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Liver disease in children

Silvana Neves Ferraz de Assunção, Ney Christian Amaral Boa Sorte, Crésio De Aragão Dantas Alves, Patrícia S Almeida Mendes, Carlos Roberto Brites Alves, Luciana Rodrigues Silva
INTRODUCTION: Non-alcoholic fatty liver disease is characterized by the intrahepatic deposition of fat. It is the most prevalent liver disease in the world, affecting obese children and adolescents. Its pathophysiology is not fully understood, although it is often related to insulin resistance. This in turn would be due to an inflammatory condition common to obesity. Thus, the objective of this study was to describe the behavior of proinflammatory cytokines in obese children and adolescents, with and without non-alcoholic fatty liver disease...
January 16, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
Seyed Mohsen Dehghani, Hazhir Javaherizadeh, Masoomeh Heidary, Naser Honar, Maryam Ataollahi, Homa Ilkanipour, Hossein Moravej
INTRODUCTION AND AIM: The aim of this study was to evaluate nutritional status in children without prior hospital admission or evidence of chronic disease. SUBJECTS AND METHODS: The current study is a cross-sectional and observational study which was conducted for assessing the nutritional status of children. In this study, consecutive sampling was used, with a sample size about 400 children aged 6 months to 18 years at first hospital admission. All subjects were hospitalized consecutively in the Pediatric Emergency Department of the Nemazee Teaching Hospital of Shiraz University of Medical Sciences (Shiraz, Islamic Republic of Iran)...
January 10, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
Seyed Saeed Seyedian, Eskandar Hajiani, Seyed Jalal Hashemi, Abdolrahim Masjedizadeh, Ali Akbar Shayesteh, Pezhman Alavinejad, Ahmad Hormati, Ali Javaherforoushzadeh, Mehdi Khabazkhoob
Introduction: Nonalcoholic fatty liver disease (NAFLD) is raising prevalence among children, and adolescence population in developed and developing countries as a major public health concern. The present study aims to determine the relationship between serum ferritin level and transient elastography findings in patients suffering from NAFLD. Materials and Methods: The demographic and biochemical profile of included individuals such as body mass index, age, level of serum transaminases, fasting blood sugar, lipid profile, and serum ferritin level were determined and a transient elastography was performed for all of them...
October 2017: Journal of Family Medicine and Primary Care
Y J Xue, J Wu, Y X Zuo, Y P Jia, A D Lu, L P Zhang
Objective: To explore the clinical features and prognostic factors of Ph-positive and/or BCR-ABL positive acute lymphoblastic leukemia (Ph+ ALL) in children. Methods: The clinical data of 68 Ph+ ALL children who were treated at Peking University People's Hospital from December 2006 to December 2016 was retrospectively reviewed. Survival analysis were estimated by Kaplan-Meier method. Univariate analysis was estimated by Log-rank test and Chi-square, and multivariate analysis was estimated by Cox proportional hazards regression model...
March 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Nashwa M Samra, Shaimaa Emad El Abrak, Hanaa H El Dash, Mona El Said El Raziky, Manal A El Sheikh
BACKGROUND: Hepatic osteodystrophy caused by vitamin D and calcium malabsorption is thought to develop in children with cholestatic liver disease leading to secondary hyperparathyroidism and rickets or osteomalacia. The aim of this study was to evaluate the dental and bone mineral densities and the serum level of vitamin D in cholestatic infants and children and to correlate this process with clinical and laboratory parameters. METHODS: This is a cross-sectional study that include 50 patients presenting with cholestasis...
March 15, 2018: Clinics and Research in Hepatology and Gastroenterology
María José Garcés Hernández, Miguel Klünder, Nayely Garibay Nieto, Juan Carlos López Alvarenga, Jenny Vilchis Gil, Samuel Flores Huerta, Rosa Quispe Siccha, Joselin Hernandez
OBJECTIVE: Visceral adiposity index (VAI) is a mathematical model associated with cardiometabolic risk in adults, but studies on children failed to support this association. Our group has proposed a pediatric VAI model using pediatric ranges, but it has not yet been evaluated and needs further adjustments. The objective of this study was to further adjust the proposed pediatric VAI by age, creating a new pediatric metabolic index (PMI), and assess the correlation of the PMI with insulin resistance indexes and hepatic enzymes...
