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Metabolic liver disease children

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https://www.readbyqxmd.com/read/29884776/lysosomal-acid-lipase-deficiency-a-rare-pathology-the-first-pediatric-patient-reported-in-colombia
#1
Verónica Botero, Victor H Garcia, Catalina Gomez-Duarte, Ana M Aristizabal, Ana M Arrunategui, Gabriel J Echeverri, Harry Pachajoa
BACKGROUND Lysosomal acid lipase deficiency is a rare genetic metabolic lipid storage disease, with a high morbidity, and mortality, in children and adults. It is characterized by a mutation in the LIPA gene that causes an alteration of lipid metabolism, resulting in deposits of cholesterol esters and triglycerides in organs such as the liver, blood vessels, and gastrointestinal tract. Lysosomal acid lipase deficiency is predominantly caused by the mutation c.894G>A, seen in approximately 50-70% of patients...
June 9, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29864682/non-alcoholic-fatty-liver-disease-severity-and-metabolic-complications-in-obese-children-impact-of-omega-3-fatty-acids
#2
Schohraya Spahis, Fernando Alvarez, Nejma Ahmed, Josée Dubois, Ramy Jalbout, Massimiliano Paganelli, Kelly Grzywacz, Edgard Delvin, Noel Peretti, Emile Levy
Although n-3 polyunsaturated fatty acids (PUFA) revealed promising therapeutic results in non-alcoholic fatty liver disease (NAFLD), which is considered as the most prevalent cause of chronic hepatic disease, inconsistencies are calling for further confirmatory trials to demonstrate therapeutic efficacy and safety. The study, registered as NCT02201160 on www.clinicaltrials.gov, was designed to compare two groups of NAFLD with a different severity, and to evaluate the efficacy of n-3 PUFA supplementation. Twenty young male participants of French Canadian origin with NAFLD were enrolled and classified into moderate (mNAFLD) and severe (sNAFLD) fatty liver groups, according to transaminase levels, ultrasonography, NAFLD Activity Score and Fatty Liver Index (FLI)...
April 10, 2018: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/29861326/the-rate-of-hepatic-artery-complications-is-higher-in-pediatric-liver-transplant-recipients-with-metabolic-liver-diseases-than-with-biliary-atresia
#3
Suk Kyun Hong, Nam-Joon Yi, Hak Chang, Sung-Woo Ahn, Hyo-Sin Kim, Kyung Chul Yoon, Hyeyoung Kim, Seong Oh Park, Ung Sik Jin, Kyung Won Minn, Kwang-Woong Lee, Kyung-Suk Suh
BACKGROUND: Liver transplantation (LT) is an excellent treatment option for patients with biliary atresia (BA) who fail portoenterostomy surgery. LT is also increasingly performed in patients with metabolic liver diseases. This study compared the outcomes in pediatric patients who underwent LT for metabolic liver diseases and BA. BASIC PROCEDURES: Data from 237 pediatric patients who underwent primary LT at Seoul National University Hospital from 1988 to 2015, including 33 with metabolic liver diseases and 135 with BA, were retrospectively analyzed...
April 27, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29860977/omega-3-fatty-acids-and-fatty-liver-disease-in-children
#4
Claudia Della Corte, Salvatore Iasevoli, Andrea Dello Strologo, Mariateresa Sanseviero, Valerio Nobili
Nonalcoholic fatty liver disease (NAFLD) represents the most common cause of chronic liver disease in Industrialized Countries in adults and children. It is estimated that NAFLD will become the main indication for liver transplantation in the next decade. NAFLD is also considered the hepatic feature of metabolic syndrome and therefore it is generally associated to the risk of developing some metabolic complications, with negative impact on patient's survival. Today, no pharmacological treatment has been identified for NAFLD, and behavioral approach, based on diet and regular physical exercise, represent the current recommended treatment, even if with disappointing results...
2018: Advances in Food and Nutrition Research
https://www.readbyqxmd.com/read/29795465/paternal-cholestasis-exacerbates-obesity-associated-hypertension-in-male-offspring-but-is-prevented-by-paternal-ursodeoxycholic-acid-treatment
#5
Vanessa Pataia, Georgia Papacleovoulou, Vanya Nikolova, Anne-Maj Samuelsson, Stephanie Chambers, Eugene Jansen, Paul D Taylor, Lucilla Poston, Catherine Williamson
BACKGROUND: Obesity is a heterogeneous phenotype and risk associations to non-communicable diseases such as cardiovascular disease and type 2 diabetes are influenced by several factors. The paternal metabolic status at the time of conception influences offspring susceptibility to developing obesity and adiposity-associated cardiometabolic disease. Cholestatic liver diseases are characterized by raised circulating serum bile acid levels and dyslipidemia, and are commonly treated with ursodeoxycholic acid (UDCA)...
