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Metabolic liver disease children

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https://www.readbyqxmd.com/read/28529199/can-fetuin-a-be-a-marker-for-insulin-resistance-and-poor-glycemic-control-in-children-with-type-1-diabetes-mellitus
#1
Ülkü Gül Sıraz, Murat Doğan, Nihal Hatipoğlu, Sabahttin Muhtaroğlu, Selim Kurtoğlu
OBJECTIVE: Backgroud Metabolic impairment in Type 1 Diabetes Mellitus (DM) with poor glycemic control causes insulin resistance, Non-Alcoholic Fatty Liver Disease (NAFLD) and atherosclerosis; thus, increased Carotid-intima media thickness (CIMT). The Fetuin-A has protective effect in cardiovascular disorders, which is increased in hepatosteatosis. We aimed to investigate reliability of Fetuin-A levels in early detection of diabetic complications in children with T1DM and to identify a cut-off value that may show poor metabolic control...
May 22, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28521872/non-alcoholic-fatty-liver-disease-an-update-with-special-focus-on-the-role-of-gut-microbiota
#2
REVIEW
Michael Doulberis, Georgios Kotronis, Dimitra Gialamprinou, Jannis Kountouras, Panagiotis Katsinelos
Non-alcoholic fatty liver disease (NAFLD) is a significant global health burden in children, adolescents and adults with substantial rise in prevalence over the last decades. Accumulating data from manifold studies support the idea of NAFLD as a hepatic manifestation of metabolic syndrome, being rather a systemic metabolic disease than a liver confined pathology. Emerging data support that the gut microbiome represents a significant environmental factor contributing to NAFLD development and progression. Apart from other regimens, probiotics may have a positive role in the management of NAFLD through a plethora of possible mechanisms...
June 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28520503/serum-alanine-aminotransferase-trends-and-their-relationship-with-obesity-and-metabolic-syndrome-in-united-states-adolescents-1999-2014
#3
Cyrelle R Fermin, Arthur M Lee, Stephanie L Filipp, Matthew J Gurka, Mark D DeBoer
INTRODUCTION: Nonalcoholic fatty liver disease (NAFLD), characterized by hepatocyte dysfunction, fat accumulation, and fibrosis, is the most common cause of chronic liver disease in children. Elevated levels of serum alanine aminotransferase (ALT) are used clinically to identify potential liver dysfunction. Our goal was to assess for changes in the national prevalence of elevated ALT over time and potential relationship to trends in the metabolic syndrome (MetS) severity and elevated body mass index (BMI)...
May 18, 2017: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/28512764/maternal-fructose-consumption-can-affect-offspring-metabolic-outcomes
#4
Alejandro Gugliucci
High sugar consumption is associated with non-alcoholic fatty liver disease (NAFLD) and excess visceral adipose tissue (VAT) which are in turn linked to type 2 diabetes mellitus (T2DM), dyslipidemia, and cardiovascular disease (CVD) in adults and children. This article is protected by copyright. All rights reserved.
May 17, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28511023/efficacy-of-docosahexaenoic-acid-choline-vitamin-e-dha-cho-ve-in-paediatric-nash-a-randomized-controlled-clinical-trial
#5
Evelyn Zöhrer, Alisi Anna, Jörg Jahnel, Antonella Moscla, Claudia Della Corte, Annalisa Crudele, Günter Fauler, Valerio Nobili
Non-alcoholic steatohepatitis (NASH), a progressive form of non-alcoholic fatty liver disease (NAFLD), is one of the most common hepatic diseases in children. We conducted a randomized controlled clinical trial on children with biopsy-proven NASH based on a combinatorial nutritional approach compared to placebo. Participants were assigned to lifestyle modification plus placebo, or lifestyle modification plus a mix containing docosahexaenoic acid, choline and vitamin E (DHA-CHO-VE). Forty children and adolescents concluded the trial...
