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Metabolic liver disease children

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https://www.readbyqxmd.com/read/29046229/fetal-overnutrition-and-adolescent-hepatic-fat-fraction-the-exploring-perinatal-outcomes-in-children-study
#1
Anna Bellatorre, Ann Scherzinger, Elizabeth Stamm, Mercedes Martinez, Brandy Ringham, Dana Dabelea
OBJECTIVE: To determine if fetal overnutrition resulting from maternal obesity or gestational diabetes mellitus (GDM) is associated with increased liver fat during adolescence, adjusting for past and current metabolic risk factors. STUDY DESIGN: Data come from a historical prospective cohort study (Exploring Perinatal Outcomes in Children) of 254 mother-child pairs in Colorado who participated in 2 research visits at T1 (mean age 10.4, SD = 1.5 years) and at T2 (mean age 16...
October 15, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29046209/-paroxysmal-muscle-weakness-liver-enlargement-and-hypoglycemia-in-a-boy
#2
Ya-Jie Cui, Chun-Lan Song, Yi-Bing Cheng
A boy aged 11 years was admitted due to intermittent weakness and difficulty in walking for 6 years, and hepatomegaly, glycopenia and unconsciousness for 4 years. The laboratory examinations showed severe metabolic acidosis, hypoglycemia, and abnormal liver function. CT scan showed marked liver enlargement with fat density shadow. The boy was given fluid infusion, correction of acidosis, intravenous injection of glucose, L-carnitine, compound vitamin B, and coenzyme Q10, but he was in a persistent coma and it was difficult to correct refractory metabolic acidosis and hypoglycemia...
October 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29039873/tillf%C3%A3-rlitliga-referensintervall-kr%C3%A3-vs-f%C3%A3-r-v%C3%A3-rdering-av-p-alp-nya-pediatriska-referensintervall-f%C3%A3-r-alkaliskt-fosfatas-har-klinisk-betydelse-f%C3%A3-r-att-hitta-r%C3%A3-tt-till-diagnosen
#3
Per Magnusson
Age- and gender-specific reference intervals are pivotal to ensure appropriate interpretation of plasma alkaline phosphatase activities in the lower range Hypophosphatasia (HPP) is an inborn error of metabolism caused by loss-of-function mutations of the ALPL gene that mainly express alkaline phosphatase (ALP) in bone and liver. The clinical expression of HPP is highly variable and is classified into six different forms mainly affecting bone and tooth mineralization. The prognosis for each of these HPP forms depends upon the severity of the skeletal disease which reflects the age at presentation...
October 16, 2017: Läkartidningen
https://www.readbyqxmd.com/read/28991837/serum-osteocalcin-levels-in-children-with-nonalcoholic-fatty-liver-disease
#4
Saleh Amin, Doaa El Amrousy, Shaymaa Elrifaey, Rasha Gamal, Hossam Hodeib
OBJECTIVES: to investigate the relationship between osteocalcin and non alcoholic fatty liver disease (NAFLD) in obese children. METHOD: 60 obese children with NAFLD were taken as patient group and 60 non - NAFLD children with matches age, sex and BMI were taken as 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...
October 6, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28988228/the-relationship-between-non-alcoholic-fatty-liver-disease-and-small-intestinal-bacterial-overgrowth-among-overweight-and-obese-children-and-adolescents
#5
Oana Belei, Laura Olariu, Andreea Dobrescu, Tamara Marcovici, Otilia Marginean
BACKGROUND: The increasing rate of obesity and overweight among children has highlighted nonalcoholic fatty liver disease (NAFLD) as the most common cause of chronic pediatric liver diseases. There are many publications supporting the idea that gut microbiota is altered in NAFLD. The aim of this study was to evaluate the prevalence of NAFLD among overweight and obese children with and without small intestinal bacterial overgrowth (SIBO) compared to a control group and to assess if intestinal dysbiosis represents a risk factor for NAFLD...
October 9, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28986475/pediatric-cytochrome-p450-activity-alterations-in-nonalcoholic-steatohepatitis
#6
Hui Li, Canet J Mark, John D Clarke, Dean Billheimer, Stavra A Xanthakos, Joel E Lavine, Robert P Erickson, Nathan J Cherrington
Variable drug responses(VDRs) are dependent upon individual variation in the activity of drug-metabolizing enzymes including cytochrome P450s(CYPs). As the most common chronic liver disease in children and adults, nonalcoholic steatohepatitis(NASH) has been identified as a source of significant inter-individual variation in hepatic drug metabolism. Compared to adults, children present age-related differences in pharmacokinetics and pharmacodynamics. The purpose of this study was to determine the impact of fatty liver disease severity on the activity of a variety of CYPs in children and adolescents...
October 6, 2017: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/28979846/the-transcription-factor-atf7-mediates-in-vitro-fertilization-induced-gene-expression-changes-in-mouse-liver
#7
Yang Liu, Toshio Maekawa, Keisuke Yoshida, Hideki Kaneda, Bruno Chatton, Shigeharu Wakana, Shunsuke Ishii
Assisted reproductive technologies, including in vitro fertilization (IVF), are now frequently used, and increasing evidence indicates that IVF causes gene expression changes in children and adolescents that increase the risk of metabolic diseases. Although such gene expression changes are thought to be due to IVF-induced epigenetic changes, the mechanism remains elusive. We tested whether the transcription factor ATF7-which mediates stress-induced changes in histone H3K9 tri- and dimethylation, typical marks of epigenetic silencing-is involved in the IVF-induced gene expression changes...
