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Cholestasis

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https://www.readbyqxmd.com/read/28721532/-chronic-cholestatic-liver-diseases-differential-diagnosis-pathogenesis-and-current-treatment-in-adults
#1
S Hohenester, U Beuers
In the long-term course chronic cholestasis regularly leads to fibrotic restructuring and ultimately to functional failure of the liver, independent of the cause. Cholestatic diseases are often clinically asymptomatic. In order to avoid progression, early diagnosis of the underlying disease and a targeted therapy are therefore decisive. The differential diagnoses of chronic cholestasis are broad; therefore, algorithms are of assistance in the diagnostic work-up. A better understanding of the pathogenesis is now leading to the development of new therapeutic agents in addition to ursodeoxycholic acid, which has long been known for its anticholestatic effects...
July 18, 2017: Der Internist
https://www.readbyqxmd.com/read/28717906/undiagnosed-liver-fibrosis-in-patients-undergoing-pancreatoduodenectomy-for-pancreatic-adenocarcinoma
#2
Andrew Gdowski, Houssam Osman, Umar Butt, Steve Foster, Dhiresh Rohan Jeyarajah
BACKGROUND: Chronic obstruction of the biliary system may cause hepatic fibrosis and liver failure. The purpose of this study was to define the incidence of unrecognized liver fibrosis in patients undergoing pancreaticoduodenectomy (PD). METHODS: Retrospective data were collected on patients undergoing PD during a 21-month period. Each patient had a core needle biopsy at the time of surgery by a hepatobiliary surgeon. RESULTS: This study identified 36 consecutive patients who were referred to a tertiary center and underwent pancreatoduodenectomy during a period of 21 months...
July 17, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/28717101/mechanism-based-risk-assessment-strategy-for-drug-induced-cholestasis-using-the-transcriptional-benchmark-dose-derived-by-toxicogenomics
#3
Taisuke Kawamoto, Yuichi Ito, Osamu Morita, Hiroshi Honda
Cholestasis is one of the major causes of drug-induced liver injury (DILI), which can result in withdrawal of approved drugs from the market. Early identification of cholestatic drugs is difficult due to the complex mechanisms involved. In order to develop a strategy for mechanism-based risk assessment of cholestatic drugs, we analyzed gene expression data obtained from the livers of rats that had been orally administered with 12 known cholestatic compounds repeatedly for 28 days at three dose levels. Qualitative analyses were performed using two statistical approaches (hierarchical clustering and principle component analysis), in addition to pathway analysis...
2017: Journal of Toxicological Sciences
https://www.readbyqxmd.com/read/28716071/primary-solitary-plasmacytoma-of-the-liver-successful-treatment-with-fractionated-stereotactic-radiotherapy-cyberknife%C3%A2-a-case-report
#4
Thomas Chalopin, Isabelle Barillot, Jean-Paul Biny, Flavie Arbion, Marie Besson, Maria Santiago-Ribeiro, Eric Piver, Olivier Herault, Emmanuel Gyan, Lotfi Benboubker
BACKGROUND: Solitary plasmacytoma of the liver is a very rare and aggressive form of plasma cell dyscrasia. To the best of our knowledge, very few cases have been reported without systemic disease. We reported a rare case of hepatic solitary plasmacytoma that successfully responded to fractionated stereotactic radiotherapy. CASE PRESENTATION: A 64-year-old white French man had monoclonal gammopathy of the immune globulin G lambda type; he developed a cholestasis and cytolysis with the discovery of a subscapular nodule...
