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https://www.readbyqxmd.com/read/29242796/bilateral-femoral-neck-fractures-in-cerebrotendinous-xanthomatosis-treated-by-hip-arthroplasties-the-first-case-report-and-literature-review
#1
Witchuree Wejjakul, Swist Chatmaitri, Thongek Wattanarojanaporn, Anuwat Pongkunakorn, Chupong Ittiwut, Vorasuk Shotelersuk
Introduction: Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive lipid storage disease caused by mutations of the CYP27A1 gene and deficiency of the sterol-27-hydroxylase enzyme in bile acid biosynthesis. It is characterized by the accumulation of cholestanol and bile alcohols in plasma, the formation of xanthomatous lesions in various tissues, and organ degeneration. This disorder is also associated with osteoporosis and increased risk of fracture. To date, only two CTX patients with femoral neck fractures have been reported...
September 2017: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/29241453/bile-acid-is-a-significant-host-factor-shaping-the-gut-microbiome-of-diet-induced-obese-mice
#2
Xiaojiao Zheng, Fengjie Huang, Aihua Zhao, Sha Lei, Yunjing Zhang, Guoxiang Xie, Tianlu Chen, Chun Qu, Cynthia Rajani, Bing Dong, Defa Li, Wei Jia
BACKGROUND: Intestinal bacteria are known to regulate bile acid (BA) homeostasis via intestinal biotransformation of BAs and stimulation of the expression of fibroblast growth factor 19 through intestinal nuclear farnesoid X receptor (FXR). On the other hand, BAs directly regulate the gut microbiota with their strong antimicrobial activities. It remains unclear, however, how mammalian BAs cross-talk with gut microbiome and shape microbial composition in a dynamic and interactive way. RESULTS: We quantitatively profiled small molecule metabolites derived from host-microbial co-metabolism in mice, demonstrating that BAs were the most significant factor correlated with microbial alterations among all types of endogenous metabolites...
December 14, 2017: BMC Biology
https://www.readbyqxmd.com/read/29240010/well-preserved-renal-function-in-children-with-untreated-chronic-liver-disease
#3
Ulla B Berg, Antal Németh
OBJECTIVES: Based on studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease. Therefore we investigated renal function in our children with chronic liver disease. METHODS: In a retrospective study of 277 children with chronic liver disease, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol...
December 12, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29239298/optimized-conditions-for-the-in-vitro-excystment-of-calicophoron-daubneyi-metacercariae
#4
Kathryn M Huson, Charlotte Wild, Caroline Fenn, Mark W Robinson
Paramphistomosis, caused by Calicophoron daubneyi, is an emerging infection of ruminants throughout Western Europe. Despite its prevalence, many questions remain regarding the basic biology of this parasite and how it interacts with its host. Consequently, there is a need to develop methods to study C. daubneyi in vitro to improve our understanding of rumen fluke biology. Towards this, we aimed to identify a suitable protocol for in vitro excystment of C. daubneyi metacercariae. Six methods that have been used to excyst metacercariae from a number of trematode species were tested with C...
December 14, 2017: Parasitology
https://www.readbyqxmd.com/read/29238888/synthesis-and-evaluation-of-bile-acid-amides-of-formula-see-text-cyanostilbenes-as-anticancer-agents
#5
Devesh S Agarwal, Rajnish Prakash Singh, K Lohitesh, Prabhat N Jha, Rajdeep Chowdhury, Rajeev Sakhuja
A series of amino-substituted [Formula: see text]-cyanostilbene derivatives and their bile acid (cholic and deoxycholic acid) amides were designed and synthesized. A comparative study on the anticancer and antibacterial activity evaluation on the synthesized analogs was carried against the human osteosarcoma (HOS) cancer cell line, and two gram -ve (E. coli and S. typhi) and two gram [Formula: see text]ve (B. subtilis and S. aureus) bacterial strains. All the cholic acid [Formula: see text]-cyanostilbene amides showed an [Formula: see text] in the range 2-13 [Formula: see text] against human osteosarcoma cells (HOS) with the most active analog (6g) possessing an [Formula: see text] of [Formula: see text]...
