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Chronic liver disease children

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https://www.readbyqxmd.com/read/29035331/nutritional-needs-and-support-for-children-with-chronic-liver-disease
#1
REVIEW
Christine H Yang, Brandon J Perumpail, Eric R Yoo, Aijaz Ahmed, John A Kerner
Malnutrition has become a dangerously common problem in children with chronic liver disease, negatively impacting neurocognitive development and growth. Furthermore, many children with chronic liver disease will eventually require liver transplantation. Thus, this association between malnourishment and chronic liver disease in children becomes increasingly alarming as malnutrition is a predictor of poorer outcomes in liver transplantation and is often associated with increased morbidity and mortality. Malnutrition requires aggressive and appropriate management to correct nutritional deficiencies...
October 16, 2017: Nutrients
https://www.readbyqxmd.com/read/29031874/an-update-on-the-physiopathology-and-therapeutic-management-of-cholestatic-pruritus-in-children
#2
A Thébaut, D Debray, E Gonzales
Pruritus is a disabling symptom accompanying chronic cholestasis. In extreme cases, the refractory nature of pruritus can result in a need for invasive therapies including liver transplantation. The pathogenesis of pruritus in cholestatic disease is poorly understood. It may involve a specific neural pathway (similar to that associated with pain) regulated by several pruritogenic substances such as bile acids, opioids, serotonin, and the more recently identified lysophosphatidic acid. While the therapeutic management of cholestatic pruritus is well established in adults, there is no consensus in children, in light of the difficulty of conducting controlled clinical studies...
October 11, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29024698/pediatric-clif-sofa-score-is-the-best-predictor-of-28-day-mortality-in-children-with-decompensated-chronic-liver-disease
#3
Rishi Bolia, Anshu Srivastava, Surender Kumar Yachha, Ujjal Poddar
BACKGROUND AND AIMS: Early identification of children with decompensated chronic liver disease (DCLD) at risk of short-term mortality helps improve outcome. We evaluated the predictors of outcome and role of Child Pugh [CP], Pediatric End- stage Liver Disease [PELD] and pediatric chronic liver failure sequential organ failure assessment [pCLIF-SOFA] score in prognosticating 28 days mortality in DCLD children. METHODS: DCLD children were prospectively evaluated with a clinico-laboratory proforma and followed till 28 days to determine outcome...
October 9, 2017: Journal of Hepatology
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
#4
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
#5
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/28981167/serum-complement-factor-5a-levels-are-associated-with-non-alcoholic-fatty-liver-il-6-disease-in-obese-children
#6
Wei Hu, Min Wang, Chunyan Yin, Shuangshaung Li, Yuesheng Liu, Yanfeng Xiao
AIM: Nonalcoholic fatty liver disease (NAFLD) is a leading cause of progressive and chronic liver injury. Complement factor 5a (C5a) may be involved in many inflammation disorders. This study investigated levels of systemic C5a in patients with and without NAFLD and lean controls METHODS: A cross-sectional study was conducted from July 2012 to June 2013 among 96 Chinese children, aged 6-17 years, recruited from the Pediatric Department of the Second Affiliated Hospital of Xi'an Jiao Tong University: 40 obese children with NAFLD, 31 obese children without NAFLD and 25 lean controls...
October 5, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#7
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28970719/autoimmune-hepatitis-standard-treatment-and-systematic-review-of-alternative-treatments
#8
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Autoimmune hepatitis is a rare chronic inflammatory liver disease, affecting all ages, characterised by elevated transaminase and immunoglobulin G levels, positive autoantibodies, interface hepatitis at liver histology and good response to immunosuppressive treatment. If untreated, it has a poor prognosis. The aim of this review is to summarize the evidence for standard treatment and to provide a systematic review on alternative treatments for adults and children. Standard treatment is based on steroids and azathioprine, and leads to disease remission in 80%-90% of patients...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28948665/performance-of-fibrosis-prediction-scores-in-paediatric-non-alcoholic-fatty-liver-disease
#9
Jasmine A Jackson, Juna V Konomi, Michael V Mendoza, Alyssa Krasinskas, Ran Jin, Shelley Caltharp, Marialena Mouzaki, Miriam B Vos
AIM: Non-alcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. The phenotype of NAFLD varies widely, and non-invasive predictors of disease severity are scarce and are needed to tailor clinical management. METHODS: We compared liver fibrosis by histology with proposed non-invasive predictors of fibrosis, including alanine transaminase (ALT), aspartate transaminase (AST), AST/ALT ratio, AST to platelet ratio index, fibrosis-4, paediatric NAFLD fibrosis index and paediatric NAFLD fibrosis score...
