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Pediatric hepatitis

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https://www.readbyqxmd.com/read/29334124/use-of-antiviral-medications-in-drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-a-case-of-infantile-dress
#1
Maggie L Chow, David Kim, Sonia Kamath, David Peng, Minnelly Luu
A 3-month-old girl with Sturge-Weber syndrome presented with a morbilliform rash, eosinophilia, and fulminant liver failure to our tertiary pediatric hospital. She was diagnosed with drug reaction with eosinophilia and systemic symptoms complicated by viremia and evidence of viral hepatitis on liver biopsy. We discuss the role of viral reactivation in drug reaction with eosinophilia and systemic symptoms and the relevance of antiviral therapy in management.
January 15, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29332587/lysosomal-acid-lipase-deficiency-could-dyslipidemia-drive-the-diagnosis
#2
Ornella Guardamagna
LAL-deficiency (LAL-D) is a rare and systemic condition, secondary to LIPA gene mutations, responsible for lysosomal accumulation of cholesteryl esters and triglycerides, whose manifestations are very heterogeneous in terms of age of onset, severity and type of clinical and radiological manifestations. Dyslipidemia, hepatomegaly and hepatosteatosis with increased levels of transaminases are the most common features. The increased risk of premature atherosclerosis and cardiovascular disorders,, secondary to a generalized alteration of lipid profile and lipoprotein dysfunction associated with LAL-D, has been increasingly pointed out...
January 11, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29331407/decreased-health-related-quality-of-life-in-children-and-adolescents-with-autoimmune-hepatitis
#3
Ana Beatriz Bozzini, Luciana Neder, Clovis A Silva, Gilda Porta
OBJECTIVE: The objective of this study was to evaluate the health-related quality of life in children and adolescents with autoimmune hepatitis. METHODS: A cross-sectional assessment with the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was completed for 80 patients with autoimmune hepatitis and 45 healthy controls. Demographic data, prednisone dose, disease remission state, disease severity, and abdominal pain were also evaluated. RESULTS: Based on the child self-reports, physical, emotional, school, and total scores were significantly lower in autoimmune hepatitis patients when compared with controls (p<0...
January 10, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29319634/hyperoxia-and-hypocapnia-during-pediatric-extracorporeal-membrane-oxygenation-associations-with-complications-mortality-and-functional-status-among-survivors
#4
Katherine Cashen, Ron Reeder, Heidi J Dalton, Robert A Berg, Thomas P Shanley, Christopher J L Newth, Murray M Pollack, David Wessel, Joseph Carcillo, Rick Harrison, J Michael Dean, Robert Tamburro, Kathleen L Meert
OBJECTIVES: To determine the frequency of hyperoxia and hypocapnia during pediatric extracorporeal membrane oxygenation and their relationships to complications, mortality, and functional status among survivors. DESIGN: Secondary analysis of data collected prospectively by the Collaborative Pediatric Critical Care Research Network. SETTING: Eight Collaborative Pediatric Critical Care Research Network-affiliated hospitals. PATIENTS: Age less than 19 years and treated with extracorporeal membrane oxygenation...
January 9, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#5
F Ferrari, G Ranucci, M Aloi, L Della Volpe, F Viola, E Miele, S Cucchiara, R Iorio
BACKGROUND AND AIMS: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long-term course and prognosis of pediatric SC is 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...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29316006/extubation-in-the-operating-room-after-pediatric-liver-transplant-a-retrospective-cohort-study
#6
Harshad G Gurnaney, Scott D Cook-Sather, Abraham Shaked, Kim M Olthoff, Elizabeth B Rand, Arul M Lingappan, Mohamed A Rehman
BACKGROUND: Early extubation immediately following liver transplantation is increasingly common in adult practice. Some pediatric institutions have begun to adopt this strategy. Careful patient selection is essential in minimizing risk. METHODS: This retrospective cohort study evaluated infants and children who underwent liver transplantation between July 2011 and December 2014. Our primary objective was to determine early extubation rate. Secondary objectives were to identify clinical factors associated with successful early extubation compared with delayed extubation and to examine significant postoperative complications, intensive care unit length of stay, and hospital length of stay...