December 13, 2017: Molecular Diversity
https://www.readbyqxmd.com/read/29238877/cryptogenic-cholestasis-in-young-and-adults-atp8b1-abcb11-abcb4-and-tjp2-gene-variants-analysis-by-high-throughput-sequencing
#6
Giovanni Vitale, Stefano Gitto, Francesco Raimondi, Alessandro Mattiaccio, Vilma Mantovani, Ranka Vukotic, Antonietta D'Errico, Marco Seri, Robert B Russell, Pietro Andreone
BACKGROUND: Mutations in ATP-transporters ATPB81, ABCB11, and ABCB4 are responsible for progressive familial intrahepatic cholestasis (PFIC) 1, 2 and 3, and recently the gene for tight junction protein-2 (TJP2) has been linked to PFIC4. AIM: As these four genes have been poorly studied in young people and adults, we investigated them in this context here. METHODS: In patients with cryptogenic cholestasis, we analyzed the presence of mutations by high-throughput sequencing...
December 13, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29238458/bacteriocinogenic-potential-and-virulence-traits-of-enterococcus-faecium-and-e-faecalis-isolated-from-human-milk
#7
Soodabeh Khalkhali, Naheed Mojgani
Background and Objectives: Human milk is a continuous supply of Lactic Acid bacteria (LAB), including enterococci with probiotic potentials. The aim of this study was to analyze two Enterococcus species, isolated from human milk for their probiotic potential, bacteriocin producing ability and virulence traits. Materials and Methods: Enterococcus faecium TA0033 and E. faecalis TA102 were tested for acid and bile tolerance, survival in simulated gastric and intestinal conditions...
August 2017: Iranian Journal of Microbiology
https://www.readbyqxmd.com/read/29237721/rev-erba-regulates-cyp7a1-through-repression-of-liver-receptor-homolog-1
#8
Tianpeng Zhang, Mengjing Zhao, Danyi Lu, Shuai Wang, Fangjun Yu, Lianxia Guo, Shijun Wen, Baojian Wu
REV-ERBα is a nuclear heme receptor (as known as a transcriptional repressor) that orchestrates circadian rhythms and cell metabolism. Here we investigate the role of LRH-1 in REV-ERBα regulation of CYP7A1 and cholesterol metabolism. We first characterized the tertiary amine GSK2945 as a highly specific Rev-erbα/REV-ERBα antagonist using cell-based assays and confirmed expression of Rev-erbα in mouse liver. GSK2945 treatment increased hepatic Cyp7a1 level and lowered plasma cholesterol in wild-type mice...
December 13, 2017: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/29235891/lipid-biomarkers-for-the-peroxisomal-and-lysosomal-disorders-their-formation-metabolism-and-measurement
#9
Ryuichi Mashima, Masamitsu Maekawa
Lipid biomarkers play important roles in the diagnosis of and monitoring of treatment in peroxisomal disorders and lysosomal storage disorders. Today, a variety of lipids, including very long chain fatty acids, glycolipids, bile acids and the oxidation products of cholesterol, have been considered as biomarkers for these disorders. In this brief review, the authors summarized the recent advances regarding these lipid biomarkers in terms of their formation, metabolism and measurement in these disorders. An understanding of these biomarkers will offer a key to the development of novel diagnoses and help create more effective therapies in the future...
December 13, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/29235297/-simultaneous-determination-of-8-constituents-of-desmodii-styracifolii-herba-in-rat-bile-with-hplc-ms-ms-and-their-excretion-pharmacokinetics
#10
Xue-Yan Fan, Xin Wang, Xiao-Chen Liu, Chun-Ying Wang
To establish a HPLC-MS/MS quantitative method for analysis of 8 constituents in rat bile. The method was applied in the biliary excretion study after oral administration of Desmodii Styracifolii Herba extract. The rat bile samples were collected during 0-1, 1-2, 2-4, 4-6, 6-8, 8-12, 12-24 h. Diamonsil C₁₈column (4.6 mm×150 mm, 5 μm) was adopted and eluted with methanol and 0.01% acetic acid in a gradient mode. The flow rate was 0.8 mL•min⁻¹, and the column temperature was set at 40 ℃. The detection was carried out by a triple quadrupole linearion trap mass spectrometer in the negative ion mode with an electrospray source...