May 24, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/29785076/role-of-osteoprotegerin-receptor-activator-of-nuclear-factor-kappa-b-receptor-activator-of-nuclear-factor-kappa-b-ligand-axis-in-nonalcoholic-fatty-liver-disease
#6
REVIEW
Lucia Pacifico, Gian Marco Andreoli, Miriam D'Avanzo, Delia De Mitri, Pasquale Pierimarchi
Concomitantly with the increase in the prevalences of overweight/obesity, nonalcoholic fatty liver disease (NAFLD) has worldwide become the main cause of chronic liver disease in both adults and children. Patients with fatty liver display features of metabolic syndrome (MetS), like insulin resistance (IR), glucose intolerance, hypertension and dyslipidemia. Recently, epidemiological studies have linked obesity, MetS, and NAFLD to decreased bone mineral density and osteoporosis, highlighting an intricate interplay among bone, adipose tissue, and liver...
May 21, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29761652/vitamin-d-status-and-associated-genetic-polymorphisms-in-a-cohort-of-uk-children-with-non-alcoholic-fatty-liver-disease
#7
P S Gibson, A Quaglia, A Dhawan, H Wu, S Lanham-New, K H Hart, E Fitzpatrick, J B Moore
BACKGROUND: Vitamin D deficiency has been associated with non-alcoholic fatty liver disease (NAFLD). However, the role of polymorphisms determining vitamin D status remains unknown. OBJECTIVES: The objectives of this study were to determine in UK children with biopsy-proven NAFLD (i) their vitamin D status throughout a 12-month period and (ii) interactions between key vitamin D-related genetic variants (nicotinamide adenine dinucleotide synthase-1/dehydrocholesterol reductase-7, vitamin D receptor, group-specific component, CYP2R1) and disease severity...
May 14, 2018: Pediatric Obesity
https://www.readbyqxmd.com/read/29752170/treating-obstructive-sleep-apnea-and-chronic-intermittent-hypoxia-improves-the-severity-of-nonalcoholic-fatty-liver-disease-in-children
#8
Shikha S Sundaram, Ann C Halbower, Jelena Klawitter, Zhaoxing Pan, Kristen Robbins, Kelley E Capocelli, Ronald J Sokol
OBJECTIVE: To determine the effects of treating obstructive sleep apnea/nocturnal hypoxia on pediatric nonalcoholic fatty liver disease (NAFLD) severity and oxidative stress. STUDY DESIGN: Biopsy proven participants (n = 9) with NAFLD and obstructive sleep apnea/hypoxia were studied before and after treatment with continuous positive airway pressure (CPAP) for sleep disordered breathing, including laboratory testing and markers of oxidative stress, urine F(2)-isoprostanes...
May 8, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29713608/investigation-of-blood-betatrophin-levels-in-obese-children-with-non-alcoholic-fatty-liver-disease
#9
Fatih Battal, Hakan Türkön, Nilüfer Aylanç, Hakan Aylanç, Şule Yıldırım, Nazan Kaymaz, Sema Uysal
Purpose: The prevalence of obesity has significantly increased among children and adolescents worldwide and is becoming an important health care problem in parallel with the increased prevalence of obesity pediatric non-alcoholic fatty liver disease. Betatrophin is a newly define hormone that is commonly secreted by liver and plays role in glucose tolerance. This study aimed to investigate the relationship between serum betatrophin levels and non-alcoholic fatty liver disease in obese children...
April 2018: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29701975/characterizing-the-biochemical-response-to-schistosoma-mansoni-infection-and-treatment-with-praziquantel-in-preschool-and-school-aged-children
#10
Gordana Panic, Jean T Coulibaly, Nikita Harvey, Jennifer Keiser, Jonathan Swann
Schistosomiasis is a widespread chronic neglected tropical disease prevalent mostly in children in under-resourced rural areas. Its pathological effects have been clinically characterized, yet the molecular-level effects are understudied. In this study, the biochemical effects of Schistosoma mansoni infection and praziquantel treatment were studied in 130 preschool aged and 159 school aged infected children and 11 noninfected children in Azaguié, Côte d'Ivoire. Urine samples were collected prior to receiving 20, 40, or 60 mg/kg of praziquantel or a placebo, as well as 24 h post-treatment, and at the 3-week follow up...
