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Pediatric Liver

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https://www.readbyqxmd.com/read/28937543/cholangiocarcinoma-among-children-and-adolescents-a-review-of-the-literature-and-seer-database-analysis
#1
Jennifer R Newsome, Rajkumar Venkatramani, Andras Heczey, Heather E Danysh, Douglas S Fishman, Tamir Miloh
OBJECTIVES: Cholangiocarcinoma (CCA) is a biliary malignancy found primarily in adults. The incidence of CCA in children is unknown. The aim of this study was to describe characteristics of CCA in children and adolescents. METHODS: Using the Surveillance, Epidemiology, and End Results Program (SEER 18) database, we identified incident cases of CCA diagnosed at <20 years of age during the period of 1973-2013. Additionally, we reviewed published case reports describing pediatric patients with CCA...
September 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28935574/taurocholate-induces-biliary-differentiation-of-liver-progenitor-cells-causing-hepatic-stellate-cell-chemotaxis-in-the-ductular-reaction-role-in-pediatric-cystic-fibrosis-liver-disease
#2
Katarzyna N Pozniak, Michael A Pearen, Tamara N Pereira, Cynthia Sm Kramer, Priyakshi Kalita-De Croft, Sujeevi K Nawaratna, Manuel A Fernandez-Rojo, Geoffrey N Gobert, Janina Ee Tirnitz-Parker, John K Olynyk, Ross W Shepherd, Peter J Lewindon, Grant A Ramm
Cystic fibrosis liver disease (CFLD) in children causes progressive fibrosis leading to biliary cirrhosis, however its cause(s) and early pathogenesis are unclear. We hypothesised that a bile acid-induced Ductular Reaction (DR) drives fibrogenesis. We evaluated the DR by cytokeratin-7 immunohistochemistry in liver biopsies, staged for fibrosis, from 60 children with CFLD and demonstrated that the DR was significantly correlated with hepatic fibrosis stage and biliary taurocholate levels. To examine the mechanisms involved in DR induction liver progenitor cells (LPCs) were treated with taurocholate and key events in DR evolution were assessed: LPC proliferation, LPC biliary differentiation and hepatic stellate cell (HSC) chemotaxis...
September 18, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28934973/liver-chemistry-in-new-onset-henoch-sch%C3%A3-nlein-syndrome
#3
Giulia Rosti, Gregorio P Milani, Emanuela A Laicini, Emilio F Fossali, Mario G Bianchetti
BACKGROUND: Henoch-Schönlein syndrome is a systemic small-vessel leukocytoclastic vasculitis that usually present with cutaneous, gastrointestinal, articular and renal manifestations. Little is known on liver involvement in this syndrome. This study investigated liver chemistry and creatine kinase in Henoch-Schönlein children. CASE PRESENTATION: Alanine aminotransferase, aspartate aminotransferase, γ-glutamyltransferase, lactate dehydrogenase, total bilirubin, prothrombin time and creatine kinase were assessed in 75 consecutive pediatric patients (41 boys and 34 girls aged from 2...
September 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28928955/pediatric-liver-failure-we-came-we-saw-but-have-we-conquered
#4
REVIEW
Sara Kathryn Smith, Philip Rosenthal
Although there have been advances made in the diagnosis and management of pediatric acute liver failure, there is still no consensus regarding the definition or standardized evaluation, and an inability to predict outcomes, specifically irreversible brain injury, in many patients exists. Much of the research surrounding pediatric acute liver failure in the last several years has centered on the development of predictive scoring systems to enhance diagnosis and treatment decisions. In this article, we will discuss our current understanding of liver failure and updated management strategies in children with acute liver failure...
