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https://www.readbyqxmd.com/read/29341979/wilson-s-disease-in-children-a-position-paper-by-the-european-society-for-paediatric-gastroenterology-hepatology-and-nutrition-committee
#1
Piotr Socha, Wojciech Janczyk, Anil Dhawan, Ulrich Baumann, Lorenzo D'Antiga, Stuart Tanner, Raffaele Iorio, Pietro Vajro, Roderick Houwen, Björn Fischler, Antal Dezsofi, Nedim Hadzic, Loreto Hierro, Jörg Jahnel, Valérie McLin, Valerio Nobili, Francoise Smets, Henkjan J Verkade, Dominique Debray
BACKGROUND: Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, while neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24h-urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism and molecular analysis of mutations in the ATP7B gene...
October 26, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29341950/clinical-characteristics-of-ulceroglandular-tularemia-in-two-bulgarian-regions-2014-2015-a-report-of-five-cases
#2
Liliya M Pekova, Magdalena P Baymakova, Pavlina O Parousheva, Mariya D Fartunova, Nikolay E Dimitrov, Iskra A Tomova
We present here the first five human cases with tularemia from two regions in South Bulgaria in which there had been no previous report of the infection. The cases occurred over a period of 8 months (December 2014 - August 2015). They were treated at the Department of Infectious Diseases in Stara Zagora University Hospital, Bulgaria. We present the clinical, epidemiological and laboratory data for four men and one woman (age range 52 to 73 years). Three men were hunters, four patients took part in handling, preparing/skinning and cooking the game animals...
December 20, 2017: Folia Medica
https://www.readbyqxmd.com/read/29341929/the-infection-of-red-seabream-iridovirus-in-mandarin-fish-siniperca-chuatsi-and-the-host-immune-related-gene-expression-profiles
#3
Xiaodan Liu, Nan Chen, Xiaojian Gao, Yingying Zhang, Xixi Li, Yue Zhang, Xuwen Bing, Hezhong Huang, Xiaojun Zhang
Red Sea bream iridovirus (RSIV) was initially isolated from marine fish, which belongs to Megalocytivirus, Iridoviridae. It can cause great economic losses in fish culture with high morbidity and mortality. In the present study, the pathogenicity and immune response associated with a RSIV genotype megalocytivirus infection were determined in mandarin fish (Siniperca chuatsi). Fish challenged showed typical clinical signs of iridovirus infection, including acute haemorrhages and enlarged visceral organs. Histopathological analysis revealed that extensive necrosis, vacuolization and inflammation were presented in the stomach, spleen, kidney and liver of the diseased fish...
January 13, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29341357/a-multicenter-pilot-survey-to-clarify-the-clinical-features-of-patients-with-acute-on-chronic-liver-failure-in-japan
#4
Nobuaki Nakayama, Hayato Uemura, Yoshihito Uchida, Tomoaki Tomiya, Akio Ido, Kazuaki Inoue, Takuya Genda, Yasuhiro Takikawa, Isao Sakaida, Shuji Terai, Osamu Yokosuka, Masahito Shimizu, Hajime Takikawa, Satoshi Mochida
AIMS: To establish diagnostic criteria for acute-on-chronic liver failure (ACLF) in Japan, a multicenter pilot survey was conducted to examine usefulness of oversea criteria in patients with chronic liver diseases manifesting acute decompensation. METHODS: Patients fulfilling the APASL, EASL or Chinese Medical Association (CMA) criteria for decompensation were enrolled from 8 institutes, and the clinical features were evaluated. RESULTS: Among 112 patients, 109 patients (97...
January 17, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29340282/should-vasoconstrictors-be-considered-in-a-cirrhotic-patient-with-acute-non-variceal-upper-gastrointestinal-bleeding
#5
Xingshun Qi, Hongyu Li, Xiaodong Shao, Zhendong Liang, Xia Zhang, Ji Feng, Hao Lin, Xiaozhong Guo
Varices manifest as a major etiology of upper gastrointestinal bleeding in patients with chronic liver diseases, such as liver cirrhosis and hepatocellular carcinoma. By contrast, non-variceal upper gastrointestinal bleeding is rare. Pharmacological treatment differs between patients with variceal and non-variceal bleeding. Vasoconstrictors are recommended for the treatment of variceal bleeding, rather than non-variceal bleeding. In contrast, pump proton inhibitors are recommended for the treatment of non-variceal bleeding, rather than variceal bleeding...
