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alpha-1 liver disease

Omesh Goyal, Sandeep Singh Sidhu, Natasha Sehgal, Sandeep Puri
OBJECTIVES: Hepatorenal syndrome (HRS) is a functional renal failure occurring in end stage liver disease, which is associated with poor prognosis. Terlipressin has been shown to be effective in treatment of HRS. More recently, it was suggested that noradrenaline, an alpha-adrenergic drug may be also effective in HRS. We aimed to compare the efficacy of noradrenaline versus terlipressin in treatment of HRS type 1. METHODS: Consecutive patients with cirrhosis and HRS type 1 were enrolled and randomised into 2 groups- Group A received intravenous noradrenaline infusion (0...
September 2016: Journal of the Association of Physicians of India
Yong Xie, Yi Zhang, Zebin Guo, Hongliang Zeng, Baodong Zheng
This study aimed to investigate the effects of total alkaloids from Nelumbinis Plumula (NPA) on insulin resistance (IR) of high-fat diet- (HFD-) induced nonalcoholic fatty liver disease (NAFLD). Rats were fed with HFD for 8 weeks to induce NAFLD. Then, the effect of NPA on ameliorating IR in HFD-induced NAFLD was evaluated. Fasting serum insulin was determined using an enzyme-linked immunosorbent assay (ELISA) kit for insulin following the manufacturer's protocol. Some inflammatory cytokines such as tumor necrosis factor alpha (TNF-α) and interleukin-6 (IL-6) were determined using ELISA kits to assess the inflammatory burden in rats...
2016: Journal of Diabetes Research
Hyelin Na, Ho Lee, Min-Ho Lee, Han Jeong Lim, Hyeon-Ji Kim, Yoon Jeon, Hae-Lim Kang, Mi-Ock Lee
AIMS: To elucidate the role of nuclear receptor subfamily 1, group D, member 1 (Nr1d1) in hepatic lipid metabolism and pathogenesis of nonalcoholic fatty liver diseases, Nr1d1 gene mutant mice, in which the DNA-binding domain (exons 3 and 4) was deleted (Nr1d1 Δex3/4), were challenged with a high-fat diet (HFD), and the gene expression patterns that responded to this alteration were profiled. MAIN METHODS: The Nr1d1 Δex3/4 mice were fed an HFD for 12weeks. Liver tissues were examined by histology, and lipid droplets were detected by Oil-Red O staining...
October 5, 2016: Life Sciences
Jangsun Hwang, Youngmin Seo, Yeonho Jo, Jaewoo Son, Jonghoon Choi
C-reactive protein (CRP) is a pentameric protein that is present in the bloodstream during inflammatory events, e.g., liver failure, leukemia, and/or bacterial infection. The level of CRP indicates the progress and prognosis of certain diseases; it is therefore necessary to measure CRP levels in the blood accurately. The normal concentration of CRP is reported to be 1-3 mg/L. Inflammatory events increase the level of CRP by up to 500 times; accordingly, CRP is a biomarker of acute inflammatory disease. In this study, we demonstrated the preparation of DNA aptamer-conjugated peripheral blood mononuclear cells (Apt-PBMCs) that specifically capture human CRP...
October 6, 2016: Scientific Reports
Laura Lucarini, Mariaconcetta Durante, Cecilia Lanzi, Alessandro Pini, Giulia Boccalini, Laura Calosi, Flavio Moroni, Emanuela Masini, Guido Mannaioni
Idiopathic pulmonary fibrosis is a severe disease characterized by excessive myofibroblast proliferation, extracellular matrix and fibrils deposition, remodelling of lung parenchyma and pulmonary insufficiency. Drugs able to reduce disease progression are available, but therapeutic results are unsatisfactory; new and safe treatments are urgently needed. Poly(ADP-ribose) polymerases-1 (PARP-1) is an abundant nuclear enzyme involved in key biological processes: DNA repair, gene expression control, and cell survival or death...
