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https://www.readbyqxmd.com/read/28433114/hepatic-features-of-wilson-disease
#1
Salih Boga, Aftab Ala, Michael L Schilsky
In Wilson disease (WD) defective AT7B function leads to biliary copper excretion and pathologic copper accumulation, particularly in liver and brain, where it induces cellular damage. Liver disease most often precedes neurologic or psychiatric manifestations. In most patients with neurologic or psychiatric symptoms there is some degree of liver disease at the time of disease presentation. Hepatic manifestations of WD can be extremely variable. Patients with clinically asymptomatic WD are often found by family screening or identified on routine laboratory testing...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433112/wilson-disease-liver-pathology
#2
Maciej Pronicki
The liver in Wilson disease may demonstrate a wide range of damage patterns. Some patients may present almost no detectable microscopic pathology, while others display lesions consistent with fulminant hepatitis or acute liver failure. Most liver biopsy specimens show moderate to severe steatosis, variable degree of portal and/or lobular inflammation, and fibrosis eventually progressing to cirrhosis. Additional findings include liver cell degeneration and ballooning, Mallory hyaline bodies, liver cell necrosis, and glycogenation of periportal hepatocytic nuclei...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28433109/pathogenesis-of-wilson-disease
#3
Ivo Florin Scheiber, Radan Brůha, Petr Dušek
Wilson disease is an autosomal-recessive disorder originating from a genetic defect in the copper-transporting ATPase ATP7B that is required for biliary copper secretion and loading of ceruloplasmin with copper. Impaired ATP7B function in Wilson disease results in excessive accumulation of copper in liver, brain, and other tissues. Toxic copper deposits may induce oxidative stress, modify expression of genes, directly inhibit proteins, and impair mitochondrial function, leading to hepatic, neuropsychiatric, renal, musculoskeletal, and other symptoms...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28431795/shear-wave-liver-elastography-with-a-propagation-map-diagnostic-performance-and-inter-observer-correlation-for-hepatic-fibrosis-in-chronic-hepatitis
#4
Eun Sun Lee, Jong Beum Lee, Hwi Ryong Park, Jeongin Yoo, Ji In Choi, Hyun Woong Lee, Hyung Joon Kim, Byung Ihn Choi, Hyun Jeong Park, Sung Bin Park
The aim of this study was to determine the performance of shear wave elastography (SWE) with a propagation map in the diagnosis of hepatic fibrosis, and to assess its reliability with transient elastography (TE) as the reference standard. Our prospective study included 115 consecutive patients with suspected or alleged chronic hepatitis. Patients underwent SWE by two different operators and TE by sonographers on the same day. The correlation coefficient of the intra-class correlation test between an experienced radiologist and a third-year radiology resident was 0...
April 18, 2017: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/28428712/notch-signaling-mediated-by-tgf-%C3%AE-smad-pathway-in-concanavalin-a-induced-liver-fibrosis-in-rats
#5
Yi Wang, Ruo-Wu Shen, Bing Han, Zhen Li, Le Xiong, Feng-Yu Zhang, Bei-Bei Cong, Bei Zhang
AIM: To explore the exact interaction between Notch and transforming growth factor (TGF)-β signaling in liver fibrosis. METHODS: We established a rat model of liver fibrosis induced by concanavalin A. Peripheral blood mononuclear cells (PBMCs) were isolated from the modeled rats, and cultured with γ-secretase inhibitor DAPT and TGF-β inhibitor for 24 h. The mRNA levels of Notch and TGF-β signaling were detected by quantitative real-time polymerase chain reaction...
