Value of brush cytology for dominant strictures in primary sclerosing cholangitis

NARCIS (Netherlands)

Ponsioen, C. Y.; Vrouenraets, S. M.; van Milligen de Wit, A. W.; Sturm, P.; Tascilar, M.; Offerhaus, G. J.; Prins, M.; Huibregtse, K.; Tytgat, G. N.

1999-01-01

Around 10% of patients with primary sclerosing cholangitis (PSC) develop cholangiocarcinoma, which is cholangiographically often indistinguishable from a benign dominant stricture. The aim of the present study was to assess the value of brush cytology in discriminating between benign and malignant

Primary sclerosing cholangitis and pregnancy

Directory of Open Access Journals (Sweden)

Casper Q. Kammeijer

2011-08-01

Full Text Available Primary sclerosing cholangitis is a progressive disease, and coincidentally in pregnancy it is rare. It is characterized by progressive inflammation and destruction of bile ducts finally resulting in liver failure. A rare case of primary sclerosing cholangitis in pregnancy is presented. The course of the pregnancy was marked by threatened preterm delivery and exacerbation of cholestasis. She was successfully treated with ursodeoxycholic acid (UDCA. Although, primary sclerosing cholangitis has both maternal and fetal effects on pregnancy, the overall outcome is favorable. Only few cases have been reported using high dose ursodeoxycholic acid for primary sclerosing cholangitis in pregnancy, it often improves pruritus but has no protection against stillbirth. Data on the safety to the fetus or neonate and long-term outcome are scarce.

Glucocorticosteroids for primary sclerosing cholangitis

DEFF Research Database (Denmark)

Giljaca, Vanja; Poropat, Goran; Stimac, Davor

2010-01-01

Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and ultimately, liver cirrhosis...... sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids, and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis...

Radiological diagnosis of primary sclerosing cholangitis: value of ERC and CT

International Nuclear Information System (INIS)

Kollmann, F.D.; Maeurer, J.; Hintze, R.E.; Adler, A.; Veltzke, W.; Lohmann, R.; Felix, R.

1994-01-01

To evaluate the use of computed tomography (CT) in primary sclerosing cholangitis (PSC) as compared to endoscopic-retrograde cholangiography (ERC), imaging studies of 24 patients were reviewed. 19 patients were studied by ERC, 19 by CT and 14 by both. In 17 cases, ERC confirmed PSC. One cholangiogram suggested a tumor. CT reflected PSC in only 11 cases, while three patients displayed a mass lesion. Thus, ERC remains the standard imaging technique for diagnosing PSC, whereas CT proves beneficial in excluding hepatic masses. (orig.) [de

Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

NARCIS (Netherlands)

Weismueller, Tobias J.; Trivedi, Palak J; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Faerkkilae, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew L.; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; de Valle, Maria Benito; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We

Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

NARCIS (Netherlands)

Weismuller, Tobias J.; Trivedi, Palak J.; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Farkkila, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; Benito de Valle, Maria; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D.; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

2017-01-01

Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. We performed a retrospective

Impact of Microbes on the Pathogenesis of Primary Biliary Cirrhosis (PBC and Primary Sclerosing Cholangitis (PSC

Directory of Open Access Journals (Sweden)

Jochen Mattner

2016-11-01

Full Text Available Primary biliary cirrhosis (PBC and primary sclerosing cholangitis (PSC represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is considered to be a classical autoimmune disease, as PSC and PBC patients do not respond to immune-suppressants. Furthermore, the decreased bile flow resulting from the immune-mediated tissue assault and the subsequent accumulation of toxic bile products in PBC and PSC not only perpetuates biliary epithelial damage, but also alters the composition of the intestinal and biliary microbiota and its mutual interactions with the host. Consistent with the close association of PSC and inflammatory bowel disease (IBD, the polyclonal hyper IgM response in PBC and (auto-antibodies which cross-react to microbial antigens in both diseases, an expansion of individual microbes leads to shifts in the composition of the intestinal or biliary microbiota and a subsequent altered integrity of epithelial layers, promoting microbial translocation. These changes have been implicated in the pathogenesis of both devastating disorders. Thus, we will discuss here these recent findings in the context of novel and alternative therapeutic options.

Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis

NARCIS (Netherlands)

Liu, Jimmy Z.; Hov, Johannes Roksund; Folseraas, Trine; Ellinghaus, Eva; Rushbrook, Simon M.; Doncheva, Nadezhda T.; Andreassen, Ole A.; Weersma, Rinse K.; Weismüller, Tobias J.; Eksteen, Bertus; Invernizzi, Pietro; Hirschfield, Gideon M.; Gotthardt, Daniel Nils; Pares, Albert; Ellinghaus, David; Shah, Tejas; Juran, Brian D.; Milkiewicz, Piotr; Rust, Christian; Schramm, Christoph; Müller, Tobias; Srivastava, Brijesh; Dalekos, Georgios; Nöthen, Markus M.; Herms, Stefan; Winkelmann, Juliane; Mitrovic, Mitja; Braun, Felix; Ponsioen, Cyriel Y.; Croucher, Peter J. P.; Sterneck, Martina; Teufel, Andreas; Mason, Andrew L.; Saarela, Janna; Leppa, Virpi; Dorfman, Ruslan; Alvaro, Domenico; Floreani, Annarosa; Onengut-Gumuscu, Suna; Rich, Stephen S.; Thompson, Wesley K.; Schork, Andrew J.; Næss, Sigrid; Thomsen, Ingo; Mayr, Gabriele; König, Inke R.; Hveem, Kristian; Cleynen, Isabelle; Gutierrez-Achury, Javier; Ricaño-Ponce, Isis; van Heel, David; Björnsson, Einar; Sandford, Richard N.; Durie, Peter R.; Melum, Espen; Vatn, Morten H.; Silverberg, Mark S.; Duerr, Richard H.; Padyukov, Leonid; Brand, Stephan; Sans, Miquel; Annese, Vito; Achkar, Jean-Paul; Boberg, Kirsten Muri; Marschall, Hanns-Ulrich; Chazouillères, Olivier; Bowlus, Christopher L.; Wijmenga, Cisca; Schrumpf, Erik; Vermeire, Severine; Albrecht, Mario; Rioux, John D.; Alexander, Graeme; Bergquist, Annika; Cho, Judy; Schreiber, Stefan; Manns, Michael P.; Färkkilä, Martti; Dale, Anders M.; Chapman, Roger W.; Lazaridis, Konstantinos N.; Franke, Andre; Anderson, Carl A.; Karlsen, Tom H.

