Segmental Arterial Mediolysis Presenting as a Pancreatic Mass in a Pediatric Patient: A Case Report.

We describe a case of segmental arterial mediolysis (SAM) in a 2-year-old male who presented with symptoms of acute pancreatitis. SAM is a vascular entity of unknown etiology that involves medium-sized arteries in which the integrity of the vessel wall is compromised, resulting in increased susceptibility to ischemia, hemorrhage, and dissection. The clinical presentation is variable and can range from abdominal pain to more ominous findings of abdominal hemorrhage or organ infarction.

Accuracy of noninvasive fibrosis scores in predicting advanced fibrosis in pediatric patients after liver transplantation.

Background: Liver biopsy is the gold standard to stage fibrosis in liver disease. Several scoring systems have been studied to predict advanced fibrosis in liver disease. Those scores have not been validated in pediatric liver transplant patients.

Eosinophilic esophagitis: A review of the pediatric population and consideration of upcoming therapies.

Eosinophilic esophagitis (EoE) is a disease entity that has become increasingly recognized in the pediatric population over the last decade and was first recognized as early as 1990. EoE is a clinicopathologic diagnosis with signs and symptoms varying between age groups. The clinical presentation of EoE is variable ranging from milder nonspecific symptoms, such as abdominal pain, vomiting, and dyspepsia, to more severe presentations such as failure to thrive, dysphagia and even food impaction and is dependent on age of diagnosis 2.

Performance Characteristics, Intra- and Inter-operator Agreement of Transient Elastography in Pediatric Nonalcoholic Fatty Liver Disease.

Background And Aims: Transient elastography (TE) is a valuable tool in assessment of hepatic steatosis and fibrosis using liver stiffness measurement (LSM) and controlled attenuation parameter (CAP), respectively. Although widely used in adults, little is known about performance characteristics and reproducibility of TE (using Fibroscan device) in evaluation of pediatric nonalcoholic fatty liver disease (NAFLD).

Methods: We prospectively recruited children with NAFLD.

Comparison between non-alcoholic fatty liver disease screening guidelines in children and adolescents.

Background & Aim: There is currently no agreement on the screening strategy for non-alcoholic fatty liver disease (NAFLD) in children at risk. The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) recommends screening for NAFLD using alanine aminotransferase (ALT) in obese/overweight children, while the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) recommends using both ALT and abdominal ultrasound. The aim of this study was to assess the prevalence of suspected NAFLD in obese children based on the 2 screening strategies.

Nonalcoholic Fatty Liver Disease in Children: Not a Small Matter.

The prevalence of nonalcoholic fatty liver disease (NAFLD) has increased substantially in the past two decades and NAFLD has now become the most common cause of chronic liver disease in children and adolescents. NAFLD is a broad clinicopathologic spectrum ranging from simple steatosis to varying degrees of necroinflammation called nonalcoholic steatohepatitis (NASH), leading to fibrosis and subsequently to cirrhosis. Despite the increasing prevalence and progressive nature of NAFLD even among children, therapy for NAFLD in both adults and children are limited.

Prevalence of Suspected Nonalcoholic Fatty Liver Disease in Lean Adolescents in the United States.

Objectives: Nonalcoholic fatty liver disease (NAFLD) can develop in lean subjects referred to as lean NAFLD. We aim to evaluate the prevalence and risk factors of NAFLD in lean adolescents in the United States (US).

Methods: Cross sectional data from 1482 lean subjects (body mass index <85th percentile) ages between 12 and 18 years, who were enrolled in the National Health and Examination Survey during the 2005 to 2014 cycles were included.

The search for noninvasive methods to identify liver fibrosis in children with nonalcoholic fatty liver disease.

Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the obesity epidemic. Recent studies have clearly shown that the stage of fibrosis in adults with NAFLD is the most important histological feature in long-term outcomes and the development of liver-related complications. Despite the paucity of data regarding the natural history of pediatric NAFLD, its progression to cirrhosis and end-stage liver disease requiring liver transplantation is well documented.

Accuracy of Noninvasive Fibrosis Scores in Predicting the Presence of Fibrosis in Patients after Liver Transplantation.

Objectives: Several scoring systems have been developed to noninvasively predict the presence of advanced fibrosis in patients with chronic liver disease. Hepatitis C virus and nonalcoholic fatty liver disease are the 2 most common indications for orthotopic liver transplant and are associated with disease recurrence that can lead to fibrosis progression. Here, we evaluated the performance of commonly used fibrosis scores in assessing the presence of advanced fibrosis in patients after orthotopic liver transplant.

A 9-Day-Old With Weight Loss and Diarrhea.

A 9-day-old infant girl presented with diarrhea and weight loss of 19% since birth. She was born via spontaneous vaginal delivery at 39 weeks' gestation to a mother positive for group B who received adequate intrapartum prophylaxis. The infant was formula-fed every 2 to 3 hours with no reported issues with feeding or swallowing.

Nonalcoholic Fatty Liver Disease in Children: Hepatic and Extrahepatic Complications.

Nonalcoholic fatty liver disease (NAFLD) is considered the hepatic manifestation of metabolic syndrome and has become the most common form of chronic liver disease in children and adolescents. The histologic spectrum of NAFLD is broad ranging, from the relatively benign form of simple steatosis to the aggressive form of nonalcoholic steatohepatitis, eventually leading to fibrosis and cirrhosis. NAFLD has also been recognized as an independent risk factor for extrahepatic complications, such as cardiovascular disease, type 2 diabetes mellitus, sleep disorders, and osteoporosis.

Prevalence of Nonalcoholic Steatohepatitis-Associated Cirrhosis in the United States: An Analysis of National Health and Nutrition Examination Survey Data.

Objectives: Nonalcoholic fatty liver disease (NAFLD) includes a wide spectrum of manifestations ranging from nonalcoholic fatty liver (NAFL) to nonalcoholic steatohepatitis (NASH), fibrosis and eventually cirrhosis. The prevalence of NAFLD has been shown to be increasing over time; however, the prevalence of NASH cirrhosis and advanced fibrosis over time has not been well studied. Estimate the changes in prevalence of NASH cirrhosis and NAFLD-associated advanced fibrosis among adults in the United States.

Survival outcomes scores (SOFT, BAR, and Pedi-SOFT) are accurate in predicting post-liver transplant survival in adolescents.

SOFT and BAR scores utilize recipient, donor, and graft factors to predict the 3-month survival after LT in adults (≥18 years). Recently, Pedi-SOFT score was developed to predict 3-month survival after LT in young children (≤12 years). These scoring systems have not been studied in adolescent patients (13-17 years).

Reduced lysosomal acid lipase activity - A potential role in the pathogenesis of non alcoholic fatty liver disease in pediatric patients.

Background: Within the spectrum of nonalcoholic fatty liver disease (NAFLD), recent evidence suggests that adult patients with nonalcoholic steatohepatitis (NASH) have significantly lower blood lysosomal acid lipase (LAL) activity than those with steatosis. This has not been studied in pediatric patients with NAFLD.

Aim: Investigate blood LAL activity in pediatric patients with NAFLD and assess its correlation with histological severity.