April 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28428284/acute-presentation-of-autoimmune-hepatitis-a-multicentre-study-with-detailed-histological-evaluation-in-a-large-cohort-of-patients
#6
Hiep Nguyen Canh, Kenichi Harada, Hirofumi Ouchi, Yasunori Sato, Koichi Tsuneyama, Masayoshi Kage, Masayuki Nakano, Kaname Yoshizawa, Atsushi Takahashi, Masanori Abe, Jong-Hon Kang, Kazuhiko Koike, Ayano Inui, Tomoo Fujisawa, Akinobu Takaki, Teruko Arinaga-Hino, Takuji Torimura, Yoshiyuki Suzuki, Keiichi Fujiwara, Mikio Zeniya, Hiromasa Ohira, Atsushi Tanaka, Hajime Takikawa
AIMS: Although liver biopsy is crucial to diagnose and guide treatment decisions, a detailed histological analysis of autoimmune hepatitis (AIH) with clinically acute presentations has not yet been performed. This study aimed to characterise the histological features and explore potential histological hallmarks to diagnose the acute presentation of AIH. METHODS: We systematically evaluated liver specimens of 87 adult patients with acute presentation of AIH retrospectively enrolled from Japanese multicentre facilities...
April 20, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28427912/hepataprotective-effects-of-ginsenoside-rg1-a-review
#7
REVIEW
Yan Gao, Shifeng Chu, Zhao Zhang, Naihong Chen
BACKGROUND AND ETHNOPHARMACOLOGICAL RELEVANCE: Ginseng has been used as efficient tonic and for the treatment of various diseases including hepatic disorders. Ginseng saponins, also known as ginsenosides, are principal constituents and have been treated to be responsible for disparate ginseng health benefits. The current review mainly focuses on ginsenoside Rg1, a compound isolated from traditional Chinese herbal medicine Panax ginseng Meyer. AIMS: To summary the hepataprotective effects and related mechanisms of ginsenoside Rg1, we conclude this review by combining the literature and our own researches...
April 17, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28427234/diversity-of-the-association-of-serum-levels-and-genetic-variants-of-mhc-class-i-polypeptide-related-chain-a-with-liver-fibrosis-in-chronic-hepatitis-c
#8
Chung-Feng Huang, Ching-I Huang, Ming-Lun Yeh, Shu-Chi Wang, Kuan-Yu Chen, Yu-Min Ko, Ching-Chih Lin, Yi-Shan Tsai, Pei-Chien Tsai, Zu-Yau Lin, Shinn-Cherng Chen, Chia-Yen Dai, Jee-Fu Huang, Wan-Long Chuang, Ming-Lung Yu
BACKGROUND/AIMS: Genetic variants of MHC class I polypeptide-related chain A (MICA) at rs2596542 have been associated with hepatocellular carcinoma. The linkage between serum MICA (sMICA) and liver fibrosis in chronic hepatitis C is elusive. RESULTS: Linear regression analysis revealed that sMICA were independently correlated to α-fetoprotein (β: 0.149; 95% confidence interval [CI]: 0.001, 0.003; P = 0.007)and MICA rs2596542 GG genotype (β: 0.209; 95% CI: 0.153, 0...
March 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28427123/-predictive-value-of-liver-pathologic-changes-in-determining-the-effectiveness-of-entecavir-treatment-for-chronic-hepatitis-b-an-analysis-of-1-366-cases
#9
M M Sun, W Zhang, G Chen, Y An, Y Q Wang, Q H Shang
Objective: To analyze the relationship between baseline liver pathologic changes and the effectiveness of entecavir(ETV) and investigate the predictive value of baseline liver pathologic changes in determining the effectiveness of ETV, to provide reliable basis for precision medicine in patients with chronic hepatitis B(CHB). Methods: A total of 1 366 cases with CHB were retrospectively recruited who underwent liver biopsy between January 2006 to June 2016 and were treated with ETV over 96 weeks.The relationship between baseline liver pathologic changes and the antiviral responses to ETV at 48, 96 weeks were compared...