2013-01-01

Primary sclerosing cholangitis (PSC) is a severe liver disease of unknown etiology leading to fibrotic destruction of the bile ducts and ultimately to the need for liver transplantation. We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped

No negative impact of serum IgG4 levels on clinical outcome in 435 patients with primary sclerosing cholangitis from Japan.

Science.gov (United States)

Tanaka, Atsushi; Tazuma, Susumu; Nakazawa, Takahiro; Isayama, Hiroyuki; Tsuyuguchi, Toshio; Inui, Kazuo; Takikawa, Hajime

2017-04-01

Several studies have demonstrated that elevated serum IgG4 levels are associated with poor outcomes of primary sclerosing cholangitis (PSC), but the impact of serum IgG4 levels on PSC remains controversial. In this study, we aimed to determine prognostic factors of patients with PSC and to investigate the association between serum IgG4 levels and the clinical features and prognosis of PSC in a Japanese cohort. We retrospectively analyzed follow-up data for 435 patients with PSC (UMIN000018438). Patients with distinct etiologies of sclerosing cholangitis including IgG4-related sclerosing cholangitis (IgG4-SC) were excluded from this study. Serum IgG4 levels were tested at the time of diagnosis in 216 of 435 patients with PSC, and were elevated in 27 patients (>134 mg/dl, 12.5%). Clinical features at diagnosis were comparable between patients with normal and elevated serum IgG4 levels, with the exception of serum albumin. The overall and liver-transplantation free survival rate was comparable between the groups. Multivariate analysis indicated that age, albumin, and bilirubin, but not IgG4, at the time of diagnosis affected PSC prognosis. The current study showed that serum IgG4 levels at diagnosis do not affect PSC prognosis in a Japanese cohort that excluded patients with IgG4-SC. © 2017 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

Bile acids for primary sclerosing cholangitis

DEFF Research Database (Denmark)

Chen, Weikeng; Gluud, C

2003-01-01

Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear.......Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear....

Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis

NARCIS (Netherlands)

Liu, Jimmy Z.; Hov, Johannes Roksund; Folseraas, Trine; Ellinghaus, Eva; Rushbrook, Simon M.; Doncheva, Nadezhda T.; Andreassen, Ole A.; Weersma, Rinse K.; Weismueller, Tobias J.; Eksteen, Bertus; Invernizzi, Pietro; Hirschfield, Gideon M.; Gotthardt, Daniel Nils; Pares, Albert; Ellinghaus, David; Shah, Tejas; Juran, Brian D.; Milkiewicz, Piotr; Rust, Christian; Schramm, Christoph; Mueller, Tobias; Srivastava, Brijesh; Dalekos, Georgios; Noethen, Markus M.; Herms, Stefan; Winkelmann, Juliane; Mitrovic, Mitja; Braun, Felix; Ponsioen, Cyriel Y.; Croucher, Peter J. P.; Sterneck, Martina; Teufel, Andreas; Mason, Andrew L.; Saarela, Janna; Leppa, Virpi; Dorfman, Ruslan; Alvaro, Domenico; Floreani, Annarosa; Onengut-Gumuscu, Suna; Rich, Stephen S.; Thompson, Wesley K.; Schork, Andrew J.; Naess, Sigrid; Thomsen, Ingo; Mayr, Gabriele; Koenig, Inke R.; Hveem, Kristian; Cleynen, Isabelle; Gutierrez-Achury, Javier; Ricano-Ponce, Isis; van Heel, David; Bjoernsson, Einar; Sandford, Richard N.; Durie, Peter R.; Melum, Espen; Vatn, Morten H.; Silverberg, Mark S.; Duerr, Richard H.; Padyukov, Leonid; Brand, Stephan; Sans, Miquel; Annese, Vito; Achkar, Jean-Paul; Boberg, Kirsten Muri; Marschall, Hanns-Ulrich; Chazouilleres, Olivier; Bowlus, Christopher L.; Wijmenga, Cisca; Schrumpf, Erik; Vermeire, Severine; Albrecht, Mario; Rioux, John D.; Alexander, Graeme; Bergquist, Annika; Cho, Judy; Schreiber, Stefan; Manns, Michael P.; Farkkila, Martti; Dale, Anders M.; Chapman, Roger W.; Lazaridis, Konstantinos N.; Franke, Andre; Anderson, Carl A.; Karlsen, Tom H.

Primary sclerosing cholangitis (PSC) is a severe liver disease of unknown etiology leading to fibrotic destruction of the bile ducts and ultimately to the need for liver transplantation(1-3). We compared 3,789 PSC cases of European ancestry to 25,079 population controls across 130,422 SNPs genotyped

Variations in primary sclerosing cholangitis across the age spectrum.