March 2018: Endocrine Practice
Eleonora Scorletti, Christopher D Byrne
For many years it has been known that high doses of long chain omega-3 fatty acids are beneficial in the treatment of hypertriglyceridaemia. Over the last three decades, there has also been a wealth of in vitro and in vivo data that has accumulated to suggest that long chain omega-3 fatty acid treatment might be beneficial to decrease liver triacylglycerol. Several biological mechanisms have been identified that support this hypothesis; notably, it has been shown that long chain omega-3 fatty acids have a beneficial effect: a) on bioactive metabolites involved in inflammatory pathways, and b) on alteration of nuclear transcription factor activities such as peroxisome proliferator-activated receptors (PPARs), sterol regulatory element-binding protein 1c (SREBP-1c) and carbohydrate-responsive element-binding protein (ChREBP), involved in inflammatory pathways and liver lipid metabolism...
March 12, 2018: Molecular Aspects of Medicine
B M Kamath, A Baker, R Houwen, L Todorova, N Kerkar
BACKGROUND: Alagille syndrome (ALGS) is an inherited multisystem disorder typically manifesting as cholestasis, and potentially leading to end-stage liver disease and death. AIM: To perform the first systematic review of the epidemiology, natural history and burden of ALGS with a focus on the liver component. METHODS: Electronic databases and proceedings from key congresses were searched in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2009 guidelines...
March 14, 2018: Journal of Pediatric Gastroenterology and Nutrition
Benjamin Saul Raywood Allin, Nigel J Hall, Andrew R Ross, Sean S Marven, Jennifer J Kurinczuk, Marian Knight
OBJECTIVE: Outcome reporting heterogeneity impedes identification of gold standard treatments for children born with gastroschisis. Use of core outcome sets (COSs) in research reduces outcome reporting heterogeneity and ensures that studies are relevant to patients. The aim of this study was to develop a gastroschisis COS. DESIGN AND SETTING: Systematic reviews and stakeholder nomination were used to identify candidate outcomes that were subsequently prioritised by key stakeholders in a three-phase online Delphi process and face-to-face consensus meeting using a 9-point Likert scale...
March 14, 2018: Archives of Disease in Childhood. Fetal and Neonatal Edition
Toshihiro Yasui, Tatsuya Suzuki, Fujio Hara, Shunsuke Watanabe, Naoko Uga, Atsuki Naoe, Yasuhiro Kondo
OBJECTIVES: In pediatric patients, renal dysfunction after living-donor liver transplant is a major issue that is difficult to evaluate. Recently, predictive equations for Japanese children have been introduced. MATERIALS AND METHODS: We conducted a retrospective study by prospectively collecting data on 26 patients under 16 years old who underwent living-donor liver transplant between June 2004 and March 2015. Serum creatinine and cystatin C levels were measured...
March 9, 2018: Experimental and Clinical Transplantation
C Ricardo Ramirez, M L Álvarez Gómez, C A Agudelo Vélez, S Zuluaga Penagos, R A Consuegra Peña, K Uribe Hernández, I C Mejía Gil, E M Cano Londoño, M Elorza Parra, J G Franco Vásquez
OBJECTIVE: To evaluate the clinical characteristics, prevalence and factors associated with delirium in critical patients from 5 to 14 years of age. DESIGN: An analytical, cross-sectional observational study was made. Delirium was assessed with the Pediatric-Confusion Assessment Method for the Intensive Care Unit (pCAM-ICU) and motor classification was established with the Delirium Rating Scale Revised-98. SETTING: A pediatric Intensive Care Unit...
March 9, 2018: Medicina Intensiva
Salvatore Giordano, Maria Di Gangi, Maria Concetta Failla, Lucrezia Bruno, Veronica Falcone, Piera Dones
Bronchiolitis is the most common disease of the lower respiratory tract occurring in children during their first year of life, becoming the most frequent cause of hospitalization. Although the disease can also be caused by other viruses, more than 70% of bronchiolitis cases are caused by respiratory syncytial virus (RSV). RSV bronchiolitis clinically presents rhinitis, coughing, increased breathing and eating difficulties; the symptoms are usually mild, but in some cases may be so severe as to require hospitalization...
March 1, 2018: Le Infezioni in Medicina
Annu Aggarwal, Mohit Bhatt
Background: Wilson disease (WD) is an inherited neurometabolic disorder that results in excessive copper deposition in the liver and the brain, affecting children and young adults. Without treatment the disease is invariably fatal. Though treatments for WD have been available since the 1950s, the disease continues to be associated with considerable morbidity and mortality because of missed diagnosis, and delayed or inadequate treatment. In this paper we survey WD-related literature in order to review recent advances in WD treatment...
