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E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leukocyte antigen (HLA) allele and haplotype frequencies of the Finnish population are unique because of the restricted and homogenous gene population. There are no published data on HLA genotype associations in paediatric autoimmune liver diseases in Scandinavia. This study characterised the HLA genotypes of children with autoimmune liver or biliary disease in Finland. METHODS: The study cohort comprised 19 paediatric patients (13 female) aged 3-15 years treated for autoimmune liver or biliary disease at the Children's Hospital, Helsinki University Hospital, between 2000 and 2011 and followed up for 4...
October 19, 2016: Acta Paediatrica
Cheal Wung Huh, Sung Ill Jang, Beom Jin Lim, Hee Wook Kim, Jae Keun Kim, Jun Sung Park, Ja Kyung Kim, Se Joon Lee, Dong Ki Lee
Common bile duct (CBD) stones are generally associated with greater elevations of alkaline phosphatase and gamma-glutamyl transpeptidase levels than aspartate aminotransferase and alanine aminotransferase levels. However, some patients with CBD stones show markedly increased aminotransferase levels, sometimes leading to the misdiagnosis of liver disease. Therefore, the aim of this study was to investigate the clinicopathologic features of patients with CBD stones and high aminotransferase levels.This prospective cohort study included 882 patients diagnosed with CBD stones using endoscopic retrograde cholangiopancreatography (ERCP)...
October 2016: Medicine (Baltimore)
Akane Yamabe, Atsushi Irisawa, Ikuo Wada, Goro Shibukawa, Mariko Fujisawa, Ai Sato, Ryo Igarashi, Takumi Maki, Koki Hoshi
Background and study aims: Biliary stent dysfunction is mainly caused by biliary sludge that forms as a result of bacterial adherence and subsequent biofilm formation on the inner surface of the stent. Silver ions arewell known to have excellent antimicrobial activity against a wide range of microorganisms. In this study, we designed and constructed silver-coated plastic stent (PS) and investigated whether the silver coating prevented bacterial adherence and biofilm formation through the use of electron microscopy...
October 2016: Endoscopy International Open
Eiko Hasegawa, Naoki Sawa, Junichi Hoshino, Tatsuya Suwabe, Noriko Hayami, Masayuki Yamanouchi, Akinari Sekine, Rikako Hiramatsu, Aya Imafuku, Masahiro Kawada, Yoshifumi Ubara, Tsunao Imamura, Kenmei Takaichi
We herein present a rare case of an autosomal dominant polycystic kidney disease (ADPKD) patient with Caroli's disease, a congenital embryonic biliary tree ductal plate abnormality often associated with autosomal recessive polycystic kidney disease. A 76-year-old woman with ADPKD on hemodialysis was admitted to our hospital with recurrent cholangitis and hepatobiliary stones. Caroli's disease was diagnosed according to typical imaging findings of cystic intrahepatic bile duct dilatation and the central dot sign...
2016: Internal Medicine
Tomohiro Watanabe, Kouhei Yamashita, Masatoshi Kudo
An increased number of clinicopathological studies on autoimmune pancreatitis, cholangitis, and sialoadenitis have led to the recognition of immunoglobulin G4-related disease (IgG4-RD) as a novel disorder, characterized by elevated levels of serum IgG4 and infiltration of IgG4-expressing plasma cells in the affected organs. Although the immunological background associated with the development of IgG4-RD remains poorly understood, recent studies have suggested involvement of the innate immune response in its pathogenesis...
October 16, 2016: Current Topics in Microbiology and Immunology
Boyang Zheng, Catherine Vincent, Marvin J Fritzler, Jean-Luc Senécal, Martial Koenig, France Joyal
OBJECTIVE: Systemic sclerosis (SSc) is a well-established disease associated with primary biliary cholangitis (PBC). However, the original 1980 American College of Rheumatology (ACR) criteria have poor sensitivity, especially for the detection of earlier SSc in previous studies. The objective was to evaluate the prevalence of SSc in patients with PBC using more sensitive 2001 LeRoy and Medsger criteria and the 2013 ACR/European League Against Rheumatism (EULAR) classification criteria...