December 2017: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/29340156/hepatotoxicity-in-hyperthyroid-patient-after-consecutive-methimazole-and-propylthiouracil-therapies
#6
Fernando Gomez-Peralta, Pablo Velasco-Martínez, Cristina Abreu, María Cepeda, Marta Fernández-Puente
Methimazole (MMI) and propylthiouracil (PTU) are widely used antithyroid drugs (ATD) that have been approved for the treatment of hyperthyroidism. Hepatotoxicity may be induced by these drugs, though they exert dissimilar incidence rates of hepatotoxicity and, possibly, with different underlying pathogenic mechanisms. We report the case of a 55-year-old woman with no relevant medical history diagnosed with hyperthyroidism due to Graves' disease, who developed two episodes of acute hepatitis concurrent with the consecutive administration of two different ATDs, first MMI and then PTU...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29339102/spirulina-ameliorates-methotrexate-hepatotoxicity-via-antioxidant-immune-stimulation-and-proinflammatory-cytokines-and-apoptotic-proteins-modulation
#7
Asmaa F Khafaga, Yasser S El-Sayed
AIMS: Methotrexate (MTX) is an efficient cytotoxic drug used against various carcinogenic, inflammatory and autoimmune diseases; however, the hepatotoxicity of MTX limits its use. Therefore, the present study aimed to evaluate the potential hepatoprotective and immune-stimulant effect of Spirulina platensis (SP) against MTX acute toxicity. MAIN METHODS: Thirty-two male Wistar rats were randomly allocated into the following four groups (n = 8): control, MTX (20 mg/kg bwt, single ip injection), SP (500 mg/kg bwt, oral gavage daily for 21 days), and MTX+SP...
January 12, 2018: Life Sciences
https://www.readbyqxmd.com/read/29337905/the-role-of-mirnas-in-the-pathophysiology-of-liver-diseases-and-toxicity
#8
REVIEW
Florian Schueller, Sanchari Roy, Mihael Vucur, Christian Trautwein, Tom Luedde, Christoph Roderburg
Both acute and chronic liver toxicity represents a major global health burden and an important cause of morbidity and lethality worldwide. Despite epochal progress in the treatment of hepatitis C virus infections, pharmacological treatment strategies for most liver diseases are still limited and new targets for prevention or treatment of liver disease are urgently needed. MicroRNAs (miRNAs) represent a new class of highly conserved small non-coding RNAs that are involved in the regulation of gene expression by targeting whole networks of so called "targets"...
January 16, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29337025/hepatosplenic-t-cell-lymphoma-a-review-of-clinicopathologic-features-pathogenesis-and-prognostic-factors
#9
Mariko Yabe, Roberto N Miranda, L Jeffrey Medeiros
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and clinically aggressive type of T-cell lymphoma that arises most often in adolescents and young adults. Patients with HSTCL commonly present with B-symptoms and cytopenias which may suggest a diagnosis of acute leukemia initially. Patients present with extranodal disease involving the spleen, liver and bone marrow; lymphadenopathy is usually absent. The lymphoma cells can show a spectrum of cell sizes and are of T-cell lineage, often negative for CD4 and CD8 and positive for T-cell receptor γδ or, less often, αβ...
January 11, 2018: Human Pathology
https://www.readbyqxmd.com/read/29336497/effects-of-treatment-with-maraviroc-a-ccr5-inhibitor-on-a-human-hepatic-stellate-cell-line
#10
N Coppola, A Perna, A Lucariello, S Martini, M Macera, M A Carleo, G Guerra, V Esposito, A De Luca
After an acute liver damage, tissue regeneration repairs lesions with degradation of deposed fibrotic material, while mechanisms of tissue restoration are persistently activated following several repeated injuries, inducing deposition of extracellular matrix. (ECM). Factors responsible for ECM remodeling have been identified in a pathway involving a family of zinc-dependent enzyme matrix metalloproteinases (MMPs), together with tissue inhibitor of metalloproteinases (TIMPs). Recent experimental models suggested a role of CCR5 receptor in the genesis of liver fibrosis...