October 4, 2016: Journal of Cellular and Molecular Medicine
Nikolas Bernhard, Robert Bals, Sebastian Fähndrich
Alpha-1-antitrypsin deficiency is a genetic risk factor for the development of chronic obstructive airway disease (COPD) and liver cirrhosis. The disease is widely underdiagnosed. The hallmarks of therapy are smoking cessation, prevention from environmental dust exposure and augmentation therapy. Findings from the recently published prospective, placebo-controlled and randomized RAPID trial proved effectiveness of AAT augmentation therapy for slowing progression of emphysema, measured by CT lung density. CT lung density may be more sensitive than forced exspiratory volume in one second (FEV1) or monoxid diffusion capacity (DLCO)...
September 2016: Deutsche Medizinische Wochenschrift
Noha G Bader El Din, Sally Farouk, Reem El-Shenawy, Marwa K Ibrahim, Reham M Dawood, Mostafa M Elhady, Ahmed M Salem, Naglaa Zayed, Ahmed Khairy, Mostafa K El Awady
AIM: To investigate the association of tumor necrosis factor alpha (TNFα) -G308A polymorphism with different liver pathological changes in treatment-naïve Egyptian patients infected with hepatitis C virus (HCV) genotype 4. METHODS: This study included 180 subjects, composed of 120 treatment-naïve chronic HCV patients with different fibrosis grades (F0-F4) and 60 healthy controls. The TNFα -G308A region was amplified by PCR and the different genotypes were detected by restriction fragment length polymorphism analysis...
September 14, 2016: World Journal of Gastroenterology: WJG
Ming-Wei Liu, Rong Liu, Hai-Yin Wu, Wei Zhang, Jing Xia, Min-Na Dong, Wen Yu, Qiang Wang, Feng-Mei Xie, Rui Wang, Yun-Qiao Huang, Chuan-Yun Qian
Xuebijing injection (XBJ) has long been used to treat infectious diseases in China. The therapeutic effect of XBJ is probably associated with anti-inflammatory effects. However, the precise mechanisms responsible for the effects of XBJ remain unknown. The present study was conducted in order to evaluate the protective effects of XBJ in a rat model of D-galactosamine (D-Gal)- and lipopolysaccharide (LPS)‑induced acute liver injury. In the present study, the rats were injected with D-Gal and LPS intraperitoneally to induce acute liver injury...
September 23, 2016: International Journal of Molecular Medicine
Ki Tae Suk, Eun Jin Kim, Dong Joon Kim, Hyo Sun Kim, Chang Seok Bang, Tae Young Park, Gwang Ho Baik, Sung Eun Kim, Ji Won Park, Sang Hoon Park, Myung Seok Lee, Hyoung Su Kim, Myoung Kuk Jang, Seung Ha Park, Eunhee Choi, Chang H Kim, Hotaik Sung, Choong Kee Park
BACKGROUND & GOALS: Early identification of hepatocellular carcinoma (HCC) is associated with improved survival for patients with chronic liver disease (CLD). We evaluated the prognostic significance of hemodynamic stage (HS) and clinical stage (CS) in predicting HCC in CLD patients. METHODS: Between January 2006 and May 2014, 801 patients with CLD who underwent hepatic venous pressure gradient (HVPG) measurement were prospectively enrolled. HS was classified by HVPG (mm Hg) as follows: HS-1 (HVPG≤6), HS-2 (6<HVPG≤10), HS-3 (10<HVPG≤12), HS-4 (12<HVPG≤20), and HS-5 (20<HVPG)...
September 22, 2016: Journal of Clinical Gastroenterology
Aurèle Besse-Patin, Mélissa Léveillé, Daniel Oropeza, Bich N Nguyen, Annick Prat, Jennifer L Estall
BACKGROUND & AIMS: Inefficient fatty acid oxidation in mitochondria and increased oxidative damage are features of non-alcoholic fatty liver disease (NAFLD). In rodent models and patients with NAFLD, hepatic expression of PPARG coactivator 1 alpha (PPARGC1A or PGC1A) is inversely correlated with liver fat and disease severity. A common polymorphism in this gene (rs8192678, encoding Gly482Ser) has been associated with NAFLD. We investigated whether reduced expression of PGC1A contributes to development of NAFLD using mouse models, primary hepatocytes and human cell lines...
