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Cholestatic liver diseases

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https://www.readbyqxmd.com/read/28230981/high-potency-phenylquinoxalinone-cystic-fibrosis-transmembrane-conductance-regulator-cftr-activators
#1
Jung-Ho Son, Jie S Zhu, Puay-Wah Phuan, Onur Cil, Andrew P Teuthorn, Colton K Ku, Sujin Lee, Alan S Verkman, Mark J Kurth
We previously identified phenylquinoxalinone CFTRact-J027 (4) as a cystic fibrosis transmembrane conductance regulator (CFTR) activator with an EC50 of ~200 nM, and demonstrated its therapeutic efficacy in mouse models of constipation. Here, structure-activity studies were done on 36 synthesized phenylquinoxalinone analogs to identify compounds with improved potency and altered metabolic stability. Synthesis of the phenylquinoxalinone core was generally accomplished by condensation of 1,2-phenylenediamines with substituted phenyloxoacetates...
February 23, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28223721/current-and-future-therapies-for-inherited-cholestatic-liver-diseases
#2
REVIEW
Wendy L van der Woerd, Roderick Hj Houwen, Stan Fj van de Graaf
Familial intrahepatic cholestasis (FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phenotypes range from benign recurrent intrahepatic cholestasis (BRIC), associated with recurrent cholestatic attacks, to progressive FIC (PFIC). Patients often suffer from severe pruritus and eventually progressive cholestasis results in liver failure. Currently, first-line treatment includes ursodeoxycholic acid in patients with ABCB4 deficiency (PFIC3) and partial biliary diversion in patients with ATP8B1 or ABCB11 deficiency (PFIC1 and PFIC2)...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28208962/the-effect-of-nutritional-status-on-outcome-of-hospitalization-in-paediatric-liver-disease-patients
#3
Yasmeen Mansi, Shereen Abdel Ghaffar, Shaymaa Sayed, Hanaa El-Karaksy
INTRODUCTION: Liver is an important organ for metabolism. It has a major role in integrating the various biochemical pathways of metabolism. Thus, children with chronic liver disease are at high risk for developing undernutrition, with important prognostic implications. AIM: To evaluate the nutritional status of hospitalized paediatric liver disease patients and its effect on outcome. MATERIALS AND METHODS: We prospectively analysed the nutritional status of 59 consecutive patients during their first 24 hours of admission, at the Hepatology Unit, using the following indices: weight/age, height/age, weight/height, Body Mass Index (BMI), arm circumference and triceps skinfold, subcapular skinfold, and mid upper arm circumference...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28197333/a-case-of-primary-biliary-cirrhosis-mimicking-acute-hepatitis-b-in-the-clinic-republic-of-korea
#4
Woo Hyuk Kwon, Hong Min Park, Jeong Jun Park, Sung Hoon Lee, Yong Kyu Lee
Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen...
January 2017: Korean Journal of Family Medicine
https://www.readbyqxmd.com/read/28196718/the-neuropeptide-galanin-is-up-regulated-during-cholestasis-and-contributes-to-cholangiocyte-proliferation
#5
Matthew McMillin, Gabriel Frampton, Stephanie Grant, Sharon DeMorrow
During the course of cholestatic liver diseases, mitotically dormant cholangiocytes proliferate and subsequently acquire a neuroendocrine phenotype. Galanin is a neuroendocrine factor responsible for regulation of physiological responses, such as feeding behavior and mood, and has been implicated in the development of fatty liver disease, although its role in biliary hyperplasia is unknown. Biliary hyperplasia was induced in rats via bile duct ligation (BDL) surgery, and galanin was increased in serum and liver homogenates from BDL rats...
