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Metabolic liver disease children

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https://www.readbyqxmd.com/read/28803487/a-mechanism-based-approach-to-management-of-children-with-end-stage-liver-disease
#1
Allah B Haafiz
Due to parallel advances in surgical and acute care disciplines, liver transplantation (LT) has revolutionized the outlook for children with end-stage liver disease (ESLD). Contrary to advances in technical aspects of LT and the peri-operative care, pre-transplant management of ESLD remains quite a formidable challenge. Areas covered: This review provides mechanisms based management strategies to address common complications of ESLD including malnutrition, amended metabolic pathways, gastrointestinal dysfunction, and development of ascites...
August 14, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28759573/alternation-of-plasma-fatty-acids-composition-and-desaturase-activities-in-children-with-liver-steatosis
#2
Man-Chin Hua, Hui-Min Su, Tsung-Chieh Yao, Ming-Ling Kuo, Ming-Wei Lai, Ming-Han Tsai, Jing-Long Huang
OBJECTIVE: The aim of this study was to investigate changes in plasma fatty acids proportions and estimated desaturase activities for variable grading of liver steatosis in children. METHODS: In total, 111 schoolchildren (aged 8-18 years) were included in the analysis from March 2015 to August 2016. Anthropometric evaluation, liver ultrasound examination and scoring for nonalcoholic fatty liver disease (NAFLD score = 0-6), and biochemical and plasma fatty acids analysis were performed...
2017: PloS One
https://www.readbyqxmd.com/read/28759094/conversion-of-sugar-to-fat-is-hepatic-de-novo-lipogenesis-leading-to-metabolic-syndrome-and-associated-chronic-diseases
#3
Jean-Marc Schwarz, Michael Clearfield, Kathleen Mulligan
Epidemiologic studies suggest a link between excess sugar consumption and obesity, fatty liver disease, metabolic syndrome, and type 2 diabetes mellitus. One important pathway that may link these metabolic diseases to sugar consumption is hepatic conversion of sugar to fat, a process known as de novo lipogenesis (DNL). Mechanistic studies have shown that diets high in simple sugars increase both DNL and liver fat. Importantly, removal of sugar from diets of children with obesity for only 9 days consistently reduced DNL and liver fat and improved glucose and lipid metabolism...
August 1, 2017: Journal of the American Osteopathic Association
https://www.readbyqxmd.com/read/28755186/liver-transplantation-for-hereditary-tyrosinaemia-type-1-in-the-united-kingdom
#4
Patrick McKiernan
Fourteen children have undergone liver transplantation for hereditary tyrosinaemia type 1 (HT1) at Birmingham Children's hospital (BCH) since 1989; six were treated prior to the availability of Nitisinone in 1993 and eight in the post Nitisinone era. Prior to 1993 essentially all children with HT1 were referred for transplantation. In the Nitisinone era only those with unresponsive liver failure or suspected malignancy were considered for transplantation. Those who were treated pre-emptively following newborn screening have no evidence of liver disease and none have required transplantation...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28751535/how-to-use-tests-for-disorders-of-copper-metabolism
#5
Jane Armer, Christian De Goede
In paediatrics, one of our main aims in the diagnostic process is to identify any treatable conditions. The copper metabolism disorder Wilson's disease (WD) is one such condition that is caused by mutations in the ATP7B gene. Delay in treatment could result in irreversible disability or even death. Although liver disease is the most common presenting feature in children, some children may initially present with a subtle neurological presentation only. In patients presenting with dystonia, tremor, dysarthria or with a deterioration in school performance, there should be a high index of suspicion for WD...