January 16, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29336434/acg-clinical-guideline-alcoholic-liver-disease
#11
Ashwani K Singal, Ramon Bataller, Joseph Ahn, Patrick S Kamath, Vijay H Shah
Alcoholic liver disease (ALD) comprises a clinical-histologic spectrum including fatty liver, alcoholic hepatitis (AH), and cirrhosis with its complications. Most patients are diagnosed at advanced stages and data on the prevalence and profile of patients with early disease are limited. Diagnosis of ALD requires documentation of chronic heavy alcohol use and exclusion of other causes of liver disease. Prolonged abstinence is the most effective strategy to prevent disease progression. AH presents with rapid onset or worsening of jaundice, and in severe cases may transition to acute on chronic liver failure when the risk for mortality, depending on the number of extra-hepatic organ failures, may be as high as 20-50% at 1 month...
January 16, 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29336092/development-and-validation-of-a-scoring-system-to-predict-progression-to-acute-on-chronic-liver-failure-in-patients-with-acute-exacerbation-of-chronic-hepatitis-b
#12
Yi Ren, Lulu Liu, Ying Li, Fangwan Yang, Yihuai He, Yanping Zhu, Xinxin Hu, Shide Lin
AIM: The aim of this study was to develop and validate a scoring system to predict the progression to acute-on-chronic liver failure (ACLF) in patients with acute exacerbation (AE) of chronic hepatitis B (CHB). METHODS: The baseline characteristics of 474 patients with AE of CHB were retrospectively reviewed; 280 and 194 patients were randomly assigned to the derivation and validation cohorts, respectively. Univariate risk factors associated with ACLF development were entered into a multivariate logistic regression...
January 15, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29334786/acute-on-chronic-liver-failure-in-chronic-hepatitis-b-an-update
#13
Rui-Hong Zhao, Yu Shi, Hong Zhao, Wei Wu, Ji-Fang Sheng
Acute-on-chronic liver failure is a common pattern of end-stage liver disease in clinical practice and occurs frequently in patients with chronic hepatitis B or HBV-related cirrhosis. New progress in recent years leads to a better understanding of this disease. Areas covered: This review updates the current comprehensive knowledge about HBV-ACLF from epidemiological studies, experimental studies, and clinical studies and provide new insights into the definition, diagnostic criteria, epidemiology, nature history, pathogenesis, treatment and prognostication of HBV-ACLF...
January 16, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29334663/acute-toxicity-screening-of-different-extractions-components-and-constituents-of-polygonum-multiflorum-thunb-on-zebrafish-danio-rerio-embryos-in-vivo
#14
Jian-Bo Yang, Wan-Fang Li, Yue Liu, Qi Wang, Xian-Long Cheng, Feng Wei, Ai-Guo Wang, Hong-Tao Jin, Shuang-Cheng Ma
Polygonum multiflorum Thunb. has been used widely in East Asia in treatment of diseases associated with aging. However, there are many reports referred to the toxicity of P. multiflorum, especially for liver adverse reactions. The toxicity of it is caused by over dosage or by the herb itself remains unclear. The aim of this study was to study the toxicity of different extractions, components and constituents of P. multiflorum, which were assessed in zebrafish embryos. Firstly, the difference of extracting solvent to the toxicity of P...
January 12, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29333079/type-1-autoimmune-hepatitis-presenting-with-severe-autoimmune-neutropenia
#15
Petros Doumtsis, Theodora Oikonomou, Ioannis Goulis, Kaliopi Zachou, George Dalekos, Evangelos Cholongitas
Autoimmune hepatitis (AIH) is a progressive, chronic liver disease characterized by unresolving hepatocellular inflammation of autoimmune origin. The clinical spectrum may vary from asymptomatic presentation, to non-specific symptoms such as fatigue, arthralgias, nausea and abdominal pain, to acute severe liver disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration on histological examination, hypergammaglobulinemia, and positive autoantibodies. AIH is associated with other autoimmune diseases and its course is often accompanied by various non-specific hematological disorders...