February 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28195083/progressive-familial-intrahepatic-cholestasis-a-comprehensive-review-of-a-challenging-liver-disease
#6
REVIEW
Kavita Gaur, Puja Sakhuja
Cholestatic liver disease in children represents a diagnostic and therapeutic challenge. The requirement of a multidisciplinary approach, high levels of professional expertise, and the costs of genetic testing are a few of the reasons why such patients may suffer for want of an accurate diagnosis. Progressive familial intrahepatic cholestasis (PFIC) is a hereditary cholestatic liver disease, afflicted children often progressing to liver failure. Despite its potential to cause significant morbidity, it has seldom been studied in India...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28194394/assessment-of-school-readiness-in-chronic-cholestatic-liver-disease-a-pilot-study-examining-children-with-and-without-liver-transplantation
#7
Anna Gold, Alaine Rogers, Elizabeth Cruchley, Stephanie Rankin, Arpita Parmar, Binita M Kamath, Yaron Avitzur, Vicky Lee Ng
Background. Assessment of school readiness evaluates physical, social-emotional, and neuropsychological domains essential for educational success. Cognitive testing of preschool aged children with chronic liver disease may guide more timely interventions and focused efforts by health care providers. Patients and Methods. Children with chronic cholestatic liver disease diagnosed as an infant and still with their native liver (NL) and children who received a liver transplant (LT) before age of 2 years underwent testing with a battery of well-validated pediatric psychometric measures...
2017: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28178654/hepatitis-b-virus-surface-proteins-accelerate-cholestatic-injury-and-tumor-progression-in-abcb4-knockout-mice
#8
Daniel Zahner, Hannah Glimm, Tomomitsu Matono, Yuri Churin, Diran Herebian, Ertan Mayatepek, Kernt Köhler, Stefan Gattenlöhner, Anne Stinn, Annette Tschuschner, Martin Roderfeld, Elke Roeb
: Understanding of the pathophysiology of cholestasis associated carcinogenesis could challenge the development of new personalized therapeutic approaches and thus improve prognosis. Simultaneous damage might aggravate hepatic injury, induce chronic liver disease and even promote carcinogenesis. We aimed to study the effect of Hepatitis B virus surface protein (HBsAg) on cholestatic liver disease and associated carcinogenesis in a mouse model combining both impairments. Hybrids of Abcb4-/- and HBsAg transgenic mice were bred on fibrosis susceptible background BALB/c...
February 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28178326/quantitative-liver-proteomics-identifies-fgf19-targets-that-couple-metabolism-and-proliferation
#9
Vittoria Massafra, Alexandra Milona, Harmjan R Vos, Boudewijn M T Burgering, Saskia W C van Mil
Fibroblast growth factor 19 (FGF19) is a gut-derived peptide hormone that is produced following activation of Farnesoid X Receptor (FXR). FGF19 is secreted and signals to the liver, where it contributes to the homeostasis of bile acid (BA), lipid and carbohydrate metabolism. FGF19 is a promising therapeutic target for the metabolic syndrome and cholestatic diseases, but enthusiasm for its use has been tempered by FGF19-mediated induction of proliferation and hepatocellular carcinoma. To inform future rational design of FGF19-variants, we have conducted temporal quantitative proteomic and gene expression analyses to identify FGF19-targets related to metabolism and proliferation...
2017: PloS One
https://www.readbyqxmd.com/read/28176238/vitamin-d-status-in-patients-with-cirrhosis-of-the-liver-and-their-relatives-a-case-control-study-from-north-india
#10
Ravikant Kumar, Pavan Kumar, Kandarp Nath Saxena, Manjul Mishra, Vivek Kumar Mishra, Anju Kumari, Manisha Dwivedi, Sri Prakash Misra
BACKGROUND AND AIM: Liver diseases interfere with the production of the metabolites of vitamin D required for activation, thus resulting in abnormal calcium and bone metabolism. Previous studies show inconsistent results of vitamin D level in non-cholestatic liver diseases. Our aim was to determine the prevalence of vitamin D insufficiency in cirrhosis as compared to apparently normal relatives and its relationship with etiology and severity. METHODS: One hundred and sixty cirrhotic patients attending the Department of Gastroenterology and Hepatology, M L N Medical College, Allahabad, were enrolled, and 25-hydroxy vitamin D [25(OH)D] and calcium levels assessed...
