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https://www.readbyqxmd.com/read/29766815/old-and-new-treatments-for-pediatric-autoimmune-hepatitis
#1
Silvia Nastasio, Giuseppe Maggiore, Marco Sciveres, Lorenza Matarazzo
Autoimmune hepatitis is a rare inflammatory disease of the liver that most frequently affects children and young adults. It is a multifactorial disease of unknown etiology, characteristically progressive in nature, and if left untreated, may lead to cirrhosis and terminal liver failure. It has been known for several decades now that immunosuppressive treatment convincingly alters the outcome of most patients with autoimmune hepatitis and as such it should be started as soon as diagnosis is made. Primary goals of treatment are: normalization of hepatocellular function, extinction of the hepatic necroinflammatory process, and maintenance of a stable remission, thus preventing progression to cirrhosis and its complications...
May 16, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29751874/nonalcoholic-steatohepatitis-histopathology-basics-within-a-broader-context
#2
REVIEW
Michael H Schild, Cynthia D Guy
Nonalcoholic fatty liver disease (NAFLD) is a major health concern and the prevalence continues to increase in many industrialized and developing countries around the world. NAFLD affects adults and children. NAFLD-related cirrhosis is expected to become the top indication for liver transplantation in the near future, and the incidence of NAFLD-related hepatocellular carcinoma is also increasing. Nonalcoholic steatohepatitis is the more severe form of NAFLD. The pathogenesis of NALFD/nonalcoholic steatohepatitis is complex and new concepts continue to evolve...
June 2018: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/29740791/nonalcoholic-fatty-liver-disease-in-children-not-a-small-matter
#3
REVIEW
Praveen Kumar Conjeevaram Selvakumar, Mohammad Nasser Kabbany, Naim Alkhouri
The prevalence of nonalcoholic fatty liver disease (NAFLD) has increased substantially in the past two decades and NAFLD has now become the most common cause of chronic liver disease in children and adolescents. NAFLD is a broad clinicopathologic spectrum ranging from simple steatosis to varying degrees of necroinflammation called nonalcoholic steatohepatitis (NASH), leading to fibrosis and subsequently to cirrhosis. Despite the increasing prevalence and progressive nature of NAFLD even among children, therapy for NAFLD in both adults and children are limited...
May 9, 2018: Paediatric Drugs
https://www.readbyqxmd.com/read/29700810/refining-the-phenotype-associated-with-biallelic-dnajc21-mutations
#4
G D'Amours, F Lopes, J Gauthier, V Saillour, C Nassif, R Wynn, N Alos, T Leblanc, Y Capri, S Nizard, E Lemyre, J L Michaud, V-A Pelletier, Y D Pastore, J-F Soucy
Inherited bone marrow failure syndromes (IBMFS) are caused by mutations in genes involved in genomic stability. Although they may be recognized by the association of typical clinical features, variable penetrance and expressivity are common, and clinical diagnosis is often challenging. DNAJC21, which is involved in ribosome biogenesis, was recently linked to bone marrow failure. However, the specific phenotype and natural history remain to be defined. We correlate molecular data, phenotype, and clinical history of five unreported affected children and all individuals reported in the literature...
April 26, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29689122/efficacy-and-safety-of-peginterferon-alfa-2a-40kd-in-children-with-chronic-hepatitis-b-the-peg-b-active-study
#5
Stefan Wirth, Hongfei Zhang, Winita Hardikar, Kathleen B Schwarz, Etienne Sokal, Weibo Yang, Huimin Fan, Vyacheslav Morozov, Qing Mao, Hong Deng, Yang Huang, Lei Yang, Nicolas Frey, Clare Nasmyth-Miller, Vedran Pavlovic, Cynthia Wat
Children with chronic hepatitis B (CHB) represent an area of unmet medical need, due to increased lifetime risk of CHB sequelae and limited therapeutic options compared with adult CHB patients. The PEG-B-ACTIVE (NCT01519960) phase III study evaluated peginterferon (PegIFN) alfa-2a treatment in children aged 3 to <18 years with CHB. A total of 161 hepatitis B e antigen (HBeAg)-positive immune-active patients without advanced fibrosis/cirrhosis were randomized (2:1) to PegIFN alfa-2a (Group A, n = 101) or no treatment (Group B, n = 50); patients with advanced fibrosis were assigned to PegIFN alfa-2a (Group C, n = 10)...