October 15, 2016: Journal of Rheumatology
Artur Gião Antunes, Bruno Peixe, Horácio Guerreiro
No abstract text is available yet for this article.
October 13, 2016: Gastroenterología y Hepatología
Nurdan Tözün, Eser Vardareli
Gastrointestinal (GI) cancers are the leading cause of mortality worldwide. These cancers are the end result of a complex interplay between gene and environment. Bacteria, parasites, and viruses have been implicated in some cancers. Recent data have put at focus the gut microbiome as the key player firing tumorigenesis. Experimental and human studies have provided evidence on the role of microbiota in cancer development. Although subject to changes in different settings such as antibiotic treatment, diet or lifestyle, our microbiome is quite stable and is capable of increasing susceptibility to cancer or decrease and halt its progression...
November 2016: Journal of Clinical Gastroenterology
Cynthia Buness, Keith D Lindor, Tamir Miloh
Primary sclerosing cholangitis (PSC), a rare progressive liver disease characterized by cholestasis and bile duct fibrosis, has no accepted, effective therapy known to delay or arrest its progression. We report a 15 year old female patient diagnosed with PSC and moderate chronic active ulcerative colitis (UC) who achieved normalization of her liver enzymes and bile ducts, and resolution of her UC symptoms with colonic mucosal healing, after treatment with a single drug therapy of the antibiotic oral vancomycin...
September 2016: Pediatric Gastroenterology, Hepatology & Nutrition
Memmet Mihmanli, Ufuk Oguz Idiz, Cemal Kaya, Uygar Demir, Ozgur Bostanci, Sinan Omeroglu, Emre Bozkurt
Echinococcus granulosus (E. granulosus) and Echinococcus multilocularis (E. multilocularis) infections are the most common parasitic diseases that affect the liver. The disease course is typically slow and the patients tend to remain asymptomatic for many years. Often the diagnosis is incidental. Right upper quadrant abdominal pain, hepatitis, cholangitis, and anaphylaxis due to dissemination of the cyst are the main presenting symptoms. Ultrasonography is important in diagnosis. The World Health Organization classification, based on ultrasonographic findings, is used for staging of the disease and treatment selection...
October 8, 2016: World Journal of Hepatology
Rodrigo Liberal, Charlotte R Grant
Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues...
October 8, 2016: World Journal of Hepatology
Gen Tohda, Masahiro Ohtani, Masaki Dochin
AIM: To investigate the efficacy and safety of emergency endoscopic retrograde cholangiopancreatography (ERCP) in elderly patients with acute cholangitis. METHODS: From June 2008 to May 2016, emergency ERCPs were performed in 207 cases of acute cholangitis at our institution. Patients were classified as elderly if they were aged 80 years and older (n = 102); controls were under the age of 80 years (n = 105). The patients' medical records were retrospectively reviewed for comorbidities, laboratory data, etiology of cholangitis (presence of biliary stones, biliary stricture and malignancy), details of the ERCP (therapeutic approaches, technical success rates, procedure duration), ERCP-related complications and mortality...
October 7, 2016: World Journal of Gastroenterology: WJG
Hanno Ehlken, Raluca Wroblewski, Christophe Corpechot, Lionel Arrivé, Tim Rieger, Johannes Hartl, Susanne Lezius, Peter Hübener, Kornelius Schulze, Roman Zenouzi, Marcial Sebode, Moritz Peiseler, Ulrike W Denzer, Alexander Quaas, Christina Weiler-Normann, Ansgar W Lohse, Olivier Chazouilleres, Christoph Schramm
BACKGROUND: Patients with primary sclerosing cholangitis (PSC) develop progressive liver fibrosis and end-stage liver disease. Non-invasive and widely available parameters are urgently needed to assess disease stage and the risk of clinical progression. Transient elastography (TE) has been reported to predict fibrosis stage and disease progression. However, these results have not been confirmed in an independent cohort and comparison of TE measurement to other non-invasive means is missing...
