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https://www.readbyqxmd.com/read/28813782/urea-cycle-pathway-targeted-therapeutic-action-of-naringin-against-ammonium-chloride-induced-hyperammonemic-rats
#1
Arumugam Ramakrishnan, Natesan Vijayakumar
Ammonia is a well-known neurotoxin that causes liver disease and urea cycle disorder. Excessive ammonia content in the blood leads to hyperammonemic condition and affects both excitatory and inhibitory neurotransmission including brain edema and coma. Naringin, a plant bioflavonoid present in various citrus fruits and mainly extracted from the grape fruit. This study was designed to assess the protective effect of naringin on ammonium chloride (NH4Cl) induced hyperammonemic rats. Experimental hyperammonemia was induced by intraperitoneal injections (i...
August 12, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28813453/metabolomics-reveals-that-vine-tea-ampelopsis-grossedentata-prevents-high-fat-diet-induced-metabolism-disorder-by-improving-glucose-homeostasis-in-rats
#2
Wenting Wan, Baoping Jiang, Le Sun, Lijia Xu, Peigen Xiao
BACKGROUND: Vine tea (VT), derived from Ampelopsis grossedentata (Hand.-Mazz.) W.T. Wang, is an alternative tea that has been consumed widely in south China for hundreds of years. It has been shown that drinking VT on a daily basis improves hyperlipidemia and hyperglycemia. However, little is known about the preventive functions of VT for metabolic dysregulation and the potential pathological mechanisms involved. This paper elucidates the preventive effects of VT on the dysregulation of lipid and glucose metabolism using rats maintained on a high-fat-diet (HFD) in an attempt to explain the potential mechanisms involved...
2017: PloS One
https://www.readbyqxmd.com/read/28812468/the-first-reported-case-of-meckel-gruber-syndrome-associated-with-abnormal-karyotype-mosaic-trisomy-17
#3
Zuzana Cierna, Pavol Janega, Frantisek Grochal, Vladimir Ferianec, Tatiana Braxatorisova, Lucia Strieskova, Jana Malova, Petra Jungova, Tomas Szemes
Meckel-Gruber syndrome (MKS) is a rare lethal autosomal recessive disorder with typical anomalies including encephalocele, multicystic renal dysplasia, congenital liver fibrosis, and polydactyly. MKS is caused by mutations of genes localized on different chromosomes. Karyotypes of published Meckel-Gruber syndrome cases are without any aberrations. We present a male fetus with meningoencephalocele, multicystic renal dysplasia, congenital liver fibrosis, and other anomalies. Standard cytogenetic examination of cultured fetal skin and muscle fibroblasts showed mosaic trisomy 17...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28811650/diet-induced-obesity-leads-to-metabolic-dysregulation-in-offspring-via-endoplasmic-reticulum-stress-in-a-sex-specific-manner
#4
J H Park, Y Yoo, M Cho, J Lim, A M Lindroth, Y J Park
BACKGROUND/OBJECTIVES: Exposure to metabolic stress has been suggested to influence the susceptibility to metabolic disorders in offspring according to epidemiological and animal studies. Nevertheless, molecular mechanisms remain unclear. We investigated impacts of diet-induced paternal obesity on metabolic phenotypes in offspring and its underlying molecular mechanism. SUBJECTS/METHODS: Male founder mice (F0), fed with control diet (CD) or high fat diet (HFD), were mated with CD-fed females...