April 18, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#10
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
RATIONALE: Hepatic steatosis develops after liver transplant in 30% of adults, and non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in non-transplanted children. However, post-transplant steatosis has been minimally studied in pediatric liver transplant recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. RESULTS: In this single-center study of pediatric patients transplanted 1988-2015 (n=318), 31% of those with any post-transplant biopsy (n=271) had ≥1 biopsy with steatosis...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28425454/association-of-serum-autotaxin-levels-with-liver-fibrosis-in-patients-with-chronic-hepatitis-c
#11
Tomoo Yamazaki, Satoru Joshita, Takeji Umemura, Yoko Usami, Ayumi Sugiura, Naoyuki Fujimori, Soichiro Shibata, Yuki Ichikawa, Michiharu Komatsu, Akihiro Matsumoto, Koji Igarashi, Eiji Tanaka
Metabolized by liver sinusoidal endothelial cells, autotaxin (ATX) is a secreted enzyme considered to be associated with liver damage. We sought to clarify the diagnostic ability of ATX for liver fibrosis in 593 biopsy-confirmed hepatitis C virus (HCV)-infected patients. The diagnostic accuracy of ATX was compared with clinical parameters and the established fibrosis biomarkers Wisteria floribunda agglutinin-positive Mac-2-binding protein, FIB-4 index, AST-to-platelet ratio, and Forn's index. Median ATX levels were consistently higher in female controls and patients than in their male counterparts (P < 0...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28425419/a-rare-bsep-mutation-associated-with-a-mild-form-of-progressive-familial-intrahepatic-cholestasis-type-2
#12
Orith Waisbourd-Zinman, Lea F Surrey, Anna E Schwartz, Pierre A Russo, Jessica Wen
Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425416/gnpat-rs11558492-is-not-a-major-modifier-of-iron-status-study-of-italian-hemochromatosis-patients-and-blood-donors
#13
Federico Greni, Luca Valenti, Raffaella Mariani, Irene Pelloni, Raffaela Rametta, Fabiana Busti, Giulia Ravasi, Domenico Girelli, Silvia Fargion, Stefania Galimberti, Alberto Piperno, Sara Pelucchi
BACKGROUND AND AIM: HFE-related Hemochromatosis (HH) is characterized by marked phenotype heterogeneity, probably due to the combined action of acquired and genetic factors. Among them, GNPAT rs11558492 was proposed as genetic modifier of iron status, but results are still controversial. To shed light on these discrepancies, we genotyped 298 Italian p.C282Y homozygotes and 169 healthy controls. MATERIAL AND METHODS: Allele and genotype frequencies were analysed and compared with those reported in Exome Variant Server (EVS)...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425404/transient-elastography-vs-aspartate-aminotransferase-to-platelet-ratio-index-in-hepatitis-c-a-meta-analysis
#14
A Z Mattos, A A Mattos
BACKGROUND AND RATIONALE: Many different non-invasive methods have been studied with the purpose of staging liver fibrosis. The objective of this study was verifying if transient elastography is superior to aspartate aminotransferase to platelet ratio index for staging fibrosis in patients with chronic hepatitis C. MATERIAL AND METHODS: A systematic review with meta-analysis of studies which evaluated both non-invasive tests and used biopsy as the reference standard was performed...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425401/mre-in-nafld-promising-but-further-validation-is-required
#15
John D Ryan, Emmanuel A Tsochatzis
MR elastography is a novel method for non-invasive fibrosis assessment, not yet sufficiently validated. In a recent study in 104 patients Park, Gastroenterology 2017; 152: 598-602), MRE was compared to transient elastography for the diagnosis of fibrosis is nonalcoholic fatty liver disease. The current viewpoint critically appraises this study.
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425016/a-simple-bedside-blood-test-fibrofast-fib-5-is-superior-to-fib-4-index-for-the-differentiation-between-non-significant-and-significant-fibrosis-in-patients-with-chronic-hepatitis-c
#16
G Shiha, S Seif, A Eldesoky, M Elbasiony, R Soliman, A Metwally, K Zalata, N Mikhail
INTRODUCTION: A simple non-invasive score (Fibrofast, FIB-5) was developed using five routine laboratory tests (ALT, AST, alkaline phosphatase, albumin and platelets count) for the detection of significant hepatic fibrosis in patients with chronic hepatitis C. The FIB-4 index is a non-invasive test for the assessment of liver fibrosis, and a score of ≤1.45 enables the correct identification of patients who have non-significant (F0-1) from significant fibrosis (F2-4), and could avoid liver biopsy...
