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https://www.readbyqxmd.com/read/28943567/a-rare-case-of-ureteral-carcinoma-with-recurrence-in-the-duodenum-requiring-double-stenting
#1
Hiroyuki Matsubayashi, Hirotoshi Ishiwatari, Masaki Tanaka, Tomohiro Iwai, Toru Matsui, Shinya Fujie, Naomi Kakushima, Sayo Ito, Ryo Yamashita, Masato Abe, Keiko Sasaki, Hiroyuki Ono
A 61-year-old man who had undergone total nephrouretectomy eight months earlier for right ureteral carcinoma was referred for the investigation of elevated serum hepatobiliary enzymes. Computed tomography revealed a small mass invading the lower bile duct. Duodenoscopy revealed a central ulcerative tumor near the major papilla, and a biopsy histologically confirmed metastatic ureteral carcinoma. Endoscopic biliary stenting ameliorated the cholangitis, and gemcitabine-based chemotherapy was initiated. The patient was stable for a year until a duodenal stenosis developed and required duodenal stenting...
September 25, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28943450/pathological-features-of-primary-sclerosing-cholangitis-identified-by-bile-proteomic-analysis
#2
C Rupp, K A Bode, Y Leopold, P Sauer, D N Gotthardt
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. Previous bile proteomic analyses in patients with PSC have revealed changes in disease activity specific to malignant transformation. In this study, we established a reference bile duct-derived bile proteome for PSC that can be used to evaluate biliary pathophysiology. Samples were collected from patients with PSC or with choledocholithiasis (control) (n=6 each). Furthermore, patients with PSC-associated cholangiocarcinoma (CC) and with CC without concomitant PSC were analyzed...
September 21, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28941329/tokyo-guidelines-2018-initial-management-of-acute-biliary-infection-and-flowchart-for-acute-cholangitis
#3
Fumihiko Miura, Kohji Okamoto, Tadahiro Takada, Steven M Strasberg, Horacio J Asbun, Henry A Pitt, Harumi Gomi, Joseph S Solomkin, David Schlossberg, Ho-Seong Han, Myung-Hwan Kim, Tsann-Long Hwang, Miin-Fu Chen, Wayne Shih-Wei Huang, Seiki Kiriyama, Takao Itoi, O James Garden, Kui-Hin Liau, Akihiko Horiguchi, Keng-Hao Liu, Cheng-Hsi Su, Dirk Joan Gouma, Giulio Belli, Christos Dervenis, Palepu Jagannath, Angus C W Chan, Wan Yee Lau, Itaru Endo, Kenji Suzuki, Yoo-Seok Yoon, Eduardo de Santibañes, Mariano Eduardo Giménez, Eduard Jonas, Harjit Singh, Goro Honda, Koji Asai, Yasuhisa Mori, Keita Wada, Ryota Higuchi, Manabu Watanabe, Toshiki Rikiyama, Naohiro Sata, Nobuyasu Kano, Akiko Umezawa, Shuntaro Mukai, Hiromi Tokumura, Jiro Hata, Kazuto Kozaka, Yukio Iwashita, Taizo Hibi, Masamichi Yokoe, Taizo Kimura, Seigo Kitano, Masafumi Inomata, Koichi Hirata, Yoshinobu Sumiyama, Kazuo Inui, Masakazu Yamamoto
The initial management of patients with suspected acute biliary infection starts with the measurement of vital signs to assess whether or not the situation is urgent. If the case is judged to be urgent, initial medical treatment should be started immediately including respiratory/circulatory management if required, without waiting for the definitive diagnosis. The patient's medical history is then taken; an abdominal examination is performed; blood tests, urinalysis, and diagnostic imaging are carried out; and a diagnosis is made using the diagnostic criteria for cholangitis/cholecystitis...