April 24, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29685617/validation-of-transient-elastography-cut-points-to-assess-advanced-liver-fibrosis-in-children-and-young-adults-the-boston-children-s-hospital-experience
#6
Christine K Lee, Paul D Mitchell, Roshan Raza, Sarah Harney, Shanna M Wiggins, Maureen M Jonas
OBJECTIVE: To derive an optimal liver stiffness measurement cut point to discriminate METAVIR fibrosis stage F4 and to validate both METAVIR fibrosis stage F3-F4 and F4 cut points in a separate cohort. STUDY DESIGN: Patients at Boston Children's Hospital with liver stiffness measurement from 2006 to 2016 and liver biopsy ≤12 months before screening were eligible. Patients enrolled 2006-2011 were used to calibrate liver stiffness measurement cut points and those enrolled 2011-2016 for validation...
April 20, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29604222/biliary-atresia-clinical-and-research-challenges-for-the-21-st-century
#7
Jorge A Bezerra, Rebecca G Wells, Cara L Mack, Saul J Karpen, Jay H Hoofnagle, Edward Doo, Ronald J Sokol
Biliary atresia (BA) is a fibroinflammatory disease of the intra- and extrahepatic biliary tree. Without medical treatment, surgical hepatic portoenterosmy (HPE) may restore bile drainage, but progression of the intrahepatic disease results in complications of portal hypertension and advanced cirrhosis in most children. Recognizing that further progress in the field is unlikely without a better understanding of the underlying cause(s) and pathogenesis of the disease, the National Institutes of Diabetes and Digestive and Kidney Diseases sponsored a research workshop focused on innovative and promising approaches and on identifying future areas of research...
March 31, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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
#8
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...
March 27, 2018: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/29565146/stunting-wasting-and-mid-upper-arm-circumference-status-among-children-admitted-to-nemazee-teaching-hospital
#9
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
https://www.readbyqxmd.com/read/29543694/systematic-review-the-epidemiology-natural-history-and-burden-of-alagille-syndrome
#10
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
https://www.readbyqxmd.com/read/29517271/the-search-for-noninvasive-methods-to-identify-liver-fibrosis-in-children-with-nonalcoholic-fatty-liver-disease
#11
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 2018: Biomarkers in Medicine
https://www.readbyqxmd.com/read/29515345/adult-presentation-of-noncirrhotic-portal-hypertension-and-ascites-following-treatment-for-wilms-tumor-in-childhood
#12
Linda Kievit, Pia Kræmer, Stephen Hamilton-Dutoit, Henning Grønbæk
A 37-year-old male, who at the age of 8 years had been treated for right-sided Wilms' tumor with nephrectomy, radiotherapy, and chemotherapy, presented with noncirrhotic portal hypertension (NCPH), grade 2 esophageal varices, and ascites. A CT scan demonstrated hypoplasia of liver segments 2 and 3. A liver biopsy showed portal tract fibrosis without cirrhosis, with histological features of NCPH. Liver vein catheterization showed a normal portal pressure gradient of 5 mm Hg while spleen to hepatic vein pressure was 29 mm Hg...
January 2018: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29505478/retrospective-analysis-of-children-with-%C3%AE-1-antitrypsin-deficiency
#13
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
https://www.readbyqxmd.com/read/29471561/nonalcoholic-fatty-liver-disease-in-children
#14
Jake P Mann, Luca Valenti, Eleonora Scorletti, Christopher D Byrne, Valerio Nobili
Nonalcoholic steatohepatitis, a progressive form of nonalcoholic fatty liver disease (NAFLD), is one of the most common hepatic diseases in children who present with particular risk factors including obesity, sedentary lifestyle, and/or a predisposing genetic background. The worldwide prevalence of NAFLD in children is a worrying phenomenon because this disease is closely associated with the development of both cirrhosis and cardiometabolic syndrome in adulthood. To date, the etiopathogenesis of primary NAFLD in children is unknown...
