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Keisuke Tachibana, Tomohiro Yuzuriha, Ryotaro Tabata, Syohei Fukuda, Takashi Maegawa, Rika Takahashi, Keiichi Tanimoto, Hirofumi Tsujino, Kazuto Nunomura, Bangzhong Lin, Yoshiharu Matsuura, Toshiya Tanaka, Takao Hamakubo, Juro Js Sakai, Tatsuhiko Kodama, Tadayuki Kobayashi, Kenji Ishimoto, Hiroyuki Miyachi, Takefumi Doi
Peroxisome proliferator-activated receptor alpha (PPARα) is a ligand-activated transcription factor that belongs to the superfamily of nuclear hormone receptors. PPARα is mainly expressed in the liver, where it activates fatty acid oxidation and lipoprotein metabolism and improves plasma lipid profiles. Therefore, PPARα activators are often used to treat patients with dyslipidemia. To discover additional PPARα activators as potential compounds for use in hypolipidemic drugs, here we established human hepatoblastoma cell lines with luciferase reporter expression from the promoters containing peroxisome proliferator responsive elements (PPRE) and tetracycline-regulated expression of full-length human PPARα to quantify the effects of chemical ligands on PPARα activity...
May 15, 2018: Journal of Biological Chemistry
Kaori Isono, Yuki Ohya, Kwang-Jong Lee, Shintaro Hashimoto, Masashi Kadohisa, Masataka Sakisaka, Keiichi Uto, Shintaro Hayashida, Hidekazu Yamamoto, Hirotoshi Yamamoto, Yasuhiko Sugawara, Yukihiro Inomata
LT is a practical therapeutic alternative for unresectable hepatoblastoma; however, deciding when to perform LT is difficult. The aim of this study was to optimize the timing of LT for hepatoblastoma using pretransplant trends in AFP levels. Trends in pretransplant AFP levels and their influence on post-transplant outcomes were retrospectively evaluated. All patients who underwent living donor LT for hepatoblastoma in our institution since 2002 were included. Variables analyzed included history of prior tumor resection, pretransplant AFP responses to chemotherapy, metastatic disease at diagnosis, and post-transplant chemotherapy...
May 15, 2018: Pediatric Transplantation
Kala Gnanasekaran Kiruthiga, Banumathi Ramakrishna, Soumitra Saha, Sudipta Sen
Background: Hepatoblastoma (HB) has different histological subtypes, with varying prognosis. Though the survival has drastically improved, subsets of patients are not responsive to therapy. Therefore, it becomes important to determine the factors which affect the behaviour of the tumour. This study was aimed to look at the histopathological subtypes and compare with immunohistochemical (IHC) expression of CK19, beta-catenin and EpCAM and survival. Methods: This study included 55 cases of HB...
April 2018: Journal of Gastrointestinal Oncology
Rosa Nguyen, M Beth McCarville, April Sykes, Shenghua Mao, Jianrong Wu, Max R Langham, Wayne L Furman
BACKGROUND: Neoadjuvant chemotherapy is given to children with unresectable hepatoblastoma to increase the rate and safety of curative complete surgical resection. Elevated levels of serum alpha-fetoprotein (sAFP) decline with tumor shrinkage. In this single-institution retrospective study, we determined early dynamic changes of sAFP levels and tumor volume in children during therapy for unresectable hepatoblastoma. METHODS: We correlated early dynamic changes of sAFP levels and tumor volume and the sum of the longest primary tumor and measurable metastatic disease diameters as per RECIST 1...
May 9, 2018: International Journal of Clinical Oncology
Niki Christou, Nabil Dib, Etienne Chuffart, Abdelkader Taibi, Sylvaine Durand-Fontanier, Denis Valleix, Muriel Mathonnet
Patients with congenital agenesis of the portal vein may develop hepatocellular tumors due to enhanced arterial blood flow. These tumors may be benign (FNH, adenomas) or malignant (hepatoblastoma, HCC). Liver resection can be proposed, and preoperative arterial embolization may decrease blood loss during surgery. Liver transplantation with PV reconstruction is also an option.
May 2018: Clinical Case Reports
Angela D Trobaugh-Lotrario, Dolores López-Terrada, Peng Li, James H Feusner
Familial adenomatous polyposis (FAP) due to APC mutation is associated with an increased risk of hepatoblastoma. All cases of hepatoblastoma in patients with FAP reported in the literature were reviewed. One hundred and nine patients were identified. Thirty-five patients (of 49 with data) were diagnosed with hepatoblastoma prior to a later diagnosis of FAP (often in association with advanced colorectal carcinoma), emphasizing a need to identify patients earlier with germline APC mutations for early colorectal carcinoma screening...