May 16, 2017: Applied Physiology, Nutrition, and Metabolism, Physiologie Appliquée, Nutrition et Métabolisme
https://www.readbyqxmd.com/read/28506362/current-concepts-in-pediatric-nonalcoholic-fatty-liver-disease
#6
REVIEW
Sarah E Fleet, Jay H Lefkowitch, Joel E Lavine
Nonalcoholic fatty liver disease (NAFLD) represents a spectrum of disease. Its increasing prevalence is a direct result of historically high rates of obesity. Hepatocyte lipid accumulation is the first step in a cascade of metabolic and inflammatory events thought to precipitate NAFLD. Histologic findings provide insight into these events. Lifestyle modification remains the primary therapy in children. Current recommendations include vitamin E treatment in those with biopsy-proven NASH. Trials of novel drugs are ongoing in adults...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28502444/nonalcoholic-fatty-liver-disease-in-children-hepatic-and-extrahepatic-complications
#7
REVIEW
Praveen Kumar Conjeevaram Selvakumar, Mohammad Nasser Kabbany, Valerio Nobili, Naim Alkhouri
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...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28497759/long-term-challenges-and-perspectives-of-pre-adolescent-liver-disease
#8
REVIEW
Nedim Hadžić, Ulrich Baumann, Pat McKiernan, Valerie McLin, Valerio Nobili
Chronic liver disease is a growing problem that has substantial effects on public health. Many paediatric liver conditions are precursors of adult chronic liver disease, cirrhosis, and hepatocellular carcinoma. Clinical management of Wilson's disease, autoimmune liver disease, and chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chronic liver disease and indication for liver transplantation, is similar in children and adults. In the past 10 or so years, paediatric hepatology has expanded into neighbouring clinical areas, such as metabolic liver diseases and systemic conditions with liver involvement...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28492501/urinary-metabolomics-in-pediatric-obesity-and-nafld-identifies-metabolic-pathways-metabolites-related-to-dietary-habits-and-gut-liver-axis-perturbations
#9
Jacopo Troisi, Luca Pierri, Annamaria Landolfi, Francesca Marciano, Antonella Bisogno, Federica Belmonte, Carmen Palladino, Salvatore Guercio Nuzio, Pietro Campiglia, Pietro Vajro
To get insight into still elusive pathomechanisms of pediatric obesity and non-alcoholic fatty liver disease (NAFLD) we explored the interplay among GC-MS studied urinary metabolomic signature, gut liver axis (GLA) abnormalities, and food preferences (Kid-Med). Intestinal permeability (IP), small intestinal bacterial overgrowth (SIBO), and homeostatic model assessment-insulin resistance were investigated in forty children (mean age 9.8 years) categorized as normal weight (NW) or obese (body mass index <85th or >95th percentile, respectively) ± ultrasonographic bright liver and hypertransaminasemia (NAFLD)...
May 11, 2017: Nutrients
https://www.readbyqxmd.com/read/28471905/predictive-variables-for-abnormal-comprehensive-metabolic-panel-testing-and-potential-cost-savings-in-children-receiving-pediatric-emergency-department-care
#10
Matthew David Huckaby, Shayla Freeman, Christopher Thurmond, Matthew Cooper, Joseph D Losek
OBJECTIVE: The aim of this study was to determine variables predictive of abnormal comprehensive metabolic panel (CMP) results in pediatric emergency department (PED) patients and the potential cost savings of a basic metabolic panel (BMP) versus a CMP. METHODS: This is a retrospective cross-sectional descriptive study of children (<18 y) at an urban academic PED (annual census, 22,000). Clinical data included 12 clinical variables: right upper quadrant pain, overdose, emesis, liver disorder, malignancy, heart disease, bleeding disorder, jaundice, right upper quadrant tenderness, hepatomegaly, ascites/peripheral edema and shock, and the liver function test (LFT) results not in a BMP (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, total bilirubin, total protein, and albumin)...
May 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28458462/partial-lipodystrophy-in-patients-who-have-undergone-hematopoietic-stem-cell-transplantation-during-childhood-an-institutional-cross-sectional-survey
#11
Masanori Adachi, Yuji Oto, Koji Muroya, Junko Hanakawa, Yumi Asakura, Hiroaki Goto
Partial lipodystrophy (PD), a condition similar to metabolic syndrome without obesity, is one of the late complications of hematopoietic stem cell transplantation (HSCT) performed during childhood. We aimed to investigate the prevalence and risk factors of PD. A cross-sectional survey was performed in a children's hospital, targeting patients treated for a malignancy or hematological disorder, and who were disease-free for > 24 mo. PD was defined as gluteal lipoatrophy and lipohypertrophy of the cheeks or neck associated with diabetes and/or fatty liver disease...
2017: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/28452835/vitamin-d-status-cardiometabolic-liver-and-mental-health-status-in-obese-youth-attending-a-pediatric-weight-management-centre
#12
Krista MacDonald, Kristine Godziuk, Jason Yap, Rena LaFrance, Mohammad Ansarian, Andrea Haqq, Diana R Mager
BACKGROUND: Vitamin D (VitD) deficiency and obesity are reaching epidemic proportions in North America, particularly in those with co-morbid conditions such as diabetes or liver disease. The study objective was to determine the prevalence of suboptimal vitD status and interrelationships with anthropometric, cardio-metabolic, liver, mental health and lifestyle (sleep/screen time) parameters in an ambulatory population of obese children. METHODS: Children (2-18 years) old attending a pediatric weight management clinic (n = 217) were retrospectively reviewed...
April 27, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28439217/pediatric-metabolic-syndrome-pathophysiology-and-laboratory-assessment
#13
Victoria Higgins, Khosrow Adeli
Pediatric overweight and obesity is an emerging public health priority as rates have rapidly increased worldwide. Obesity is often clustered with other metabolic abnormalities including hypertension, dyslipidemia, and insulin resistance, leading to increased risk of cardiovascular disease. This cluster of risk factors, termed the metabolic syndrome, has traditionally been reported in adults. However, with the increased prevalence of pediatric obesity, the metabolic syndrome is now evident in children and adolescents...