January 8, 2018: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29301447/defibrotide-sodium-for-the-treatment-of-hepatic-veno-occlusive-disease-sinusoidal-obstruction-syndrome
#7
Paul G Richardson, Brandon M Triplett, Vincent T Ho, Nelson Chao, Fiona L Dignan, Michelle Maglio, Mohamad Mohty
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is an unpredictable condition associated with endothelial-cell damage due to conditioning for hematopoietic stem-cell transplantation (HSCT) or chemotherapy without HSCT. Mortality in patients with VOD/SOS and multi-organ dysfunction (MOD) may be >80%. Areas covered: Defibrotide is the only approved drug for the treatment of severe hepatic VOD/SOS after HSCT in the European Union and hepatic VOD/SOS with renal or pulmonary dysfunction in the United States...
January 5, 2018: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/29298340/the-association-between-a-body-shape-index-and-cardiovascular-risk-in-overweight-and-obese-children-and-adolescents
#8
Chiara Mameli, Nir Y Krakauer, Jesse C Krakauer, Alessandra Bosetti, Chiara Matilde Ferrari, Norma Moiana, Laura Schneider, Barbara Borsani, Teresa Genoni, Gianvincenzo Zuccotti
A Body Shape Index (ABSI) and normalized hip circumference (Hip Index, HI) have been recently shown to be strong risk factors for mortality and for cardiovascular disease in adults. We conducted an observational cross-sectional study to evaluate the relationship between ABSI, HI and cardiometabolic risk factors and obesity-related comorbidities in overweight and obese children and adolescents aged 2-18 years. We performed multivariate linear and logistic regression analyses with BMI, ABSI, and HI age and sex normalized z scores as predictors to examine the association with cardiometabolic risk markers (systolic and diastolic blood pressure, fasting glucose and insulin, total cholesterol and its components, transaminases, fat mass % detected by bioelectrical impedance analysis) and obesity-related conditions (including hepatic steatosis and metabolic syndrome)...
2018: PloS One
https://www.readbyqxmd.com/read/29290748/therapeutic-drug-monitoring-of-prolonged-infusion-aztreonam-for-multi-drug-resistant-pseudomonas-aeruginosa-a-case-report
#9
Jeffrey J Cies, Richard J LaCoursiere, Wayne S Moore, Arun Chopra
Aztreonam, a broad-spectrum monobactam, is typically reserved for multidrug resistant (MDR) infections. Pharmacokinetic (PK) data to guide dosing in children, however, are limited to healthy volunteers or nonintensive care unit (ICU) patients. Impaired antibiotic delivery into tissue remains a major concern and may explain the high morbidity and mortality associated with MDR infections. Therefore, evaluating the PK changes in pediatric ICU patients is necessary to elucidate the most appropriate antimicrobial regimen...
November 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29290659/balloon-dilatation-for-treatment-of-hepatic-venous-outflow-obstruction-following-pediatric-liver-transplantation
#10
Zhi-Yuan Zhang, Long Jin, Guang Chen, Tian-Hao Su, Zhi-Jun Zhu, Li-Ying Sun, Zhen-Chang Wang, Guo-Wen Xiao
AIM: To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction (HVOO) following pediatric liver transplantation. METHODS: A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures (two patients underwent two balloon dilatation procedures) were included in this analysis...
December 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29287014/treatment-of-chronic-hepatitis-c-virus-infection-in-children-a-position-paper-by-the-hepatology-committee-of-european-society-of-paediatric-gastroenterology-hepatology-and-nutrition
#11
Giuseppe Indolfi, Loreto Hierro, Antal Dezsofi, Jörg Jahnel, Dominique Debray, Nedim Hadzic, Piotr Czubowski, Girish Gupte, Yael Mozer-Glassberg, Wendy van der Woerd, Françoise Smets, Henkjan J Verkade, Björn Fischler
OBJECTIVES: In 2017, the European Medicines Agency (EMA) and the Food and Drug Administration (FDA) approved the use of the fixed-dose combination of ledipasvir/sofosbuvir and of the combination of sofosbuvir and ribavirin for treatment of adolescents (12-17 years, weighing more than 35 kg) with chronic hepatitis C virus (HCV) genotype 1, 4, 5 and 6 and genotype 2 and 3 infections, respectively. Although trials with direct acting antivirals (DAAs) are ongoing for younger children, the only available treatment in US and Europe for those < 12 years is still the dual therapy of pegylated interferon (PEG IFN) and ribavirin...