May 2, 2018: Pediatric Blood & Cancer
Akiko Inoue, Ryo Suzuki, Keisuke Urabe, Yutaro Kawamura, Midori Masuda, Kanta Kishi, Kimitaka Takitani, Hiroshi Katayama, Hideki Tomiyama, Michihiro Hayashi, Shintaro Nemoto, Kazuhisa Uchiyama, Hiroshi Tamai
Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease-free and the fourth is alive with remaining of the tumor...
April 27, 2018: Pediatric Blood & Cancer
Jacquelyn M Powers, Martha M Pacheco, Jonathan E Wickiser
Children with metastatic hepatoblastoma have a poor prognosis, even with dose intensification of cisplatin and doxorubicin. Vincristine and irinotecan have demonstrated activity in high risk disease. This report describes a 3-year-old girl with metastatic hepatoblastoma with unresectable disease after 5 cycles of cisplatin, 5-fluorouracil, vincristine, and doxorubicin who had a complete response of her metastatic disease to vincristine and irinotecan (intravenous and oral forms), allowing surgical resection of her liver disease...
April 17, 2018: Journal of Pediatric Hematology/oncology
Emilie Indersie, Katarzyna B Hooks, Caroline Capdevielle, Monique Fabre, Nathalie Dugot-Senant, Angélique Desplat, Sébastien Lepreux, Aksam Merched, Christophe F Grosset, Martin Hagedorn
Hepatoblastoma (HBL) is a pediatric liver cancer with defined molecular alterations driving its progression. Here, we describe an animal model for HBL on the chick chorioallantoic membrane (CAM), which recapitulates relevant features of HBL in patients. Expression of classic tumor-associated proteins such as β-catenin, EpCAM and CK19 was maintained in acini-like organized tumors on CAM, as was synthesis of AFP, a tumor marker used for monitoring patient response. RNA sequencing revealed an unexpected molecular evolution of HBL cells on the CAM, with significant deregulation of more than 6,000 genes including more than half of all HOX genes...
March 23, 2018: Oncotarget
Nianwu He, Lingmin Tian, Xichuan Zhai, Xiaowen Zhang, Yan Zhao
This study was designed to investigate the chemical characterization and bioactivity of the Trametes lactinea (Berk.) Pat polysaccharides (TLP). The crude TLP was fractionated into two fractions, namely TLP-1 and TLP-2 with Cellulose DEAE-52 and Sephadex G-150. HPLC and FT-IR analysis showed that TLP-1 and TLP-2 were heteropolysaccharides mainly composed of glucose with the average molecular weights of 443.19 kDa and 388.83 kDa, respectively. TLP-1 from water elution possessed of higher reducing power and scavenging activities against 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical, superoxide radical and hydroxyl radical than TLP-2 eluted by 0...
April 12, 2018: International Journal of Biological Macromolecules
Nathalie R Wingert, Marcelo D Arbo, Gabriela Göethel, Bárbara da Costa, Louise F Altknecht, Solange C Garcia, Martin Steppe
Degradation kinetics of oral anticoagulant rivaroxaban (RIV) was assessed in acid and alkaline media and while exposed to UVC radiation. Among all stress conditions tested, kinetic degradation process was better described by a zero-order model. A stability indicating method was validated for the analysis of the anticoagulant RIV in tablets by high-performance liquid chromatography. Robustness was evaluated with a two-level Plackett-Burman experimental design. The effect of acute exposition of the human hepatoblastoma HepG2 cell line to RIV stressed samples (100 and 500 µM) was assessed through in vitro toxicity tests...
April 12, 2018: Drug and Chemical Toxicology
Sandra Diaz-Parra, Gema Lozano-Sanchez, Olga Escobosa-Sanchez, David Moreno-Perez, Antonio Morales-Martinez, Carolina Armengol-Niell, Tomás Acha-Garcia
Severe combined immunodeficiency is an inherited disease with profoundly defective T cells, B cells, and natural killer cells. X-linked severe combined immunodeficiency is the most common form. In this report, we describe a 4-month-old male infant who was admitted to our hospital with progressive breathlessness and abdominal mass. He was diagnosed with hepatoblastoma and presented a pneumocystis jirovecii pneumonia at the beginning of chemotherapy. Definitive diagnosis of X-linked severe combined immunodeficiency was established by DNA analysis of the interleukin 2 receptor gamma chain gene...
April 3, 2018: Journal of Pediatric Hematology/oncology
Nhatrang Le, Douglas C Rivard, Rebecca M Rentea, Michelle Manalang, Walter Andrews, Bartholomew Kane, Richard J Hendrickson
Portal vein embolization (PVE) is a pre-operative treatment modality in adults undergoing hepatectomy with concerns of post-operative liver failure from insufficient future liver remnant (FLR). PVE induces growth in the FLR. The success of this technique is well described in adults, but not in young children with hepatoblastoma.
