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https://www.readbyqxmd.com/read/28436333/epidemiology-of-hepatitis-c-infection-in-pakistan-current-estimate-and-major-risk-factors
#1
Aiman Arshad, Usman Ali Ashfaq
In Pakistan, hepatitis C virus (HCV) is a major healthcare problem, with acute and chronic infections responsible for liver damage, cirrhosis, and hepatocellular carcinoma. Under the Human Development Index of the United Nations, Pakistan is ranked 134th of 174 countries due to its poor educational and health standards. This study was designed to study HCV and its genotype prevalence in different cities and provinces of Pakistan and describe the major routes of HCV transmission. Literature searches were performed in PubMed, Mendeley, and Google Scholar...
2017: Critical Reviews in Eukaryotic Gene Expression
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#2
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
RATIONALE: Hepatic steatosis develops after liver transplant in 30% of adults, and non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in non-transplanted children. However, post-transplant steatosis has been minimally studied in pediatric liver transplant recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. RESULTS: In this single-center study of pediatric patients transplanted 1988-2015 (n=318), 31% of those with any post-transplant biopsy (n=271) had ≥1 biopsy with steatosis...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28426447/measles-cases-in-children-requiring-hospital-access-in-an-academic-pediatric-hospital-in-italy-2008-2013
#3
Marta Ciofi Degli Atti, Antonietta Filia, Antonino Bella, Annamaria Sisto, Maria Antonietta Barbieri, Antonino Reale, Massimiliano Raponi
BACKGROUND: The Lazio Region is one of the Italian regions where sustained measles transmission continues to occur. We investigated measles cases reported by the Emergency Department (ED) of the largest pediatric hospital in Italy, located in Lazio. METHODS: We reviewed clinical records of all measles cases from 0 to 18 years of age evaluated in the ED in 2008-2013. We compared demographic and clinical characteristics of patients admitted to the inpatient setting with those of patients discharged home, to assess possible determinants of hospital admission...
April 18, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28396861/non-invasive-investigations-for-the-diagnosis-of-fontan-associated-liver-disease-in-pediatric-and-adult-fontan-patients
#4
Amyna Fidai, Frederic Dallaire, Nanette Alvarez, Yvonne Balon, Robin Clegg, Michael Connelly, Frank Dicke, Deborah Fruitman, Joyce Harder, Kimberley Myers, David J Patton, Tim Prieur, Erika Vorhies, Robert P Myers, Steven R Martin, Steven C Greenway
Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28395968/-aetiology-outcomes-and-prognostic-indicators-of-paediatric-acute-liver-failure
#5
Juan José Gilbert Pérez, Belén Jordano Moreno, Mónica Rodríguez Salas
INTRODUCTION: Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF. OBJECTIVES: To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator. PATIENTS AND METHODS: A retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group...
April 7, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28391883/childhood-adult-onset-lysosomal-acid-lipase-deficiency-a-serious-metabolic-and-vascular-phenotype-beyond-liver-disease-four-new-pediatric-cases
#6
Pierre Poinsot, Sophie Collardeau Frachon, Lioara Restier, André Sérusclat, Mathilde Di Filippo, Sybil Charrière, Philippe Moulin, Alain Lachaux, Noel Peretti
BACKGROUND: The childhood/adult-onset lysosomal acid lipase deficiency (LALD; late-onset LALD) is a rare genetic disease. Children present severe fatty liver disease with early cirrhosis. Before enzyme replacement therapy, statins were the standard treatment to improve the severe dyslipidemia. However, late-onset LALD should be considered as a systemic metabolic disease: chronic hyper-low-density lipoprotein and hypo-high-density lipoprotein cholesterolemia induces early atherosclerosis in addition to the liver morbidity...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#7
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28386718/globus-pallidus-mr-signal-abnormalities-in-children-with-chronic-liver-disease-and-or-porto-systemic-shunting
#8
Sylviane Hanquinet, Claire Morice, Delphine S Courvoisier, Vladimir Cousin, Mehrak Anooshiravani, Laura Merlini, Valérie A McLin
OBJECTIVES: Detection of subclinical hepatic encephalopathy in children is difficult. We aimed to assess the changes in imaging of the central nervous system in children with chronic liver disease using MR imaging, diffusion, and (1)H -spectroscopy. METHODS: Forty three children with chronic liver disease and/or porto-systemic shunting (111.4±56.9 months) and 24 controls (72.0±51.8 months) underwent brain MRI/spectroscopy on a 1.5T to examine T1, T2, ADC, Cho/Cr, ml/Cr, Glx/Cr ratio spectroscopy in the globus pallidus...
