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Cirrhosis in children

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/29515345/adult-presentation-of-noncirrhotic-portal-hypertension-and-ascites-following-treatment-for-wilms-tumor-in-childhood
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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...
February 15, 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
#15
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/29349944/investigation-of-clinical-and-pathological-relationships-between-adult-and-pediatric-type-nash-in-korean-children
#16
Jungmin Yoon, Byung Han Park, Ja Hye Kim, Jin Hwa Moon, Young Ho Lee, Se Min Jang, Yong Joo Kim
BACKGROUND: Histologically, nonalcoholic steatohepatitis (NASH) is categorized into adult-type (type 1) and pediatric-type (type 2). The origination of the histological difference between the two types and how they differ clinically remain uncertain. We aimed to understand the incidence and clinical characteristics of the two types of NASH in Korean children, and to investigate the association between their pathological type and clinical characteristics, using anthropometric and laboratory data...
January 29, 2018: Journal of Korean Medical Science
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
#17
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/29316057/a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#18
Federica Ferrari, Giusy Ranucci, Marina Aloi, Luca Della Volpe, Franca Viola, Erasmo Miele, Salvatore Cucchiara, Raffaele Iorio
AIM: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease that is being increasingly diagnosed in childhood. The long-term course and prognosis of pediatric SC are poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers, during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age, 10.4 years; male patients, 73...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29286012/hepatitis-a-b-c-and-hiv-seroprevalence-among-syrian-refugee-children-admitted-to-outpatient-clinics
#19
Şükran Köse, Ilker Ödemiş, Didem Çelik, Bengü Gireniz Tatar, Ilkay Akbulut, Dilek Yilmaz Çiftdoğan
Viral hepatitis is the most common cause of serious health problems such as liver cirrhosis and hepatocellular carcinoma (HCC). Leading to immunodeficiency disorders through different mechanisms, Human Immunodeficiency Virus (HIV) causes the development of severe secondary infections. Hepatitis A (HAV) is thought to spread by the faecal-oral route, while Hepatitis B (HBV), Hepatitis C (HCV) and HIV are mostly transmitted vertically during childhood. In our study, we aimed to determine the seroprevalence of HAV, HBV, HCV and HIV among Syrian refugee children who were admitted to outpatient clinics...
December 1, 2017: Le Infezioni in Medicina
https://www.readbyqxmd.com/read/29240010/well-preserved-renal-function-in-children-with-untreated-chronic-liver-disease
#20
Ulla B Berg, Antal Németh
OBJECTIVES: On the basis of studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease (CLD). Therefore, we investigated renal function in children with CLD. METHODS: In a retrospective study of 277 children with CLD, 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...
April 2018: Journal of Pediatric Gastroenterology and Nutrition
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