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https://www.readbyqxmd.com/read/28538506/allogeneic-hematopoietic-cell-transplantation-for-dyskeratosis-congenita-a-report-of-3-cases
#1
Shinichi Tamura, Toshihiko Imamura, Takayo Urata, Miki Kobayashi, Mari Gen, Toshihiro Tomii, Junko Do, Shinya Osone, Hiroyuki Ishida, Hajime Hosoi, Hiroshi Kuroda
Although bone marrow failure in patients with dyskeratosis congenita (DKC) can be successfully treated with allogeneic hematopoietic cell transplantation (allo-HCT) using a reduced intensity conditioning (RIC) regimen, the outcome of nonhematological disorders in patients with DKC treated with allo-HCT using RIC has not been fully elucidated. Here, we describe the clinical course of nonhematological disorders after allo-HCT with RIC in 3 consecutive patients with DKC. Allo-HCT with RIC was feasible in all cases; however, patient 1 developed lethal pulmonary disease and patient 2 experienced progression of hepatic fibrosis...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28535186/fxr-gankyrin-axis-is-involved-in-development-of-pediatric-liver-cancer
#2
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/28534541/-effectiveness-of-second-generation-direct-acting-antivirals-in-chronic-hepatitis-c
#3
R Juanbeltz Zurbano, J M Zozaya Urmeneta, J Repáraz Padrós, J Castilla Catalán, M T Sarobe Carricas, J I Úriz-Otano, M Gracia Ruiz de Alda, R San Miguel Elcano
BACKGROUND: Second-generation direct-acting antivirals (DAA) have shown high sustained virologic response (SVR) for the treatment of chronic hepatitis C in clinical trials. The objective of this study is to estimate DAA effectiveness in treatment of this disease. METHODS: Hepatitis C virus (HCV) monoinfected patients and HCV-human immunodeficiency virus (HIV) coinfected pa-tients who started interferon-free DAA based regimens during 2015 were included. The primary effectiveness outcome was SVR, defined as an undetectable viral load 12 weeks after the end of treatment...
April 30, 2017: Anales del Sistema Sanitario de Navarra
https://www.readbyqxmd.com/read/28534001/frequency-of-acute-hepatitis-following-acute-paraphenylene-diamine-intoxication
#4
Rizwan Ishtiaq, Sadaf Shafiq, Ali Imran, Qazi Masroor Ali, Raheel Khan, Hassan Tariq, Daniyal Ishtiaq
INTRODUCTION: Paraphenylene diamine (PPD) ingestion is manifesting as one of the more common ways of committing suicide in Southern Punjab, Pakistan, especially Bahawalpur. PPD is an ingredient of a compound commonly known "Kala Pathar" which means "Black Stone" in Urdu. It is readily available in the market at low cost and is used to dye hair and fur. Its intoxication inhibits cellular oxidation and affects the muscles causing rhabdomyolysis. This leads to myoglobinuria followed by renal failure and edema of face and throat resulting in respiratory difficulty...
April 21, 2017: Curēus
https://www.readbyqxmd.com/read/28533939/efficacy-of-peptide-receptor-radionuclide-therapy-with-177-lu-octreotate-in-metastatic-pulmonary-neuroendocrine-tumors-a-dual-centre-analysis
#5
Amir Sabet, Alexander R Haug, Collin Eiden, Christoph J Auernhammer, Birgit Simon, Peter Bartenstein, Hans J Biersack, Samer Ezziddin
There is lack of data on the specific benefit of peptide receptor radionuclide therapy (PRRT) in neuroendocrine tumors (NET) of pulmonary origin. This dual- centre study aimed to assess outcome and toxicity of standardized PRRT with (177)Lu-octreotate in a patient population of advanced pulmonary NET of grade 1-2. We retrospectively assessed 22 consecutively patients treated with 4 intended cycles at 3 monthly intervals (mean activity per cycle 7.8±0.68 GBq). In a median follow-up period of 54 months, no significant nephrotoxicity (≥ grade 3) was observed...
2017: American Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28533924/interferon-free-treatments-in-patients-with-hepatitis-c-genotype-1-4-infections-in-a-real-world-setting
#6
Huascar Ramos, Pedro Linares, Ester Badia, Isabel Martín, Judith Gómez, Carolina Almohalla, Francisco Jorquera, Sara Calvo, Isidro García, Pilar Conde, Begoña Álvarez, Guillermo Karpman, Sara Lorenzo, Visitación Gozalo, Mónica Vásquez, Diana Joao, Marina de Benito, Lourdes Ruiz, Felipe Jiménez, Federico Sáez-Royuela, Asociación Castellano Y Leonesa de Hepatología ACyLHE
AIM: To investigated the real-world effectiveness and safety of various regimens of interferon-free treatments in patients infected with hepatitis C virus (HCV). METHODS: We performed an observational study to analyze different antiviral treatments administered to 462 HCV-infected patients, of which 56.7% had liver cirrhosis. HCV RNA after 4 wk of treatment and at 12 wk after treatment sustained virologic response (SVR) as well as serious adverse events (SAEs) was analyzed first for the whole cohort and then separately in patients who met or did not meet the inclusion criteria of a clinical trial (CT-met and CT-unmet, respectively)...
