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Metabolic liver disease children

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https://www.readbyqxmd.com/read/29471012/pediatric-fatty-liver-disease-pefld-all-is-not-nafld-pathophysiological-insights-and-approach-to-management
#1
REVIEW
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
https://www.readbyqxmd.com/read/29470286/genetic-variants-associated-with-obesity-and-insulin-resistance-in-hispanic-boys-with-nonalcoholic-fatty-liver-disease
#2
John C Rausch, Joel E Lavine, Naga Chalasani, Xiuqing Guo, Soonil Kwon, Jeffrey B Schwimmer, Jean P Molleston, Rohit Loomba, Elizabeth M Brunt, Yii-Der Ida Chen, Mark O Goodarzi, Kent D Taylor, Katherine P Yates, Jerome I Rotter
BACKGROUND AND OBJECTIVES: Nonalcoholic fatty liver disease (NAFLD) disproportionately affects Hispanic boys. Further, obesity and insulin resistance are major risk factors for NAFLD. No gene localization studies had been performed on children with biopsy-proven NAFLD. This study aims to identify genomic variants associated with increased adiposity and insulin resistance in a population of children with varying histologic severity of NAFLD. METHODS: We conducted a genome-wide association scan (GWAS) including 624,297 single-nucleotide polymorphisms (SNPs) distributed among all 22 autosomal chromosomes in 234 Hispanic boys (up to 18 years of age) who were consecutively recruited in a prospective cohort study in the Nonalcoholic Steatohepatitis Clinical Research Network Studies...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29438686/evaluation-of-nonalcoholic-fatty-liver-disease-using-magnetic-resonance-in-obese-children-and-adolescents
#3
Patrícia O Benetolo, Maria I M Fernandes, Ieda R L Del Ciampo, Jorge Elias-Junior, Regina Sawamura
OBJECTIVE: To determine the frequency of nonalcoholic fatty liver disease using nuclear magnetic resonance as a noninvasive method. METHODOLOGY: This was a cross-sectional study conducted on 50 children and adolescents followed up at an outpatient obesity clinic. The subjects were submitted to physical examination, laboratory tests (transaminases, liver function tests, lipid profile, glycemia, and basal insulin) and abdominal nuclear magnetic resonance (calculation of hepatic, visceral, and subcutaneous fat)...
February 10, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29379536/bifidobacteria-and-lactobacilli-in-the-gut-microbiome-of-children-with-non-alcoholic-fatty-liver-disease-which-strains-act-as-health-players
#4
Valerio Nobili, Lorenza Putignani, Antonella Mosca, Federica Del Chierico, Pamela Vernocchi, Anna Alisi, Laura Stronati, Salvatore Cucchiara, Marco Toscano, Lorenzo Drago
Introduction: Non-alcoholic fatty liver disease (NAFLD), considered the leading cause of chronic liver disease in children, can often progress from non-alcoholic fatty liver (NAFL) to non-alcoholic steatohepatitis (NASH). It is clear that obesity is one of the main risk factors involved in NAFLD pathogenesis, even if specific mechanisms have yet to be elucidated. We investigated the distribution of intestinal bifidobacteria and lactobacilli in the stools of four groups of children: obese, obese with NAFL, obese with NASH, and healthy, age-matched controls (CTRLs)...
January 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29360619/hypophosphatasia-biochemical-hallmarks-validate-the-expanded-pediatric-clinical-nosology
#5
Michael P Whyte, Stephen P Coburn, Lawrence M Ryan, Karen L Ericson, Fan Zhang
Hypophosphatasia (HPP) is the inborn-error-of-metabolism due to loss-of-function mutation(s) of the ALPL (TNSALP) gene that encodes the tissue non-specific isoenzyme of alkaline phosphatase (TNSALP). TNSALP represents a family of cell-surface phosphohydrolases differing by post-translational modification that is expressed especially in the skeleton, liver, kidney, and developing teeth. Thus, the natural substrates of TNSALP accumulate extracellularly in HPP including inorganic pyrophosphate (PPi), a potent inhibitor of mineralization, and pyridoxal 5'-phosphate (PLP), the principal circulating form of vitamin B6...
