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Ursodeoxycholic acid

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https://www.readbyqxmd.com/read/29242416/etiology-and-outcome-of-cholelithiasis-in-turkish-children
#1
Maşallah Baran, Yeliz Çağan Appak, Gökhan Tümgör, Miray Karakoyun, Tunç Özdemir, Gökhan Köylüoğlu
OBJECTIVE: The aim of this study was to examine the etiology of gallstones in children and responses to ursodeoxycholic acid (UDCA) treatment. METHODS: 74 children with cholelithiasis were recruited, and underwent ultrasonography to detect gallstones. All relevant clinical information was recorded in a structured proforma. RESULTS: The commonest risk factor was a family history of gallstones. Most children responded to UDCA treatment in the first six months; children with hemolytic diseases showed no response to UDCA...
December 14, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29231188/major-hepatic-complications-in-ursodeoxycholic-acid-treated-patients-with-primary-biliary-cholangitis-risk-factors-and-time-trends-in-incidence-and-outcome
#2
Maren H Harms, Willem J Lammers, Douglas Thorburn, Christophe Corpechot, Pietro Invernizzi, Harry L A Janssen, Pier M Battezzati, Frederik Nevens, Keith D Lindor, Annarosa Floreani, Cyriel Y Ponsioen, Marlyn J Mayo, George N Dalekos, Tony Bruns, Albert Parés, Andrew L Mason, Xavier Verhelst, Kris V Kowdley, Jorn C Goet, Gideon M Hirschfield, Bettina E Hansen, Henk R van Buuren
OBJECTIVES: In this era of near universal ursodeoxycholic acid (UDCA) treatment for primary biliary cholangitis (PBC), progression to cirrhosis still occurs in an important proportion of patients. The aim of this study was to describe the incidence of cirrhosis-associated complications in patients with PBC and assess risk factors and impact on survival. METHODS: Cohorts of UDCA-treated patients from 16 European and North-American liver centers were included. We used Cox proportional hazards assumptions and Kaplan-Meier estimates...
December 12, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29228183/plasma-oxysterols-biomarkers-for-diagnosis-and-treatment-in-spastic-paraplegia-type-5
#3
Cecilia Marelli, Foudil Lamari, Dominique Rainteau, Alexandre Lafourcade, Guillaume Banneau, Lydie Humbert, Marie-Lorraine Monin, Elodie Petit, Rabab Debs, Giovanni Castelnovo, Elisabeth Ollagnon, Julie Lavie, Julie Pilliod, Isabelle Coupry, Patrick J Babin, Claire Guissart, Imen Benyounes, Urielle Ullmann, Gaetan Lesca, Christel Thauvin-Robinet, Pierre Labauge, Sylvie Odent, Claire Ewenczyk, Claude Wolf, Giovanni Stevanin, David Hajage, Alexandra Durr, Cyril Goizet, Fanny Mochel
The hereditary spastic paraplegias are an expanding and heterogeneous group of disorders characterized by spasticity in the lower limbs. Plasma biomarkers are needed to guide the genetic testing of spastic paraplegia. Spastic paraplegia type 5 (SPG5) is an autosomal recessive spastic paraplegia due to mutations in CYP7B1, which encodes a cytochrome P450 7α-hydroxylase implicated in cholesterol and bile acids metabolism. We developed a method based on ultra-performance liquid chromatography electrospray tandem mass spectrometry to validate two plasma 25-hydroxycholesterol (25-OHC) and 27-hydroxycholesterol (27-OHC) as diagnostic biomarkers in a cohort of 21 patients with SPG5...
December 8, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29226620/comparative-potency-of-obeticholic-acid-and-natural-bile-acids-on-fxr-in-hepatic-and-intestinal-in%C3%A2-vitro-cell-models
#4
Yuanyuan Zhang, Carl LaCerte, Sanjay Kansra, Jonathan P Jackson, Kenneth R Brouwer, Jeffrey E Edwards
Obeticholic acid (OCA) is a semisynthetic farnesoid X receptor (FXR) agonist, an analogue of chenodeoxycholic acid (CDCA) which is indicated for the treatment of primary biliary cholangitis (PBC) in combination with ursodeoxycholic acid (UDCA). OCA efficiently inhibits bile acid synthesis and promotes bile acid efflux via activating FXR-mediated mechanisms in a physiologically relevant in vitro cell system, Sandwich-cultured Transporter Certified ™ human primary hepatocytes (SCHH). The study herein evaluated the effects of UDCA alone or in combination with OCA in SCHH...