March 29, 2018: Pediatric Surgery International
Mohammad K Parvez, Ahmad H Arbab, Mohammed S Al-Dosari, Adnan J Al-Rehaily, Perwez Alam, Khalid E Ibrahim, Mansour S Alsaid, Syed Rafatullah
Atriplex suberecta I. Verd is a known phytomedicinal species of Atriplex ; however, studies into its bioactivity remain inconclusive. The in vitro and in vivo antioxidative and hepatoprotective potential of A. suberecta ethanol-extract (ASEE) was assessed in the present study. 1,1-diphenyl-2-picrylhydrazyl radical scavenging and β-carotene bleaching assays revealed that ASEE possesses free radical scavenging and anti-lipid peroxidative activities. These results were supported by the in vitro protection of HepG2 hepatoblastoma cells via abating 2,7-dichlorofluorescein-activated oxidative and apoptotic molecules (caspase-3/-7)...
April 2018: Experimental and Therapeutic Medicine
Benji Lv, Lianhai Zhang, Runchen Miao, Xiaohong Xiang, Shunbin Dong, Ting Lin, Ke Li, Qu Kai
Hepatoblastoma (HB), as a common pediatric liver malignancy, is composed of a variety of subgroups with different clinical outcomes. Long-noncoding RNA (lncRNA) has crucial roles in cancer biology. However, the association between lncRNA and HB has not been fully investigated. In this study, we screened lncRNA expression profiles that were annotated from the GSE75271 dataset. A total of 225 differentially expressed lncRNAs (DELs) were identified based on comparison between three prognostic subgroups, and seven of them (XR_241302, XR_923061, NR_038322, XR_951687, XR_934593, NR_120317 and XR_93406) that exhibited highly predictive accuracies were selected for functional analysis...
February 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Candace C Style, Stephanie M Cruz, Patricio E Lau, Timothy C Lee, David E Wesson, Oluyinka O Olutoye
PURPOSE: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital. METHODS: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes. RESULTS: Forty-seven children with a diagnosis of BWS were identified...
May 2018: Journal of Pediatric Surgery
Jose J G Marin, Oscar Briz, Elisa Herraez, Elisa Lozano, Maitane Asensio, Silvia Di Giacomo, Marta R Romero, Luis M Osorio-Padilla, Ana I Santos-Llamas, Maria A Serrano, Carolina Armengol, Thomas Efferth, Rocio I R Macias
A characteristic shared by most frequent types of primary liver cancer, i.e., hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) in adults, and in a lesser extent hepatoblastoma (HB) mainly in children, is their high refractoriness to chemotherapy. This is the result of synergic interactions among complex and diverse mechanisms of chemoresistance (MOC) in which more than 100 genes are involved. Pharmacological treatment, although it can be initially effective, frequently stimulates the expression of MOC genes, which results in the relapse of the tumor, usually with a more aggressive and less chemosensitive phenotype...
March 12, 2018: Clinics and Research in Hepatology and Gastroenterology
Chunqing Dou, Mingming Han, Bao Zhang, Liyuan Sun, Xin Jin, Tao Li
Previous studies have indicated that chrysotoxene may be a potential drug used to treat tumors, however the effect of chrysotoxene on hepatoblastoma remains unknown. Therefore, the present study aimed to investigate the cytotoxic effect and elucidate the potential molecular mechanism of chrysotoxene on human hepatoblastoma HepG2 cells. Chrysotoxene (5-40 µg/ml) exhibited cytotoxic activity against HepG2 cells with inhibitory rates of 24.67-84.06% (half maximal inhibitory concentration, 19.64 µg/ml), observed from a Cell Counting Kit-8 assay...
April 2018: Oncology Letters
Lin Xie, Jay Onysko, Howard Morrison
INTRODUCTION: Surveillance of childhood cancer incidence trends can inform etiologic research, policy and programs. This study presents the first population-based report on demographic and geographic variations in incidence trends of detailed pediatric diagnostic groups in Canada. METHODS: The Canadian Cancer Registry data were used to calculate annual age-standardized incidence rates (ASIRs) from 1992 to 2010 among children less than 15 years of age by sex, age and region for the 12 main diagnostic groups and selected subgroups of the International Classification of Childhood Cancer (ICCC), 3rd edition...
March 2018: Health Promotion and Chronic Disease Prevention in Canada
Shwetha V Kumar, Philip J Lupo, Lisa A Pompeii, Heather E Danysh
The environmental determinants of pediatric embryonal tumors remain unclear. Because of the growing concern over the impact of exposures to traffic-related air pollution on pediatric cancer, we conducted a population-based study evaluating the impact of maternal residential proximity to major roadways on the risk of pediatric embryonal tumors in offspring. We identified children diagnosed with neuroblastoma, Wilms tumor, retinoblastoma, or hepatoblastoma at <5 years of age from the Texas Cancer Registry and selected unaffected controls from birth certificates...
March 13, 2018: International Journal of Environmental Research and Public Health
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