8.5% of overweight and 24.9% of obese children. In obese children the prevalence of NAFLD was 22.3% for age group 6-11.9 years and 35.5% for age group 12-19 years. There was no significant difference between girls and boys (P= 0.521). Age was similar in children with and without NAFLD (P= 0.766). An increase of 10 U/L of ALT, AST and ALK increased the odds of NAFLD 6%, 5% and 3%, respectively. An increase of 10 mg/dl of triglycerides and glucose were associated with a 12% and 8% increase and one of TSH with a 15% increase in the odds of NAFLD. An increase of 1 unit of HOMA-IR was associated with a 21% increase in the odds of NAFLD. Conclusions ALT, AST, ALK and HOMA-IR can predict the progression of NAFLD. Findings emphasize on the importance of prevention of obesity and early intervention to prevent abnormalities among obese children.

Introduction: Acute liver failure (ALF) is a syndrome defined by jaundice, coagulopathy (INR > 1.5) and hepatic encephalopathy in patients with no evidence of prior liver disease. Toxins and drugs are a frequent cause of ALF in children. Material and methods: The aim of our study was to establish the causes of toxic ALF in children followed up in our hospital in the period of January 2000 to August 2018. We retrospectively studied all hospital records of patients who developed ALF after mushroom/drug exposure and had been admitted to our hospital, the main pediatric toxicology center in northwestern Romania. Results: In the last 18 years, 123 patients were admitted to our clinic with toxic ALF (89 patients secondary to mushroom ingestion and 34 patients after drug exposure). In the 2000-2012 period accidental mushroom poisoning was the leading cause of toxic ALF. Unfortunately, during the last years, voluntary drug ingestions have increased dramatically. The most commonly incriminated drug was acetaminophen (52.94%). Conclusions: ALF in mushroom poisoning is associated with a high mortality in children, despite optimal medical therapy. This etiology was one of the most important causes of death in our cohort. The difficulty in accessing emergency liver transplantation is an obstacle common to many Eastern European pediatric centers. Fortunately, in the last 5 years the incidence of mushroom intoxications has decreased in our area. It is worrying that over the last few years there has been an increased incidence of toxic ALF after drug exposure (for suicidal purposes or due to lenient regulations for prescribing hepatotoxic medications).

This study aimed to analyse vitamin D-binding protein (Gc-globulin) serum levels in acute liver failure (ALF) in children in relation to disease outcomes and correlations with other known markers used to evaluate the severity of ALF. Our study included 34 children (mean age 4.87 ± 5.30 years) with ALF of different causes (metabolic, 26.47%; autoimmune, 23.53%; toxic, 20.59%; infection, 17.65%; unknown, 11.76%) and 30 children without any liver injury (mean age 6.11 ± 4.26 years). The outcome was poor in 14 patients (41.18%), including one child with liver transplantation (2.94%). Serum Gc-globulin levels were significantly lower in ALF patients compared to the control group (151.57 ± 171.8 mg/L vs. 498.63 ± 252.50 mg/L; p < 0.000001), with an optimum cut-off of 163.5 mg/L (Area Under the Curve, AUC, 0.8921; sensitivity, 76.50%; specificity, 100%). Levels were also lower in patients with poor outcomes compared to survivors (59.34 ± 33.73 mg/L vs. 216.12 ± 199.69 mg/L; p < 0.0001), with an optimum cut-off 115 mg/L (AUC, 0.7642; sensitivity, 100%; specificity, 50%). Gc-globulin serum levels were variable according to ALF aetiology, i.e., lower in metabolic, infectious, or unknown causes compared to autoimmune and toxic causes. Gc-globulin serum levels were decreased in children with ALF and lower in those with poor outcomes compared with survivors. Gc-globulin serum levels were correlated with other known parameters used to evaluate the severity of ALF and could help to identify patients at high risk for poor outcomes.