December 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29279846/autoantibodies-against-cyp-2c19-a-novel-serum-marker-in-pediatric-de-novo-autoimmune-hepatitis
#12
Maria Grazia Clemente, Roberto Antonucci, Claudia Mandato, Lucia Cicotto, Antonella Meloni, Bruno Gridelli, Stefano De Virgiliis, Michael P Manns, Pietro Vajro
Diagnosis of de novo autoimmune hepatitis (AIH) after orthotopic liver transplantation (OLT) is challenging especially in the absence of hyper-γ-globulinemia. Circulating autoantibodies are not sensitive nor specific in de novo AIH but when positive increase the diagnostic probability. We report the discovery of novel liver microsomal (LM) autoantibodies against CYP-2C19 in a 9-year-old boy with "de novo" AIH developed 7 years after OLT. Graft dysfunction presented with hypertransaminasemia (up to 400 IU/L), while serum γ-globulins remained within the normal range for age...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29274889/new-onset-autoimmune-hepatitis-during-anti-tumor-necrosis-factor-alpha-treatment-in-children
#13
Amanda Ricciuto, Binita M Kamath, Thomas D Walters, Karen Frost, Nicholas Carman, Peter C Church, Simon C Ling, Anne M Griffiths
OBJECTIVES: To evaluate a large anti-tumor necrosis factor (TNF)-treated pediatric inflammatory bowel disease cohort for drug-induced liver injury (DILI) following presentation of an index case with suspected DILI with autoimmune features after infliximab exposure. To characterize the incidence, natural history, and risk factors for liver enzyme elevation with anti-TNF use. STUDY DESIGN: We reviewed the index case and performed a retrospective cohort study of 659 children receiving anti-TNF therapy between 2000 and 2015 at a tertiary pediatric inflammatory bowel disease center...
December 21, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29273893/intraoperative-doppler-sonogram-in-pediatric-liver-transplants-a-pictorial-review-of-intraoperative-and-early-postoperative-complications
#14
A Luana Stanescu, Shawn E Kamps, André A S Dick, Marguerite T Parisi, Grace S Phillips
A spectrum of vascular complications can be seen in pediatric liver transplant patients, including occlusion and hemodynamically significant narrowing of the vessels that provide inflow to or outflow from the graft. Intraoperative Doppler ultrasound (US) has the potential benefit of identifying vascular complications in pediatric liver transplant patients prior to abdominal closure. Importantly, intraoperative Doppler US can be used as a problem-solving tool in situations such as position-dependent kinking of the portal or hepatic veins, or in suspected vasospasm of the hepatic artery...
December 22, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/29260287/change-in-liver-spleen-and-bone-marrow-magnetic-resonance-imaging-signal-intensity-over-time-in-children-with-solid-abdominal-tumors
#15
Michael Sirignano, Jonathan R Dillman, Brian D Weiss, Charles T Quinn, Bin Zhang, Weizhe Su, Andrew T Trout
BACKGROUND: Reticuloendothelial system MRI signal hypointensity is common in pediatric oncology patients with solid abdominal tumors. OBJECTIVE: To assess changes in liver, spleen and bone marrow T2-weighted MRI signal intensity over time and their relationship to blood transfusion history in children with solid abdominal tumors. MATERIALS AND METHODS: In this retrospective study we measured liver, spleen and bone marrow signal intensity on axial T2-weighted MR images obtained December 2009 through February 2016 in children with hepatoblastoma, neuroblastoma, ganglioneuroblastoma and Wilms tumor...