March 2017: EJIFCC
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#14
Filipa Dias Costa, Rita Moinho, Sandra Ferreira, Paula Garcia, Luísa Diogo, Isabel Gonçalves, Carla Pinto
INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...
April 11, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28395968/-aetiology-outcomes-and-prognostic-indicators-of-paediatric-acute-liver-failure
#15
Juan José Gilbert Pérez, Belén Jordano Moreno, Mónica Rodríguez Salas
INTRODUCTION: Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF. OBJECTIVES: To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator. PATIENTS AND METHODS: A retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group...
April 7, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28391883/childhood-adult-onset-lysosomal-acid-lipase-deficiency-a-serious-metabolic-and-vascular-phenotype-beyond-liver-disease-four-new-pediatric-cases
#16
Pierre Poinsot, Sophie Collardeau Frachon, Lioara Restier, André Sérusclat, Mathilde Di Filippo, Sybil Charrière, Philippe Moulin, Alain Lachaux, Noel Peretti
BACKGROUND: The childhood/adult-onset lysosomal acid lipase deficiency (LALD; late-onset LALD) is a rare genetic disease. Children present severe fatty liver disease with early cirrhosis. Before enzyme replacement therapy, statins were the standard treatment to improve the severe dyslipidemia. However, late-onset LALD should be considered as a systemic metabolic disease: chronic hyper-low-density lipoprotein and hypo-high-density lipoprotein cholesterolemia induces early atherosclerosis in addition to the liver morbidity...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28374256/analysis-of-magnetic-resonance-imaging-findings-of-children-with-neurologic-complications-after-liver-transplantation
#17
Mehmet Öztürk, İsmail Akdulum, Nurullah Dağ, Ahmet Sığırcı, Serdal Güngör, Sezai Yılmaz
OBJECTIVE: To analyze the magnetic resonance imaging findings in children diagnosed with neurologic complications after liver transplantation (LT). MATERIALS AND METHODS: A total of 39 patients diagnosed with neurologic complications following LT between 2010 and 2016. Neuroradiologic imaging was performed using cranial magnetic resonance imaging (MRI). Descriptive statistics regarding age, gender, type of complication, diagnostic and therapeutic modalities were calculated and presented as number and percentage...
April 3, 2017: La Radiologia Medica
https://www.readbyqxmd.com/read/28364818/liver-transplantation-in-alpha-1-antitrypsin-deficiency
#18
REVIEW
Virginia C Clark
Alpha-1 antitrypsin (AAT) deficiency is a common inherited metabolic disorder caused by a point mutation in the SERPIN1A gene. A small portion of homozygous PI*ZZ individuals develop severe liver disease that requires liver transplantation. Posttransplant survival is excellent. The largest burden of advanced liver disease lies within the adult population rather than children. Evaluation of lung function in adults before transplant is essential because of the underlying risk for chronic obstructive pulmonary disease...
May 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28359257/plasma-urotensin-ii-levels-in-children-and-adolescents-with-chronic-kidney-disease-a-single-centre-study
#19
Anastasia Garoufi, Styliani Drapanioti, Antonios Marmarinos, Varvara Askiti, Andromachi J Mitsioni, Maria Mila, Georgia Grigoriadou, Dimitrios Georgakopoulos, Constantinos J Stefanidis, Dimitrios Gourgiotis
BACKGROUND: Increased plasma Urotensin II (UII) levels have been found in adults with renal diseases. Studies in children are scarce. The objective of the study is to estimate plasma UII levels in subjects with chronic kidney disease (CKD) stages 3 to 5 and renal transplant recipients (RTR). In addition, the correlation of UII with anthropometric features and biochemical parameters was assessed. METHODS: Fifty-four subjects, aged 3 to 20 years old, 23 with CKD, 13 with end-stage kidney disease (ESKD) undergoing hemodialysis (HD) and 18 RTR were enrolled...
March 31, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28348471/role-of-allopurinol-in-optimizing-thiopurine-therapy-in-patients-with-autoimmune-hepatitis-a-review
#20
REVIEW
Shivani Deswal, Anshu Srivastava
Autoimmune hepatitis (AIH) is a chronic immune mediated liver disease characterized by elevated transaminases, hyper gammaglobulinemia, presence of autoantibodies and interface hepatitis in the absence of a known etiology of liver disease. Thiopurines (azathioprine [AZA]/6-mercaptopurine [6MP]) and steroids remain the first line of treatment of AIH in both children and adults. However, a small proportion of AIH patients are either non-responders or develop side effects with AZA. The metabolism of AZA is complex and mediated by multiple enzymes...
March 2017: Journal of Clinical and Experimental Hepatology
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