2017: F1000Research
https://www.readbyqxmd.com/read/28927979/enlarging-vascular-stents-after-pediatric-liver-transplantation
#5
Yi-Ting Yeh, Cheng-Yen Chen, Hsiou-Shan Tseng, Hsin-Kai Wang, Hsin-Lin Tsai, Niang-Cheng Lin, Chou-Fu Wei, Chinsu Liu
BACKGROUND: Endovascular intervention with stent placement to treat portal vein (PV) and hepatic vein (HV) stenosis after pediatric liver transplantation (LT) is still controversial in small children owing to the potential risk of functional stenosis after growth. The aim of this study is to evaluate the safety and efficacy of stent placement in this population. METHODS: Between 2004 and 2016, 6 children (all <3 years) received HV (n = 2) and PV (n = 4) stents placement among 46 pediatric LT patients at our institution...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28926121/new-pediatric-percentiles-of-liver-enzyme-serum-levels-alt-ast-ggt-effects-of-age-sex-bmi-and-pubertal-stage
#6
Sarah Bussler, Mandy Vogel, Diana Pietzner, Kristian Harms, Theresa Buzek, Melanie Penke, Norman Händel, Antje Körner, Ulrich Baumann, Wieland Kiess, Gunter Flemming
The present study aims to clarify the effects of sex, age, BMI and puberty on transaminase serum levels in children and adolescents and to provide new age- and sex-related percentiles for alanine aminotransferase (ALT), aspartate aminotransferase (AST) and γ-glutamyltransferase (GGT). Venous blood and anthropometric data were collected from 4,126 cases. Excluded were cases of participants with potential hepatotoxic medication, with evidence of potential illness at the time of blood sampling and non-normal BMI (BMI < 10(th) or > 90(th) )...
September 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28923827/mutant-ctnnb1-and-histological-heterogeneity-define-metabolic-subtypes-of-hepatoblastoma
#7
Stefania Crippa, Pierre-Benoit Ancey, Jessica Vazquez, Paolo Angelino, Anne-Laure Rougemont, Catherine Guettier, Vincent Zoete, Mauro Delorenzi, Olivier Michielin, Etienne Meylan
Hepatoblastoma is the most common malignant pediatric liver cancer. Histological evaluation of tumor biopsies is used to distinguish among the different subtypes of hepatoblastoma, with fetal and embryonal representing the two main epithelial components. With frequent CTNNB1 mutations, hepatoblastoma is a Wnt/β-catenin-driven malignancy. Considering that Wnt activation has been associated with tumor metabolic reprogramming, we characterized the metabolic profile of cells from hepatoblastoma and compared it to cells from hepatocellular carcinoma...
September 18, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28921939/extreme-hepatic-resections-for-the-treatment-of-advanced-hepatoblastoma-are-planned-close-margins-an-acceptable-approach
#8
Adriana Fonseca, Abha Gupta, Furqan Shaikh, Raveena Ramphal, Vicky Ng, Ian McGilvray, J Ted Gerstle
BACKGROUND: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. OBJECTIVE: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. METHODS: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015...
September 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28921748/increased-risk-organ-transplantation-in-the-pediatric-population
#9
Sean M Wrenn, Peter W Callas, Trishul Kapoor, Alia F Aunchman, Adam N Paine, Jaime A Pineda, Carlos E Marroquin
IRD organs are classified by the Public Health Service to be at above-average risk for harboring human immunodeficiency virus, hepatitis C, and hepatitis B. Traditionally underutilized, there exists even greater reluctance for their use in pediatric patients. We performed a retrospective analysis via the United Network for Organ Sharing database of all pediatric renal and hepatic transplants performed from 2004 to 2008 in the United States. Primary outcomes were patient and graft survival. Proportional hazards regression was performed to control for potentially confounding factors...