July 27, 2017: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/28737569/elevated-hemoglobin-level-is-associated-with-advanced-fibrosis-in-pediatric-nonalcoholic-fatty-liver-disease
#6
Valentina Giorgio, Antonella Mosca, Arianna Alterio, Anna Alisi, Antonio Grieco, Valerio Nobili, Luca Miele
OBJECTIVES: Hemoglobin (Hb) and red blood cell distribution width (RDW) have been reported to be a risk marker of metabolic syndrome and nonalcoholic fatty liver disease (NAFLD). No study exists on pediatric populations. We aimed to determine the association between hematological parameters, and the severity of disease in children with biopsy-proven NAFLD. METHODS: A total of 117 children (85 boys, mean age 12 years) with ultrasound evidence of NAFLD undergoing liver biopsy for diagnosis of nonalcoholic steatohepatitis (NASH), were prospectively enrolled between January 2011 and May 2013 in the setting of a tertiary care center...
August 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28699612/relationship-between-immune-parameters-and-non-alcoholic-fatty-liver-disease-in-obese-children
#7
Jun-Qi Shi, Wen-Xia Shen, Xiang-Zhi Wang, Ke Huang, Chao-Chun Zou
OBJECTIVE: To investigate the relationship between immune parameters and non-alcoholic fatty liver disease (NAFLD) in obese children. DESIGN: Case-control study. SETTING: Zhejiang Province, China on July to September 2015. PARTICIPANTS: A total of 117 obese children and 209 healthy non-obese children were studied as the obese and control groups. Depending on the severity of NAFLD, the obese group was divided into subgroups 1 (without NAFLD), 2 (with simple fatty liver) and 3 (with steatohepatitis)...
July 11, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28689548/-non-alcoholic-fatty-liver-disease-in-children-and-adolescents
#8
Jessica Björklund, Tea Lund Laursen, Konstantin Kazankov, Karen Louise Thomsen, Stephen Hamilton-Dutoit, Elisabeth Stenbøg, Henning Grønbæk
Non-alcoholic fatty liver disease (NAFLD) is characterized by liver fat accumulation and non-alcoholic steatohepatitis (NASH) with inflammation and fibrosis, which may lead to cirrhosis also in childhood. NAFLD/NASH in children are related to obesity and the metabolic syndrome, and incidence and prevalence are expected to increase. Children having liver steatosis and elevated liver enzymes are most often asymptomatic, and a liver biopsy is necessary for correct diagnosis and staging. The treatment should focus on lifestyle changes, as pharmacological therapy needs further evaluation...
July 3, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28685316/-liver-biopsy-in-children-and-adolescents-preliminary-morphological-examinations-in-diffuse-liver-disease
#9
REVIEW
C Lackner, A S Knisely
BACKGROUND: Frequencies of types of liver disease differ between adults and nonadults (NA). OBJECTIVES: The particular problems encountered in interpreting liver-biopsy findings in NA often require referral in consultation.  To permit this efficiently, we recommend specific approaches to light microscopy, with special stains and immunohistochemistry, as well as to ultrastructural study. METHODS: Prosection and the choice of special stains are described, and are discussed in relation to clinical questions...
July 2017: Der Pathologe
https://www.readbyqxmd.com/read/28677866/increased-incidence-of-non-alcoholic-fatty-liver-disease-in-male-rat-offspring-exposed-to-fluoxetine-during-fetal-and-neonatal-life-involves-the-nlrp3-inflammasome-and-augmented-de-novo-hepatic-lipogenesis
#10
Nicole E De Long, Daniel B Hardy, Noelle Ma, Alison C Holloway
Up to 10% of women take selective serotonin reuptake inhibitors (SSRI) during pregnancy. Children exposed to SSRIs in utero have an increased risk of being overweight suggesting that fetal exposure to SSRIs can cause permanent metabolic changes. We have previously shown in rats that fetal and neonatal exposure to the SSRI antidepressant fluoxetine results in metabolic perturbations including increased hepatic triglyceride content; a hallmark of non-alcoholic fatty liver disease (NAFLD). Therefore, the aim of this study was to identify the mechanism(s) underlying the fluoxetine-induced increase in intrahepatic triglyceride content...