September 19, 2016: Gastroenterology
Tung-Hung Su, Tsung-Hui Hu, Chi-Yi Chen, Yi-Hsiang Huang, Wan-Long Chuang, Chun-Che Lin, Chia-Chi Wang, Wei-Wen Su, Ming-Yao Chen, Cheng-Yuan Peng, Rong-Nan Chien, Yi-Wen Huang, Horng-Yuan Wang, Chih-Lin Lin, Sheng-Shun Yang, Tsung-Ming Chen, Lein-Ray Mo, Shih-Jer Hsu, Kuo-Chih Tseng, Tsai-Yuan Hsieh, Fat-Moon Suk, Chi-Tan Hu, Ming-Jong Bair, Cheng-Chao Liang, Yung-Chao Lei, Tai-Chung Tseng, Chi-Ling Chen, Jia-Horng Kao
BACKGROUND & AIMS: Oral antiviral therapy may reduce the disease progression of chronic hepatitis B (CHB) patients. We aimed to further investigate the efficacy of long-term entecavir therapy in reduction of the risk of hepatocellular carcinoma (HCC), cirrhotic events and mortality in a large group of CHB-related cirrhosis patients. METHODS: The C-TEAM (Cirrhosis-Taiwanese EntecAvir Multicenter) study was a nationwide, multicenter, retrospective-prospective cohort study in Taiwan...
September 16, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Gurmit Gill, Hammad Bajwa, Peter Strouhal, Harit N Buch
BACKGROUND: Interferon alpha is a widely used therapeutic agent in the treatment of hepatitis C virus infection. Clinical thyroid disease is seen in nearly 15 % of patients receiving interferon alpha for hepatitis C virus infection. The mechanism of thyroid dysfunction with interferon alpha is either autoimmune or inflammatory. We report a case of young woman who developed biphasic thyroid dysfunction posing a diagnostic challenge, while receiving interferon alpha treatment for hepatitis C virus infection...
2016: Journal of Medical Case Reports
Napat Angkathunyakul, Francesca Rosini, Nigel Heaton, Pierre Foskett, Alberto Quaglia
AIMS: Both homo- and heterozygous AAT deficiency patients are risk of hepatocellular carcinoma (HCC) but also of cholangiocarcinoma and combined hepatocellular-cholangiocarcinoma. We report a series of bile duct adenoma (BDA) and intrahepatic cholangiocarcinoma (ICC), in AAT deficiency adult patients, observed in our institution over a five year period. Our observational study includes a detailed investigation of their immunohistochemical profile and BRAF V600E mutation status. METHODS AND RESULTS: Eleven biliary lesions from 5 AAT deficiency patients (6 BDAs from 3 cirrhotic patients with concurrent other liver diseases, 3 BDAs and 2 ICCs from 2 non-cirrhotic patients) were identified between 2010 and 2015 during routine histology...
September 14, 2016: Histopathology
J Stollenwerk, M Schepke, E Biecker
BACKGROUND: Altered liver function tests are a common finding in clinical practice. Our retrospective study aimed to identify the diagnoses in a non-selected cohort of patients with altered liver tests and to investigate whether alpha-1 antitrypsin genotyping should be part of the diagnostic workup. PATIENTS AND METHODS: 501 patients who were admitted to our outpatient clinic for further evaluation of altered liver function tests were included in the study. The patients underwent a standardized diagnostic program with history taking, physical examination, laboratory tests and ultrasonography...
September 2016: Zeitschrift Für Gastroenterologie
M Lu, J Bai, B Xu, Q Y Sun, F X Wei, X F Tang, H F Zhang, J Li, G L Wang, Q Q Yin, S Y Li
Ammonia in poultry houses not only affects worker health but also induces a variety of poultry diseases. Alpha-lipoic acid (LA) is an effective antioxidant that protects cells against oxidative injury during various toxic and pathological processes. This study was designed to evaluate the mitigating effects of LA supplementation on ammonia stress and hepatic proteome changes in broilers. Male broilers (22 d old) were allocated to 3 groups: (1) a control group without ammonia stress (CTRL); (2) exposure to 70 ppm ammonia (AM); and (3) exposure to 70 ppm ammonia and dietary administration of 300 mg/kg LA (AM+LA)...