April 6, 2017: European Radiology
https://www.readbyqxmd.com/read/28364818/liver-transplantation-in-alpha-1-antitrypsin-deficiency
#9
REVIEW
Virginia C Clark
Alpha-1 antitrypsin (AAT) deficiency is a common inherited metabolic disorder caused by a point mutation in the SERPIN1A gene. A small portion of homozygous PI*ZZ individuals develop severe liver disease that requires liver transplantation. Posttransplant survival is excellent. The largest burden of advanced liver disease lies within the adult population rather than children. Evaluation of lung function in adults before transplant is essential because of the underlying risk for chronic obstructive pulmonary disease...
May 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28359257/plasma-urotensin-ii-levels-in-children-and-adolescents-with-chronic-kidney-disease-a-single-centre-study
#10
Anastasia Garoufi, Styliani Drapanioti, Antonios Marmarinos, Varvara Askiti, Andromachi J Mitsioni, Maria Mila, Georgia Grigoriadou, Dimitrios Georgakopoulos, Constantinos J Stefanidis, Dimitrios Gourgiotis
BACKGROUND: Increased plasma Urotensin II (UII) levels have been found in adults with renal diseases. Studies in children are scarce. The objective of the study is to estimate plasma UII levels in subjects with chronic kidney disease (CKD) stages 3 to 5 and renal transplant recipients (RTR). In addition, the correlation of UII with anthropometric features and biochemical parameters was assessed. METHODS: Fifty-four subjects, aged 3 to 20 years old, 23 with CKD, 13 with end-stage kidney disease (ESKD) undergoing hemodialysis (HD) and 18 RTR were enrolled...
March 31, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28357028/limitations-and-opportunities-of-non-invasive-liver-stiffness-measurement-in-children
#11
REVIEW
Guido Engelmann, Jasmin Quader, Ulrike Teufel, Jens Peter Schenk
Changes in liver structure are an important issue in chronic hepatopathies. Until the end of the 20(th) century, these changes could only be determined by histological analyses of a liver specimen obtained via biopsy. The well-known limitations of this technique (i.e., pain, bleeding and the need for sedation) have precluded its routine use in follow-up of patients with liver diseases. However, the introduction of non-invasive technologies, such as ultrasound and magnetic resonance imaging, for measurement of liver stiffness as an indirect marker of fibroses has changed this situation...
March 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28353468/primary-budd-chiari-syndrome-in-children-king-s-college-hospital-experience
#12
Susana Nobre, Rajeev Khanna, Natalie Bab, Eirini Kyrana, Sue Height, John Karani, Pauline Kane, Nigel Heaton, Anil Dhawan
Primary Budd-Chiari syndrome (BCS) is a rare cause of liver disease in children in the western world. Here we present a retrospective review of children with Primary BCS presenting from Jan 2001 to Nov 2015 to our hospital. Seven children were identified. Their presentation was mostly chronic. All had predisposing factors for thrombosis and were started on anticoagulation. Radiological interventions (2 transjugular intrahepatic portosystemic shunts (TIPSS) and 1 hepatic vein stenting), liver transplant and mesocaval shunt were done in 3, 2 and 1 patients, respectively; one child underwent bone-marrow transplantation following TIPSS, and one child was managed only medically...
March 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28348471/role-of-allopurinol-in-optimizing-thiopurine-therapy-in-patients-with-autoimmune-hepatitis-a-review
#13
REVIEW
Shivani Deswal, Anshu Srivastava
Autoimmune hepatitis (AIH) is a chronic immune mediated liver disease characterized by elevated transaminases, hyper gammaglobulinemia, presence of autoantibodies and interface hepatitis in the absence of a known etiology of liver disease. Thiopurines (azathioprine [AZA]/6-mercaptopurine [6MP]) and steroids remain the first line of treatment of AIH in both children and adults. However, a small proportion of AIH patients are either non-responders or develop side effects with AZA. The metabolism of AZA is complex and mediated by multiple enzymes...
March 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28332779/intrafamilial-phenotypic-variability-in-a-polish-family-with-sensenbrenner-syndrome-and-biallelic-wdr35-mutations
#14
Joanna Walczak-Sztulpa, Anna Wawrocka, Agata Sobierajewicz, Lukasz Kuszel, Jan Zawadzki, Ryszard Grenda, Anna Swiader-Lesniak, Beata Kocyla-Karczmarewicz, Anna Wnuk, Anna Latos-Bielenska, Krystyna H Chrzanowska
Sensenbrenner syndrome (cranioectodermal dysplasia, CED) is a very rare autosomal recessive ciliopathy. Cranioectodermal dysplasia is characterized by craniofacial, skeletal, and ectodermal abnormalities. About 50 patients have been described to date. Sensenbrenner syndrome belongs to a group of ciliary chondrodysplasias and is a genetically heterogeneous disorder. Mutations in five genes: IFT122, WDR35, IFT43, WDR19, and IFT52 have been associated with CED. All known genes encode proteins that are part of the intraflagellar transport complex, which plays an important role in the assembly and maintenance of cilia...