September 25, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28892185/prevalence-comorbidities-and-mortality-of-toxic-shock-syndrome-in-united-states-children-and-adults
#10
Mark A Strom, Derek Y Hsu, Jonathan I Silverberg
Toxic Shock Syndrome (TSS) is a superantigen-mediated illness characterized by rash, hypotension and multi-organ dysfunction. The predictors of TSS and related morbidity and mortality are poorly defined. We analyzed data on 61,959,084 hospitalizations from the 2003-2012 Nationwide Inpatient Sample, a 20% stratified sample of US hospitalizations. ICD-9-CM coding was used to identify 4,491 hospitalizations with a diagnosis of TSS. Incidence, in-hospital mortality rate, comorbidities, length of stay and costs of care due to TSS were determined...
September 11, 2017: Microbiology and Immunology
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#11
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28882581/premature-ovarian-senescence-and-high-miscarriage-rate-impair-fertility-in-women-with-hepatitis-c-virus-infection
#12
Aimilia Karampatou, Xue Han, Loreta A Kondili, Gloria Taliani, Alessia Ciancio, Filomena Morisco, Rosina Maria Critelli, Enrica Baraldi, Veronica Bernabucci, Giulia Troshina, Maria Guarino, Simonetta Tagliavini, Federica D'Ambrosio, Laura Bristot, Laura Turco, Stefano Rosato, Stefano Vella, Tommaso Trenti, Isabella Neri, Antonio La Marca, Shivaji Manthena, Andrea S Goldstein, Savino Bruno, Yanjun Bao, Yuri Sanchez Gonzalez, Erica Villa
BACKGROUND AND AIMS: Premenopausal Hepatitis C Virus positive (HCV+) women have failing ovarian function.We thus investigated reproductive history,risk of infertility and pregnancy outcomes in HCV+ women of childbearing age. METHODS: Three different groups were studied:1) Clinical cohort:100 HCV+ women with chronic liver disease (CLD), age matched with 50 HBV+ women with CLD and with 100 healthy women consecutively observed in 3 Italian Gastroenterology Units;2) 1998 HCV+ women enrolled in the Italian Platform for the Study of Viral Hepatitis Therapies (PITER);3) 6...
September 4, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28837506/coagulopathy-in-children-with-liver-disease
#13
Patricia Sachi Kawada, Aisha Bruce, Patti Massicotte, Mary Bauman, Jason Yap
It was thought that a high International Normalized Ratio (INR) predicted bleeding in patients with chronic liver disease (CLD) and patients were 'autoanticoagulated'. Contrary to this belief, while patients with CLD experienced bleeding, they also developed thromboses. In the last decade, the prevailing literature challenged the idea that an elevated INR increased bleeding risk. The global assays of coagulation such as: thromboelastography (TEG)/rotational thromboelastometry (ROTEM) and thrombin generation assays (TGA) provide additional insight into coagulation processes...
August 23, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28814423/portal-hypertension-and-its-management-in-children
#14
REVIEW
Tassos Grammatikopoulos, Patrick James McKiernan, Anil Dhawan
Portal hypertension (PHT), defined as raised intravascular pressure in the portal system, is a complication of chronic liver disease or liver vascular occlusion. Advances in our ability to diagnose and monitor the condition but also predict the risk of gastrointestinal bleeding have enabled us to optimise the management of children with PHT either at a surveillance or at a postbleeding stage. A consensus among paediatric centres in the classification of varices can be beneficial in streamlining future paediatric studies...