September 23, 2017: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/28941291/dysregulation-of-circulating-tfr-tfh-ratio-in-primary-biliary-cholangitis
#4
Jiajia Zheng, Tiancheng Wang, Li Zhang, Liyan Cui
Follicular helper T (Tfh) cells and follicular regulatory T (Tfr) cells are critical for the development and maintenance of germinal center (GC) and humoral immune responses. Accumulating evidence has demonstrated that the dysregulation of either Tfh cells or Tfr cells contributes to the pathogenesis of autoimmune diseases. We aim to investigate the roles of circulating Tfh cells and circulating Tfr cells in the pathogenesis of primary biliary cholangitis (PBC). 34 PBC patients and 27 health individuals were enrolled in this study...
September 23, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28940041/a-review-of-long-term-outcome-and-quality-of-life-of-patients-after-kasai-operation-surviving-with-native-livers
#5
REVIEW
Kenneth K Y Wong, Carol W Y Wong
Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy...
September 22, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28940004/adjuvant-therapy-in-biliary-atresia-hopelessly-optimistic-or-potential-for-change
#6
REVIEW
Mark Davenport
Given that the aetiology of biliary atresia (BA) is complex and that there is a multiplicity of possible pathogenic mechanisms then it is perhaps not surprising that the evidence for effect of a number of different agents is contradictory. Post-operative cholangitis for instance is common, bacterial in origin and various antibiotic regimens have been tested (although none in a randomized trial) but continuation beyond the early post-operative period does not appear to offer any greater protection. There is an inflammatory reaction in about 25-35% of cases of BA illustrated by abnormal expression of class II antigen and upregulation of ICAM, VCAM and E-selectin with an infiltrate of immune-activated T cells (predominantly CD4 + Th1 and Th17) and NK cells and a systemic surge in inflammatory cytokines (e...
September 22, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28939398/the-emergency-medicine-focused-review-of-cholangitis
#7
Rachel Ely, Brit Long, Alex Koyfman
BACKGROUND: Cholangitis is a life-threatening infection of the biliary tract. Historically, the mortality secondary to cholangitis approached 100%. However, with early recognition, antibiotics, resuscitation, and surgical or endoscopic intervention, patient outcomes have significantly improved, although there is still progress to be made. OBJECTIVE OF REVIEW: The objective of this review is to provide an emergency medicine-centered approach to the risk factors, presentations, and various diagnostic and treatment modalities in cholangitis...
September 19, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28936401/assessment-of-the-visual-analogue-score-in-the-evaluation-of-the-pruritus-of-cholestasis
#8
Nora V Bergasa, E Anthony Jones
Background and Aims: A visual analogue score (VAS), based on application of a visual analogue scale, has been widely used to assess pruritus in clinical studies of patients with cholestatic liver disease. A VAS is a numerical score of the severity of the perception of pruritus, and, hence, is inherently subjective. The objective of this study was to assess the reliability of a VAS as an index of pruritus in cholestatic patients. Methods: In 8 patients with chronic pruritus due to primary biliary cholangitis, values for a VAS of pruritus were compared with corresponding measurements of scratching activity, which were generated by a monitoring system specifically designed to quantitate this activity...
September 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/28933417/serum-wisteria-floribunda-agglutinin-positive-mac-2-binding-protein-could-not-always-predict-early-cirrhosis-in-non-viral-liver-diseases
#9
Yuki Haga, Tatsuo Kanda, Reina Sasaki, Masato Nakamura, Koji Takahashi, Shuang Wu, Shin Yasui, Makoto Arai, Shingo Nakamoto, Osamu Yokosuka
BACKGROUND: Wisteria floribunda agglutinin-positive human Mac-2-binding protein (WFA(+)-M2BP) is a novel non-invasive marker of liver fibrosis. The goal of the study was to investigate whether the novel serum biomarker WFA(+)-M2BP or other non-invasive markers are useful for the prediction of liver fibrosis in patients with nonalcoholic steatohepatitis (NASH), autoimmune hepatitis (AIH), and primary biliary cholangitis (PBC). METHODS: We examined a significant correlation between serum WFA(+)-M2BP levels and histological staging of fibrosis in several chronic liver diseases, such as NASH, AIH, and PBC...