September 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28921384/can-diffusion-weighted-imaging-distinguish-between-benign-and-malignant-pediatric-liver-tumors
#10
Pablo Caro-Domínguez, Abha A Gupta, Govind B Chavhan
BACKGROUND: There are limited data on utility of diffusion-weighted imaging (DWI) in the evaluation of pediatric liver lesions. OBJECTIVE: To determine whether qualitative and quantitative DWI can be used to differentiate benign and malignant pediatric liver lesions. MATERIALS AND METHODS: We retrospectively reviewed MRIs in children with focal liver lesions to qualitatively evaluate lesions noting diffusion restriction, T2 shine-through, increased diffusion, hypointensity on DWI and apparent diffusion coefficient (ADC) maps, and intermediate signal on both, and to measure ADC values...
September 18, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28918602/population-pharmacokinetics-and-optimal-sampling-strategy-for-model-based-precision-dosing-of-melphalan-in-patients-undergoing-hematopoietic-stem-cell-transplantation
#11
Kana Mizuno, Min Dong, Tsuyoshi Fukuda, Sharat Chandra, Parinda A Mehta, Scott McConnell, Elias J Anaissie, Alexander A Vinks
BACKGROUND: High-dose melphalan is an important component of conditioning regimens for patients undergoing hematopoietic stem cell transplantation. The current dosing strategy based on body surface area results in a high incidence of oral mucositis and gastrointestinal and liver toxicity. Pharmacokinetically guided dosing will individualize exposure and help minimize overexposure-related toxicity. OBJECTIVE: The purpose of this study was to develop a population pharmacokinetic model and optimal sampling strategy...
September 16, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28917237/screening-for-nutritional-risk-in-hospitalized-children-with-liver-disease
#12
Tiantian Song, Ying Mu, Xue Gong, Wenyan Ma, Li Li
BACKGROUND AND OBJECTIVES: Malnutrition is a major contributor to morbidity and mortality from pediatric liver disease. We investigated the prevalence of both malnutrition and high nutritional risk in hospitalized children with liver disease as well as the rate of in-hospital nutritional support. METHODS AND STUDY DESIGN: A total of 2,874 hospitalized children and adolescents with liver disease aged 1 to 17 years (inclusive) were enrolled. Malnutrition was screened by anthropometric measures (height-for-age, weight-for-height, weight-for-age, and BMI- for-age z-scores)...
2017: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#13
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28913888/viral-upper-respiratory-infection-at-pediatric-liver-transplant-is-associated-with-hepatic-artery-thrombosis
#14
Jordan A Kohler, Flor M Munoz, John A Goss, Tamir A Miloh
No abstract text is available yet for this article.
September 15, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28912999/tacrolimus-aggravated-tube-feeding-syndrome-with-acute-renal-failure-in-a-pediatric-liver-transplant-recipient
#15
R Kula, M Melter, J Kunkel, C Dörfler, S Alikadic, B Knoppke, R Zant
Acute renal failure can be caused by calcineurin inhibitors (CNIs), due to arteriolopathy and altered tubular function. Within this context, we present the case of a 14-month-old liver transplant recipient who suffered an acute polyuric renal failure during a short episode of hypercaloric feeding. In our case, CNI-induced distal RTA led to nephrocalcinosis and therefore to secondary nephrogenic diabetes insipidus. The diet with high renal solute load consequently resulted in an acute polyuric renal failure with severe hypernatremic dehydration...
2017: Case Reports in Transplantation
https://www.readbyqxmd.com/read/28910276/igf2-stimulates-fetal-growth-in-a-sex-and-organ-dependent-manner
#16
Veronica White, Alicia Jawerbaum, Maria Belen Mazzucco, Martin Gauster, Gernot Desoye, Ursula Hiden
BACKGROUND: IGF2 is a key determinant of fetal growth, and altered expression of IGF2 is implicated in fetal growth disorders and maternal metabolic derangements including gestational diabetes. Here we studied how increased levels of IGF2 in late pregnancy affect fetal growth. METHODS: We employed a rat model of repeated intra-fetal IGF2 administration in late pregnancy, i.e. during GD19-GD21, and measured the consequences on fetal organ weight and expression of insulin/IGF-axis components...