May 6, 2017: World Journal of Gastrointestinal Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28533911/hepatic-encephalopathy
#7
Peter Ferenci
Hepatic encephalopathy (HE) is a reversible syndrome of impaired brain function occurring in patients with advanced liver diseases. The precise pathophysiology of HE is still under discussion; the leading hypothesis focus on the role of neurotoxins, impaired neurotransmission due to metabolic changes in liver failure, changes in brain energy metabolism, systemic inflammatory response and alterations of the blood brain barrier. HE produces a wide spectrum of nonspecific neurological and psychiatric manifestations...
May 2017: Gastroenterology Report
https://www.readbyqxmd.com/read/28533473/u-s-food-and-drug-administration-approval-summary-pembrolizumab-for-the-treatment-of-recurrent-or-metastatic-head-and-neck-squamous-cell-carcinoma-with-disease-progression-on-or-after-platinum-containing-chemotherapy
#8
Erin Larkins, Gideon M Blumenthal, Weishi Yuan, Kun He, Rajeshwari Sridhara, Sriram Subramaniam, Hong Zhao, Chao Liu, Jingyu Yu, Kirsten B Goldberg, Amy E McKee, Patricia Keegan, Richard Pazdur
On August 5, 2016, the U.S. Food and Drug Administration granted accelerated approval to pembrolizumab (KEYTRUDA injection, Merck Sharp & Dohme Corp., Kenilworth, NJ) for treatment of patients with recurrent or metastatic head and neck squamous cell carcinoma (HNSCC) with disease progression on or after platinum-containing chemotherapy. Approval was based on the objective response rate (ORR) and duration of response (DoR) in a cohort of patients in a nonrandomized multi-cohort trial (KEYNOTE-012) that included 174 patients with recurrent or metastatic HNSCC who had disease progression on or after platinum-containing chemotherapy...
May 22, 2017: Oncologist
https://www.readbyqxmd.com/read/28527109/erratum-to-clinical-pharmacokinetics-of-sacubitril-valsartan-lcz696-a-novel-angiotensin-receptor-neprilysin-inhibitor
#9
Surya Ayalasomayajula, Thomas Langenickel, Parasar Pal, Sreedevi Boggarapu, Gangadhar Sunkara
Sacubitril/valsartan (LCZ696) is indicated for the treatment of heart failure with reduced ejection fraction. Absorption of sacubitril/valsartan and conversion of sacubitril (prodrug) to sacubitrilat (neprilysin inhibitor) was rapid with maximum plasma concentrations of sacubitril, sacubitrilat, and valsartan (angiotensin receptor blocker) reaching within 0.5, 1.5-2.0, and 2.0-3.0 h, respectively. With a twofold increase in dose, an increase in the area under the plasma concentration-time curve was proportional for sacubitril, ~1...
May 19, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28526886/fk866-attenuates-acute-hepatic-failure-through-c-jun-n-terminal-kinase-jnk-dependent-autophagy
#10
Enshuang Guo, Renlong Li, Jiankun Yang, Jun Zhang, Anyi Li, Yan Yang, Shenpei Liu, Anding Liu, Xiaojing Jiang
FK866 exhibits a protective effect on D-galactosamine (GaIN)/lipopolysaccharide (LPS) and concanavalin A (ConA)-induced acute liver failure (ALF), but the mechanism by which FK866 affords this benefit has not yet been elucidated. Autophagy has a protective effect on acute liver injury. However, the contribution of autophagy to FK866-conferred hepatoprotection is still unclear. This study aimed to investigate whether FK866 could attenuate GaIN/LPS and ConA-induced ALF through c-jun-N-terminal kinase (JNK)-dependent autophagy...