October 2017: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/29235014/increased-bile-acid-signals-after-duodenal-jejunal-bypass-improve-non-alcoholic-steatohepatitis-nash-in-a-rodent-model-of-diet-induced-nash
#11
Takahiro Tsuchiya, Takeshi Naitoh, Munenori Nagao, Naoki Tanaka, Kazuhiro Watanabe, Hirofumi Imoto, Tomohiro Miyachi, Fuyuhiko Motoi, Michiaki Unno
BACKGROUND: The increasing incidence of non-alcoholic steatohepatitis (NASH) has resulted in it becoming a common cause of liver-related mortality; however, no efficient treatment has been established. It has been reported that bariatric surgery improves metabolic comorbidities, such as diabetes mellitus and NASH. Although the mechanism is unclear, it is thought that the changes in bile acid (BA) signaling via its nuclear receptor, farnesoid X receptor (FXR), produce various metabolic effects...
December 12, 2017: Obesity Surgery
https://www.readbyqxmd.com/read/29235009/duodenal-exclusion-but-not-sleeve-gastrectomy-preserves-insulin-secretion-making-it-the-more-effective-metabolic-procedure
#12
Claudia Laessle, Gergana Nenova, Goran Marjanovic, Gabriel Seifert, Lampros Kousoulas, Bernd Jaenigen, Stefan Fichtner-Feigl, Jodok Matthias Fink
INTRODUCTION: There is an ongoing debate on which procedure provides the best treatment for type 2 diabetes. Furthermore, the pathomechanisms of diabetes improvement of partly anatomically differing operations is not fully understood. METHODS: A loop duodenojejunostomy (DJOS) with exclusion of one third of intestinal length, a sleeve gastrectomy (SG), or a combination of DJOS + SG was performed in 8-week-old male ZDF rats. One, three, and six months after surgery, an oral glucose tolerance test and measurements of GLP-1, GIP, insulin, and bile acids were conducted...
December 12, 2017: Obesity Surgery
https://www.readbyqxmd.com/read/29234972/staging-treatment-and-future-approaches-of-gallbladder-carcinoma
#13
REVIEW
Sanjeev Kumar Shukla, Govind Singh, K S Shahi, Bhuvan, Prabhat Pant
BACKGROUND: Gallbladder cancer is the most common malignant cancer of the bile ducts and third most common gastrointestinal malignant in the world for public health. Its relatively low incidence and confused symptoms result in advanced disease at the time of presentation, contributing to poor prognosis and reduced survival associated with this disease. The main function of the gallbladder is to store excreted bile acids from the liver in preparation for a meal. Its main risk factor is prolonged exposure to biliary calculations, although bacterial infections and other inflammatory conditions are associated...
December 12, 2017: Journal of Gastrointestinal Cancer
https://www.readbyqxmd.com/read/29234315/food-associated-lactobacillus-plantarum-and-yeasts-inhibit-the-genotoxic-effect-of-4-nitroquinoline-1-oxide
#14
Roberta Prete, Rosanna Tofalo, Ermanno Federici, Aurora Ciarrocchi, Giovanni Cenci, Aldo Corsetti
Lactic acid bacteria and yeasts, representing the prevailing microbiota associated with different foods generally consumed without any cooking, were identified and characterized in vitro for some functional properties, such as acid-bile tolerance and antigenotoxic activity. In particular, 22 Lactobacillus plantarum strains and 14 yeasts were studied. The gastro-intestinal tract tolerance of all the strains was determined by exposing washed cell suspensions at 37°C to a simulated gastric juice (pH 2.0), containing pepsin (0...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29234196/glycochenodeoxycholic-acid-does-not-increase-transforming-growth-factor-beta-expression-in-bile-duct-epithelial-cells-or-collagen-synthesis-in-myofibroblasts
#15
Anna Wang, Dorothy Yu, Yuewen Gong, Jessie Garber, Gerald Y Minuk
Background/Aims: Primary sclerosing cholangitis (PSC) is a chronic, progressive hepatobiliary disorder characterized by extensive fibrosis and stricturing of the intra- and/or extra-hepatic bile ducts: Previous studies have documented low phosphatylcholine (PC) concentrations in PSC bile. The aim of this study was to determine whether low PC levels in bile facilitate toxic bile acid induced injury of biliary tract epithelial cells resulting in enhanced transforming growth factor-beta (TGF-β) expression and increased collagen synthesis by myofibroblasts...