December 19, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/29249967/psoralea-corylifolia-l-attenuates-nonalcoholic-steatohepatitis-in-juvenile-mouse
#16
Lishan Zhou, Jianqiao Tang, Xiaoli Xiong, Hui Dong, Juan Huang, Shunchang Zhou, Lingling Zhang, Huan Qin, Suqi Yan
Psoralea corylifolia L. (PC) is a traditional Chinese herb used to treat yang deficiency of the spleen and kidney in pediatric disease. Recent studies have shown its liver protection and anti-oxidative effects. The aim of this study was to explore the effect and mechanism of PC on nonalcoholic steatohepatitis in juvenile mice. The juvenile mouse model of nonalcoholic fatty liver disease/nonalcoholic steatohepatitis (NAFLD/NASH) was established by being fed a high-fat diet in maternal-offspring manner. PC granules were prepared and the quality was assessed...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29234203/cholemic-or-bile-cast-nephropathy-in-a-child-with-liver-failure
#17
Vikrant Sood, Bikrant B Lal, Suman Lata, Archana Rastogi, Seema Alam
Cholemic or bile cast nephropathy is an under-reported entity characterized by acute renal dysfunction in patients with hepatic insult. Limited literature is available regarding its clinical presentation, pathogenesis and prognosis. We hereby present a pediatric case who presented with acute on chronic liver failure with renal dysfunction secondary to cholemic nephropathy.
December 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29232664/vaccination-practices-in-pediatric-dialysis-patients-across-europe-a-european-pediatric-dialysis-working-group-and-european-society-for-pediatric-nephrology-dialysis-working-group-study
#18
Sevcan A Bakkaloğlu, Yesim Özdemir Atikel, Fabio Paglialonga, Constantinos J Stefanidis, Varvara Askiti, Enrico Vidal, Gema Ariceta, Engin Melek, Enrico Verrina, Nikoleta Printza, Karel Vondrak, Aleksandra Zurowska, Ilona Zagozdzon, Mesiha Ekim, Elif Nursel Özmert, Stephanie Dufek, Augustina Jankauskiene, Claus Peter Schmitt, Eszter Lévai, Johan Vande Walle, Nur Canpolat, Tuula Holtta, Michel Fischbach, Guenter Klaus, Christoph Aufricht, Rukshana Shroff, Alberto Edefonti
BACKGROUND: Data on the immunization practices in pediatric chronic kidney disease (CKD) patients are scarce. The purpose of this study was to evaluate current vaccination practices for children on dialysis across European pediatric nephrology centers. METHODS: A total of 18 tertiary pediatric nephrology centers from 12 European countries were included in the study. The data on universal national immunization programs and immunization practices for children with chronic disease or risk were recorded from European Center for Disease Prevention and Control and the World Health Organization...
December 12, 2017: Nephron
https://www.readbyqxmd.com/read/29228658/dna-methylation-landscape-of-hepatoblastomas-reveals-arrest-at-early-stages-of-liver-differentiation-and-cancer-related-alterations
#19
Mariana Maschietto, Tatiane Cristina Rodrigues, André Yoshiaki Kashiwabara, Érica Sara Souza de Araujo, Talita Ferreira Marques Aguiar, Cecilia Maria Lima da Costa, Isabela Werneck da Cunha, Luciana Dos Reis Vasques, Monica Cypriano, Helena Brentani, Silvia Regina Caminada de Toledo, Peter Lees Pearson, Dirce Maria Carraro, Carla Rosenberg, Ana C V Krepischi
Hepatoblastomas are uncommon embryonal liver tumors accounting for approximately 80% of childhood hepatic cancer. We hypothesized that epigenetic changes, including DNA methylation, could be relevant to hepatoblastoma onset. The methylomes of eight matched hepatoblastomas and non-tumoral liver tissues were characterized, and data were validated in an independent group (11 hepatoblastomas). In comparison to differentiated livers, hepatoblastomas exhibited a widespread and non-stochastic pattern of global low-level hypomethylation...
November 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29228631/identification-of-differentially-expressed-genes-lncrnas-and-mirnas-which-are-associated-with-tumor-malignant-phenotypes-in-hepatoblastoma-patients
#20
Sida Liu, Fujing Xie, Xiaohong Xiang, Sinan Liu, Shunbin Dong, Kai Qu, Ting Lin
Hepatoblastoma (HB) is one of the most common hepatic malignancies in the pediatric population. HB are composed of a variety of tumors, which derived from different origins and had varying clinical outcomes. However, the unclear underlying mechanisms of HB limited exploring novel biomarkers and effective therapeutic targets. We searched microarray datasets on Gene Expression Omnibus (GEO) database and selected GSE75271 and GSE75283 datasets for comprehensive analysis. Weighted gene correlation network analysis (WGCNA) was employed to identify genes which were associated with tumor malignant phenotypes, including HB subtypes, Cairo classification and tumor stage...
November 14, 2017: Oncotarget
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