August 16, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28811612/coenzyme-q10-improves-lipid-metabolism-and-ameliorates-obesity-by-regulating-camkii-mediated-pde4-inhibition
#5
Zhe Xu, Jia Huo, Xin Ding, Mu Yang, Lin Li, Jian Dai, Kazunori Hosoe, Hiroshi Kubo, Masayuki Mori, Keiichi Higuchi, Jinko Sawashita
Our recent studies revealed that supplementation with the reduced form of coenzyme Q10 (CoQ10H2) inhibits oxidative stress and slows the process of aging in senescence-accelerated mice. CoQ10H2 inhibits adipocyte differentiation and regulates lipid metabolism. In the present study, we show that dietary supplementation with CoQ10H2 significantly reduced white adipose tissue content and improved the function of brown adipose tissue by regulating expression of lipid metabolism-related factors in KKAy mice, a model of obesity and type 2 diabetes...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28811499/the-role-of-dermcidin-isoform-2-in-the-occurrence-and-severity-of-diabetes
#6
Suman Bhattacharya, Md Mobidullah Khan, Chandradipa Ghosh, Sarbashri Bank, Smarajit Maiti
Diabetes is now epidemic worldwide. Several hundred-million peoples are presently suffering from this disease with other secondary-disorders. Stress, hypertension, sedentary life-style, carbohydrate/lipid metabolic-disorders due to genetic or environmental factors attributes to type-1 and/or type-2 diabetes. Present investigation demonstrates that stress-induced protein dermcidin isoform-2 (DCN-2) which appears in the serum of diabetic-patients play a key-role in this disease pathogenesis/severity. DCN-2 suppresses insulin production-release from liver/pancreas...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28811490/mammary-tumors-induce-central-pro-inflammatory-cytokine-expression-but-not-behavioral-deficits-in-balb-c-mice
#7
William H Walker Ii, Jeremy C Borniger, Surbhi, Abigail A Zalenski, Stevie L Muscarella, Julie A Fitzgerald, Ning Zhang, Monica M Gaudier-Diaz, A Courtney DeVries
Breast cancer survivors are more likely to develop mood disorders and cognitive deficits than women in the general population. Previous studies suggest that peripheral tumors elicit central pro-inflammatory cytokine production, in turn leading to depression and cognitive deficits. In the current study, two cohorts of female Balb/C mice received bilateral orthotopic injections of syngeneic 67NR, 4T07, or 4T1cells (1 × 10(5) cells per injection) to induce mammary tumors. Approximately three weeks later, learned fear (via fear conditioning) or depressive-like behavior (via tail suspension and forced swim test) was assessed...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28809727/models-of-non-alcoholic-fatty-liver-disease-and-potential-translational-value-the-effects-of-3-5-l-diiodothyronine
#8
Elena Grasselli, Laura Canesi, Piero Portincasa, Adriana Voci, Laura Vergani, Ilaria Demori
Non-alcoholic fatty liver disease (NAFLD) is the most common liver disorder in industrialized countries and is associated with increased risk of cardiovascular, hepatic and metabolic diseases. Molecular mechanisms on the root of the disrupted lipid homeostasis in NAFLD and potential therapeutic strategies can benefit of in vivo and in vitro experimental models of fatty liver. Here, we describe the high fat diet (HFD)-fed rat in vivo model, and two in vitro models, the primary cultured rat fatty hepatocytes or the FaO rat hepatoma fatty cells, mimicking human NAFLD...
August 8, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28809446/deferasirox-for-managing-iron-overload-in-people-with-thalassaemia
#9
REVIEW
Claudia Bollig, Lisa K Schell, Gerta Rücker, Roman Allert, Edith Motschall, Charlotte M Niemeyer, Dirk Bassler, Joerg J Meerpohl
BACKGROUND: Thalassaemia is a hereditary anaemia due to ineffective erythropoiesis. In particular, people with thalassaemia major develop secondary iron overload resulting from regular red blood cell transfusions. Iron chelation therapy is needed to prevent long-term complications.Both deferoxamine and deferiprone are effective; however, a review of the effectiveness and safety of the newer oral chelator deferasirox in people with thalassaemia is needed. OBJECTIVES: To assess the effectiveness and safety of oral deferasirox in people with thalassaemia and iron overload...