February 2018: Seminars in Liver Disease
https://www.readbyqxmd.com/read/29460791/hypercholesterolemia-and-hypertriglyceridemia-as-risk-factors-of-liver-dysfunction-in-children-with-inflammation-receiving-total-parenteral-nutrition
#15
Ana Paula Brigatto Simões Golucci, André Moreno Morcillo, Taís Daiene Russo Hortencio, Antônio Fernando Ribeiro, Roberto José Negrão Nogueira
BACKGROUND AND AIMS: Hepatic dysfunction, due to parenteral nutrition, may become severe and lead to cirrhosis and hepatic failure, especially in newborns and infants. This study aimed to evaluate the association between the exclusive use of total parenteral nutrition (TPN) and changes in the hepatic profile, and to investigate the relationship between age, hypertriglyceridemia, and hypercholesterolemia, and the occurrence of laboratory liver dysfunction. METHODS: A descriptive and historical cohort study was conducted, evaluating 195 pediatric patients (age: 1 month to 19 years) who received TPN...
February 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29456414/high-prevalence-of-hepatitis-b-antibody-loss-and-a-case-report-of-de-novo-hepatitis-b-virus-infection-in-a-child-after-living-donor-liver-transplantation
#16
Palittiya Sintusek, Nawarat Posuwan, Piyaporn Wanawongsawad, Suttiruk Jitraruch, Yong Poovorawan, Voranush Chongsrisawat
AIM: To assess the seroprevalence of hepatitis B virus (HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation. METHODS: This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss...
February 14, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29446799/autoimmune-hepatitis-in-children
#17
Saumya Pathak, Deepak Kamat
Autoimmune hepatitis (AIH) is an immune-mediated, inflammatory liver disease. Clinical presentation of AIH in children is highly variable. It can present acutely, chronically, or silently. There are two main types of AIH-type 1 and type 2, which are differentiated and defined by the presence of specific autoantibodies. AIH eventually progresses to cirrhosis when left untreated, and occasionally even with treatment. AIH must be suspected and excluded in all children presenting with signs of acute, prolonged, or severe liver disease...
February 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29446109/systematic-review-the-natural-history-of-alpha-1-antitrypsin-deficiency-and-associated-liver-disease
#18
REVIEW
S A Townsend, R G Edgar, P R Ellis, D Kantas, P N Newsome, A M Turner
BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is estimated to affect three million people worldwide. It causes liver disease in a proportion of carriers of the PiS and PiZ allele due to the formation and retention of polymers within the endoplasmic reticulum of hepatocytes. The reason for this selective penetrance is not known. Although clinical trials are underway, liver transplantation is the only effective treatment for liver disease due to AATD. AIMS: To report the prevalence and natural history of liver disease among individuals with AATD, and assess the outcomes of liver transplantation through systematic review...
April 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29434181/efficacy-of-hepatitis-b-vaccination-among-children-in-special-region-of-yogyakarta-indonesia-evaluation-of-humoral-and-cellular-immunity
#19
Caessar Pronocitro, Nenny Sri Mulyani, Afif Avicenna Ghufron, Yugata Halimawan Hazazi, Bambang Ardianto, Didik Setyo Heriyanto
Hepatitis B remains a global burden, with estimated 15 to 40 percents of infected individuals eventually suffer from liver cirrhosis, liver failure, and hepatocellular carcinoma. Vaccination aims to form anti-HBs antibody with protective titer to prevent infection. CD4 T cell lymphocytes are known to play a major role in establishing immunity after vaccination. This study aimed to investigate protective titer rate among Indonesian children in Special Region of Yogyakarta following hepatitis B vaccination and correlation between anti-HBs titer and CD4 count...
January 17, 2018: Kobe Journal of Medical Sciences
https://www.readbyqxmd.com/read/29369303/report-from-the-international-conference-on-viral-hepatitis-2017
#20
Vicente Soriano, Benjamin Young, Nancy Reau
The International Conference on Viral Hepatitis 2017 brought exciting news on the treatment of viral hepatitis. The most recent estimates of the burden for hepatitis B virus and hepatitis C virus (HCV) infections were presented. The current gaps and prospects for regional and global eradication of viral hepatitis were discussed on the light of the WHO roadmap until 2030. Debates focused on hepatitis C and expectations using the new approved HCV pan-genotypic, once daily, oral direct-acting antivirals (DAAs), glecaprevir-pibrentasvir, and sofosbuvir-velpatasvir-voxilaprevir...
January 2018: AIDS Reviews
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