Science.gov (United States)

Eaton, John E; McCauley, Bryan M; Atkinson, Elizabeth J; Juran, Brian D; Schlicht, Erik M; de Andrade, Mariza; Lazaridis, Konstantinos N

2017-10-01

Primary sclerosing cholangitis (PSC) typically develops in middle-age adults. Little is known about phenotypic differences when PSC is diagnosed at various ages. Therefore, we sought to compare the clinical characteristics of a large PSC cohort based on the age when PSC was diagnosed. We performed a multicenter retrospective review to compare the features of PSC among those diagnosed between 1-19 (n = 95), 20-59 (n = 662), and 60-79 years (n = 102). Those with an early diagnosis (ED) of PSC were more likely to have small-duct PSC (13%) than those with a middle-age diagnosis (MD) (5%) and late diagnosis (LD) groups (2%), P early in life are more likely to have small-duct PSC and less likely to have disease-related complications. Clinicians should be vigilant for underlying cholangiocarcinoma among those with PSC diagnosed late in life. © 2017 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Bile acids for primary sclerosing cholangitis

DEFF Research Database (Denmark)

Poropat, Goran; Giljaca, Vanja; Stimac, Davor

2011-01-01

Primary sclerosing cholangitis is a progressive chronic cholestatic liver disease that usually leads to the development of cirrhosis. Studies evaluating bile acids in the treatment of primary sclerosing cholangitis have shown a potential benefit of their use. However, no influence on patients...

Sclerosing cholangitis: Clinicopathologic features, imaging spectrum, and systemic approach to differential diagnosis

Energy Technology Data Exchange (ETDEWEB)

Seo, Ni Eun [Dept. of Radiology, Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, So Yeon; Lee, Seung Soo; Byun, Jae Ho; Kim, Hyoung Jung; Kim, Jin Hee; Lee, Moon Gyu [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

2016-02-15

Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

Sclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis.

Science.gov (United States)

Seo, Nieun; Kim, So Yeon; Lee, Seung Soo; Byun, Jae Ho; Kim, Jin Hee; Kim, Hyoung Jung; Lee, Moon-Gyu

2016-01-01

Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

A survey of infectious agents as risk factors for primary sclerosing cholangitis: are Chlamydia species involved?

NARCIS (Netherlands)

Ponsioen, Cyriel Y.; Defoer, Jacqueline; ten Kate, Fiebo J. W.; Weverling, Gerrit J.; Tytgat, Guido N. J.; Pannekoek, Yvonne; Wertheim-Dillen, Pauline M. E.

2002-01-01

Objectives The aetiology of primary sclerosing cholangitis (PSC) is unknown, and the role of micro-organisms has been studied only to a limited extent. We tested the hypothesis that past or persisting infection with common viruses or atypical bacteria might play a role in genetically susceptible

Primary sclerosing cholangitis associated with increased peripheral eosinophils and serum IgE.

Science.gov (United States)

Shimomura, I; Takase, Y; Matsumoto, S; Kuyama, J; Nakajima, T; Maeda, H; Sugase, T; Hata, A; Hanada, M; Okuno, M

1996-10-01

Symptoms of cholestasis, including epigastralgia, fever, and jaundice, with marked increases in peripheral eosinophils and serum IgE in a 20-year-old man are reported here. Endoscopic retrograde cholangio-pancreatography (ERCP) detected constrictions of the bile ducts, compatible with primary sclerosing cholangitis (PSC). The symptoms and blood parameters of liver dysfunction were associated with the degree of eosinophilia and high serum IgE levels. During corticosteroid therapy, all of these parameters improved, and morphologic improvements of the bile ducts were also observed. The pathogenesis of PSC may be explained, in part, by the concept of hypereosinophilic syndrome or allergic reaction.

Risk factors and outcome in patients with primary sclerosing cholangitis with persistent biliary candidiasis.

Science.gov (United States)

Rupp, Christian; Bode, Konrad Alexander; Chahoud, Fadi; Wannhoff, Andreas; Friedrich, Kilian; Weiss, Karl-Heinz; Sauer, Peter; Stremmel, Wolfgang; Gotthardt, Daniel Nils

2014-10-23

Candidiasis is commonly observed in patients with primary sclerosing cholangitis (PSC), but the clinical risk factors associated with its presence have not been fully investigated. In this study, we aimed to analyse the incidence, risk factors, and transplantation-free survival in primary sclerosing cholangitis (PSC) patients with persistent biliary candidiasis. We retrospectively analysed patients diagnosed with PSC who were admitted to our department during 2002 to 2012. One-hundred fifty patients whose bile cultures were tested for fungal species were selected, and their clinical and laboratory parameters were investigated. The results of endoscopic retrograde cholangiography (ERC) and bile cultures were analysed using chart reviews. The cases of biliary candidiasis were sub-classified as transient or persistent. Thirty out of 150 (20.0%) patients had biliary candidiasis. Although all patients demonstrated comparable baseline characteristics, those with biliary candidiasis showed significantly reduced transplantation-free survival (p candidiasis. A subgroup analysis showed reduced survival with a greater necessity for orthotopic liver transplantation (OLT) only in patients with persistence of Candida (p = 0.007). The survival in the patients with transient biliary candidiasis was comparable to that in candidiasis-free patients. In a multivariate regression analysis that included Mayo risk score (MRS), sex, age, dominant stenosis, inflammatory bowel disease, autoimmune hepatitis overlap syndrome, and number of times ERC was performed, biliary candidiasis was an independent risk factor for reduced survival (p = 0.008). Risk factors associated with acquisition of biliary candidiasis were age at PSC diagnosis and number of ERCs. The persistence of biliary candidiasis is associated with markedly reduced transplantation-free survival in PSC patients. By contrast, actuarial survival in patients with transient biliary candidiasis approaches that for patients without any

Microbiological analysis of bile and its impact in critically ill patients with secondary sclerosing cholangitis.