December 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29233934/chronic-infusion-of-taurolithocholate-into-the-brain-increases-fat-oxidation-in-mice
#16
Hannah M Eggink, Lauren L Tambyrajah, Rosa van den Berg, Isabel Mol, Jose K van den Heuvel, Martijn Koehorst, Albert K Groen, Anita Boelen, Andries Kalsbeek, Johannes A Romijn, Patrick C N Rensen, Sander Kooijman, Maarten R Soeters
Bile acids can function in the postprandial state as circulating signaling molecules in the regulation of glucose and lipid metabolism via the transmembrane receptor TGR5 and nuclear receptor FXR. Both receptors are present in the central nervous system, but their function in the brain is unclear. Therefore, we investigated the effects of intracerebroventricular (icv) administration of taurolithocholate (tLCA), a strong TGR5 agonist, and GW4064, a synthetic FXR agonist, on energy metabolism. We determined the effects of chronic icv infusion of tLCA, GW4064, or vehicle on energy expenditure, body weight and composition as well as tissue specific fatty acid uptake in mice equipped with osmotic minipumps...
December 12, 2017: Journal of Endocrinology
https://www.readbyqxmd.com/read/29233036/increased-levels-of-the-novel-hepatokine-fetuin-b-in-patients-with-intrahepatic-cholestasis-of-pregnancy
#17
Nadiye Koroglu, Ahmet Tayyar, Esra Nur Tola, Gonca Yetkin Yildirim, Ilkbal Temel Yuksel, Berna Aslan Cetin, Ismail Dag, Deniz Kanber Acar
OBJECTIVES: To investigate liver-derived plasma protein fetuin B levels in healthy pregnant women and pregnant women with intrahepatic cholestasis of pregnancy (ICP). MATERIALS AND METHODS: Forty women with ICP and 40 healthy pregnant women were included in this cross-sectional study. The serum fetuin B levels of these patients were analyzed. The patients were followed up to delivery. RESULTS: Maternal age, gravida, parity, BMI at assessment, and gestational age at blood sampling were similar between the ICP and control groups (p > ...
December 12, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29229992/mirna-21-ablation-protects-against-liver-injury-and-necroptosis-in-cholestasis
#18
Marta B Afonso, Pedro M Rodrigues, André L Simão, Maria M Gaspar, Tânia Carvalho, Paula Borralho, Jesús M Bañales, Rui E Castro, Cecília M P Rodrigues
Inhibition of microRNA-21 (miR-21) prevents necroptosis in the mouse pancreas. Necroptosis contributes to hepatic necro-inflammation in the common bile duct ligation (BDL) murine model. We aimed to evaluate the role of miR-21 in mediating deleterious processes associated with cholestasis. Mechanistic studies established a functional link between miR-21 and necroptosis through cyclin-dependent kinase 2-associated protein 1 (CDK2AP1). miR-21 expression increased in the liver of primary biliary cholangitis (PBC) patients and BDL wild-type (WT) mice at both 3 and 14 days...
December 11, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29229736/comparison-of-salmonella-enterica-serovars-typhi-and-typhimurium-reveals-typhoidal-specific-responses-to-bile
#19
Rebecca Johnson, Matt Ravenhall, Derek Pickard, Gordon Dougan, Alexander Byrne, Gad Frankel
Salmonella enterica serovars Typhi and Typhimurium cause typhoid fever and gastroenteritis respectively. A unique feature of typhoid infection is asymptomatic carriage within the gallbladder, which is linked with S Typhi transmission. Despite this, S Typhi responses to bile have been poorly studied. RNA-Seq of S Typhi Ty2 and a clinical S Typhi isolate belonging to the globally dominant H58 lineage (129-0238), as well as S Typhimurium 14028, revealed that 249, 389 and 453 genes respectively were differentially expressed in the presence of 3% bile compared to control cultures lacking bile...
December 11, 2017: Infection and Immunity
https://www.readbyqxmd.com/read/29229136/the-gut-liver-axis-impact-of-a-mouse-model-of-small-bowel-bacterial-overgrowth
#20
Qingqing Wang, Bin Wang, Vijay Saxena, Lili Miles, Josh Tiao, Joel E Mortensen, Jaimie D Nathan
BACKGROUND: The mechanisms by which intestinal bacteria impact liver diseases remain poorly understood. The aim of this study was to develop a mouse model of small-bowel bacterial overgrowth and to determine its impact on hepatobiliary injury. MATERIALS AND METHODS: A jejunal self-filling blind loop (SFBL) was created in C57BL/6 mice. Three weeks after surgery, the mice were euthanized, and bacterial cultures of luminal content of the loop and extraintestinal tissues were performed...
January 2018: Journal of Surgical Research
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