September 14, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28903524/pediatric-dental-clinic-associated-outbreak-of-mycobacterium-abscessus-infection
#17
Lindsay A Hatzenbuehler, Melissa Tobin-D'Angelo, Cherie Drenzek, Gianna Peralta, Lisa C Cranmer, Evan J Anderson, Sarah S Milla, Shelly Abramowicz, Jumi Yi, Joseph Hilinski, Roy Rajan, Matthew K Whitley, Verlia Gower, Frank Berkowitz, Craig A Shapiro, Joseph K Williams, Paula Harmon, Andi L Shane
Background: Mycobacterium abscessus is an uncommon cause of invasive odontogenic infection. Methods: M abscessus-associated odontogenic infections occurred in a group of children after they each underwent a pulpotomy. A probable case-child was defined as a child with facial or neck swelling and biopsy-confirmed granulomatous inflammation after a pulpotomy between October 1, 2013, and September 30, 2015. M abscessus was isolated by culture in confirmed case-children...
September 1, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/28902943/use-of-fibrinogen-and-thrombin-sponge-in-pediatric-split-liver-transplantation
#18
Fernando Pompeu Piza Vicentine, Adriano Miziara Gonzalez, Barbara Burza Beninni, Ramiro Anthero de Azevedo, Marcelo Moura Linhares, Alberto Goldenberg, Gaspar de Jesus Lopes, Jose Luiz Martins, Alcides Augusto Salzedas
Purpose: To analyze the use of this sponge in pediatric patients undergoing split-liver transplantation. Methods: Retrospective study, including 35 pediatric patients undergoing split-liver transplantation, divided into two groups according to the use of the sponge: 18 patients in Group A (no sponge) and 17 in Group B (with sponge). Results: The characteristics of recipients and donors were similar. We observed greater number of reoperation due to bleeding in the wound area in Group A (10 patients - 55...
August 2017: Acta Cirúrgica Brasileira
https://www.readbyqxmd.com/read/28902846/downregulation-of-microrna-145-may-contribute-to-liver-fibrosis-in-biliary-atresia-by-targeting-add3
#19
Yongqin Ye, Zhihan Li, Qi Feng, Zimin Chen, Zhouguang Wu, Jianyao Wang, Xiaoshuo Ye, Dahao Zhang, Lei Liu, Wei Gao, Lihui Zhang, Bin Wang
BACKGROUND AND OBJECTIVES: Biliary atresia (BA) is a pediatric liver disease characterized by fibro-obliteration and obstruction of the extrahepatic biliary system, that invariably leads to cirrhosis and even death, if left untreated for extended time. However, its pathology and etiology still remained unknown. In this study, we tested the expression of adducin 3 (ADD3), the gene identified as a susceptibility gene in BA by GWAS, and uncovered its upstream regulatory microRNA in the pathogenesis of BA...
2017: PloS One
https://www.readbyqxmd.com/read/28898314/-associated-factors-to-non-operative-management-failure-of-hepatic-and-splenic-lesions-secondary-to-blunt-abdominal-trauma-in-children
#20
Adriana Echavarria Medina, Carlos Hernando Morales Uribe, Luis Guillermo Echavarria R, Viviana María Vélez Marín, Jorge Alberto Martínez Montoya, David Fernando Aguillón
INTRODUCTION: The non operative management (NOM) is the standard management of splenic and liver blunt trauma in pediatric patients.Hemodynamic instability and massive transfusions have been identified as management failures. Few studies evaluate whether there exist factors allowing anticipation of these events. The objective was to identify factors associated with the failure of NOM in splenic and liver injuries for blunt abdominal trauma. PATIENTS AND METHOD: Retrospective analysis between 2007-2015 of patients admitted to the pediatric surgery at University Hospital Saint Vincent Foundation with liver trauma and/or closed Spleen...
2017: Revista Chilena de Pediatría
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