June 1, 2018: Journal of Proteome Research
https://www.readbyqxmd.com/read/29699915/dyslipidemia-is-associated-with-pediatric-nonalcoholic-fatty-liver-disease
#11
Shima Dowla, Stella Aslibekyan, Amy Goss, Kevin Fontaine, Ambika P Ashraf
BACKGROUND: With the increasing prevalence of childhood obesity, nonalcoholic fatty liver disease (NAFLD) has emerged as the most common cause of pediatric chronic liver disorder. Factors underlying the pathophysiology of NAFLD remain poorly defined. OBJECTIVE: This study aimed to describe the metabolic characteristics of children with NAFLD differing in race/ethnicity and to test associations between dyslipidemia and NAFLD. METHODS: A retrospective chart review was conducted at a tertiary referral university hospital among 309 children with a diagnosis of NAFLD...
April 3, 2018: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/29684480/fetuin-b-links-vitamin-d-deficiency-and-pediatric-obesity-direct-negative-regulation-by-vitamin-d
#12
Gillian E Walker, Antonia Follenzi, Valentina Bruscaggin, Marcello Manfredi, Simonetta Bellone, Emilio Marengo, Luigi Maiuri, Flavia Prodam, Gianni Bona
Vitamin D (VD) deficiency (VDD) correlates to obesity, with VD a recognized mediator of metabolic diseases. From a previous proteomic study identifying adiponectin as a link between VDD and pediatric obesity, herein we analysed another protein (SSP2301) increased with VDD. A focused 2D-electrophoretic analysis identified 4 corresponding plasma proteins, with one predicted to be fetuin B (FETUB). FETUB was studied due to its emerging role in metabolic diseases and cytogenetic location (3q27.3) with adiponectin...
April 21, 2018: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/29680189/vitamin-d-non-sufficiency-is-prevalent-in-children-with-chronic-liver-disease-in-a-tropical-country
#13
Way Seah Lee, Muhammad Yazid Jalaludin, Shin Yee Wong, Sik Yong Ong, Hee Wei Foo, Ruey Terng Ng
BACKGROUND: To determine vitamin D status in children with chronic liver disease (CLD) in a tropical country. METHODS: Cross-sectional study in Malaysian children with CLD. Factors affecting serum vitamin D level (definition: deficient < 30 nmol/L; insufficient 30-50 nmol/L; sufficient ≥ 50 nmol/L) was analyzed. RESULTS: Of the 59 children studied (males 32, 54%; median age 6.8 ± 5.3 years), the three most common causes were biliary atresia (n = 25), autoimmune hepatitis (n = 16) and sclerosing cholangitis (n = 6)...
April 5, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29668570/value-of-serum-zinc-in-diagnosing-and-assessing-severity-of-liver-disease-in-children-with-wilson-disease
#14
Palittiya Sintusek, Eirini Kyrana, Anil Dhawan
Supplemental Digital Content is available in the textABSTRACT OBJECTIVES:: Wilson disease (WD) is a rare inborn error of copper metabolism with diverse manifestations. There has been no study of zinc (Zn), the copper's antagonist, in WD diagnosis and severity so far. Our aims were to evaluate serum Zn in WD and its correlation with the disease severity score (revised WD index). Although the ATP7B mutation analysis is highly accurate for WD diagnosis, it may not be readily available in a resource-limiting setting...
April 17, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29651819/biochemical-predictors-of-early-onset-non-alcoholic-fatty-liver-disease-in-young-children-with-obesity
#15
Ju Young Kim, Jinmin Cho, Hye Ran Yang
BACKGROUND: The prevalence of metabolic syndrome (MS) and non-alcoholic fatty liver disease (NAFLD) and their associated risk factors are not well-established in young children with obesity. The purpose of this study was to evaluate the prevalence of early onset NAFLD and identify its biochemical predictors in obese children aged less than 10 years. METHODS: Anthropometric measurements, blood pressure, laboratory tests, and abdominal ultrasonography (USG) were performed in all subjects...