July 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28714273/%C3%AE-catenin-regulation-of-farnesoid-x-receptor-signaling-and-bile-acid-metabolism-during-murine-cholestasis
#5
Michael D Thompson, Akshata Moghe, Pamela Cornuet, Rebecca Marino, Jianmin Tian, Pengcheng Wang, Xiaochao Ma, Marc Abrams, Joseph Locker, Satdarshan P S Monga, Kari Nejak-Bowen
Cholestatic liver diseases result from impaired bile flow and are characterized by inflammation, atypical ductular proliferation (ADP), and fibrosis. The Wnt/β-catenin pathway plays a role in bile duct development, yet its role in cholestatic injury remains indeterminate. Liver-specific β-catenin knockout (KO) mice and wild-type (WT) littermates were subjected to cholestatic injury via bile duct ligation or short-term exposure to 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) diet. Intriguingly, KO exhibit a dramatic protection from liver injury, fibrosis, and ADP, which coincided with significantly decreased total hepatic bile acids (BA)...
July 17, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28712532/influence-of-different-intravenous-lipid-emulsions-on-growth-development-and-laboratory-and-clinical-outcomes-in-hospitalised-paediatric-patients-a-systematic-review
#6
REVIEW
Roy-Rosshan Edward, Jacqueline K Innes, Luise V Marino, Philip C Calder
BACKGROUND & AIMS: Fats in the form of lipid emulsions (LEs) are an integral part of intravenous nutrition. The fatty acid composition of different LEs varies. The exact composition of a LE may influence cell and tissue function and clinical outcome. Currently, it is not clear which LE might be best for paediatric patients. We conducted a systematic review of the effects of different intravenous LEs in hospitalised paediatric patients. METHODS: Randomised controlled trials published in a peer reviewed journal, written in the English language, and comparing two or more different intravenous LEs in hospitalised paediatric patients were included...
July 8, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28710680/development-of-a-novel-tool-to-assess-the-impact-of-itching-in-pediatric-cholestasis
#7
Binita M Kamath, Linda Abetz-Webb, Ciara Kennedy, Bonnie Hepburn, Martha Gauthier, Nathan Johnson, Sharon Medendorp, Alejandro Dorenbaum, Lora Todorova, Benjamin L Shneider
OBJECTIVES: The aim was to develop a clinical outcome assessment (COA) for itching in children with cholestatic pruritus. METHODS: This prospective study aimed to enroll patients aged 4-30 years with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis type 1 and caregivers of patients aged 5 months to 14 years. Eligible patients experienced itching during ≥3 of the 7 days before enrollment and had not undergone liver transplant or surgical interruption of the enterohepatic circulation...
July 14, 2017: Patient
https://www.readbyqxmd.com/read/28709963/animal-models-of-biliary-injury-and-altered-bile-acid-metabolism
#8
REVIEW
Valeria Mariotti, Mario Strazzabosco, Luca Fabris, Diego F Calvisi
In the last 25years, a number of animal models, mainly rodents, have been generated with the goal to mimic cholestatic liver injuries and, thus, to provide in vivo tools to investigate the mechanisms of biliary repair and, eventually, to test the efficacy of innovative treatments. Despite fundamental limitations applying to these models, such as the distinct immune system and the different metabolism regulating liver homeostasis in rodents when compared to humans, multiple approaches, such as surgery (bile duct ligation), chemical-induced (3,5-diethoxycarbonyl-1,4-dihydrocollidine, DDC, α-naphthylisothiocyanate, ANIT), viral infections (Rhesus rotavirustype A, RRV-A), and genetic manipulation (Mdr2, Cftr, Pkd1, Pkd2, Prkcsh, Sec63, Pkhd1) have been developed...
July 11, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28702139/ramipril-associated-cholestasis-in-the-setting-of-recurrent-drug-induced-liver-injury
#9
David Forner, Tasha Kulai, Thomas Arnason, Steven E Gruchy, Magnus MacLeod
Aim: Angiotensin-converting enzyme inhibitors (ACEIs) are commonly used to treat hypertension. Although generally well tolerated, the adverse effects of ACEIs include hypotension, cough, acute kidney injury and hyperkalemia. Rare reports of ACEI-induced hepatotoxicity have been described, most notably a cholestatic pattern of injury related to captopril. A 67-year-old male presented to the emergency department with a three-week history of jaundice, pruritis and weakness. Eight weeks before, he began taking ramipril and clopidogrel...