July 5, 2017: Journal of Applied Toxicology: JAT
https://www.readbyqxmd.com/read/28676332/circulating-exosomes-in-obstructive-sleep-apnea-as-phenotypic-biomarkers-and-mechanistic-messengers-of-end-organ-morbidity
#11
REVIEW
Abdelnaby Khalyfa, Leila Kheirandish-Gozal, David Gozal
Obstructive sleep apnea (OSA), the most severe form of sleep disordered breathing, is characterized by intermittent hypoxia during sleep (IH), sleep fragmentation, and episodic hypercapnia. OSA is associated with increased risk for morbidity and mortality affecting cardiovascular, metabolic, and neurocognitive systems, and more recently with non-alcoholic fatty liver disease (NAFLD) and cancer-related deaths. Substantial variability in OSA outcomes suggests that genetically-determined and environmental and lifestyle factors affect the phenotypic susceptibility to OSA...
July 1, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28663066/successful-management-of-acute-liver-failure-in-italian-children-a-16-year-experience-at-a-referral-centre-for-paediatric-liver-transplantation
#12
A Di Giorgio, A Sonzogni, A Piccichè, G Alessio, E Bonanomi, M Colledan, L D'Antiga
BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: 55 children (median age 2...
June 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28646278/liver-involvement-in-kidney-disease-and-vice-versa
#13
REVIEW
Karen Van Hoeve, Djalila Mekahli, Eva Morava, Elena Levtchenko, Peter Witters
The liver and kidneys are often similarly affected by a single disease. This is the case in metabolic, immunological, toxic, and infectious diseases, and in the different congenital malformation syndromes. Also, an enzymatic defect in an otherwise healthy liver or the consequences of advanced liver disease by itself can cause kidney disease as a secondary phenomenon. In this review, we describe numerous pathogenic mechanisms leading to dysfunction or malformations of the liver and kidneys in children. We encourage multidisciplinary management for optimal care...
June 23, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28625218/the-response-of-male-and-female-rats-to-a-high-fructose-diet-during-adolescence-following-early-administration-of-hibiscus-sabdariffa-aqueous-calyx-extracts
#14
K G Ibrahim, E Chivandi, F B O Mojiminiyi, K H Erlwanger
Metabolic syndrome is linked to the consumption of fructose-rich diets. Nutritional and pharmacological interventions perinatally can cause epigenetic changes that programme an individual to predispose or protect them from the development of metabolic diseases later. Hibiscus sabdariffa (HS) reportedly has anti-obesity and hypocholesterolaemic properties in adults. We investigated the impact of neonatal intake of HS on the programming of metabolism by fructose. A total of 85 4-day-old Sprague Dawley rats were divided randomly into three groups...
June 19, 2017: Journal of Developmental Origins of Health and Disease
https://www.readbyqxmd.com/read/28608518/liver-transplantation-may-prevent-neurodevelopmental-deterioration-in-high-risk-patients-with-urea-cycle-disorders
#15
Jun Kido, Shirou Matsumoto, Ken Momosaki, Rieko Sakamoto, Hiroshi Mitsubuchi, Fumio Endo, Kimitoshi Nakamura
UCDs are among the most common inherited metabolic diseases in Japan. We investigated the clinical manifestations, treatment, and prognoses of 177 patients with UCDs who were evaluated and treated from January 1999 to March 2009 in Japan, using a questionnaire survey. Among these 177 patients, 42 (seven with carbamoyl phosphate synthetase 1 deficiency, 27 with ornithine transcarbamylase deficiency, seven with argininosuccinate synthetase deficiency, and one with arginase 1 deficiency) underwent living-donor LT...