2016: PloS One
Bi-Jhen Syu, Chia-En Loh, Yu-Hsin Hsueh, M Eric Gershwin, Ya-Hui Chuang
Primary biliary cirrhosis (PBC) is a progressive autoimmune liver disease with a long natural history. The pathogenesis of PBC is thought to be orchestrated by Th1 and/or Th17. In this study, we investigated the role of CD4(+) helper T subsets and their cytokines on PBC using our previous established murine model of 2-OA-OVA immunization. We prepared adeno-associated virus (AAV)-IFN-γ and AAV-IL-4 and studied their individual influences on the natural history of autoimmune cholangitis in this model. Administration of IFN-γ significantly promotes recruitment and lymphocyte activation in the earliest phases of autoimmune cholangitis but subsequently leads to downregulation of chronic inflammation through induction of the immunosuppressive molecule IL-30...
October 10, 2016: Scientific Reports
Yoshiaki Takahashi, Toshiharu Matsuura, Yusuke Yanagi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND/PURPOSE: There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. METHODS: Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score...
September 26, 2016: Journal of Pediatric Surgery
Piotr Buda, Ryszard Grenda, Anna Wieteska-Klimczak, Piotr Gietka, Lidia Skobejko-Włodarska, Karina Felberg, Janusz Książyk
Eosinophilic cystitis (EC) is a rare inflammatory disorder of the urinary tract characterized by infiltration of bladder with eosinophils. The cause remains unclear, immunological mechanisms have been implicated in pathogenesis. Potential etiological factors include: tumors, allergy, parasitic infections, trauma. The disease may have a variable course, from a mild self-limiting, through common symptoms like: dysuria, hematuria, abdominal pain, tumor, to severe renal failure, with eosinophilic infiltration of the other organs and systemic complications...
2016: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Nicholas L Webb, Ashish Jiwane, Chee Y Ooi, Scott Nightinghale, Susan E Adams, Usha Krishnan
AIM: Biliary atresia (BA) literature has focussed on the relationship between age at Kasai procedure (KP) and post-KP outcomes. This study primarily examines post-KP outcomes including, 6-month normalisation of bilirubin, 5-year native liver survival (NLS), development of portal hypertension (PHT) and incidence of ascending cholangitis at a single tertiary paediatric centre in Australia. The study also evaluated prognostic factors which may influence these aforementioned outcomes. METHODS: Retrospective chart review of all BA cases between 1999 and 2014...
October 7, 2016: Journal of Paediatrics and Child Health
Jesús A Bahena-Aponte, Guillermo Ramírez de Aguilar, Juan Carlos Torres Carrillo, Luis Espino Urbina, Jesús A Sánchez González
We present the case of a 34 years old female patient who presents with abdominal pain and elevated total and direct bilirrubins, so she underwent ERCP Reporting: a) successful sphincterotomy without complications, b) choledocholithias is endoscopically resolved, c) secondary cholangitis. She developed significant abdominal pain at 72 h, with hypovolemic shock and peritoneal irritation. She was taken to the surgery, finding a grade III liver laceration. This one was resolved with liver raffia and packing, during the same operative time cholecystectomy was performed...
July 2016: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Matías E Czerwonko, Pablo Huespe, Santiago Bertone, Pablo Pellegrini, Oscar Mazza, Juan Pekolj, Eduardo de Santibañes, Sung Ho Hyon, Martín de Santibañes
BACKGROUND: In times of modern surgery, transplantation and percutaneous techniques, pyogenic liver abscess (PLA) has essentially become a problem of biliary or iatrogenic origin. In the current scenario, diagnostic approach, clinical behavior and therapeutic outcomes have not been profoundly studied. This study analyzes the clinical and microbiological features, diagnostic methods, therapeutic management and predictive factors for recurrence and mortality of first episodes of PLA. METHODS: A retrospective single-center study was conducted including 142 patients admitted to the Hospital Italiano de Buenos Aires, between 2005 and 2015 with first episodes of PLA...
October 3, 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
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