Science.gov (United States)

Voigtländer, Torsten; Leuchs, Ensieh; Vonberg, Ralf-Peter; Solbach, Philipp; Manns, Michael P; Suerbaum, Sebastian; Lankisch, Tim O

2015-05-01

Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an emerging disease entity with unfavourable outcome. Our aim was to analyze the microbial spectrum in bile of patients with SSC-CIP and to evaluate the potential impact on the empiric antibiotic treatment in these patients. 169 patients (72 patients with SSC-CIP and 97 patients with primary sclerosing cholangitis (PSC)) were included in a prospective observational study between 2010 and 2013. Bile was obtained during endoscopic retrograde cholangiography (ERC) and microbiologically analyzed. Patients with SSC displayed a significantly different microbiological profile in bile. Enterococcus faecium, Pseudomonas aeruginosa and non-albicans species of Candida were more frequent in SSC compared to patients with PSC (p bile (p = 0.001). The antimicrobial therapy was adjusted in 64% of patients due to resistance or presence of microorganisms not covered by the initial therapy regimen. Patients with SSC-CIP have a distinct microbial profile in bile. Difficult to treat organisms are frequent and an ERC with bile fluid collection for microbiological analysis should be considered in case of insufficient antimicrobial treatment. Copyright © 2015 The British Infection Association. Published by Elsevier Ltd. All rights reserved.

Papillary bile duct dysplasia in primary sclerosing cholangitis.

Science.gov (United States)

Ludwig, J; Wahlstrom, H E; Batts, K P; Wiesner, R H

1992-06-01

A 62-year-old man with a 20-year history of chronic ulcerative colitis and a 9-year history of primary sclerosing cholangitis (PSC) underwent orthotopic liver transplantation because of symptoms related to PSC and cholangiographic features compatible with a biliary neoplasm. Study of the excised liver revealed papillary mucosal lesions in the common hepatic duct and the right and left hepatic ducts as well as cholangiectases and other features typically associated with PSC. The papillary lesions consisted of abundant fibrovascular stroma covered by biliary epithelium with low-grade and high-grade dysplasia. Some periductal glands were also dysplastic. These features distinguished papillary dysplasia from classic biliary papillomatosis. Only one focus of microinvasion was found; there were no metastases. Among 60 cases of PSC in whom the entire liver could be studied after orthotopic liver transplantation, this was the only instance of unequivocal dysplasia. However, in one specimen, papillary hyperplasia was found. Detailed macroscopic and microscopic rereview of 23 livers from our patients with the longest history of PSC (range, 5-24 years) failed to reveal any additional cases with dysplasia. It is concluded that (a) papillary mucosal lesions in PSC may represent papillary dysplasia without invasion; (b) these lesions may evolve from papillary hyperplasia; (c) the process may be largely, if not entirely, in situ; and (d) the prevalence of dysplasia and carcinoma of bile ducts may be less than the 7%-9% reported in the literature for malignancies associated with PSC.

Three ulcerative colitis susceptibility loci are associated with primary sclerosing cholangitis and indicate a role for IL2, REL, and CARD9

NARCIS (Netherlands)

Janse, Marcel; Lamberts, Laetitia E.; Franke, Lude; Raychaudhuri, Soumya; Ellinghaus, Eva; Muri Boberg, Kirsten; Melum, Espen; Folseraas, Trine; Schrumpf, Erik; Bergquist, Annika; Björnsson, Einar; Fu, Jingyuan; Jan Westra, Harm; Groen, Harry J. M.; Fehrmann, Rudolf S. N.; Smolonska, Joanna; van den Berg, Leonard H.; Ophoff, Roel A.; Porte, Robert J.; Weismüller, Tobias J.; Wedemeyer, Jochen; Schramm, Christoph; Sterneck, Martina; Günther, Rainer; Braun, Felix; Vermeire, Severine; Henckaerts, Liesbet; Wijmenga, Cisca; Ponsioen, Cyriel Y.; Schreiber, Stefan; Karlsen, Tom H.; Franke, Andre; Weersma, Rinse K.

2011-01-01

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently

Three Ulcerative Colitis Susceptibility Loci Are Associated with Primary Sclerosing Cholangitis and Indicate a Role for IL2, REL, and CARD9

NARCIS (Netherlands)

Janse, Marcel; Lamberts, Laetitia E.; Franke, Lude; Raychaudhuri, Soumya; Ellinghaus, Eva; Boberg, Kirsten Muri; Melum, Espen; Folseraas, Trine; Schrumpf, Erik; Bergquist, Annika; Bjornsson, Einar; Fu, Jingyuan; Westra, Harm Jan; Groen, Harry J. M.; Fehrmann, Rudolf S. N.; Smolonska, Joanna; van den Berg, Leonard H.; Ophoff, Roel A.; Porte, Robert J.; Weismueller, Tobias J.; Wedemeyer, Jochen; Schramm, Christoph; Sterneck, Martina; Guenther, Rainer; Braun, Felix; Vermeire, Severine; Henckaerts, Liesbet; Wijmenga, Cisca; Ponsioen, Cyriel Y.; Schreiber, Stefan; Karlsen, Tom H.; Franke, Andre; Weersma, Rinse K.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently

Primary Sclerosing Cholangitis Risk Estimate Tool (PREsTo) Predicts Outcomes in PSC: A Derivation & Validation Study Using Machine Learning.

Science.gov (United States)

Eaton, John E; Vesterhus, Mette; McCauley, Bryan M; Atkinson, Elizabeth J; Schlicht, Erik M; Juran, Brian D; Gossard, Andrea A; LaRusso, Nicholas F; Gores, Gregory J; Karlsen, Tom H; Lazaridis, Konstantinos N

2018-05-09

Improved methods are needed to risk stratify and predict outcomes in patients with primary sclerosing cholangitis (PSC). Therefore, we sought to derive and validate a new prediction model and compare its performance to existing surrogate markers. The model was derived using 509 subjects from a multicenter North American cohort and validated in an international multicenter cohort (n=278). Gradient boosting, a machine based learning technique, was used to create the model. The endpoint was hepatic decompensation (ascites, variceal hemorrhage or encephalopathy). Subjects with advanced PSC or cholangiocarcinoma at baseline were excluded. The PSC risk estimate tool (PREsTo) consists of 9 variables: bilirubin, albumin, serum alkaline phosphatase (SAP) times the upper limit of normal (ULN), platelets, AST, hemoglobin, sodium, patient age and the number of years since PSC was diagnosed. Validation in an independent cohort confirms PREsTo accurately predicts decompensation (C statistic 0.90, 95% confidence interval (CI) 0.84-0.95) and performed well compared to MELD score (C statistic 0.72, 95% CI 0.57-0.84), Mayo PSC risk score (C statistic 0.85, 95% CI 0.77-0.92) and SAP statistic 0.65, 95% CI 0.55-0.73). PREsTo continued to be accurate among individuals with a bilirubin statistic 0.90, 95% CI 0.82-0.96) and when the score was re-applied at a later course in the disease (C statistic 0.82, 95% CI 0.64-0.95). PREsTo accurately predicts hepatic decompensation in PSC and exceeds the performance among other widely available, noninvasive prognostic scoring systems. This article is protected by copyright. All rights reserved. © 2018 by the American Association for the Study of Liver Diseases.

Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial

NARCIS (Netherlands)

Beuers, U.; Spengler, U.; Kruis, W.; AYDEMIR, U.; WIEBECKE, B.; HELDWEIN, W.; WEINZIERL, M.; Pape, G. R.; Sauerbruch, T.; Paumgartner, G.

1992-01-01

The efficacy and safety of ursodeoxycholic acid for the treatment of primary sclerosing cholangitis were evaluated in a prospective, randomized, double-blind, placebo-controlled trial. Fourteen patients with primary sclerosing cholangitis documented by cholestatic serum enzyme pattern, liver

[Primary sclerosing cholangitis associated with Sjögren's syndrome, retroperitoneal fibrosis and chronic pancreatitis. Report of a case].

Science.gov (United States)

Barreda, F; Contardo, C; León, A; Navarrete, J; Figueroa, R; Attanasio, F

1989-01-01

Primary Sclerosing Cholangitis (PSC) is an unusual chronic, cholestatic disease of unknown etiology, more frequently seen in young adults in close relationship with Chronic Ulcerative Colitis. We report the case of a 30 year old woman, coming from the peruvian amazon with PSC associated with Sjögren Syndrome, Chronic Pancreatitis and Retroperitoneal Fibrosis, without colonic involvement. She was treated with external biliary drainage and controlled for 12 months. In this paper, clinical, biochemical, radiological, histological and therapeutic features are reviewed as well as its possible immunologie autoimmune origin.

A case of cutaneous scleroderma with primary sclerosing cholangitis

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H P Nandeesh

2014-01-01

Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

Leukocytapheresis Therapy Improved Cholestasis in a Patient Suffering from Primary Sclerosing Cholangitis with Ulcerative Colitis

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Minoru Itou

2009-04-01

Full Text Available Primary sclerosing cholangitis (PSC is an autoimmune disease of the hepatobiliary system for which effective therapy has not been established. Leukocytapheresis (LCAP therapy is known to effective in patients with ulcerative colitis (UC. In addition, effects of LCAP therapy were reported on some autoimmune diseases such as Crohn’s disease, rheumatoid arthritis and rapidly progressive glomerulonephritis. Here we report the case of a 29-year-old man with PSC associated with UC who was treated with LCAP therapy. He had a 16-year history of UC and a 12-year history of PSC. Although he was under treatment with prednisolone and ursodeoxycholic acid, exacerbation of UC and PSC-associated cholestasis were seen. Since he showed side effects of prednisolone, he was treated with LCAP. Not only improvement of UC, but also decreased serum alkaline phosphatase, γ-guanosine triphosphate and total bile acids, suggesting improvement of PSC-associated cholestaisis, were seen after treatment with LCAP. Our experience with this case suggests that LCAP therapy could be a new effective therapeutic strategy for patients with PSC associated with UC.

Altered Cyclosporine Absorption in a Patient with Ulcerative Colitis, Sclerosing Cholangitis and Pancreatic Insufficiency

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Mark G Swain

1991-01-01

Full Text Available Pancreatic insufficiency leading to altered cyclosporine absorption is reported in a 37-year-old man with ulcerative colitis and sclerosing cholangitis. Asymptomatic chronic pancreatitis occurs frequently in patients with ulcerative colitis, and even more commonly when there is coexistent sclerosing cholangitis. However, pancreatic insufficiency has been documented in only one patient previously with ulcerative colitis and sclerosing cholangitis. Pancreatic function testing can help to identify the complex etiology of malabsorption in these patients and is recommended in patients when liver transplantation is contemplated, as pancreatic insufficiency may alter the absorption of cyclosporine.

Increased cholestatic enzymes in two patients with long-term history of ulcerative colitis: consider primary biliary cholangitis not always primary sclerosing cholangitis.

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Polychronopoulou, Erietta; Lygoura, Vasiliki; Gatselis, Nikolaos K; Dalekos, George N

2017-09-25

Several hepatobiliary disorders have been reported in ulcerative colitis (UC) patients with primary sclerosing cholangitis (PSC) being the most specific. Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, rarely occurs in UC. We present two PBC cases of 67 and 71 years who suffered from long-standing UC. Both patients were asymptomatic but they had increased cholestatic enzymes and high titres of antimitochondrial antibodies (AMA)-the laboratory hallmark of PBC. After careful exclusion of other causes of cholestasis by MRI/magnetic resonance cholangiopancreatography (MRCP), virological and microbiological investigations, a diagnosis of PBC associated with UC was established. The patients started ursodeoxycholic acid (13 mg/kg/day) with complete response. During follow-up, both patients remained asymptomatic with normal blood biochemistry. Although PSC is the most common hepatobiliary manifestation among patients with UC, physicians must keep also PBC in mind in those with unexplained cholestasis and repeatedly normal MRCP. In these cases, a reliable AMA testing can help for an accurate diagnosis. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Genetic polymorphisms of matrix metalloproteinase 3 in primary sclerosing cholangitis

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Juran, Brian D.; Atkinson, Elizabeth J.; Schlicht, Erik M.; Larson, Joseph J.; Ellinghaus, David; Franke, Andre; Lazaridis, Konstantinos N.