January 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/29333071/factors-associated-with-waiting-time-on-the-liver-transplant-list-an-analysis-of-the-united-network-for-organ-sharing-unos-database
#16
Judy A Trieu, Mohammad Bilal, Bashar Hmoud
Background: Liver transplantation (LT) is an important treatment for acute liver failure and end-stage liver disease. In 2002, the model for end-stage liver disease (MELD) score was incorporated to prioritize patients awaiting LT. Although there is data on how the MELD score affects waiting times, there is a paucity of literature regarding other components. We aimed to evaluate the factors affecting LT waiting times in the United States. Methods: Using the United Network for Organ Sharing (UNOS) database, patients aged 12-75 years listed for LT over the years 2002-2015 were included...
January 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/29331477/atypical-presentation-of-pregnancy-related-hemolytic-uremic-syndrome
#17
Salim Baghli, Catherine Abendroth, Umar Farooq, Jennifer A Schaub
The cause of acute kidney injury during pregnancy and in the postpartum period can be particularly challenging to diagnose, especially when it is necessary to differentiate among preeclampsia; eclampsia; hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome; and thrombotic microangiopathies (TMAs). All these disease entities can present with kidney failure, microangiopathic hemolytic anemia, and thrombocytopenia. We present a teaching case of atypical hemolytic uremic syndrome in the postpartum period in a young woman who was found to have mutations of uncertain clinical significance in the complement cascade, including in C3, CFH, and CFI...
January 10, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29331340/hsp72-protects-from-liver-injury-via-attenuation-of-hepatocellular-death-oxidative-stress-and-jnk-signaling
#18
Kateryna Levada, Nurdan Guldiken, Xiaoji Zhang, Giovanna Vella, Fa-Rong Mo, Laura P James, Johannes Haybaeck, Sonja M Kessler, Alexandra K Kiemer, Thomas Ott, Daniel Hartmann, Norbert Hüser, Marianne Ziol, Christian Trautwein, Pavel Strnad
BACKGROUND AND AIMS: Heat shock protein (Hsp) 72 is a molecular chaperone that is upregulated in response to stress and possesses broad cytoprotective functions. To determine its hepatic function, we studied its expression in human liver disorders and its biological significance in newly generated transgenic animals. AIM AND METHODS: Double transgenic mice overexpressing Hsp72 (gene HSPA1A) under the control of a tissue-specific tetracycline-inducible system (Hsp72-LAP mice) were produced...
January 10, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29331278/impact-of-treatment-with-a-protein-tyrosine-kinase-inhibitor-genistein-on-acute-and-chronic-experimental-schistosoma-mansoni-infection
#19
Maysa Mohamed Kamel Sobhy, Soheir Sayed Mahmoud, Shaimaa Helmy El-Sayed, Enas Mohamed Ali Rizk, Amira Raafat, Mohamed Sherif Ismail Negm
Schistosomiasis mansoni is considered one of the most common fibrotic diseases resulting from inflammation and deposition of fibrous tissue around parasitic eggs trapped in the liver, causing morbidity and mortality. Chemotherapy against schistosomiasis is largely dependent on Praziquantel (PZQ). Yet, the huge administration of it in endemic areas and its incompetence towards the immature stages have raised serious alarms against the development of drug resistance. Few drugs are directed to reverse schistosomal liver fibrosis, particularly at the chronic and advanced stages of the disease...
January 10, 2018: Experimental Parasitology
https://www.readbyqxmd.com/read/29329599/gallbladder-ascariasis-in-kosovo-focus-on-ultrasound-and-conservative-therapy-a-case-series
#20
Vlora Ismaili-Jaha, Halim Toro, Lidvana Spahiu, Mehmedali Azemi, Teuta Hoxha-Kamberi, Muharrem Avdiu, Shqipe Spahiu-Konjusha, Luan Jaha
BACKGROUND: Ascaris lumbricoides is one of the most common intestinal infections in developing countries, including Kosovo. In contrast to migration to the bile duct, migration of the worm to the gallbladder, due to the narrow and tortuous nature of the cystic duct, is rare. When it does occur, it incites acalculous cholecystitis. CASE PRESENTATIONS: This case series describes a 16-month-old Albanian girl, a 22-month-old Albanian girl, a 4-year-old Albanian girl, and a 10-year-old Albanian boy...
January 13, 2018: Journal of Medical Case Reports
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