February 8, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28161910/associations-of-gender-and-a-proxy-of-female-menopausal-status-with-histological-features-of-drug-induced-liver-injury
#11
Ayako Suzuki, Huiman Barnhart, Jiezhun Gu, Herbert L Bonkovsky, Hans L Tillmann, Robert J Fontana, David E Kleiner
Gender and menopause may contribute to type and severity of drug-induced liver injury (DILI) by influencing host responses to injury. The aim of this study was to assess the associations of gender and female age 50 [a proxy of menopause] with histologic features of liver injury in 212 adults enrolled in the Drug-Induced Liver Injury Network (DILIN) registry METHODS: All participants had a causality score of at least 'probable', a liver biopsy within 30 days of DILI onset, and no prior chronic liver disease...
February 5, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28157235/possible-involvement-of-nitric-oxide-in-enhanced-liver-injury-and-fibrogenesis-during-cholestasis-in-cytoglobin-deficient-mice
#12
Tuong Thi Van Thuy, Le Thi Thanh Thuy, Katsutoshi Yoshizato, Norifumi Kawada
This study clarified the role of Cygb, the fourth globin in mammals originally discovered in rat hepatic stellate cells (HSCs), in cholestatic liver disease. Bile duct ligation (BDL) augmented inflammatory reactions as revealed by increased infiltrating neutrophils, CD68(+)-macrophages, and chemokine expression in Cygb(-/-) mice. In these mice, impairment of bile canalicular indicated by the loss of CD10 expression, down-regulation of bile salt transporters, increased total bile acid, and massive apoptotic and necrotic hepatocytes occurred with the release of cytochrome c, activation of caspase 3, resulting in reduced animal survival compared to wild-type mice...
February 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28150525/role-of-homocysteine-and-folic-acid-on-the-altered-calcium-homeostasis-of-platelets-from-rats-with-biliary-cirrhosis
#13
Paola Romecín, Noemí M Atucha, Esther G Navarro, M Clara Ortiz, David Iyú, Juan Antonio Rosado, Joaquín García-Estañ
Previously, we have found that intracellular calcium homeostasis is altered in platelets from an experimental model of liver cirrhosis, the bile-duct ligated (BDL) rat; these alterations are compatible with the existence of a hypercoagulable state. Different studies indicate that cholestatic diseases are associated with hyperhomocysteinemia; thus, we hypothetized that it could contribute to those platelet alterations. In the present study, we have investigated the role of homocysteine (HCY) in platelet aggregation and calcium signaling in the BDL model...
February 2, 2017: Platelets
https://www.readbyqxmd.com/read/28146070/decreased-expression-of-vitamin-d-receptor-affects-an-immune-response-in-primary-biliary-cholangitis-via-the-vdr-mirna155-socs1-pathway
#14
Agnieszka Kempinska-Podhorodecka, Malgorzata Milkiewicz, Urszula Wasik, Joanna Ligocka, Michał Zawadzki, Marek Krawczyk, Piotr Milkiewicz
Primary biliary cholangitis (PBC) is an immune-mediated cholestatic disease. Vitamin D receptor (VDR)-dependent signaling constrains an inflammatory response by targeting the miRNA155-SOCS1 (suppressor of cytokine signaling 1) axis. The VDR-miRNA155-SOCS1 pathway was investigated in the context of the autoimmune response associated with PBC. Human liver tissues from non-cirrhotic PBC (n = 22), cirrhotic PBC (n = 22), cirrhotic primary sclerosing cholangitis (PSC, n=13), controls (n = 23), and peripheral blood mononuclear cells (PBMC) obtained from PBC (n = 16) and PSC (n = 10) patients and healthy subjects (n = 11) were used for molecular analyses...
January 29, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28145671/evaluation-of-jaundice-in-adults
#15
Matthew V Fargo, Scott P Grogan, Aaron Saguil
Jaundice in adults can be an indicator of significant underlying disease. It is caused by elevated serum bilirubin levels in the unconjugated or conjugated form. The evaluation of jaundice relies on the history and physical examination. The initial laboratory evaluation should include fractionated bilirubin, a complete blood count, alanine transaminase, aspartate transaminase, alkaline phosphatase, ?-glutamyltransferase, prothrombin time and/or international normalized ratio, albumin, and protein. Imaging with ultrasonography or computed tomography can differentiate between extrahepatic obstructive and intrahepatic parenchymal disorders...