August 15, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28808418/emerging-roles-of-sirt1-in-fatty-liver-diseases
#10
REVIEW
Ren-Bo Ding, Jiaolin Bao, Chu-Xia Deng
Fatty liver diseases, which are commonly associated with high-fat/calorie diet, heavy alcohol consumption and/or other metabolic disorder causes, lead to serious medical concerns worldwide in recent years. It has been demonstrated that metabolic homeostasis disruption is most likely to be responsible for this global epidemic. Sirtuins are a group of conserved nicotinamide adenine dinucleotide (NAD(+)) dependent histone and/or protein deacetylases belonging to the silent information regulator 2 (Sir2) family...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28807459/rapidly-progressive-atherosclerosis-after-domino-liver-transplantation-from-a-teenage-donor-with-homozygous-familial-hypercholesterolemia
#11
Jessica R Golbus, Linda Farhat, Robert J Fontana, Melvyn Rubenfire
Familial hypercholesterolemia (FH) is an autosomal dominant disorder characterized by impaired clearance of low-density lipoprotein cholesterol. Given limitations in pharmacologic therapy and the significant morbidity and mortality associated with this disease, liver transplantation may be offered to select homozygous FH patients in childhood in an effort to slow progression of atherosclerotic cardiovascular disease. In rare cases, domino liver transplantation can be performed, transplanting the livers of patients with various metabolic disorders into elderly recipients whose projected survival precludes prolonged waiting on the transplant list...
July 22, 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28807226/liver-metabolomics-study-reveals-protective-function-of-phyllanthus-urinaria-against-ccl4-induced-liver-injury
#12
Qing Guo, Qian-Qian Zhang, Jia-Qing Chen, Wei Zhang, Hong-Cong Qiu, Zun-Jian Zhang, Bu-Ming Liu, Feng-Guo Xu
Phyllanthus Urinaria L. (PUL) is a traditional Chinese medicine used to treat hepatic and renal disorders. However, the mechanism of its hepatoprotective action is not fully understood. In the present study, blood biochemical indexes and liver histopathological changes were used to estimate the extent of hepatic injury. GC/MS and LC/MS-based untargeted metabolomics were used in combination to characterize the potential biomarkers associated with the protective activity of PUL against CCl4-induced liver injury in rats...
July 2017: Chinese Journal of Natural Medicines
https://www.readbyqxmd.com/read/28807197/perioperative-blood-transfusion-and-complications-in-children-undergoing-surgery-for-solid-tumors
#13
Dani O Gonzalez, Jennifer N Cooper, Erica Mantell, Peter C Minneci, Katherine J Deans, Jennifer H Aldrink
BACKGROUND: The objective was to assess whether perioperative blood transfusion (PBT) is associated with postoperative complications in children undergoing surgery for a solid tumor. METHODS: Using 2012-2014 National Surgical Quality Improvement Program Pediatric data, we identified patients aged 0-18 years who underwent surgery (biopsy or resection) for solid tumors. We compared demographic, clinical, and 30-day outcome characteristics between children who did and did not receive a PBT within 72 hours after surgery...
August 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28806319/transient-segmental-enhancement-of-pyogenic-liver-abscess-a-comparison-between-contrast-enhanced-ultrasound-and-computed-tomography
#14
Kun Sun, Wei Zhu, Yan Luo, Yongzhong Li, Xiang Zhou
OBJECTIVE: This study aimed to investigate the correlation between the transient segmental enhancement (TSE) of liver abscesses on contrast-enhanced ultrasound (CEUS) imaging and contrast-enhanced computed tomography (CT) scans. METHODS: In total, 42 abscesses in 38 patients were evaluated with real-time CEUS and contrast-enhanced CT imaging. The CT imaging and CEUS examinations were performed within one to 2 days of each other in all cases. The initial reports of the observations of TSE on CEUS scans were correlated later with the findings of TSE on contrast-enhanced CT images...
August 12, 2017: Journal of Computer Assisted Tomography
https://www.readbyqxmd.com/read/28805579/nonmotor-manifestations-of-wilson-s-disease
#15
Samar Biswas, Neelanjana Paul, Shyamal K Das
Wilson disease (WD) is an autosomal genetic disorder characterized by excessive copper deposition initially in liver (hepatic variant) followed by brain (neuropsychiatric variant) and other organs such as cornea and kidney due to defect in biliary copper excretion. Predominant presentations of neuropsychiatric variant are extrapyramidal motor dysfunctions such as dystonias, Parkinsonism, choreoathetosis, tremor, and ataxias. Nonmotor symptoms (NMS) can appear before clinical disease expression and during ongoing disease process...