September 2, 2016: Poultry Science
Luz M Medrano, Norma Rallón, Juan Berenguer, María A Jiménez-Sousa, Vicente Soriano, Teresa Aldámiz-Echevarria, Amanda Fernández-Rodríguez, Marcial García, Francisco Tejerina, Isidoro Martínez, José M Benito, Salvador Resino
BACKGROUND AND AIMS: TRIM5 and TRIM22 are restriction factors involved in innate immune response and exhibit anti-viral activity. Single nucleotide polymorphisms (SNPs) at TRIM5 and TRIM22 genes have shown to influence several viral infections such as human immunodeficiency virus (HIV), hepatitis B, as well as measles and rubella vaccination. The aim of this study is to analyze whether TRIM5 and TRIM22 polymorphisms are associated with liver fibrosis inflammation-related biomarkers and response to pegylated-interferon-alpha plus ribavirin (pegIFNα/RBV) therapy in HIV/hepatitis C virus (HCV) coinfected patients...
2016: Journal of Translational Medicine
Eun Sun Ji, Heeyoun Hwang, Gun Wook Park, Ju Yeon Lee, Hyun Kyoung Lee, Na Young Choi, Hoi Keun Jeong, Kwang Hoe Kim, Jin Young Kim, Seungho Lee, Yeong Hee Ahn, Jong Shin Yoo
Fucosylation of N-glycoproteins has been implicated in various diseases, such as hepatocellular carcinoma (HCC). However, few studies have performed site-specific analysis of fucosylation in liver-secreted proteins. In this study, we characterized the fucosylation patterns of liver-secreted proteins in HCC plasma using a workflow to identify site-specific N-glycoproteins, where characteristic B- and/or Y-ion series with and without fucose in collision-induced dissociation were used in tandem mass spectrometry...
November 2016: Analytical and Bioanalytical Chemistry
Zobair M Younossi, Maria Stepanova, Linda Henry
BACKGROUND: The hepatitis C virus (HCV) infection has tremendous clinical, health-related quality-of-life (HRQOL), and economic burden on patients and the society. To assess the comprehensive impact of HCV infection, systematic tracking of HRQOL in patients with HCV infection is important. OBJECTIVE: The aim of this study was to systematically validate an HCV-specific HRQOL instrument, the Chronic Liver Disease Questionnaire-Hepatitis C Version (CLDQ-HCV), in patients with chronic HCV infection...
July 2016: Value in Health: the Journal of the International Society for Pharmacoeconomics and Outcomes Research
Robert A Sandhaus
Alpha-1 antitrypsin deficiency (AATD) is a condition caused by the inheritance of two mutated SERPINA1 gene alleles. Individuals with AATD are at increased risk of injury to the liver and lungs. The pulmonary manifestations include precocious onset of pulmonary emphysema and bronchiectasis. For nearly three decades, treatment has been available to individuals with emphysema caused by AATD, but this therapy-augmentation of plasma and tissue alpha-1 antitrypsin levels by intravenous administration of human plasma-derived protein-was approved by regulatory authorities based on its biochemical efficacy...
August 2016: Annals of the American Thoracic Society
Cormac McCarthy, Emer P Reeves, Noel G McElvaney
Alpha-1 antitrypsin deficiency (AATD) is characterized by low levels of circulating alpha-1 antitrypsin and an increased risk for emphysema, liver disease, and panniculitis. The reduced levels of alpha-1 antitrypsin in AATD predispose the lung to unopposed proteolytic activity, predominantly from neutrophil-derived proteases, chiefly neutrophil elastase. This leads to emphysema. The mechanisms subtending the liver disease are less well understood, but are probably due to a "gain-of function" inflammatory process in the liver, stoked by intracellular retention of aberrantly folded alpha-1 antitrypsin...
August 2016: Annals of the American Thoracic Society
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