February 1, 2017: American Family Physician
https://www.readbyqxmd.com/read/28141677/the-unique-inflammatory-bowel-disease-phenotype-of-pediatric-primary-sclerosing-cholangitis-a-single-center-study
#16
Henry Shiau, Faith D Ihekweazu, Mansi Amin, Tatiana Fofanova, Tamir Miloh, Richard Kellermayer
BACKGROUND: In adults, primary sclerosing cholangitis (PSC), a cholestatic liver disease characterized by inflammation/fibrosis of intra/extra-hepatic bile ducts, associates with a milder form of inflammatory bowel disease (IBD), particularly ulcerative colitis (UC). The pediatric PSC-IBD phenotype is less well characterized. METHODS: We performed a retrospective, single-center study examining PSC-IBD patients at Texas Children's Hospital (TCH) between 2000-2015...
January 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28137455/new-onset-severe-hypercholesterolemia-in-a-patient-with-cholestatic-liver-disease-should-we-treat-the-lipids
#17
Panteleimon E Papakonstantinou, Eirini Theodoraki, Mairi Koulentaki, John A Papadakis
No abstract text is available yet for this article.
January 27, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28134655/a-central-role-for-r7bp-in-the-regulation-of-itch-sensation
#18
Mritunjay Pandey, Jian-Hua Zhang, Santosh K Mishra, Poorni R Adikaram, Benjamin Harris, John F Kahler, Anna Loshakov, Roxanne Sholevar, Allison Genis, Claire Kittock, Juraj Kabat, Sundar Ganesan, Richard R Neubig, Mark A Hoon, William F Simonds
Itch is a protective sensation producing a desire to scratch. Pathologic itch can be a chronic symptom of illnesses such as uremia, cholestatic liver disease, neuropathies and dermatitis, however current therapeutic options are limited. Many types of cell surface receptors, including those present on cells in the skin, on sensory neurons and on neurons in the spinal cord, have been implicated in itch signaling. The role of G protein signaling in the regulation of pruriception is poorly understood. We identify here two G protein signaling components whose mutation impairs itch sensation...
January 27, 2017: Pain
https://www.readbyqxmd.com/read/28128437/schisandrol-b-protects-against-cholestatic-liver-injury-through-pregnane-x-receptor
#19
Hang Zeng, Yiming Jiang, Pan Chen, Xiaomei Fan, Dongshun Li, Aiming Liu, Xiaochao Ma, Wen Xie, Peiqing Liu, Frank J Gonzalez, Min Huang, Huichang Bi
BACKGROUND AND PURPOSE: Currently, ursodeoxycholic acid is the only FDA-approved drug for limited cholestatic liver diseases, and thus the development of new therapeutic approaches is of great importance. This study aimed to evaluate the anti-cholestasis effect of Schisandrol B (SolB), a bioactive compound isolated from Schisandra sphenanthera. EXPERIMENTAL APPROACH: Hepatoprotective effect of SolB against lithocholic acid (LCA)-induced intrahepatic extrahepatic cholestasis was evaluated in mice...
January 27, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28124078/primary-biliary-cholangitis-associated-with-skin-disorders-a-case-report-and-review-of-the-literature
#20
REVIEW
Benedetta Terziroli Beretta-Piccoli, Caroline Guillod, Igor Marsteller, Roland Blum, Luca Mazzucchelli, Chiara Mondino, Pietro Invernizzi, M Eric Gershwin, Carlo Mainetti
Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease. It is often associated with extrahepatic autoimmune diseases. Skin disorders are sporadically reported in association with PBC. We report an unusual case of PBC associated with acquired reactive perforating dermatosis (ARPD) and present a review of the literature on skin disorders associated with PBC. Our patient presented to the dermatology department with generalized pruritus associated with nodular perforating skin lesions on the trunk, and cholestatic liver disease of unknown origin...
January 25, 2017: Archivum Immunologiae et Therapiae Experimentalis
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