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pediatric liver cancer

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https://www.readbyqxmd.com/read/28535186/fxr-gankyrin-axis-is-involved-in-development-of-pediatric-liver-cancer
#1
Leila Valanejad, Kyle Lewis, Mary Wright, Yanjun Jiang, Amber D'Souza, Rebekah Karns, Rachel Sheridan, Anita Gupta, Kevin Bove, David Witte, James Geller, Gregory Tiao, David L Nelson, Lubov Timchenko, Nikolai Timchenko
The development of hepatoblastoma (HBL) is associated with failure of hepatic stem cells (HSC) to differentiate into hepatocytes. Despite intensive investigations, mechanisms of the failure of HSC to differentiate are not known. We found that oncogene Gankyrin (Gank) is involved in the inhibition of differentiation of HSC via triggering degradation of tumor suppressor proteins (TSPs) Rb, p53, C/EBPα and HNF4α. Our data show that the activation of a repressor of Gank, farnesoid X receptor, FXR, after initiation of liver cancer by Diethylnitrosamine (DEN) prevents the development of liver cancer by inhibiting Gank and rescuing tumor suppressor proteins...
May 23, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/28494529/nash-therapy-omega-3-supplementation-vitamin-e-insulin-sensitizers-and-statin-drugs
#2
Stephen Caldwell
Non-alcoholic steatohepatitis (NASH) is the more aggressive form of non-alcoholic fatty liver disease (NAFLD). NASH can progress to hepatic fibrosis, cirrhosis, portal hypertension and primary liver cancer. Therapy is evolving with a substantial number of trials of promising new agents now in progress. In this article however, we will examine data for several older forms of therapy which have been fairly extensively studied over the years: Polyunsaturated Fatty Acid (PUFA) supplements, vitamin E, insulin sensitizing agents with a focus on pioglitazone and statin agents...
May 10, 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28459463/mycn-induces-neuroblastoma-in-primary-neural-crest-cells
#3
R R Olsen, J H Otero, J García-López, K Wallace, D Finkelstein, J E Rehg, Z Yin, Y-D Wang, K W Freeman
Neuroblastoma (NBL) is an embryonal cancer of the sympathetic nervous system (SNS), which causes 15% of pediatric cancer deaths. High-risk NBL is characterized by N-Myc amplification and segmental chromosomal gains and losses. Owing to limited disease models, the etiology of NBL is largely unknown, including both the cell of origin and the majority of oncogenic drivers. We have established a novel system for studying NBL based on the transformation of neural crest cells (NCCs), the progenitor cells of the SNS, isolated from mouse embryonic day 9...
May 1, 2017: Oncogene
https://www.readbyqxmd.com/read/28455541/review-of-management-and-morbidity-of-pediatric-craniopharyngioma-patients-in-a-low-middle-income-country-a-12-year-experience
#4
Nisreen Amayiri, Maisa Swaidan, Yocoub Yousef, Hadeel Halalsheh, Ramiz Abu-Hijlih, Sima Kalaldeh, Maha Barbar, Maher Elayyan, Nesreen Faqih, Maysa Al-Hussaini, Mustafa Mehyar, Ute Bartels, James Drake, Awni Musharbash, Eric Bouffet
BACKGROUND: Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few. PATIENTS AND METHODS: The study provides a retrospective chart review of pediatric patients <18 years old, diagnosed with craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan. RESULTS: Twenty-four patients (12 males) were identified...
April 28, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28454262/aberrant-klk4-gene-promoter-hypomethylation-in-pediatric-hepatoblastomas
#5
Baihui Liu, Ximao Cui, Shan Zheng, Kuiran Dong, Rui Dong
DNA methylation has a crucial role in cancer biology and has been recognized as an activator of oncogenes and inactivator of tumor suppressor genes, both of which are mechanisms for tumorigenesis. Kallikrein-related peptidase 4 (KLK4), has been suggested to be an oncogene in various types of cancer. The aim of the present study was to assess the DNA methylation patterns of the KLK4 gene in cancerous samples harvested from patients with hepatoblastoma (HB). KLK4 mRNA expression levels were detected using reverse transcription-quantitative polymerase chain reaction and assessed its DNA methylation patterns using high-throughput mass spectrometry on a matrix-assisted laser desorption/ionization time-of-flight mass array...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28351519/contrast-enhanced-ultrasound-for-the-evaluation-of-blunt-pediatric-abdominal-trauma
#6
Lindsey B Armstrong, David P Mooney, Harriet Paltiel, Carol Barnewolt, Beatrice Dionigi, Mary Arbuthnot, Chinwendu Onwubiko, Susan A Connolly, Delma Y Jarrett, Jill M Zalieckas
INTRODUCTION: Blunt abdominal trauma is a common problem in children. Computed tomography (CT) is the gold standard for imaging in pediatric blunt abdominal trauma, however up to 50% of CTs are normal and CT carries a risk of radiation-induced cancer. Contrast enhanced ultrasound (CEUS) may allow accurate detection of abdominal organ injuries while eliminating exposure to ionizing radiation. METHODS: Children aged 7-18years with a CT-diagnosed abdominal solid organ injury underwent grayscale/power Doppler ultrasound (conventional US) and CEUS within 48h of injury...