May 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28525892/rasal1-is-a-potent-regulator-of-hepatic-stellate-cell-activity-and-liver-fibrosis
#11
Akemi Takata, Motoyuki Otsuka, Takahiro Kishikawa, Mari Yamagami, Rei Ishibashi, Kazuma Sekiba, Tatsunori Suzuki, Motoko Ohno, Yui Yamashita, Takaya Abe, Ryota Masuzaki, Tsuneo Ikenoue, Kazuhiko Koike
Liver fibrosis, leading to cirrhosis and liver failure, can occur after chronic liver injury. The transition of hepatic stellate cells (HSCs) from quiescent cells into proliferative and fibrogenic cells is a central event in liver fibrosis. Here, we show that RAS protein activator like-1 (RASAL1), a RAS-GTPase-activating protein, which switches off RAS activity, is significantly decreased during HSC activation, and that HSC activation can be antagonized by forced expression of the RASAL1 protein. We demonstrate that RASAL1 suppresses HSC proliferation by regulating the Ras-MAPK pathway, and that RASAL1 suppresses HSC fibrogenic activity by regulating the PKA-LKB1-AMPK-SRF pathway by interacting with angiotensin II receptor, type 1...
May 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28524831/a-case-series-of-liver-abscess-formation-after-transcatheter-arterial-chemoembolization-for-hepatic-tumors
#12
Wei Sun, Fei Xu, Xiao Li, Chen-Rui Li
BACKGROUND: Liver abscess is a serious complication following transcatheter arterial chemoembolization (TACE). Much attention has been paid to this condition as it may interfere with the treatment process and result in a poor prognosis of the patient. This study aimed to analyze the causes of liver abscess, a complication, after TACE for hepatic tumors and to summarize management approaches. METHODS: From June 2012 to June 2014, of 1480 consecutive patients who underwent TACE at our hospital, five patients developed liver abscess after TACE procedures for hepatic tumors...
June 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28523950/levocarnitine-and-vitamin-b-complex-for-the-treatment-of-pegaspargase-induced-hepatotoxicity-a-case-report-and-review-of-the-literature
#13
Alison Blackman, Alyssa Boutin, Alexei Shimanovsky, William J Baker, Nicholas Forcello
Asparaginase is a chemotherapeutic agent that is commonly used in combination with other medications for the treatment of acute lymphoblastic leukemia. An adverse effect of asparaginase includes hepatotoxicity, which can lead to severe liver failure and death. Several reports have documented successful treatment of asparaginase-induced hepatotoxicity using levocarnitine (l-carnitine) and vitamin B complex. Herein, we report a patient with acute lymphoblastic leukemia that experienced acute liver injury following pegaspargase administration...
January 1, 2017: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/28522898/the-protective-effect-of-cordycepin-on-d-galactosamine-lipopolysaccharide-induced-acute-liver-injury
#14
Jin Li, Liping Zhong, Haibo Zhu, Fengzhong Wang
As the major active ingredient of Cordyceps militaris, cordycepin (3'-deoxyadenosine) has been well documented to alleviate inflammation and oxidative stress both in vitro and in vivo. To explore the potential protective effect of cordycepin in fulminant hepatic failure, mice were pretreated with cordycepin for 3 weeks followed by D-galactosamine (GalN)/lipopolysaccharide (LPS) injection. Then we found cordycepin (200 mg/kg) administration elevated survival rate, improved liver function, and suppressed hepatocyte apoptosis and necrosis in mice with severe hepatic damage by GalN/LPS treatment...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28521955/prevalence-of-relevant-ns5a-resistance-associated-substitutions-to-elbasvir-in-genotype-1a-hepatitis-c-virus-patients-in-spain
#15
Claudia Palladino, Beatriz Esteban-Cartelle, Irene Mate-Cano, Marta Sánchez-Carrillo, Salvador Resino, Verónica Briz
Resistance-associated substitutions (RASs) to the new HCV NS5A inhibitor elbasvir may limit its efficacy and lead to virological failure in HCV-GT1a-infected patients. There are no data outside clinical trials evaluating their prevalence and impact in grazoprevir/elbasvir in GT1a-infected patients in Spain. A multicentre cross-sectional study of 632 initial patients was conducted. In 13 of these patients, the sample could not be amplified or a consensus sequence by Sanger sequencing could not be performed. Ultimately, 617 HCV-G1a-infected individuals treated at 84 Spanish hospitals from the 17 autonomous communities plus the 2 autonomous cities of Spain were analysed...