December 2017: Pharmacology Research & Perspectives
https://www.readbyqxmd.com/read/29220537/milder-disease-stage-in-patients-with-primary-biliary-cholangitis-over-a-44-year-period-a-changing-natural-history
#5
Fiorella Murillo Perez, Jorn C Goet, Willem J Lammers, Aliya Gulamhusein, Henk R van Buuren, Cyriel Y Ponsioen, Marco Carbone, Andrew Mason, Christophe Corpechot, Pietro Invernizzi, Marlyn J Mayo, Pier Maria Battezzati, Annarosa Floreani, Albert Pares, Frederik Nevens, Kris V Kowdley, Tony Bruns, George N Dalekos, Douglas Thorburn, Gideon Hirschfield, Nicholas F LaRusso, Keith D Lindor, Kalliopi Zachou, Raoul Poupon, Palak J Trivedi, Xavier Verhelst, Harry L A Janssen, Bettina E Hansen
Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis (PBC) are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international PBC cohort of 4805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n=143), 1980-1989 (n=858), 1990-1999 (n=1754), 2000-2009 (n=1815), ≥2010 (n=235)...
December 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29204050/current-and-emerging-pharmacological-therapy-for-non-alcoholic-fatty-liver-disease
#6
EDITORIAL
Ahad Eshraghian
The main treatment of patients with non-alcoholic fatty liver disease (NAFLD) is life style modification including weight reduction and dietary regimen. Majority of patients are safely treated with this management and pharmacologic interventions are not recommended. However, a subgroup of NAFLD patients with non-alcoholic steatohepatitis (NASH) who cannot achieve goals of life style modification may need pharmacological therapy. One major obstacle is measurement of histological outcome by liver biopsy which is an invasive method and is not recommended routinely in these patients...
November 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29202273/effects-of-barley-variety-dietary-fiber-and-%C3%AE-glucan-content-on-bile-acid-composition-in-cecum-of-rats-fed-low-and-high-fat-diets
#7
Tannaz Ghaffarzadegan, Yadong Zhong, Frida Fåk Hållenius, Margareta Nyman
Diet-induced obesity and insulin resistance have been linked to changes in bile acid (BA) profiles, which in turn are highly dependent on the dietary composition and activity of the gut microbiota. The objective of the present study was to investigate whether the type and level of fiber had an effect on cecal BA composition when included in low- and high-fat diets. Groups of rats were fed two barley varieties, which resulted in three test diets containing three levels of β-glucans and two levels of dietary fiber...
December 1, 2017: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/29187717/-a-case-of-liver-dysfunction-requiring-hospital-admission-after-taking-oral-itraconazole-for-the-treatment-of-kerion-celsi
#8
Eri Ikeda, Soko Watanabe, Mizuki Sawada, Junya Ninomiya, Itaru Dekio, Sumiko Ishizaki, Mariko Fujibayashi, Masaru Tanaka, Takashi Harada
  67-year-old female patient developed drug-induced liver dysfunction after taking oral itraconazole (ITCZ) for the treatment of kerion celsi. Red papules appeared on the temporal area of the patient one month prior to her visit to our clinic. The patient presented with a nodule with yellow crust, erosion, infiltration, and hair loss on the area. Diagnosis of kerion celsi caused by Trichophyton rubrum was made from clinical, pathological, and mycological findings. Laboratory data showed normal liver function, and the patient was not taking any other medication, thus, daily oral ITCZ 100 mg was started...
2017: Medical Mycology Journal
https://www.readbyqxmd.com/read/29181702/geoepidemiology-of-primary-biliary-cholangitis-lessons-from-switzerland
#9
Benedetta Terziroli Beretta-Piccoli, Guido Stirnimann, Andreas Cerny, David Semela, Roxane Hessler, Beat Helbling, Felix Stickel, Carolina Kalid-de Bakker, Florian Bihl, Emiliano Giostra, Magdalena Filipowicz Sinnreich, Carl Oneta, Adriana Baserga, Pietro Invernizzi, Marco Carbone, Joachim Mertens
No data on primary biliary cholangitis (PBC) are available in Switzerland. We established a national patient cohort to obtain information on PBC phenotypes and disease course in Switzerland. Local databases in all university hospitals and in two large secondary centers were searched for case finding. In addition, all primary care physicians, gastroenterologists, rheumatologists, and dermatologists were invited to contribute patients from their own medical records. PBC diagnosis was centrally reviewed. Five hundred one PBC patients were identified, 474 were included in data analysis, and 449 of them were enrolled by tertiary centers...