October 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28953935/association-of-mttp-gene-variants-with-pediatric-nafld-a-candidate-gene-based-analysis-of-single-nucleotide-variations-in-obese-children
#8
Dongling Dai, Feiqiu Wen, Shaoming Zhou, Zhe Su, Guosheng Liu, Mingbang Wang, Jianli Zhou, Fusheng He
OBJECTIVE: We used targeted next-generation sequencing to investigate whether genetic variants of lipid metabolism-related genes are associated with increased susceptibility to nonalcoholic fatty liver disease (NAFLD) in obese children. METHODS: A cohort of 100 obese children aged 6 to 18 years were divided into NAFLD and non-NAFLD groups and subjected to hepatic ultrasound, anthropometric, and biochemical analyses. We evaluated the association of genetic variants with NAFLD susceptibility by investigating the single nucleotide polymorphisms in each of 36 lipid-metabolism-related genes...
2017: PloS One
https://www.readbyqxmd.com/read/28948754/histopathology-grading-and-staging-of-nonalcoholic-fatty-liver-disease
#9
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
https://www.readbyqxmd.com/read/28942965/biallelic-c1qbp-mutations-cause-severe-neonatal-childhood-or-later-onset-cardiomyopathy-associated-with-combined-respiratory-chain-deficiencies
#10
René G Feichtinger, Monika Oláhová, Yoshihito Kishita, Caterina Garone, Laura S Kremer, Mikako Yagi, Takeshi Uchiumi, Alexis A Jourdain, Kyle Thompson, Aaron R D'Souza, Robert Kopajtich, Charlotte L Alston, Johannes Koch, Wolfgang Sperl, Elisa Mastantuono, Tim M Strom, Saskia B Wortmann, Thomas Meitinger, Germaine Pierre, Patrick F Chinnery, Zofia M Chrzanowska-Lightowlers, Robert N Lightowlers, Salvatore DiMauro, Sarah E Calvo, Vamsi K Mootha, Maurizio Moggio, Monica Sciacco, Giacomo P Comi, Dario Ronchi, Kei Murayama, Akira Ohtake, Pedro Rebelo-Guiomar, Masakazu Kohda, Dongchon Kang, Johannes A Mayr, Robert W Taylor, Yasushi Okazaki, Michal Minczuk, Holger Prokisch
Complement component 1 Q subcomponent-binding protein (C1QBP; also known as p32) is a multi-compartmental protein whose precise function remains unknown. It is an evolutionary conserved multifunctional protein localized primarily in the mitochondrial matrix and has roles in inflammation and infection processes, mitochondrial ribosome biogenesis, and regulation of apoptosis and nuclear transcription. It has an N-terminal mitochondrial targeting peptide that is proteolytically processed after import into the mitochondrial matrix, where it forms a homotrimeric complex organized in a doughnut-shaped structure...
October 5, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28930295/global-burden-of-nafld-and-nash-trends-predictions-risk-factors-and-prevention
#11
REVIEW
Zobair Younossi, Quentin M Anstee, Milena Marietti, Timothy Hardy, Linda Henry, Mohammed Eslam, Jacob George, Elisabetta Bugianesi
NAFLD is one of the most important causes of liver disease worldwide and will probably emerge as the leading cause of end-stage liver disease in the coming decades, with the disease affecting both adults and children. The epidemiology and demographic characteristics of NAFLD vary worldwide, usually parallel to the prevalence of obesity, but a substantial proportion of patients are lean. The large number of patients with NAFLD with potential for progressive liver disease creates challenges for screening, as the diagnosis of NASH necessitates invasive liver biopsy...
September 20, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28912414/polish-experience-with-liver-transplantation-and-post-transplant-outcomes-in-children-with-urea-cycle-disorders
#12
Edyta Szymańska, Piotr Kaliciński, Joanna Pawłowska, Sylwia Szymańska, Maciej Pronicki, Marek Stefanowicz, Joanna Teisseyre, Dorota Broniszczak, Dariusz Rokicki
BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation...
September 15, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28902093/bile-acid-synthesis-disorders-in-arabs-a-10-year-screening-study
#13
Abdulrahman Al-Hussaini, Kenneth D R Setchell, Bader AlSaleem, James E Heubi, Khurram Lone, Anne Davit-Spraul, Emmanuel Jacquemin
OBJECTIVES: Early diagnosis of bile acid synthesis disorders (BASD) is important because, untreated, these conditions can be fatal. Our objectives were to screen children with cholestasis or unexplained liver disease for BASD and in those with confirmed BASD to evaluate the effectiveness of cholic acid therapy. METHODS: A routine serum total bile acid measurement was performed on children with cholestasis, liver cirrhosis, and liver failure. Patients were screened for BASD by fast atom bombardment ionization-mass spectrometry (FAB-MS) analysis of urine, and molecular analysis confirmed diagnosis...
September 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28880885/markers-of-intestinal-permeability-are-already-altered-in-early-stages-of-non-alcoholic-fatty-liver-disease-studies-in-children
#14
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
https://www.readbyqxmd.com/read/28803487/a-mechanism-based-approach-to-management-of-children-with-end-stage-liver-disease
#15
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 28, 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
#16
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
#17
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
#18
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
#19
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
#20
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
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