June 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28606575/good-adherence-to-the-mediterranean-diet-reduces-the-risk-for-nash-and-diabetes-in-pediatric-patients-with-obesity-the-results-of-an-italian-study
#16
Claudia Della Corte, Antonella Mosca, Andrea Vania, Arianna Alterio, Salvatore Iasevoli, Valerio Nobili
OBJECTIVE: In the last decade, it was demonstrated that the Mediterranean diet (MD) represents an ideal diet for all age groups and has an important role in the prevention of metabolic and cardiovascular diseases, as well as nonalcoholic fatty liver disease (NAFLD). The aim of the present study was to analyze the association between adherence to the MD and NAFLD, with laboratory and histologic evaluation, in a group of children and adolescents with obesity. METHODS: We enrolled 243 patients with obesity referred to our department from March 2014 to November 2015...
July 2017: Nutrition
https://www.readbyqxmd.com/read/28600116/features-of-nonalcoholic-steatohepatitis-in-severely-obese-children-and-adolescents-undergoing-sleeve-gastrectomy
#17
Aayed Alqahtani, Mohamed Elahmedi, Khalid Alswat, Maha Arafah, Mosa Fagih, Jaehoon Lee
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is an underrecognized co-morbidity of obesity. The characteristic features and severity of NAFLD in severe childhood obesity remain unknown. OBJECTIVES: To investigate features of NAFLD in obese children and identify predictors of significant disease. SETTING: Academic center with a standardized pathway for pediatric bariatric surgery and a dedicated multidisciplinary team. DESIGN: This is a baseline cross-sectional analysis for a data set obtained from a prospective clinical outcome study that included severely obese children (≤14 yr of age) and adolescents (14-21 yr of age) who underwent laparoscopic sleeve gastrectomy between March 2008 and March 2015...
April 7, 2017: Surgery for Obesity and related Diseases: Official Journal of the American Society for Bariatric Surgery
https://www.readbyqxmd.com/read/28588179/childhood-wilson-disease-bangladesh-perspective
#18
M Rukunuzzaman, A B Karim, M Nurullah, F Sultana, M W Mazumder, M A Rahman, S B Billah, F Begum, M Oliullah
Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28587303/the-role-of-lipid-and-lipoprotein-metabolism-in-non-alcoholic-fatty-liver-disease
#19
REVIEW
Francesco Massimo Perla, Maurizia Prelati, Michela Lavorato, Daniele Visicchio, Caterina Anania
Due to the epidemic of obesity across the world, nonalcoholic fatty liver disease (NAFLD) has become one of the most prevalent chronic liver disorders in children and adolescents. NAFLD comprises a spectrum of fat-associated liver conditions that can result in end-stage liver disease and the need for liver transplantation. Simple steatosis, or fatty liver, occurs early in NAFLD and may progress to nonalcoholic steatohepatitis, fibrosis and cirrhosis with increased risk of hepatocellular carcinoma. The mechanism of the liver injury in NAFLD is currently thought to be a "multiple-hit process" where the first "hit" is an increase in liver fat, followed by multiple additional factors that trigger the inflammatory activity...
June 6, 2017: Children
https://www.readbyqxmd.com/read/28579536/effects-of-dietary-fructose-restriction-on-liver-fat-de-novo-lipogenesis-and-insulin-kinetics-in-children-with-obesity
#20
Jean-Marc Schwarz, Susan M Noworolski, Ayca Erkin-Cakmak, Natalie J Korn, Michael J Wen, Viva W Tai, Grace M Jones, Sergiu P Palii, Moises Velasco-Alin, Karen Pan, Bruce W Patterson, Alejandro Gugliucci, Robert H Lustig, Kathleen Mulligan
BACKGROUND & AIMS: Consumption of sugar is associated with obesity, type 2 diabetes mellitus, nonalcoholic fatty liver disease, and cardiovascular disease. The conversion of fructose to fat in liver (de novo lipogenesis [DNL]) may be a modifiable pathogenetic pathway. We determined the effect of 9 days of isocaloric fructose restriction on DNL, liver fat, visceral fat (VAT), subcutaneous fat, and insulin kinetics in obese Latino and African American children with habitual high sugar consumption (fructose intake >50 g/d)...
June 1, 2017: Gastroenterology
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