2017: Gastroenterology and Hepatology From Bed to Bench
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#10
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28698093/bile-acids-and-intestinal-microbiota-in-autoimmune-cholestatic-liver-diseases
#11
REVIEW
You Li, Ruqi Tang, Patrick S C Leung, M Eric Gershwin, Xiong Ma
Cholestatic liver diseases, including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), are manifested as an impairment of normal bile flow and excessive accumulation of potentially toxic bile acids. Endogenous bile acids are involved in the pathogenesis and progression of cholestasis. Consequently, chronic cholestasis affects the expression of bile acids transporters and nuclear receptors, and results in liver injury. Several lines of evidence suggest that intestinal microbiota plays an important role in the etiopathogenesis of cholestatic liver diseases by regulating metabolism and immune responses...
July 8, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28697823/-clinical-feature-and-genetic-analysis-of-a-family-affected-by-congenital-bile-acid-synthesis-defect-type-2-identification-of-2-novel-mutations-in-akr1d1-gene
#12
Ying Cheng, Li Guo, Mei Deng, Yuan-Zong Song
Congenital bile acid synthesis defect type 2 (CBAS2) is an autosomal recessive disorder caused by biallelic mutations of AKR1D1 gene, which encodes the Δ4-3-oxo-steroid 5β-reductase. Cholestatic jaundice is the main clinical manifestation, accompanied by malabsorption of fat and fat-soluble vitamins. This paper reported the clinical and genetic features of a CBAS2 patient definitely diagnosed by AKR1D1 genetic analysis. An 8-month-old male infant was referred to the hospital with the complaint of jaundiced skin and sclera over 7 months...
July 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28697498/effect-of-histone-deacetylase-hdac3-on-cytokines-il-18-il-12-and-tnf-%C3%AE-in-patients-with-intrahepatic-cholestasis-of-pregnancy
#13
Yong Shao, Jing Chen, Jiao Zheng, Cai-Ru Liu
BACKGROUND/AIMS: The pathogenesis of intrahepatic cholestasis of pregnancy (ICP) is poorly understood. OBJECTIVE: This study aimed to explore the possible effect of HDAC3 (histone deacetylase) on cytokines IL-18, IL-12 and TNF-α in ICP. METHODS: Serum levels of cytokines IL-18, IL-12 and TNF-α, bile acids and hepatic function parameters were measured. The expression of HDAC3 in the placenta was determined by immunohistochemistry (IHC), western blotting and RT-PCR...
July 11, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28696845/decompensated-cirrhosis-and-sickle-cell-disease-case-reports-and-review-of-the-literature
#14
Roberta D'Ambrosio, Marco Maggioni, Maria F Donato, Pietro Lampertico, Maria D Cappellini, Giovanna Graziadei
Although its prevalence is unknown, liver involvement by sickle cell disease is not uncommon and encompasses different clinical spectra including non cholestatic and cholestatic disorders. Few data have been provided on chronic sickle cell intrahepatic cholestasis (SCIC) clinical course, although cirrhosis has been reported in sickle cell disease. However, no effective therapeutic approaches have been recognized either to prevent or treat this condition. Here we present two cases of adult sickle cell disease patients with decompensated cirrhosis...
March 2017: Hemoglobin
https://www.readbyqxmd.com/read/28694659/induction-of-chronic-cholestasis-without-liver-cirrhosis-creation-of-an-animal-model
#15
Felix Dondorf, René Fahrner, Michael Ardelt, Eleonora Patsenker, Felix Stickel, Uta Dahmen, Utz Settmacher, Falk Rauchfuß
AIM: To analyze time intervals of inflammation and regeneration in a cholestatic rat liver model. METHODS: In 36 Lewis rats, divided into six groups of 6 animals (postoperative observation periods: 1, 2, 3, 4, 6, 8 wk), the main bile duct was ligated with two ligatures and observed for the periods mentioned above. For laboratory evaluation, cholestasis parameters (bilirubin, γ-GT), liver cell parameters (ASAT, ALAT) and liver synthesis parameters (quick, albumin) were determined...