April 19, 2017: Hepatology International
https://www.readbyqxmd.com/read/28424480/ketogenic-diet-attenuates-hepatopathy-in-mouse-model-of-respiratory-chain-complex-iii-deficiency-caused-by-a-bcs1l-mutation
#17
Janne Purhonen, Jayasimman Rajendran, Matthias Mörgelin, Kristiina Uusi-Rauva, Shintaro Katayama, Kaarel Krjutskov, Elisabet Einarsdottir, Vidya Velagapudi, Juha Kere, Matti Jauhiainen, Vineta Fellman, Jukka Kallijärvi
Mitochondrial disorders are among the most prevalent inborn errors of metabolism but largely lack treatments and have poor outcomes. High-fat, low-carbohydrate ketogenic diets (KDs) have shown beneficial effects in mouse models of mitochondrial myopathies, with induction of mitochondrial biogenesis as the suggested main mechanism. We fed KD to mice with respiratory chain complex III (CIII) deficiency and progressive hepatopathy due to mutated BCS1L, a CIII assembly factor. The mutant mice became persistently ketotic and tolerated the KD for up to 11 weeks...
April 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28424263/the-kielin-chordin-like-protein-kcp-attenuates-high-fat-diet-induced-obesity-and-metabolic-syndrome-in-mice
#18
Abdul Soofi, Katherine I Wolf, Margo P Emont, Nathan Qi, Gabriel Martinez-Santibanez, Edward Grimley, Wesam Ostwani, Gregory R Dressler
Obesity and its associated complications, such as insulin resistance and non-alcoholic fatty liver disease, are reaching epidemic proportions. In mice, the TGF-beta superfamily is implicated in the regulation of white and brown adipose tissue differentiation. The Kielin/chordin-like Protein (KCP) is a secreted regulator of the TGF-beta superfamily pathways that can inhibit both TGF-beta and activin signals while enhancing bone morphogenetic protein (BMP) signaling. However, KCP's effects on metabolism and obesity have not been studied in animal models...
April 19, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28423499/imidazoline-i2-receptor-inhibitor-idazoxan-regulates-the-progression-of-hepatic-fibrosis-via-akt-nrf2-smad2-3-signaling-pathway
#19
Li Xuanfei, Chen Hao, Yi Zhujun, Liu Yanming, Gong Jianping
Liver fibrosis is a global health problem and its relationship with imidazoline I2 receptor has not been reported. This study aimed to investigate the effects and underlying mechanisms of imidazoline I2 receptor (I2R) inhibitor idazoxan (IDA) on carbon tetrachloride (CCl4)-induced liver fibrosis. In vivo liver fibrosis in mice was induced by intraperitoneally injections of CCl4 for eight weeks, and in vitro studies were performed on activated LX2 cells treated with transforming growth factor-β (TGF-β). Our results showed that IDA significantly improved liver inflammation, ameliorated hepatic stellate cells activation and reduced collagen accumulation by suppressing the pro-fibrogenic signaling of TGF-β/Smad...
March 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423468/cytoprotective-effects-of-dietary-flavonoids-against-cadmium-induced-toxicity
#20
REVIEW
Xia Li, Xinwei Jiang, Jianxia Sun, Cuijuan Zhu, Xiaoling Li, Lingmin Tian, Liu Liu, Weibin Bai
Cadmium (Cd) damages the liver, kidney, bones, reproductive system, and other organs. Flavonoids, such as anthocyanins and flavonols, which are commonly found in plant foods, have shown protective effects against Cd-induced damage. The cytoprotective effects of flavonoids against Cd-induced diseases are mainly attributable to three mechanisms. First, flavonoids clear reactive oxygen species, thereby reducing lipid peroxide production and improving the activity of antioxidation enzymes. Second, flavonoids chelate Cd, thus reducing the accumulation of Cd and altering the levels of other essential metal ions in vivo...
April 19, 2017: Annals of the New York Academy of Sciences
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