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Liver steatosis in children

Giuseppina R Umano, Sonia Caprio, Anna Di Sessa, Naga Chalasani, Daniel J Dykas, Bridget Pierpont, Allen E Bale, Nicola Santoro
OBJECTIVES: Non alcoholic fatty liver disease (NAFLD) is a leading cause of liver damage in childhood, its occurrence is influenced by genetic and environmental factors. Recently, the rs626283 polymorphism in the MBOAT7 gene has been found to be associated with alcoholic liver disease and NAFLD in adults. METHODS: In a multiethnic cohort of obese children and adolescents we genotyped the rs626283 polymorphism in the MBOAT7 gene, evaluated insulin sensitivity by an oral glucose tolerance test, and measured the intra-hepatic fat content (HFF%) by magnetic resonance imaging...
February 27, 2018: American Journal of Gastroenterology
Robert Hegarty, Maesha Deheragoda, Emer Fitzpatrick, Anil Dhawan
The recognition of a pattern of steatotic liver injury where histology mimicked alcoholic liver disease but alcohol consumption was denied, led to the identification of non-alcoholic fatty liver disease (NAFLD). Non-alcoholic fatty liver disease has since become the most common chronic liver disease in adults owing to the global epidemic of obesity. However, in pediatrics, the term NAFLD seems incongruous: alcohol consumption is largely not a factor and inherited metabolic disorders (IMD) can mimic or co-exist with a diagnosis of NAFLD...
February 19, 2018: Journal of Hepatology
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
María Medrano, Cristina Cadenas-Sanchez, Celia Álvarez-Bueno, Iván Cavero-Redondo, Jonatan R Ruiz, Francisco B Ortega, Idoia Labayen
The main purposes of this study were to elucidate the effects of supervised-exercise training (ET) interventions on hepatic fat content and on non-alcoholic fatty liver disease (NAFLD) prevalence in children and adolescents and to provide information about the optimal ET prescription (type, intensity, volume, and frequency) needed to reduce hepatic fat content in youths. Supervised-ET interventions performed in children and adolescents (6-19 years) that provided results of exercise effects on hepatic fat content or NAFLD prevalence were included...
February 13, 2018: Progress in Cardiovascular Diseases
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
February 8, 2018: Acta Neuropathologica
Yu-Cheng Lin, Pi-Feng Chang, Mei-Hwei Chang, Yen-Hsuan Ni
BACKGROUND/AIMS: There are substantial genetic components contributing to the susceptibility of nonalcoholic NAFLD disease (NAFLD). It has recently been reported that the rs641738 C>T variant in the membrane-bound O-acyltransferase domain-containing protein 7 (MBOAT7) gene increased severity of NAFLD in adults of European descent. We aimed to test the hypothesis that MBOAT7 rs641738 variant would increase hepatic steatosis and hepatocellular injury in obese children. METHODS: A total of 831 obese children aged 7-15 years were recruited...
January 4, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
Malgorzata Wojcik, Dominika Janus, Anna Kalicka-Kasperczyk, Krystyna Sztefko, Jerzy B Starzyk
INTRODUCTION AND OBJECTIVE: Vitamin D deficiency is common in obesity; however, its contribution in the development of metabolic complications remains uncertain. The aim of the study was to examine the relationships between vitamin D status and metabolic complications. MATERIAL AND METHODS: The results of blood pressure measurements, biochemical tests and ultrasound of the liver were compared in both groups. The study was conducted at the Children's University Hospital in Krakow, Poland...
December 23, 2017: Annals of Agricultural and Environmental Medicine: AAEM
Valerio Nobili, Antonella Mosca, Rita De Vito, Massimiliano Raponi, Eleonora Scorletti, Christopher D Byrne
BACKGROUND & AIMS: As dietary components are delivered directly to the periportal zone of the liver lobule, there is the potential for greater injury in this zone (zone 1) compared to the perivenous zone (zone 3). We investigated the associations between dietary fructose consumption and uric acid concentrations and differential zonal injury in periportal and perivenous zones. METHODS: A total of 271 children's histological images were scored in 5 periportal and 5 perivenous zones for steatosis, ballooning, inflammation and fibrosis severity...