November 27, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29181663/gene-tnf-polymorphism-308g-a-rs1800629-and-its-relationship-with-the-efficiency-of-ursodeoxycholic-acid-therapy-in-patients-with-nonalcoholic-stetohepatitis
#10
I V Kurbatova, L V Topchieva, O P Dudanova
Association of TNF gene polymorphism -308G>A with the development of nonalcoholic steatohepatitis in the Russian population was revealed. Carriers of allele A of the TNF gene marker -308G>A have significantly higher risk of nonalcoholic steatohepatitis development: OR=1.69 (1.05; 2.71). Allele A carriage by this marker predicts an increase in the basal HDL level and a decrease in LDL and IL-10 levels in the blood of healthy subjects. Patients with nonalcoholic steatohepatitis, differing by the TNF gene -308G>A marker genotype, differ by the time course of the markers of hepatocellular damage (ALT, AST), activity of hepatocyte apoptosis (tissue polypeptide-specific antigen), and activation of specific humoral immunity (γ-globulin) in response to therapy with ursodeoxycholic acid in a dose of 10-15 mg/kg over 4-6 weeks...
November 27, 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/29176025/a-newborn-with-combined-pituitary-hormone-deficiency-developing-shock-and-sludge
#11
Yasuhiro Ueda, Hayato Aoyagi, Toshihiro Tajima
A male neonate was born at 41 weeks of gestation with a birth weight of 3320 g. Artificial respiratory management was required due to respiratory disturbance 1 h after birth, and subsequently catecholamine-refractory low cardiac output-induced shock occurred. Severe combined pituitary hormone deficiency (CPHD) was considered based on the presence of his respiratory disturbance, hypoglycemia and micropenis. After hydrocortisone (HDC) administration, circulatory dynamics rapidly improved. Brain magnetic resonance imaging (MRI) showed aplasia of the anterior pituitary gland and ectopic posterior gland...
November 25, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29159718/genetics-and-epigenetics-in-the-pathogenesis-of-primary-biliary-cholangitis
#12
REVIEW
Satoru Joshita, Takeji Umemura, Eiji Tanaka, Masao Ota
Primary biliary cholangitis (PBC) is a chronic, slowly progressive cholestatic autoimmune liver disease predominantly afflicting women. PBC is characterized by the presence of disease-specific antimitochondrial antibodies and the histological destruction of intrahepatic bile ducts, which eventually lead to cirrhosis and hepatic failure. Fortunately, ursodeoxycholic acid therapy has improved the outcome of the vast majority of PBC cases. Although the etiology of PBC has not yet been elucidated, human leukocyte antigen (HLA) class II alleles have been consistently associated with disease onset for decades...
November 20, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29159008/caroli-s-disease-as-a-cause-of-chronic-epigastric-abdominal-pain-two-case-reports-and-a-brief-review-of-the-literature
#13
REVIEW
Pedro Cabral Correia, Bruno Morgado
Caroli's disease is a very rare congenital malformation, currently included in cystic diseases of the biliary tract, and is characterized by ectasia and dilatation of the intrahepatic bile ducts. Two clinical entities can be distinguished, Caroli's disease in which congenital hepatic impairment is limited to cystic dilatation and Caroli's syndrome in which congenital hepatic fibrosis coexists. We present two cases of atypical presentations of Caroli's disease. Case one was a 76-year-old man who was referred to our hospital for chronic non-remitting epigastric pain prior to diagnosis...
September 20, 2017: Curēus
https://www.readbyqxmd.com/read/29158853/diagnosis-and-treatment-of-autoimmune-liver-diseases-in-a-tertiary-referral-center-in-cuba
#14
Marlen Ivón Castellanos Fernández, Deyanira la Rosa Hernández, Diego Enrique Cabrera Eugenio, Wilson Palanca, Zaily Dorta Guridi, Licet González Fabián
Background: Autoimmune liver diseases (AILD) comprise a set of entities characterized by tissue damage as a result of the loss of self-tolerance. There are few reports of AILD from Caribbean countries. Objectives: The aim of our study was to investigate the clinical patterns, laboratory findings, and immunologic features, treatment responses, and prognoses of AILD in adult patients at a Cuban tertiary referral center. Methods: A prospective study was conducted at the National Institute of Gastroenterology in Havana, Cuba, from May 2012 to April 2016...
2017: Current Therapeutic Research, Clinical and Experimental
https://www.readbyqxmd.com/read/29149831/molecular-mechanisms-triggered-by-bile-acids-on-iintestinal-ca2-absorption
#15
Ana Marchionatti, Maria Rivoira, Valeria Rodriguez, Adriana Perez, Nori Tolosa de Talamoni
Bile acids (BAs) are synthesized in the liver and are among the main components of bile. For many years, it was thought that they have only a role as emulsifiers of fat in the small intestine facilitating the absorption of lipids and lipid soluble vitamins. Lately, they are also considered important signaling molecules, not only by regulating their own synthesis, but also having a role in several metabolic diseases. In this review we focus on the effect of deoxycholic, ursodeoxycholic and litocholic acids and their combination upon the intestinal Ca2+ absorption...