2017: International Review of Neurobiology
https://www.readbyqxmd.com/read/28804824/laboratory-testing-for-activated-protein-c-resistance-apcr
#16
Soma Mohammed, Emmanuel J Favaloro
Activated protein C resistance (APCR) describes a hemostatic disorder characterized by a poor anticoagulant response to activated protein C (APC). This results in an increased risk of venous thrombosis, including deep vein thrombosis and pulmonary embolism. Protein C is a natural anticoagulant that is synthesized in the liver and is activated to APC via proteolysis. APC then degrades Factors Va and VIIIa. APCR describes the reduced inability of APC to cleave Factors Va and VIIIa, which therefore promotes increased thrombin generation and potentially leads to a prothrombotic state...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804516/the-role-of-sebelipase-alfa-in-the-treatment-of-lysosomal-acid-lipase-deficiency
#17
REVIEW
Angelika L Erwin
Lysosomal acid lipase deficiency (LALD) is a lysosomal storage disorder (LSD) characterized either by infantile onset with fulminant clinical course and very poor prognosis or childhood/adult-onset disease with an attenuated phenotype. The disorder is often misdiagnosed or remains undiagnosed in children and adults due to a rather unspecific clinical presentation with dyslipidemia and steatohepatitis. Until recently, no good treatment options were available for LALD. Despite supportive and symptomatic therapies, death occurred before 1 year of age in patients with infantile-onset disease and patients with childhood/adult-onset LALD suffered from significant complications, such as liver cirrhosis, requiring liver transplantation and early-onset cardiovascular disease...
July 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28803824/the-clinical-spectrum-of-hepatic-manifestations-in-chronic-lymphocytic-leukemia
#18
REVIEW
Natalia Kreiniz, Ofrat Beyar Katz, Aaron Polliack, Tamar Tadmor
Chronic lymphocytic leukemia (CLL) is the most common leukemia in the Western world, characterized by the presence of long-lived circulating leukemic cells in the peripheral blood that may infiltrate all organs, particularly those of the reticulo-endothelial system. Liver enlargement and elevation of liver enzymes related to specific involvement by the underlying disease are well-recognized features in these patients. In CLL, the differential diagnosis of liver disorders is broad and includes liver infiltration by leukemic cells, immunologic manifestations associated with CLL, primary and secondary hepatic malignancies, drug-induced hepatotoxicity, infections, and Richter transformation...
July 23, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28802875/primary-sclerosing-cholangitis-a-comprehensive-review
#19
REVIEW
Tom H Karlsen, Trine Folseraas, Douglas Thorburn, Mette Vesterhus
Primary sclerosing cholangitis (PSC) is a rare disorder characterized by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease (IBD) is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. With no effective medical therapy, most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and lack of proven surveillance strategies, patients experience significant unmet needs...
August 9, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28802248/utility-of-rapid-whole-exome-sequencing-in-the-diagnosis-of-neonatal-niemann-pick-disease-type-c-presenting-with-fetal-hydrops-and-liver-failure
#20
Mersedeh Rohanizadegan, Sarah El-Almery, Anne O'Donnell-Luria, Ivana Mihalek, Peggy Chen, Marilyn Sanders, Kristen Leeman, Megan Cho, Christina Hung, Olaf Bodamer
Rapid whole exome sequencing (rWES) is increasingly used in critically ill newborn infants to inform about diagnosis, clinical management and prognosis. Here we report a male newborn infant with hydrops, pancytopenia and acute liver failure who was listed for liver transplantation. Given the acuity of the presentation, the procedure related morbidity and mortality and lack of diagnosis we employed rWES in the proband and both parents with a turn-around time of 10 business days. rWES returned one maternally inherited, likely pathogenic and one paternally inherited, likely pathogenic variant in NPC1 suggestive of a diagnosis of Niemann Pick disease type C (NPC)...
August 11, 2017: Cold Spring Harbor Molecular Case Studies
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