December 9, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
Emily R Perito, Veeral Ajmera, Nathan M Bass, Philip Rosenthal, Joel E Lavine, Jeffrey B Schwimmer, Katherine P Yates, Anna Mae Diehl, Jean P Molleston, Karen F Murray, Ann Scheimann, Ryan Gill, David Glidden, Bradley Aouizerat
Background: Reliable non-invasive markers to characterize inflammation, hepatocellular ballooning, and fibrosis in nonalcoholic fatty liver disease (NAFLD) are lacking. We investigated the relationship between plasma cytokine levels and features of NAFLD histology to gain insight into cellular pathways driving NASH and to identify potential non-invasive discriminators of NAFLD severity and pattern. Methods: Cytokines were measured from plasma obtained at enrollment in pediatric participants in NASH Clinical Research Network studies with liver biopsy-proven NAFLD...
September 2017: Hepatology communications
V Pampanini, E Inzaghi, D Germani, A Alterio, A Puglianiello, A Alisi, V Nobili, S Cianfarani
BACKGROUND AND AIMS: Fetuin-A has been proposed as a marker of liver damage in adults with obesity-related NAFLD. The aim of this study was to test serum fetuin-A concentrations in obese children with NAFLD diagnosed either by ultrasonography or by liver biopsy and to determine its applicability as predictive tool in pediatric NAFLD. METHODS AND RESULTS: Metabolic parameters and fetuin-A levels were investigated in 81 obese children with NAFLD diagnosed by biopsy, 79 obese children with NAFLD defined by liver ultrasonography and 23 lean subjects...
October 3, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
Katherine P Yates, Ross Deppe, Megan Comerford, Howard Masuoka, Oscar W Cummings, James Tonascia, Naga Chalasani, Raj Vuppalanchi
AIM: Serum high mobility group box 1 protein (HMGB1) is a proinflammatory molecule that could potentially serve as a biomarker for non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) due to its correlation with degree of liver fibrosis. The aim of the current study was to examine the cross-sectional and longitudinal relationships between serum HMGB1 levels and liver histology in adults and children with NAFLD participating in two large randomized controlled trials...
2017: PloS One
Jonathan R Dillman, Andrew T Trout, Emma N Costello, Suraj D Serai, Kristin S Bramlage, Rohit Kohli, Stavra A Xanthakos
OBJECTIVE: The purpose of this study is to determine the relationships between quantitative liver MRI measurements and liver biopsy findings in pediatric and young adult patients with nonalcoholic fatty liver disease (NAFLD). MATERIALS AND METHODS: Data were obtained from a registry that prospectively enrolls pediatric and young adult patients with biopsy-confirmed NAFLD at our tertiary medical center with parent or guardian and subject informed consent, as appropriate...
October 18, 2017: AJR. American Journal of Roentgenology
Michael S Middleton, Mark L Van Natta, Elhamy R Heba, Adina Alazraki, Andrew T Trout, Prakash Masand, Elizabeth M Brunt, David E Kleiner, Edward Doo, James Tonascia, Joel E Lavine, Wei Shen, Gavin Hamilton, Jeffrey B Schwimmer, Claude B Sirlin
We assessed the performance of magnetic resonance imaging (MRI) proton density fat fraction (PDFF) in children to stratify hepatic steatosis grade before and after treatment in the Cysteamine Bitartrate Delayed-Release for the Treatment of Nonalcoholic Fatty Liver Disease in Children (CyNCh) trial, using centrally-scored histology as reference. Participants had multi-echo 1.5T or 3T MRI on scanners from three manufacturers. Of 169 enrolled children, 110 (65%) and 83 (49%) had MRI and liver biopsy at baseline and at end-of-treatment (EOT; 52-weeks), respectively...
October 13, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Saleh Amin, Doaa El Amrousy, Shaymaa Elrifaey, Rasha Gamal, Hossam Hodeib
OBJECTIVES: The aim of the study was to investigate the relationship between osteocalcin and nonalcoholic fatty liver disease (NAFLD) in children with obesity. METHOD: 60 obese children with NAFLD were taken as a patient group and 60 obese children and normal liver with matching age, sex, and body mass index were taken as a control group. Anthropometric measurements, abdominal ultrasonography for diagnosis and grading of NAFLD, and laboratory investigations in the form of liver function tests, lipid profile, fasting serum glucose and insulin, and serum osteocalcin levels were done for all children...