November 16, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/29146216/-nisch-syndrome-a-rare-cause-of-neonatal-cholestasis-a-case-report
#16
S Szepetowski, C Lacoste, S Mallet, B Roquelaure, C Badens, A Fabre
NISCH syndrome is a rare autosomal recessive disease. It is characterized by scalp hypotrichosis, scarring alopecia, ichthyosis, and neonatal sclerosing cholangitis. It is caused by mutations in the CLDN1 gene encoding the claudin-1 protein, which is located at tight junctions. Fifteen cases have been reported to date and three different mutations have been identified. We report on the case of a 2-year-old boy from a consanguineous Moroccan family, presenting with NISCH syndrome and carrying the so-called Moroccan homozygous mutation (c...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29140706/a-galactosylated-pro-drug-of-ursodeoxycholic-acid-design-synthesis-characterization-and-pharmacological-effects-in-a-rat-model-of-estrogen-induced-cholestasis
#17
Francesca Di Guida, Claudio Pirozzi, Salvatore Magliocca, Anna Santoro, Adriano Lama, Roberto Russo, Maria Nieddu, Lucia Burrai, Gianpiero Boatto, Maria Pina Mollica, Federica Sodano, Loretta Lazzarato, Konstantin Chegaev, Rosaria Meli, Giuseppina Mattace Raso, Maria Grazia Rimoli
Ursodeoxycholic acid (UDCA) is considered the first-choice therapy for cholestatic disorders. To enhance solubility and exploit specific transporters in liver, we synthesized a new galactosyl pro-drug of UDCA (UDCAgal). Ethinylestradiol (EE)-induced cholestasis was used to study and compare the effects of UDCAgal with UDCA on bile flow, hepatic canalicular efflux transporter expression, and inflammation. UDCAgal resulted quite stable both at pH 7.4 and 1.2 and regenerated the parent drug after incubation in human plasma...
November 15, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/29130098/ursodeoxycholic-acid-suppresses-epithelial-mesenchymal-transition-and-cancer-stem-cell-formation-by-reducing-the-levels-of-peroxiredoxin-ii-and-reactive-oxygen-species-in-pancreatic-cancer-cells
#18
Yoon Jae Kim, Seok Hoo Jeong, Eun-Kyung Kim, Eui Joo Kim, Jae Hee Cho
Reactive oxygen species (ROS) play a key role in cancer development and progression. Ursodeoxycholic acid (UDCA) may possess antioxidant, anti-inflammatory and chemoprophylatic effects. Therefore, we aimed to investigate the effects and mechanisms of UDCA treatment on pancreatic cancer cells. The pancreatic cancer cell lines HPAC and Capan-1 were treated with 0.2 mM UDCA. To examine alterations in the levels of intracellular ROS, the DCF-DA stain was used and both stemness and epithelial-mesenchymal transition (EMT)-related genes were quantified using qRT-PCR and western blot analysis...
December 2017: Oncology Reports
https://www.readbyqxmd.com/read/29119141/case-report-ursodeoxycholic-acid-treatment-in-niemann-pick-disease-type-c-clinical-experience-in-four-cases
#19
William R H Evans, Elena-Raluca Nicoli, Raymond Y Wang, Nina Movsesyan, Frances M Platt
In this case series, we demonstrate that Ursodeoxycholic acid (UDCA) improves liver dysfunction in Niemann-Pick type C (NPC) and may restore a suppressed cytochrome p450 system. NPC disease is a progressive neurodegenerative lysosomal storage disease caused by mutations in either the NPC1 or NPC2 genes. Liver disease is a common feature presenting either acutely as cholestatic jaundice in the neonatal period, or in later life as elevated liver enzymes indicative of liver dysfunction. Recently, an imbalance in bile acid synthesis in a mouse model of NPC disease was linked to suppression of the P450 detoxification system and was corrected by UDCA treatment...
2017: Wellcome Open Research
https://www.readbyqxmd.com/read/29110023/-modern-treatment-of-primary-biliary-cholangitis
#20
REVIEW
C P Strassburg
For nearly 30 years ursodeoxycholic acid (UDCA) represented the only pharmacological treatment option available for primary biliary cholangitis (PBC). This changed at the end of 2016 when obeticholic acid was licensed in Europe for PBC patients not responding to UDCA. Novel treatment concepts involving the modulation of nuclear receptor signaling in cholestatic and other liver diseases have led to a host of new potential options, studies and drug candidates for the treatment of PBC. The analysis of large multinational cohorts has additionally confirmed the effectiveness of UDCA in slowing PBC progression, and has led to the development of new definitions for the risk assessment of PBC patients under therapy, which will be an asset for clinical decision making...
November 6, 2017: Der Internist
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