March 23, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28332028/infection-profile-in-chronic-granulomatous-disease-a-23-year-experience-from-a-tertiary-care-center-in-north-india
#15
Amit Rawat, Pandiarajan Vignesh, Avinash Sharma, Jitendra K Shandilya, Madhubala Sharma, Deepti Suri, Anju Gupta, Vikas Gautam, Pallab Ray, Shivaprakash M Rudramurthy, Arunaloke Chakrabarti, Kohsuke Imai, Shigeaki Nonoyama, Osamu Ohara, Yu L Lau, Surjit Singh
PURPOSE: Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed...
March 22, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28321162/mortality-associated-with-gastrointestinal-bleeding-in-children-a-retrospective-cohort-study
#16
Thomas M Attard, Mikaela Miller, Chaitanya Pant, Ashwath Kumar, Mike Thomson
AIM: To determine the clinical characteristics of children with gastrointestinal bleeding (GIB) who died during the course of their admission. METHODS: We interrogated the Pediatric Hospital Information System database, including International Classification of Diseases, Current Procedural Terminology and Clinical Transaction Classification coding from 47 pediatric tertiary centers extracting the population of patients (1-21 years of age) admitted (inpatient or observation) with acute, upper or indeterminate GIB (1/2007-9/2015)...
March 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28306597/diagnosis-of-viral-hepatitis
#17
Philippa J Easterbrook, Teri Roberts, Anita Sands, Rosanna Peeling
PURPOSE OF REVIEW: Chronic hepatitis B virus (HBV) and hepatitis C virus (HCV) infections and HIV-HBV and HCV coinfection are major causes of chronic liver disease worldwide. Testing and diagnosis is the gateway for access to both treatment and prevention services, but there remains a large burden of undiagnosed infection globally. We review the global epidemiology, key challenges in the current hepatitis testing response, new tools to support the hepatitis global response (2016-2020 Global Hepatitis Health Sector strategy, and 2017 WHO guidelines on hepatitis testing) and future directions and innovations in hepatitis diagnostics...
May 2017: Current Opinion in HIV and AIDS
https://www.readbyqxmd.com/read/28281846/autoimmune-liver-diseases-and-inflammatory-bowel-diseases-in-children-current-issues-and-future-perspectives
#18
Sabrina Cardile, Tommaso Alterio, Manila Candusso, Andrea Pietrobattista, Daniela Liccardo, Maria Sole Basso, Bronislava Papadatou, Fiammetta Bracci, Daniela Knafelz, Giuliano Torre
Inflammatory bowel diseases (IBDs) represent a group of intestinal disorders with a chronic and relapsing inflammation of the gut, and with a potential risk of systemic involvement of other organs and systems. Over the pediatric age, an incidence higher than 20% of developing extraintestinal manifestation during follow-up has been reported. The liver and the biliary system are frequently involved, and primary sclerosing cholangitis (PSC) is the most predominant entity with an incidence rate of 6.4-7.8% in children...
June 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28276299/immunohistochemical-analysis-of-the-stem-cell-marker-lgr5-in-pediatric-liver-disease
#19
Zahida Khan, Anne Orr, George K Michalopoulos, Sarangarajan Ranganathan
Aims In regenerating liver, hepatic progenitor cells (HPCs) are recruited in response to injury; however, few highly specific human HPC markers exist for the hepatocyte lineage. Leucine-rich repeat-containing G-protein coupled receptor 5 (LGR5), a Wnt-associated stem cell marker, has been extensively studied in intestinal stem cells, but little is known about its expression in human liver. We hypothesized that LGR5+ HPCs are induced in the regenerative response to pediatric liver injury. Methods and results Immunohistochemistry was used to characterize LGR5 expression in pediatric liver explants (n = 36)...
January 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28251116/pediatric-hepatitis-b-treatment
#20
REVIEW
Haruki Komatsu, Ayano Inui, Tomoo Fujisawa
Although the introduction of hepatitis B vaccine has been contributing to the reduction in the prevalence of hepatitis B virus (HBV) carriers worldwide, the treatment of children with chronic HBV infection is a challenge to be addressed. HBeAg seroconversion, which induces low replication of HBV, is widely accepted as the first goal of antiviral treatment in children with chronic hepatitis B. However, spontaneous HBeAg seroconversion is highly expected in children with chronic HBV infection. Therefore, the identification of children who need antiviral treatment to induce HBeAg seroconversion is essential in the management of chronic HBV infection...
February 2017: Annals of Translational Medicine
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