March 20, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28349834/downregulation-of-transcription-factor-gata4-sensitizes-human-hepatoblastoma-cells-to-doxorubicin-induced-apoptosis
#7
Tea Soini, Marjut Pihlajoki, Antti Kyrönlahti, Leif C Andersson, David B Wilson, Markku Heikinheimo
Hepatoblastoma, the most common type of pediatric liver cancer, is treated with a combination of surgery and chemotherapy. An essential drug in the treatment of hepatoblastoma is doxorubicin, which in high doses is cardiotoxic. This adverse effect is due to downregulation of cardiac expression of transcription factor GATA4, leading in turn to diminished levels of anti-apoptotic BCL2 (B-cell lymphoma 2) protein family members. GATA4 is also expressed in early fetal liver, but absent from normal postnatal hepatocytes...
March 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/28333140/metabolic-and-molecular-insights-into-an-essential-role-of-nicotinamide-phosphoribosyltransferase
#8
Li Q Zhang, Leon Van Haandel, Min Xiong, Peixin Huang, Daniel P Heruth, Charlie Bi, Roger Gaedigk, Xun Jiang, Ding-You Li, Gerald Wyckoff, Dmitry N Grigoryev, Li Gao, Linheng Li, Min Wu, J Steven Leeder, Shui Qing Ye
Nicotinamide phosphoribosyltransferase (NAMPT) is a pleiotropic protein implicated in the pathogenesis of acute respiratory distress syndrome, aging, cancer, coronary heart diseases, diabetes, nonalcoholic fatty liver disease, obesity, rheumatoid arthritis, and sepsis. However, the underlying molecular mechanisms of NAMPT in these physiological and pathological processes are not fully understood. Here, we provide experimental evidence that a Nampt gene homozygous knockout (Nampt(-/-)) resulted in lethality at an early stage of mouse embryonic development and death within 5-10 days in adult mice accompanied by a 25...
March 23, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28302000/expanded-criteria-for-hepatocellular-carcinoma-in-liver-transplant
#9
Mehmet Haberal, Aydıncan Akdur, Gökhan Moray, Gülnaz Arslan, Figen Özçay, Haldun Selçuk, Handan Özdemir
OBJECTIVES: Hepatocellular carcinoma is the sixth most common cancer worldwide and is the third highest cause of malignancy-related death. Because of its typically late diagnosis, median survival is approximately 6 to 20 months, with 5-year survival of < 12%. Hepatocellular carcinoma typically arises in the background of cirrhosis, with liver transplant regarded as the optimal therapy for selected patients. Initially, orthotopic liver transplant was limited to patients with extensive unresectable tumors, resulting in uniformly dismal outcomes due to high tumor recurrence rates...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28242319/white-paper-aga-power-practice-guide-on-obesity-and%C3%A2-weight-management-education-and-resources
#10
REVIEW
Andres Acosta, Sarah Streett, Mathew D Kroh, Lawrence J Cheskin, Katherine H Saunders, Marina Kurian, Marsha Schofield, Sarah E Barlow, Louis Aronne
The epidemic of obesity continues at alarming rates, with a high burden to our economy and society. The American Gastroenterological Association understands the importance of embracing obesity as a chronic, relapsing disease and supports a multidisciplinary approach to the management of obesity. Because gastrointestinal disorders resulting from obesity are more frequent and often present sooner than type 2 diabetes mellitus and cardiovascular disease, gastroenterologists have an opportunity to address obesity and provide an effective therapy early...
May 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#11
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28103709/radiation-absorbed-dose-estimates-for-18f-bpa-pet
#12
Yuzuru Kono, Hiroaki Kurihara, Hiroshi Kawamoto, Naoko Yasui, Naoki Honda, Hiroshi Igaki, Jun Itami
Background Boron neutron capture therapy (BNCT) is a molecular radiation therapy approach based on the (10)B (n, α) (7)Li nuclear reaction in cancer cells. In BNCT, delivery of (10)B in the form of 4-borono-phenylalanine conjugated with fructose (BPA-fr) to the cancer cells is important. The PET tracer 4-borono-2-18F-fluoro-phenylalanine (FBPA) has been used to predict the accumulation of BPA-fr before BNCT. Purpose To determine the biodistribution and dosimetric parameters in 18F-BPA PET/CT studies. Material and Methods Human biokinetic data were obtained during clinical 18F-BPA PET studies between February and June 2015 at one institution...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28039901/living-donor-liver-transplantation-in-a-pediatric-patient-with-preexisting-yolk-sac-tumor
#13
Taiichi Wakiya, Yoshikazu Toyoki, Keinosuke Ishido, Daisuke Kudo, Norihisa Kimura, Shinji Tsutsumi, Tadashi Odagiri, Akiko Suto, Chiaki Uchida, Kenichi Hakamada
There is ongoing discussion regarding the indications and timing of LT for patients with a preexisting extrahepatic malignancy. We herein report a pediatric case that underwent LDLT after therapy for YST. The patient, a 13-year-old female with biliary atresia, had undergone portoenterostomy at 2 months of age. She developed a left ovarian tumor with a high serum alpha-fetoprotein concentration at 10 years of age. She underwent left oophorectomy and was diagnosed with ovarian YST (Stage I). After surgery, hepatopulmonary syndrome progressed gradually...