June 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28691322/bile-acid-levels-and-risk-of-adverse-perinatal-outcomes-in-intrahepatic-cholestasis-of-pregnancy-a-meta-analysis
#16
Donghua Cui, Yongxing Zhong, Lin Zhang, Hechun Du
AIM: We aimed to determine the association between maternal total bile acid (TBA) levels and the risks of adverse perinatal outcomes in pregnant women with intrahepatic cholestasis of pregnancy (ICP) based on a meta-analysis study. METHODS: We searched PubMed for articles published from 2000 to 2015 with a focus on ICP and restriction to the English language. The main perinatal outcomes were preterm birth (PTB), meconium-stained amniotic fluid (MSAF), asphyxia, or respiratory distress syndrome (RDS)...
July 10, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28689603/small-bowel-dilation-in-children-with-short-bowel-syndrome-is-associated-with-mucosal-damage-bowel-derived-bloodstream-infections-and-hepatic-injury
#17
Maria Hukkinen, Annika Mutanen, Mikko P Pakarinen
BACKGROUND: Liver disease occurs frequently in short bowel syndrome. Whether small bowel dilation in short bowel syndrome could influence the risk of liver injury through increased bacterial translocation remains unknown. Our aim was to analyze associations between small bowel dilation, mucosal damage, bloodstream infections, and liver injury in short bowel syndrome patients. METHODS: Among short bowel syndrome children (n = 50), maximal small bowel diameter was measured in contrast series and expressed as the ratio to the height of the fifth lumbar vertebra (small bowel diameter ratio), and correlated retrospectively to fecal calprotectin and plasma citrulline-respective markers of mucosal inflammation and mass-bloodstream infections, liver biochemistry, and liver histology...
July 6, 2017: Surgery
https://www.readbyqxmd.com/read/28688656/notch-signaling-promotes-ductular-reactions-in-biliary-atresia
#18
Jessica A Zagory, William Dietz, Alex Park, Michael Fenlon, Jiabo Xu, Sarah Utley, Nirmala Mavila, Kasper S Wang
BACKGROUND: Biliary atresia (BA) is a congenital, progressive, fibro-obliterative disease of the extrahepatic biliary tree and the most common cause of end-stage liver disease in children. BA is characterized by extensive intrahepatic proliferating ductular reactions that may contribute to biliary fibrosis. Lineage tracing during experimental cholestasis indicates that cells within ductular reactions derive from PROM1-expressing hepatic progenitor cells. Given the role of Notch signaling in normal biliary development, we hypothesize that activated Notch signaling promotes the formation of ductular reactions in BA...
July 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28687948/role-of-hepatobiliary-scintigraphy-and-preoperative-liver-biopsy-for-exclusion-of-biliary-atresia-in-neonatal-cholestasis-syndrome
#19
Ankur Mandelia, Richa Lal, Nijagal Mutt
All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder...
July 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28685316/-liver-biopsy-in-children-and-adolescents-preliminary-morphological-examinations-in-diffuse-liver-disease
#20
REVIEW
C Lackner, A S Knisely
BACKGROUND: Frequencies of types of liver disease differ between adults and nonadults (NA). OBJECTIVES: The particular problems encountered in interpreting liver-biopsy findings in NA often require referral in consultation.  To permit this efficiently, we recommend specific approaches to light microscopy, with special stains and immunohistochemistry, as well as to ultrastructural study. METHODS: Prosection and the choice of special stains are described, and are discussed in relation to clinical questions...
July 2017: Der Pathologe
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