April 16, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29610169/erythropoietic-protoporphyria-initial-diagnosis-with-cholestatic-liver-disease
#16
Amy Coffey, Daniel H Leung, Norma M Quintanilla
The porphyrias are a group of rare metabolic disorders that result from defects in heme biosynthesis. Erythropoietic protoporphyria (EPP) is the most common inherited porphyria in children and is diagnosed in most individuals after the onset of cutaneous manifestations. Hepatobiliary disease affects the minority of individuals with EPP and usually manifests in patients with an established diagnosis of EPP. We report on a classic but rare case of EPP that masqueraded as cholestasis. An 8-year-old boy was referred to the Hepatology Clinic after an abrupt onset of jaundice with a longstanding history of dermatitis...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29604537/chronic-exposure-to-low-environmental-concentrations-and-legal-aquaculture-doses-of-antibiotics-cause-systemic-adverse-effects-in-nile-tilapia-and-provoke-differential-human-health-risk
#17
Samwel M Limbu, Li Zhou, Sheng-Xiang Sun, Mei-Ling Zhang, Zhen-Yu Du
BACKGROUND: Antibiotics used globally to treat human and animal diseases exist ubiquitously in the environment at low doses because of misuse, overdose and poor absorption after ingestion, coupled with their high-water solubility and degradation resistance. However, the systemic chronic effects of exposure to low environmental concentrations of antibiotics (LECAs) and legal aquaculture doses of antibiotics (LADAs) in fish and their human health risk are currently unknown. OBJECTIVE: To investigate the in vivo chronic effects of exposure to LECAs and LADAs using oxytetracycline (OTC) and sulfamethoxazole (SMZ) in Nile tilapia (Oreochromis niloticus) and their human health risk...
June 2018: Environment International
https://www.readbyqxmd.com/read/29601997/effectiveness-of-a-carbohydrate-restricted-diet-to-treat-non-alcoholic-fatty-liver-disease-in-adolescents-with-obesity-trial-design-and-methodology
#18
Shima Dowla, May Pendergrass, Mark Bolding, Barbara Gower, Kevin Fontaine, Ambika Ashraf, Taraneh Soleymani, Shannon Morrison, Amy Goss
BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is the most common liver disorder among children in the developed world and can progress to cirrhosis, hepatocellular carcinoma, and liver failure. No evidence-based dietary guidelines exist on the most effective diet prescription to treat NAFLD. OBJECTIVE: To compare the effect of a carbohydrate (CHO)-restricted diet vs fat-restricted diet, the current standard of care, on changes in hepatic fat infiltration, body composition, and metabolic health over an 8-week period among overweight and obese children diagnosed with NAFLD...
May 2018: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/29596181/chapter-6-0-history-of-paediatric-hepatology-in-espghan
#19
Martin Burdelski
Hepatology played an important role in ESPGAN from the beginning. In 1989, the Hepatology Committee was started. In the early 1990s H for hepatology was included in ESPGHAN. Hepatology Summer schools were held from 1995 and later monothematic conferences met the needs of many young ESPGHAN members. The role of ESPGHAN members in the progress of diagnostic and therapeutic measures in hepatitis B and C will be elucidated (Chapter 6.1) as well as the role of other ESPGHAN members in the understanding of immunological hepatic disorders of childhood (Chapter 6...
April 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29594475/individual-fatty-acids-in-erythrocyte-membranes-are-associated-with-several-features-of-the-metabolic-syndrome-in-obese-children
#20
Sara Bonafini, Angela Tagetti, Rossella Gaudino, Paolo Cavarzere, Martina Montagnana, Elisa Danese, Marco Benati, Diego Alberto Ramaroli, Sara Raimondi, Alice Giontella, Anna Mantovani, Angela Donato, Andrea Dalbeni, Pietro Minuz, Franco Antoniazzi, Claudio Maffeis, Cristiano Fava
PURPOSE: Obesity leads to the clustering of cardiovascular (CV) risk factors and the metabolic syndrome (MetS) also in children and is often accompanied by non-alcoholic fatty liver disease. Quality of dietary fat, beyond the quantity, can influence CV risk profile and, in particular, omega-3 fatty acids (FA) have been proposed as beneficial in this setting. The aim of the study was to evaluate the associations of individual CV risk factors, characterizing the MetS, with erythrocyte membrane FA, markers of average intake, in a group of 70 overweight/obese children...
March 28, 2018: European Journal of Nutrition
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