2011-01-01

Background The damaging cholestasis inherent to primary sclerosing cholangitis (PSC) results from bile duct stricturing because of progressive fibrosis. The matrix metalloproteinase 3 (MMP3) degrades a wide range of matrix components and is expressed by activated liver stellate cells, and so is a candidate for involvement with the fibrotic processes underlying PSC. Moreover, the MMP3 gene harbours polymorphisms associated with variation in its activity directly impacting clinical phenotypes. Aims We aimed to examine the influence of MMP3 polymorphisms on PSC risk and progression. Methods Nine single nucleotide polymorphisms (SNPs) tagging the common genetic variation of MMP3 were genotyped in 266 PSC patients and 407 controls. SNPs and inferred haplotypes were assessed for PSC association by logistic regression and score tests. The effect of SNPs on survival to liver transplant or death was analysed using Cox regression, and Kaplan–Meier curves were constructed. Results No association of PSC with individual SNPs or haplotypes of MMP3 was detected. However, progression to death or liver transplant was significantly associated with homozygosity for minor alleles of rs522616, rs650108 and rs683878, particularly among PSC patients with concurrent ulcerative colitis (UC) (strongest in redundant SNPs rs650108/rs683878, hazard ratio = 3.23, 95% confidence interval 1.45–7.25, P = 0.004). Conclusions Genetic variation in MMP3 influences PSC progression, possibly in the context of coexisting UC. While the functional variants and specific mechanisms remain unknown, this finding implicates the turnover of the extracellular matrix as an important and variable component of PSC pathogenesis. Efforts to understand this process could form the basis for developing effective treatments, which are currently lacking for PSC. PMID:21134112

Diagnosis of primary sclerosing cholangitis: prospective comparison of MR cholangiography with endoscopic retrograde cholangiography

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Oberholzer, K.; Mildenberger, P.; Grebe, P.; Bantelmann, M.; Thelen, M.; Lohse, A.W.; Schadeck, T.

1998-01-01

Purpose: To assess the accuracy of MR cholangiography (MRC) in the diagnosis of primary sclerosing cholangitis (PSC) in comparison to endoscopic retrograde cholangiography (ERC). Method: 20 patients with PSC were examined by ERC and MRC (1.0 T. HASTE sequence). Visualization and pathologic changes of the extra- and intrahepatic bile ducts were evaluated with both methods. Results: Mural irregularities of the common bile duct were seen with MRC in 6/7 cases, stenoses and dilatation of the common bile duct were detected correctly in all patients. Diffuse, multifocal strictures of the intrahepatic bile duct were the most common intrahepatic findings and correctly diagnosed in all patients. Mural irregularities of the intrahepatic ducts in early stages may be missed by MRC because of the limited spatial resolution. MRC is superior to ERC in visualization of nonopacified intrahepatic ducts. Conclusions: MRC is a reliable, non-invasive method to detect typical diagnostic features of PSC. It should be considered as an adjunct to ERC in patients with suspected PSC for primary diagnosis and as an alternate method for follow-up studies. (orig.) [de

Secondary sclerosing cholangitis in critically ill patients: current perspectives

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Gudnason HO

2017-06-01

Full Text Available Hafsteinn O Gudnason,1 Einar S Björnsson1,2 1Division of Gastroenterology and Hepatology, Department of Internal Medicine, Landspitali, University Hospital of Iceland, 2Faculty of Medicine, University of Iceland, Reykjavik, Iceland Abstract: Secondary sclerosing cholangitis (SSC is a term used for a group of chronic cholestatic disease affecting the intra- and/or extrahepatic biliary tree with inflammation and progressive stricture formation, which can lead to biliary cirrhosis. A newly recognized form of SSC is secondary sclerosing cholangitis in critically ill patients (SSC-CIP. Pathogenesis is believed to involve ischemic injury of intrahepatic bile ducts associated with prolonged hypotension, vasopressors administration, and/or mechanical ventilation in patients treated in the intensive care unit (ICU. Patients diagnosed with SSC-CIP have no prior history of liver disease and no known pathologic process or injury responsible for bile duct obstruction prior to ICU treatment. Reasons leading to ICU treatment are many including multitrauma, burn injury, cardiac surgery, severe pneumonia, other infections, or bleeding after abdominal surgery. Patients have in common prolonged ICU admission. SSC-CIP is associated with rapid progression to liver cirrhosis and poor survival with limited treatment options except a liver transplantation. Transplant-free survival is around 17–40 months, which is lower than in other SSC patients. During the initial stages of the disease, the clinical symptoms and biochemical profile are not specific and easily missed. Biliary casts formation may be considered pathognomonic for SSC-CIP since most patients have them in early stages of the disease. Increased awareness and early detection of the disease and its complications is considered to be crucial to improve the poor prognosis. Keywords: secondary sclerosing cholangitis, SSC-CIP, chronic cholestatic disease, sclerosing cholangitis

De-novo cholangiocarcinoma in native common bile duct remnant following OLT for primary sclerosing cholangitis.

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Landaverde, Carmen; Ng, Vivian; Sato, Alisa; Tabibian, James; Durazo, Francisco; Busuttil, Ronald

2009-01-01

Primary sclerosing cholangitis (PSC) is a chronic, progressive, inflammatory and obstructive disease of the intra- and extra-hepatic bile ducts of unknown etiology. Currently, orthotopic liver transplantation (OLT) is the only definitive treatment for PSC-related end-stage liver disease. However, PSC has been known to recur in the grafted liver. Roux-en-Y hepaticojejunostomy is more commonly performed than choledochocholedochostomy for PSC, although choledochocholedochostomy has been found to be safe and efficacious for PSC if the distal common bile duct is uninvolved at the time of OLT. Our case is unique in that it describes a patient who developed de-novo cholangiocarcinoma in the remnant portion of the native common bile duct six years after OLT with choledochocholedochostomy for PSC-associated end-stage liver disease without having PSC recurrence. In conclusion, our case report indicates that choledochocholedochostomy may not be desirable in PSC due to an increased risk of developing cholangiocarcinoma in the native common bile duct. This risk exists as well with a Roux-en-Y hepaticojejunostomy in the remaining intra-duodenal and intra-pancreatic biliary epithelium, although in theory to a lesser extent. Therefore, the risk of developing cholangiocarcinoma in the recipient common bile duct can only be completely eliminated by performing a Whipple procedure at the time of OLT.