May 15, 2017: Enfermedades Infecciosas y Microbiología Clínica
https://www.readbyqxmd.com/read/28520708/hepatitis-b-surface-antigen-screening-among-pregnant-women-and-care-of-infants-of-hepatitis-b-surface-antigen-positive-mothers-guam-2014
#16
Winston E Abara, Susan Cha, Tasneem Malik, Mia S DeSimone, Bernadette Schumann, Esther Mallada, Michael Klemme, Vince Aguon, Anne Marie Santos, Melissa Collier, Mary Kamb
Hepatitis B virus (HBV) infection is endemic among adults in the U.S. territory of Guam (1,2). Perinatal HBV transmission, which occurs at birth from an infected mother to her newborn infant, is a major mode of HBV transmission and maintains HBV endemicity (3). Approximately 90% of HBV-infected infants will develop chronic HBV infection, and approximately 25% of those will die prematurely from liver failure or hepatocellular carcinoma (4,5). Since 1988, the Advisory Committee on Immunization Practices has recommended that all pregnant women be screened for hepatitis B surface antigen (HBsAg), an indicator of HBV infection, and that infants of women who screen positive (HBsAg-positive women) receive postexposure prophylaxis (PEP) (hepatitis B vaccine and hepatitis B immunoglobulin [HBIG])...
May 19, 2017: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/28518219/impact-of-the-timing-of-hepatitis-b-virus-identification-and-anti-hepatitis-b-virus-therapy-initiation-on-the-risk-of-adverse-liver-outcomes-for-patients-receiving-cancer-therapy
#17
Jessica P Hwang, Maria E Suarez-Almazor, Scott B Cantor, Andrea Barbo, Heather Y Lin, Sairah Ahmed, Mariana Chavez-MacGregor, Christian Donato-Santana, Cathy Eng, Alessandra Ferrajoli, Michael J Fisch, Peter McLaughlin, George R Simon, Gabriela Rondon, Elizabeth J Shpall, Anna S Lok
BACKGROUND: Data on the incidence of adverse liver outcomes are limited for cancer patients with chronic (hepatitis B surface antigen [HBsAg]-positive/hepatitis B core antibody [anti-HBc]-positive) or past (HBsAg-negative/anti-HBc-positive) hepatitis B virus (HBV) after chemotherapy. This study was aimed at determining the impact of test timing and anti-HBV therapy on adverse liver outcomes in these patients. METHODS: Patients with solid or hematologic malignancies who received chemotherapy between 2004 and 2011 were retrospectively studied...
May 18, 2017: Cancer
https://www.readbyqxmd.com/read/28516570/proposal-of-size-based-surgical-indication-criteria-for-liver-hemangioma-based-on-a-nationwide-survey-in-japan
#18
Yoshihiro Sakamoto, Norihiro Kokudo, Takeyuki Watadani, Junji Shibahara, Masakazu Yamamoto, Hiroki Yamaue
BACKGROUND: Surgical indications for liver hemangioma remain unclear. METHODS: Of 510 patients undergoing surgical resection for liver hemangioma in 118 Japanese centers between 1998 and 2012, abdominal symptoms, diagnostic accuracy, and surgical outcomes were analyzed to propose size-based surgical indications. Patients were classified into four groups based on tumor size: Group A) ≤5 cm (n = 122, 24%), B) 5-10 cm (n = 164, 32%), C) 10-15 cm (n = 124, 24%), and D) >15 cm (n = 100, 20%)...
May 17, 2017: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/28516271/three-dimensional-image-fusion-guidance-for-transjugular-intrahepatic-portosystemic-shunt-placement
#19
Vania Tacher, Arthur Petit, Haytham Derbel, Luigi Novelli, Manuel Vitellius, Fourat Ridouani, Alain Luciani, Alain Rahmouni, Christophe Duvoux, Chady Salloum, Mélanie Chiaradia, Hicham Kobeiter
INTRODUCTION: To assess the safety, feasibility and effectiveness of image fusion guidance with pre-procedural portal phase computed tomography with intraprocedural fluoroscopy for transjugular intrahepatic portosystemic shunt (TIPS) placement. MATERIALS AND METHODS: All consecutive cirrhotic patients presenting at our interventional unit for TIPS creation from January 2015 to January 2016 were prospectively enrolled. Procedures were performed under general anesthesia in an interventional suite equipped with flat panel detector, cone-beam computed tomography (CBCT) and image fusion technique...
May 17, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28515612/acute-yellow-phosphorus-poisoning-causing-fulminant-hepatic-failure-with-parenchymal-hemorrhages-and-contained-duodenal-perforation
#20
Reddy Ravikanth, S Sandeep, Babu Philip
White phosphorus is well known as a potent hepatotoxin and a severe local and systemic toxin causing damage to gastrointestinal, hepatic, cardiovascular, and renal systems. It is used in the manufacture of matches, fireworks, rodenticide, and fertilizers. Death results due to acute liver failure. Management of yellow phosphorus (YP) poisoning is supportive with no antidote available. Here, we present a case of acute YP poisoning in a 25-year-old female presenting with fulminant hepatic failure and duodenal perforation...
April 2017: Indian Journal of Critical Care Medicine
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