January 2018: Journal of Pediatric Gastroenterology and Nutrition
David Kleiner
Nonalcoholic fatty liver disease (NAFLD) is the liver disease associated with obesity, diabetes and the metabolic syndrome . Although steatosis is a key histological feature, liver biopsies of patients with NAFLD can show a wide range of other findings, including portal and lobular inflammation, ballooning and apoptotic hepatocellular injury, Mallory-Denk bodies, megamitochondria and fibrosis. Nonalcoholic steatohepatitis (NASH) is a progressive subtype of NAFLD first defined by analogy to alcoholic hepatitis...
September 25, 2017: Minerva Gastroenterologica e Dietologica
Anika Nier, Anna Janina Engstler, Ina Barbara Maier, Ina Bergheim
BACKGROUND & AIMS: Recent studies have shown that patients with manifest non-alcoholic fatty liver disease (NAFLD), e.g. steatosis grade 3 or steatohepatitis with or without beginning fibrosis frequently show altered fecal microbiota composition and elevated bacterial endotoxin levels. However, if these alterations are signs of a progressing disease or are already found in initial disease stages has not yet been clarified. METHODS: Twenty children with simple steatosis (grade 1) diagnosed by ultrasound and 29 normal weight healthy control children (age <10 years) were included in the study (mean age 7...
2017: PloS One
Savinda Liyanagedera, Robert Patrick Williams, Silvio Veraldi, Valerio Nobili, Jake P Mann
Non-alcoholic fatty liver disease (NAFLD) represents a spectrum, including 'simple' steatosis, non-alcoholic steatohepatitis (NASH), and fibrosis. Increasing prevalence of NAFLD has followed the international rise in obesity and lifestyle modification is the mainstay therapy for children. To date, pharmacological trials have had varying efficacy but a large number of new agents are in early phase trials for adults. Areas covered: This review explores the effect of current and potential future paediatric NAFLD treatments in terms of histological and biochemical endpoints...
November 2017: Expert Review of Clinical Pharmacology
Michele Di Martino, Kameliya Koryukova, Mario Bezzi, Carlo Catalano
Non-invasive diagnosis and quantification of liver steatosis is important to overcome limits of liver biopsy, in order to follow up patients during their therapy and to establish a reference standard that can be used in clinical trials and longitudinal studies. Imaging offers several methods in this setting: ultrasound, which is the cheapest technique and easy to perform; magnetic resonance spectroscopy (MRS), which reflects the real content of triglycerides in a specific volume; and proton density fat fraction (PDFF) magnetic resonance, which is a simple method that reflects the distribution of the fat in the whole liver...
August 11, 2017: Children
Maurizio Elia, Joerg Klepper, Baerbel Leiendecker, Hans Hartmann
BACKGROUND: Although a larger number of antiepileptic drugs became available in the last decades, epilepsy remains drug-resistant in approximately a third of patients. Ketogenic diet (KD), first proposed at the beginning of the last century, is complex and has anticonvulsant effects, yet not completely understood. Over the last decades, different types of ketogenic diets (KDs) have been developed, namely classical KD and modified Atkins diet (MAD). They offer an effective alternative for children and adults with drug-resistant epilepsies...
August 9, 2017: Current Pharmaceutical Design
Marco Poeta, Luca Pierri, Pietro Vajro
Non-alcoholic fatty liver disease (NAFLD) is the most frequent type of chronic liver disease in the pediatric age group, paralleling an obesity pandemic. A "multiple-hit" hypothesis has been invoked to explain its pathogenesis. The "first hit" is liver lipid accumulation in obese children with insulin resistance. In the absence of significant lifestyle modifications leading to weight loss and increased physical activity, other factors may act as "second hits" implicated in liver damage progression leading to more severe forms of inflammation and hepatic fibrosis...
August 2, 2017: Children
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