March 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28038456/dna-methylation-landscape-of-hepatoblastomas-reveals-arrest-at-early-stages-of-liver-differentiation-and-cancer-related-alterations
#14
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 Cristina Victorino 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...
December 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/27986169/hepatic-steatosis-etiology-patterns-and-quantification
#15
REVIEW
Ilkay S Idilman, Ilknur Ozdeniz, Musturay Karcaaltincaba
Hepatic steatosis can occur because of nonalcoholic fatty liver disease (NAFLD), alcoholism, chemotherapy, and metabolic, toxic, and infectious causes. Pediatric hepatic steatosis is also becoming more frequent and can have distinctive features. The most common pattern is diffuse form; however, it can present in heterogenous, focal, multinodular, perilesional, perivascular, subcapsular, and lobar forms. Focal steatosis and fat sparing can occur because of the presence of veins of Sappey, pancreaticoduodenal vein, and aberrant right and left gastric veins, which drain into the liver as third inflow...
December 2016: Seminars in Ultrasound, CT, and MR
https://www.readbyqxmd.com/read/27938502/novel-advances-in-understanding-of-molecular-pathogenesis-of-hepatoblastoma-a-wnt-%C3%AE-catenin-perspective
#16
Danielle Bell, Sarangarajan Ranganathan, Junyan Tao, Satdarshan P Monga
Hepatoblastoma is the most common pediatric liver malignancy, typically striking children within the first 3 years of their young lives. While advances in chemotherapy and newer surgical techniques have improved survival in patients with localized disease, unfortunately, for the 25% of patients with metastasis, the overall survival remains poor. These tumors, which are thought to arise from hepatic progenitors or hepatoblasts, hence the name hepatoblastoma, can be categorized by histological subtyping based on their level of cell differentiation...
February 10, 2017: Gene Expression
https://www.readbyqxmd.com/read/27793881/therapeutic-innovations-for-targeting-hepatoblastoma
#17
REVIEW
Agnès Garnier, Matthias Ilmer, Roland Kappler, Michael Berger
Hepatoblastoma is the most common pediatric liver tumor. Despite recent advances in treatment with surgery and chemotherapy, the prognosis in advanced stages remains poor. The neurokinin-1 receptor (NK1R) has recently been described to be pivotal in the development of cancer. Furthermore, overwhelming evidence now exists showing that pharmacological manipulation of NK1R can cause a robust anticancer effect. Consequently, NK1R antagonists, such as the clinical drug aprepitant, are under current investigation as future innovative anticancer agents...
2016: Anticancer Research
https://www.readbyqxmd.com/read/27788486/gata4-promotes-hepatoblastoma-cell-proliferation-by-altering-expression-of-mir125b-and-dkk3
#18
Yihua Pei, Qin Yao, Sibo Yuan, Bozhen Xie, Yan Liu, Chunsheng Ye, Huiqin Zhuo
GATA4 is a zinc finger DNA-binding protein that plays an important role in mammalian liver development. However, the effects of GATA4 in hepatoblastoma (HB), a common liver cancer in pediatric patients, remain largely unknown. In this study, we demonstrate that GATA4 promotes growth and survival in the Huh6 human hepatoblastoma cell line. GATA4 expression was high in Huh6 cells, and its knockdown decreased expression of Dickkopf-related protein 3 (DKK3), a gene that may contribute to premature or undifferentiated phenotypes in HB...
November 22, 2016: Oncotarget
https://www.readbyqxmd.com/read/27685428/update-of-literature-from-cystic-fibrosis-registries-2012-2015-part-6-epidemiology-nutrition-and-complications
#19
REVIEW
Donatello Salvatore, Roberto Buzzetti, Gianni Mastella
Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries...
September 29, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27631159/the-use-of-erwinia-asparaginase-for-adult-patients-with-acute-lymphoblastic-leukemia-after-pegaspargase-intolerance
#20
Troy Z Horvat, Joshua J Pecoraro, Ryan J Daley, Larry W Buie, Amber C King, Raajit K Rampal, Martin S Tallman, Jae H Park, Dan Douer
Asparaginase administration has become a crucial component of front-line pediatric and pediatric-insipired multi-agent regimens for the treatment of acute lymphoblastic leukemia (ALL). The aim of this retrospective study was to assess the safety and feasibility of switching to Erwinia asparaginase after pegaspargase intolerance in adult ALL patients treated at Memorial Sloan Kettering Cancer Center. Our analysis included 10 patients, with a median age of 39 years (range 20-72), male predominance (90%), and a typical B-cell to T-cell ratio (70:30%) for ALL...
November 2016: Leukemia Research
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