January 19, 2018: Bone
https://www.readbyqxmd.com/read/29341979/wilson-s-disease-in-children-a-position-paper-by-the-hepatology-committee-of-the-european-society-for-paediatric-gastroenterology-hepatology-and-nutrition
#6
Piotr Socha, Wojciech Janczyk, Anil Dhawan, Ulrich Baumann, Lorenzo D'Antiga, Stuart Tanner, Raffaele Iorio, Pietro Vajro, Roderick Houwen, Björn Fischler, Antal Dezsofi, Nedim Hadzic, Loreto Hierro, Jörg Jahnel, Valérie McLin, Valerio Nobili, Francoise Smets, Henkjan J Verkade, Dominique Debray
BACKGROUND: Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, whereas neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24-hour urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism, and molecular analysis of mutations in the ATP7B gene...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29322175/association-of-psoriasis-with-comorbidity-development-in-children-with-psoriasis
#7
Megha M Tollefson, Holly K Van Houten, Dennis Asante, Xiaoxi Yao, Hilal Maradit Kremers
Importance: Children with psoriasis are at increased risk for comorbidities. Many children with psoriasis are also overweight or obese; it is unknown whether the increased risk of comorbidities in these children is independent of obesity. Objective: To determine the risk of elevated lipid levels (hyperlipidemia/hypertriglyceridemia), hypertension, metabolic syndrome, polycystic ovarian syndrome, diabetes, nonalcoholic liver disease, and elevated liver enzyme levels in children with and without psoriasis, after accounting for obesity...
January 10, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29317557/prevalence-of-nonalcoholic-fatty-liver-disease-in-normal-weight-and-overweight-preadolescent-children-in-haryana-india
#8
Manoja Kumar Das, Vidyut Bhatia, Anupam Sibal, Abha Gupta, Sarath Gopalan, Raman Sardana, Reeti Sahni, Ankur Roy, Narendra K Arora
OBJECTIVE: To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normal-weight and overweight schoolchildren. STUDY DESIGN: Cross-sectional study. SETTING: Thirteen private schools in urban Faridabad, Haryana. PARTICIPANTS: 961 school children aged 5-10 years. METHODS: Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing...
December 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29305416/involvement-of-hepcidin-in-iron-metabolism-dysregulation-in-gaucher-disease
#9
Thibaud Lefebvre, Niloofar Reihani, Raed Daher, Thierry Billette de Villemeur, Nadia Belmatoug, Christian Rose, Yves Colin-Aronovicz, Hervé Puy, Caroline Le Van Kim, Mélanie Franco, Zoubida Karim
Gaucher disease is an inherited deficiency of glucocerebrosidase leading to accumulation of glucosylceramide in tissues such as the spleen, liver, and bone marrow. The resulting lipid-laden macrophages lead to the appearance of Gaucher cells. Anemia associated with an unexplained hyperferritinemia is a frequent finding in Gaucher disease, but whether this pathogenesis is related to an iron metabolism disorder has remained unclear. To investigate this issue, we explored the iron status of a large cohort of 90 type I Gaucher disease patients, including 66 patients treated with enzyme replacement therapy...
January 5, 2018: Haematologica
https://www.readbyqxmd.com/read/29295475/nutrition-and-liver-disease
#10
EDITORIAL
Claudia Mandato, Antonella Di Nuzzi, Pietro Vajro
Malnutrition in children and adults with advanced liver disease represents a tremendous challenge as the nutritional problems are multifactorial. This Editorial comments the articles appearing in this special issue of Nutrients, "Nutrition and Liver disease" dealing with multiple diagnostic and therapeutic features that relate the outcomes of liver disease to nutrition. To improve quality of life and prevent nutrition-related medical complications, patients diagnosed with advanced liver disease should have their nutritional status promptly assessed and be supported by appropriate dietary interventions...
December 23, 2017: Nutrients
https://www.readbyqxmd.com/read/29243158/bilateral-native-nephrectomy-to-reduce-oxalate-stores-in-children-at-the-time-of-combined-liver-kidney-transplantation-for-primary-hyperoxaluria-type-1
#11
Eliza Lee, Gabriel Ramos-Gonzalez, Nancy Rodig, Scott Elisofon, Khashayar Vakili, Heung Bae Kim
OBJECTIVE: Primary hyperoxaluria type-1 (PH-1) is a rare genetic disorder in which normal hepatic metabolism of glyoxylate is disrupted resulting in diffuse oxalate deposition and end-stage renal disease (ESRD). While most centers agree that combined liver-kidney transplant (CLKT) is the appropriate treatment for PH-1, perioperative strategies for minimizing recurrent oxalate-related injury to the transplanted kidney remain unclear. We present our management of children with PH-1 and ESRD on hemodialysis (HD) who underwent CLKT at our institution from 2005 to 2015...
December 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29240010/well-preserved-renal-function-in-children-with-untreated-chronic-liver-disease
#12
Ulla B Berg, Antal Németh
OBJECTIVES: Based on studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease. Therefore we investigated renal function in our children with chronic liver disease. METHODS: In a retrospective study of 277 children with chronic liver disease, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol...