August 16, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28776642/diagnosis-of-monogenic-liver-diseases-in-childhood-by-next-generation-sequencing
#15
Amelie Stalke, Britta Skawran, Bernd Auber, Thomas Illig, Brigitte Schlegelberger, Norman Junge, Imeke Goldschmidt, Christoph Leiskau, Nils von Neuhoff, Ulrich Baumann, Eva-Doreen Pfister
Next-Generation Sequencing (NGS) has opened up novel diagnostic opportunities for children with unidentified, but suspected inherited diseases. We describe our single-center experience with NGS diagnostics in standard clinical scenarios in pediatric hepatology. We investigated 135 children with suspected inherited hepatopathies, where initially no causative pathogenic variant had been identified, with an amplicon-based NGS panel of 21 genes associated with acute and chronic hepatopathies. In 23 of these patients we detected pathogenic or likely pathogenic variants in ten different genes...
August 4, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28770975/paracetamol-acetaminophen-for-chronic-non-cancer-pain-in-children-and-adolescents
#16
REVIEW
Tess E Cooper, Emma Fisher, Brian Anderson, Nick Mr Wilkinson, David G Williams, Christopher Eccleston
BACKGROUND: Pain is a common feature of childhood and adolescence around the world, and for many young people, that pain is chronic. The World Health Organization guidelines for pharmacological treatments for children's persisting pain acknowledge that pain in children is a major public health concern of high significance in most parts of the world. While in the past, pain was largely dismissed and was frequently left untreated, views on children's pain have changed over time, and relief of pain is now seen as important...
August 2, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28767077/gut-liver-axis-derangement-in-non-alcoholic-fatty-liver-disease
#17
REVIEW
Marco Poeta, Luca Pierri, Pietro Vajro
Non-alcoholic fatty liver disease (NAFLD) is the most frequent type of chronic liver disease in the pediatric age group, paralleling an obesity pandemic. A "multiple-hit" hypothesis has been invoked to explain its pathogenesis. The "first hit" is liver lipid accumulation in obese children with insulin resistance. In the absence of significant lifestyle modifications leading to weight loss and increased physical activity, other factors may act as "second hits" implicated in liver damage progression leading to more severe forms of inflammation and hepatic fibrosis...
August 2, 2017: Children
https://www.readbyqxmd.com/read/28759094/conversion-of-sugar-to-fat-is-hepatic-de-novo-lipogenesis-leading-to-metabolic-syndrome-and-associated-chronic-diseases
#18
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/28752463/semiquantitation-of-monomer-and-polymer-alpha-1-antitrypsin-by-centrifugal-separation-and-assay-by-western-blot-of-soluble-and-insoluble-components
#19
Keith S Blomenkamp, Jeffrey H Teckman
Alpha-1 antitrypsin (a1AT) deficiency, in its classical form, is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults. The vast majority of liver disease is associated with homozygosity for the Z mutant allele, also called PiZZ. This homozygous allele synthesizes large quantities of a1AT mutant Z protein in the liver, but the mutant protein also folds improperly during biogenesis. As a result, approximately 85% of the molecules are retained within the hepatocytes instead of being appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752441/pathophysiology-of-alpha-1-antitrypsin-deficiency-liver-disease
#20
Jeffrey H Teckman, Keith S Blomenkamp
Classical alpha-1 antitrypsin (a1AT) deficiency is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults (Teckman and Jain, Curr Gastroenterol Rep 16(1):367, 2014). The vast majority of the liver disease is associated with homozygosity for the Z mutant allele, the so-called PIZZ. These homozygous individuals synthesize large quantities of a1AT mutant Z protein in the liver, but the mutant protein folds improperly during biogenesis and approximately 85% of the molecules are retained within the hepatocytes rather than appropriately secreted...
2017: Methods in Molecular Biology
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