December 14, 2016: Diseases (Basel)
https://www.readbyqxmd.com/read/28932607/percutaneous-endoscopic-management-for-oriental-cholangiohepatitis-a-case-report-and-a-brief-review-of-the-literature
#10
Khalil Aloreidi, Prince Sethi, Terry Yeager, Muslim Atiq
Oriental cholangiohepatitis (OCH) is a disease characterized by intrabiliary pigment stone formation, resulting in recurrent bouts of cholangitis. OCH is found mostly in Southeast Asia but it is occasionally recognized in Western societies. OCH etiology is largely unknown. We report our experience with a patient who presented with acute cholecystitis. Following laparoscopic cholecystectomy, she developed acute cholangitis due to multiple biliary tree stones. She underwent ERCP to clear the stones from common bile duct...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28926118/chop-induced-loss-of-intestinal-epithelial-stemness-contributes-to-bile-duct-ligation-induced-cholestatic-liver-injury
#11
Runping Liu, Xiaojiaoyang Li, Zhiming Huang, Derrick Zhao, Bhagyalaxmi Sukka Ganesh, Guanhua Lai, William M Pandak, Phillip B Hylemon, Jasmohan S Bajaj, Arun J Sanyal, Huiping Zhou
Impaired intestinal barrier function promotes the progression of various liver diseases including cholestatic liver disease. The close association of primary sclerosing cholangitis (PSC) with inflammatory bowel disease highlights the importance of the gut-liver axis. It has been reported that bile duct ligation (BDL)-induced liver fibrosis is significantly reduced in C/EBP homologous protein knock out (CHOP(-/-) ) mice. However, the underlying mechanisms remain unclear. In the current study, we demonstrate that BDL induces striking and acute hepatic ER stress responses after 1 day, which return to normal after 3 days...
September 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28922472/mirna-506-promotes-primary-biliary-cholangitis-like-features-in-cholangiocytes-and-immune-activation
#12
Oihane Erice, Patricia Munoz-Garrido, Javier Vaquero, Maria J Perugorria, Maite G Fernandez-Barrena, Elena Saez, Alvaro Santos-Laso, Ander Arbelaiz, Raul Jimenez-Agüero, Joaquin Fernandez-Irigoyen, Enrique Santamaria, Verónica Torrano, Arkaitz Carracedo, Meenakshisundaram Ananthanarayanan, Marco Marzioni, Jesus Prieto, Ulrich Beuers, Ronald P Oude Elferink, Nicholas F LaRusso, Luis Bujanda, Jose J G Marin, Jesus M Banales
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease associated with autoimmune phenomena targeting intrahepatic bile duct cells (cholangiocytes). Although PBC etiopathogenesis still remains obscure, development of anti-mitochondrial auto-antibodies against pyruvate dehydrogenase complex-E2 (PDC-E2) is a common feature. MicroRNA (miR) dysregulation occurs in liver and immune cells of PBC patients, but their functional relevance is largely unknown. We previously reported that miR-506 is overexpressed in PBC cholangiocytes and directly targets both Cl(-) /HCO3(-) anion exchanger 2 (AE2) and type III inositol 1,4,5-trisphosphate receptor (InsP3R3), leading to cholestasis...
September 18, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28922436/genetic-risk-factors-for-autoimmune-thyroid-disease-might-affect-the-susceptibility-to-and-modulate-the-progression-of-primary-biliary-cholangitis
#13
Aleksander Kuś, Magdalena Arłukowicz-Grabowska, Konrad Szymański, Ewa Wunsch, Małgorzata Milkiewicz, Rafał Płoski, Zakera Shums, Gary L Norman, Piotr Milkiewicz, Tomasz Bednarczuk, Marcin Krawczyk
BACKGROUND AND AIMS: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD). METHODS: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28921801/complications-symptoms-quality-of-life-and-pregnancy-in-cholestatic-liver-disease
#14
Kais Zakharia, Anilga Tabibian, Keith D Lindor, James H Tabibian
Cholestatic liver diseases (CLDs) encompass a variety of disorders of bile formation and/or flow which generally result in progressive hepatobiliary injury and ultimately end-stage liver disease. Many patients with CLD are diagnosed between the ages of 20-50 years, a particularly productive period of life professionally, biologically, and in other respects; it is not surprising, thus, that CLD is often associated with impaired health-related quality of life (HRQOL) and uncertainty regarding implications for and outcomes of pregnancy...