December 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29215054/in-vivo-redox-metabolic-imaging-of-mitochondria-assesses-disease-progression-in-non-alcoholic-steatohepatitis
#13
Ryosuke Nakata, Fuminori Hyodo, Masaharu Murata, Hinako Eto, Tomoko Nakaji, Takahito Kawano, Sayoko Narahara, Keiji Yasukawa, Tomohiko Akahoshi, Morimasa Tomikawa, Makoto Hashizume
Given the rising incidence of non-alcoholic fatty liver disease (NAFLD) in both adults and children, the development of a non-invasive diagnostic method for assessing disease progression to non-alcoholic steatohepatitis (NASH) has become an important research goal. Currently available non-invasive imaging technologies are only able to assess fat accumulation in the liver. Therefore, these methods are not suitable for a precise diagnosis of NASH. The standard diagnostic technique for NASH, liver biopsy, has several drawbacks, including the higher risk of complications that accompanies invasive procedures...
December 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29209135/first-case-of-cross-auxiliary-double-domino-donor-liver-transplantation
#14
Zhi-Jun Zhu, Lin Wei, Wei Qu, Li-Ying Sun, Ying Liu, Zhi-Gui Zeng, Liang Zhang, En-Hui He, Hai-Ming Zhang, Ji-Dong Jia, Zhong-Tao Zhang
We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy (FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the first stage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava (IVC)...
November 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29187144/non-alcoholic-fatty-liver-disease-and-its-associations-among-adolescents-in-an-urban-sri-lankan-community
#15
Shaman Rajindrajith, Arunasalam Pathmeswaran, Chamilka Jayasinghe, Dulani Kottahachchi, Anuradhani Kasturiratne, Shamila T de Silva, Madunil A Niriella, Anuradha S Dassanayake, Arjuna P de Silva, H Janaka de Silva
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is a common problem across the world. We aimed to determine the prevalence of NAFLD and its associations in Sri Lankan adolescents living in an urban Sri Lankan community. METHOD: The study population consisted of the birth cohort of the year 2000, residing in the Ragama Medical Officer of Health area. Socio-demographic and anthropometric data [anthropometric measurements, blood pressure and total body fat distribution] of these adolescents were collected by trained data collectors...
November 29, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/29147139/insights-into-the-management-of-wilson-s-disease
#16
REVIEW
Mohmadshakil Kathawala, Gideon M Hirschfield
Wilson's disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations...
November 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/29134448/hyperammonemia-associated-with-distal-renal-tubular-acidosis-or-urinary-tract-infection-a-systematic-review
#17
Caterina M Clericetti, Gregorio P Milani, Sebastiano A G Lava, Mario G Bianchetti, Giacomo D Simonetti, Olivier Giannini
BACKGROUND: Hyperammonemia usually results from an inborn error of metabolism or from an advanced liver disease. Individual case reports suggest that both distal renal tubular acidosis and urinary tract infection may also result in hyperammonemia. METHODS: A systematic review of the literature on hyperammonemia secondary to distal renal tubular acidosis and urinary tract infection was conducted. RESULTS: We identified 39 reports on distal renal tubular acidosis or urinary tract infections in association with hyperammonemia published between 1980 and 2017...
November 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29132978/intensive-care-in-severe-malaria-report-from-the-task-force-on-tropical-diseases-by-the-world-federation-of-societies-of-intensive-and-critical-care-medicine
#18
REVIEW
Dilip R Karnad, Mohd Basri Mat Nor, Guy A Richards, Tim Baker, Pravin Amin
Severe malaria is common in tropical countries in Africa, Asia, Oceania and South and Central America. It may also occur in travelers returning from endemic areas. Plasmodium falciparum accounts for most cases, although P vivax is increasingly found to cause severe malaria in Asia. Cerebral malaria is common in children in Africa, manifests as coma and seizures, and has a high morbidity and mortality. In other regions, adults may also develop cerebral malaria but neurological sequelae in survivors are rare...
November 8, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/29122442/serum-fetuin-a-levels-in-obese-children-with-biopsy-proven-nonalcoholic-fatty-liver-disease
#19
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
https://www.readbyqxmd.com/read/29118811/obese-children-with-metabolic-syndrome-have-3-times-higher-risk-to-have-nonalcoholic-fatty-liver-disease-compared-with-those-without-metabolic-syndrome
#20
Dimitrios Papandreou, Mirey Karavetian, Zacharoula Karabouta, Eleni Andreou
Background: The aim of this study was to investigate the relationship between metabolic syndrome (MS) and nonalcoholic fatty liver disease (NAFLD) in obese children. One hundred and twenty-five subjects aged 11-12 years old participated in the study. Methods: Anthropometric and biochemical indices were measured, including lipid and liver profile, blood glucose, serum insulin, and liver ultrasound. Results: Forty-four children (58.6%) were found to have MS...
2017: International Journal of Endocrinology
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