2018: Tremor and Other Hyperkinetic Movements
Vicky L Ng, Lisa G Sorensen, Estella M Alonso, Emily M Fredericks, Wen Ye, Jeff Moore, Saul J Karpen, Benjamin L Shneider, Jean P Molleston, Jorge A Bezerra, Karen F Murray, Kathleen M Loomes, Philip Rosenthal, Robert H Squires, Kasper Wang, Ronen Arnon, Kathleen B Schwarz, Yumirle P Turmelle, Barbara H Haber, Averell H Sherker, John C Magee, Ronald J Sokol
OBJECTIVES: To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment. STUDY DESIGN: Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition...
March 5, 2018: Journal of Pediatrics
Chetan Mandelia, Mohammad Nasser Kabbany, Praveen Kumar Conjeevaram Selvakumar, Naim Alkhouri
Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the obesity epidemic. Recent studies have clearly shown that the stage of fibrosis in adults with NAFLD is the most important histological feature in long-term outcomes and the development of liver-related complications. Despite the paucity of data regarding the natural history of pediatric NAFLD, its progression to cirrhosis and end-stage liver disease requiring liver transplantation is well documented. Given the high prevalence of NAFLD in children and adults, there is an urgent need to find safe and cost-effective alternatives to biopsy to determine the stage of liver fibrosis...
March 8, 2018: Biomarkers in Medicine
Wail A Hayajneh, Vincent J Daniels, Cerise K James, Muhammet Nabi Kanıbir, Matthew Pilsbury, Morgan Marks, Michelle G Goveia, Elamin H Elbasha, Erik Dasbach, Camilo J Acosta
BACKGROUND: As the socioeconomic conditions in Jordan have improved over recent decades the disease and economic burden of Hepatitis A has increased. The purpose of this study is to assess the potential health and economic impact of a two-dose hepatitis A vaccine program covering one-year old children in Jordan. METHODS: We adapted an age-structured population model of hepatitis A transmission dynamics to project the epidemiologic and economic impact of vaccinating one-year old children for 50 years in Jordan...
March 7, 2018: BMC Infectious Diseases
Atakan Comba, Fatma Demirbaş, Gönül Çaltepe, Esra Eren, Ayhan G Kalayci
BACKGROUND: α-1 Antitrypsin (AAT) deficiency is the most frequently occurring genetic liver disorder. The association among classical α-1 antitrypsin deficiency (AATD), chronic liver disease, and cirrhosis is common in adult patients but rare in children. AIM: To assess the clinical characteristics of children with AATD and to compare symptoms between homozygous and heterozygous children. MATERIALS AND METHODS: The study included 20 children who were found to have mutant Pi alleles...
March 2, 2018: European Journal of Gastroenterology & Hepatology
Syeda Sara Batool Hamdani, Huma Arshad Cheema, Anjum Saeed, Hassan Suleman Malik, Tayaba Sehar
BACKGROUND: Wilson disease (WD) is one of the most common metabolic liver diseases in older children. It has a strong genetic background with autosomal recessive inheritance. WD is a multisystem disorder with predominant hepatic and neurological manifestations and variable age of presentation. The data on cardiac manifestations in children is very limited and only few adult studies are available in the literature. This study was planned to determine the frequency and spectrum of Electrocardiographic (ECG) changes in pediatric WD...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
Bikrant Bihari Lal, Seema Alam, Rajeev Khanna, Dinesh Rawat
There are no evidence-based recommendations on the ideal dose and regimen for supplementation of vitamin D in children with chronic liver disease (CLD). This study aimed to compare the safety and efficacy of weekly and stoss regimens for treatment of vitamin D deficiency in these children. Children between the ages of 1 to 18 years with CLD and hypovitaminosis D defined by 25-OH vitamin D (25(OH)D) < 30µg/l were included. They were randomized to receive either stoss regimen (600,000 IU on day 1) or weekly (60,000 IU weekly) regimen of vitamin D...
March 4, 2018: European Journal of Pediatrics
Akash Deep, Romit Saxena, Bipin Jose
Acute kidney injury (AKI) is a common accompaniment in patients with liver disease. The causes, risk factors, manifestations and management of AKI in these patients vary according to the liver disease in question (acute liver failure, acute-on-chronic liver failure, post-liver transplantation or metabolic liver disease). There are multiple causes of AKI in patients with liver disease-pre-renal, acute tubular necrosis, post-renal, drug-induced renal failure and hepatorenal syndrome (HRS). Definitions of AKI in liver failure are periodically revised and updated, but pediatric definitions have still to see the light of the day...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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