September 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28921595/the-interplay-of-type-i-and-type-ii-interferons-in-murine-autoimmune-cholangitis-as-a-basis-for-sex-biased-autoimmunity
#15
Heekyong R Bae, Deborah L Hodge, Guo-Xiang Yang, Patrick S C Leung, Sathi Babu Chodisetti, Julio C Valencia, Michael Sanford, John M Fenimore, Ziaur S M Rahman, Koichi Tsuneyama, Gary L Norman, M Eric Gershwin, Howard A Young
We have reported a murine model of autoimmune cholangitis, generated by altering the AU rich element by deletion of the IFN-γ 3'UTR region (coined ARE-Del(-/-) ), that has striking similarities to human primary biliary cholangitis (PBC) with female predominance. Previously, we suggested that the gender bias of autoimmune cholangitis was secondary to intense and sustained type I and II IFN signaling. Based on this thesis, and to define the mechanisms that lead to portal inflammation, we specifically addressed the hypothesis that type I IFNs are the driver of this disease...
September 16, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#16
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28917018/temporal-trends-in-utilization-and-outcomes-of-endoscopic-retrograde-cholangiopancreatography-in-acute-cholangitis-due-to-choledocholithiasis-from-1998-to-2012
#17
Malav P Parikh, Niyati M Gupta, Prashanthi N Thota, Rocio Lopez, Madhusudhan R Sanaka
BACKGROUND AND AIMS: Expeditious endoscopic retrograde cholangiopancreatography (ERCP) in acute cholangitis with biliary decompression is associated with better outcomes. In this study, we evaluated the temporal trends of ERCP utilization and healthcare outcomes among patients hospitalized with acute cholangitis due to choledocholithiasis (CDC) from 1998 to 2012. METHODS: We identified patients with a combined diagnosis of cholangitis and choledocholithiasis from the national inpatient sample database...
September 15, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#18
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28913623/updated-strategies-for-the-management-pathogenesis-and-molecular-genetics-of-different-forms-of-ichthyosis-syndromes-with-prominent-hair-abnormalities
#19
REVIEW
Madiha Rasheed, Shaheen Shahzad, Afifa Zaeem, Imran Afzal, Asma Gul, Sumbal Khalid
Syndromic ichthyosis is rare inherited disorders of cornification with varied disease complications. This disorder appears in seventeen subtypes associated with severe systematic manifestations along with medical, cosmetic and social problems. Syndromic ichthyosis with prominent hair abnormalities covers five major subtypes: Netherton syndrome, trichothiodystrophy, ichthyosis hypotrichosis syndrome, ichthyosis hypotrichosis sclerosing cholangitis and ichthyosis follicularis atrichia photophobia syndrome. These syndromes mostly prevail in high consanguinity states, with distinctive clinical features...
September 14, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28913620/a-brief-review-on-prognostic-models-of-primary-biliary-cholangitis
#20
REVIEW
Sha Chen, Weijia Duan, Hong You, Jidong Jia
Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts. If left untreated, PBC may eventually result in end-stage liver disease. For better management of PBC and optimal allocation of medical resources, it is pivotal to accurately estimate the prognosis of patients with PBC. This article will briefly review the models that predict long-term outcome of PBC patients, with special focus on the applicability, strengths and limitations of the widely used models reported from 1983 to 2016...
September 14, 2017: Hepatology International
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