Celiac disease (CD), ulcerative colitis (UC), and primary sclerosing cholangitis (PSC) in one patient: a family study Enfermedad celiaca (EC), colitis ulcerosa (CU) y colangitis esclerosante primaria (CEP) asociadas en el mismo paciente: estudio familiar

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V. Cadahía; L. Rodrigo; D. Fuentes; S. Riestra; R. de Francisco; M. Fernández

2005-01-01

We discuss the case of a 17-year-old male who at the age of 7 was diagnosed with celiac disease (CD) together with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). The patient was treated with gluten-free diet and immunosuppressive drugs (azathioprine), and currently remains asymptomatic. The patient's younger, 12-year-old sister was diagnosed with CD when she was 1.5 years old, and at 7 years she developed type-I diabetes mellitus, which was difficult to control. A family st...

Reduced Coffee Consumption Among Individuals with Primary Sclerosing Cholangitis but Not Primary Biliary Cirrhosis

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Lammert, Craig; Juran, Brian D.; Schlicht, Erik; Xie, Xiao; Atkinson, Elizabeth J.; de Andrade, Mariza; Lazaridis, Konstantinos N.

2014-01-01

Background & Aims Coffee consumption has been associated with decreased risk of liver disease and related outcomes. However, coffee drinking has not been investigated among patients with cholestatic autoimmune liver diseases, primary biliary cirrhosis (PBC), or primary sclerosing cholangitis (PSC). We investigated the relationship between coffee consumption and risk of PBC and PSC in a large North American cohort. Methods Lifetime coffee drinking habits were determined from responses to questionnaires from 606 patients with PBC, 480 with PSC, and 564 healthy volunteers (controls). Patients (those with PBC or PSC) were compared to controls utilizing the Wilcoxon rank sum test for continuous variables and c2 method for discrete variables. Logistic regression was used to analyze the estimate the effects of different coffee parameters (time, frequency, and type of coffee consumption) after adjusting for age, sex, smoking status, and education level. Results Patients with PBC and controls did not differ in coffee parameters. However, 24% of patients with PSC had never drank coffee compared to 16% of controls (Pcoffee drinking coffee (46.6% vs 66.7% for controls, Pcoffee protected against proctocolectomy (hazard ratio=0.34, PCoffee consumption is lower among patients with PSC, but not PBC, compared to controls. PMID:24440215

Gene Expression by PBMC in Primary Sclerosing Cholangitis: Evidence for Dysregulation of Immune Mediated Genes

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Christopher A. Aoki

2006-01-01

Full Text Available Primary sclerosing cholangitis (PSC is a chronic disease of the bile ducts characterized by an inflammatory infiltrate and obliterative fibrosis. The precise role of the immune system in the pathogenesis of PSC remains unknown. We used RNA microarray analysis to identify immune-related genes and pathways that are differentially expressed in PSC. Messenger RNA (mRNA from peripheral blood mononuclear cells (PBMC was isolated from both patients with PSC and age and sex matched healthy controls. Samples from 5 PSC patients and 5 controls were analyzed by microarray and based upon rigorous statistical analysis of the data, relevant genes were chosen for confirmation by RT-PCR in 10 PSC patients and 10 controls. Using unsupervised hierarchical clustering, gene expression in PSC was statistically different from our control population. Interestingly, genes within the IL-2 receptor beta, IL-6 and MAP Kinase pathways were found to be differently expressed in patients with PSC compared to controls. Further, individual genes, TNF-α induced protein 6 (TNFaip6 and membrane-spanning 4-domains, subfamily A (ms4a were found to be upregulated in PSC while similar to Mothers against decapentaplegic homolog 5 (SMAD 5 was downregulated. In conclusion, several immune-related pathways and genes were differentially expressed in PSC compared to control patients, giving further evidence that this disease is systemic and immune-mediated.

Pancreatic changes in patients with primary sclerosing cholangitis: MR cholangiopancreatography and MRI findings

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Ozkavukcu, Esra; Erden, Ayse; Erden, Ilhan

2009-01-01

Purpose: To evaluate the possible pancreatic changes and their frequencies in patients with primary sclerosing cholangitis (PSC) on MR cholangiopancreatography (MRCP), and conventional abdominal MRI. Materials and Methods: Patient group consisted of 29 PSC (13 male, 16 female) cases, whereas cohort 1 consisted of 12 female patients with primary biliary cirrhosis, and cohort 2 consisted of 17 patients (6 male, 11 female) with non-immune chronic liver disease. Two radiologists retrospectively evaluated the MR examinations paying special attention to the pancreatic size (atrophy or enlargement), T1- and T2-signal intensity of the pancreas, focal pancreatic lesion, capsule-like rim, peripancreatic edema or fluid, fascial thickening, and pancreatic ducts (dilatation or narrowing). The results are expressed as percentages. Three groups were compared using Pearson chi-square test for each feature. However, only p-value for 'dilatation of the pancreatic duct' was determined, whereas p-value could not be calculated because of the insufficient number of subjects/sequences for the other features. Results: Twelve PSC patients (41.3%) had pancreatic abnormalities. The most common pancreatic changes in PSC patients were decreased T1-signal intensity (44%) and dilatation of the pancreatic duct (13.8%), respectively. Increased T2-signal intensity was also shown in 2 PSC patients (6.9%). Conclusion: Even PSC patients without any sign of pancreatitis, can show MR changes in the pancreatic parenchyma or the pancreatic duct. The etiologies of these changes, and whether they are unique to PSC, are still controversial. Histopathological studies bringing light to these pancreatic changes are needed.

IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

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Allon Kahn

2015-01-01

Full Text Available IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

Imaging and estimation of the prognostic features of primary sclerosing cholangitis by ultrasonography and MR cholangiography

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Oikarinen, H.; Paeaekkoe, E.; Suramo, I.; Paeivaensalo, M.; Tervonen, O.; Lehtola, J.; Aukee, J.

2001-01-01

Purpose: To evaluate the ability of US and MR cholangiography (MRC) to detect bile duct changes and prognostic signs of primary sclerosing cholangitis (PSC) seen at endoscopic retrograde cholangiography (ERC). Material and Methods: In a prospective study, 9 patients with PSC underwent US, MRC, MR imaging and ERC of the bile ducts and the liver. Eight age- and sex-matched control patients were examined with MRC, MR imaging and ERC. A segmental comparison was performed to assess the ability of MRC-MR and US to reveal the accurate ductal involvement in different segments of the biliary tree and the specific criteria of poor prognostic outcome in PSC. The ability of MRC-MR to detect the presence of PSC in different patients was analysed blindly. Results: MRC-MR depicted changes of PSC correctly in 9 patients (radiologist 1) and in 8 patients with 1 false-positive finding (radiologist 2) in the blinded analysis. In the segmental comparison, MRC missed especially bile duct dilatation. MRC was too pessimistic in the evaluation of the outcome. US detected features suggestive of PSC in 8 patients (radiologist 3). US was unable to show the predictors of poor outcome. Conclusion: MRC and US seem to be useful in the detection of PSC. US is unable and MRC is too pessimistic to estimate the outcome of PSC

Characterization of Intestinal Microbiota in Ulcerative Colitis Patients with and without Primary Sclerosing Cholangitis.

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Kevans, D; Tyler, A D; Holm, K; Jørgensen, K K; Vatn, M H; Karlsen, T H; Kaplan, G G; Eksteen, B; Gevers, D; Hov, J R; Silverberg, M S

2016-03-01

There is an unexplained association between ulcerative colitis [UC] and primary sclerosing cholangitis [PSC], with the intestinal microbiota implicated as an important factor. The study aim was to compare the structure of the intestinal microbiota of patients with UC with and without PSC. UC patients with PSC [PSC-UC] and without PSC [UC] were identified from biobanks at Oslo University Hospital, Foothills Hospital Calgary and Mount Sinai Hospital Toronto. Microbial DNA was extracted from colonic tissue and sequencing performed of the V4 region of the 16S rRNA gene on Illumina MiSeq. Sequences were assigned to operational taxonomic units [OTUs] using Quantitative Insights Into Microbial Ecology [QIIME]. Microbial alpha diversity, beta diversity, and relative abundance were compared between PSC-UC and UC phenotypes. In all, 31 PSC-UC patients and 56 UC patients were included. Principal coordinate analysis [PCoA] demonstrated that city of sample collection was the strongest determinant of taxonomic profile. In the Oslo cohort, Chao 1 index was modestly decreased in PSC-UC compared with UC [p = 0.04] but did not differ significantly in the Calgary cohort. No clustering by PSC phenotype was observed using beta diversity measures. For multiple microbial genera there were nominally significant differences between UC and PSC-UC, but results were not robust to false-discovery rate correction. No strong PSC-specific microbial associations in UC patients consistent across different cohorts were identified. Recruitment centre had a strong effect on microbial composition. Future studies should include larger cohorts to increase power and the ability to control for confounding factors. Copyright © 2015 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.

MicroRNAs in Serum and Bile of Patients with Primary Sclerosing Cholangitis and/or Cholangiocarcinoma.

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Voigtländer, Torsten; Gupta, Shashi K; Thum, Sabrina; Fendrich, Jasmin; Manns, Michael P; Lankisch, Tim O; Thum, Thomas

2015-01-01

Patients with primary sclerosing cholangitis (PSC) are at high risk for the development of cholangiocarcinoma (CC). Analysis of micro ribonucleic acid (MiRNA) patterns is an evolving research field in biliary pathophysiology with potential value in diagnosis and therapy. Our aim was to evaluate miRNA patterns in serum and bile of patients with PSC and/or CC. Serum and bile from consecutive patients with PSC (n = 40 (serum), n = 52 (bile)), CC (n = 31 (serum), n = 19 (bile)) and patients with CC complicating PSC (PSC/CC) (n = 12 (bile)) were analyzed in a cross-sectional study between 2009 and 2012. As additional control serum samples from healthy individuals were analyzed (n = 12). The miRNA levels in serum and bile were determined with global miRNA profiling and subsequent miRNA-specific polymerase chain reaction-mediated validation. Serum analysis revealed significant differences for miR-1281 (p = 0.001), miR-126 (p = 0.001), miR-26a (p = 0.001), miR-30b (p = 0.001) and miR-122 (p = 0.034) between patients with PSC and patients with CC. All validated miRNAs were significantly lower in healthy individuals. MiR-412 (p = 0.001), miR-640 (p = 0.001), miR-1537 (p = 0.003) and miR-3189 (p = 0.001) were significantly different between patients with PSC and PSC/CC in bile. Patients with PSC and/or CC have distinct miRNA profiles in serum and bile. Furthermore, miRNA concentrations are different in bile of patients with CC on top of PSC indicating the potential diagnostic value of these miRNAs.

Detection of Cholangiocarcinoma with Magnetic Resonance Spectroscopy of Bile in Patients with and without Primary Sclerosing Cholangitis

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Albiin, N.; Smith, I.C.P.; Arnelo, U.; Lindberg, B.; Bergquist, A.; Dolenko, B.; Bryksina, N.; Bezabeh, T.

2008-01-01

Background: Early detection of cholangiocarcinoma (CC) is very difficult, especially in patients with primary sclerosing cholangitis (PSC) who are at increased risk of developing CC. Purpose: To evaluate 1 H magnetic resonance spectroscopy ( 1 H-MRS) of bile as a diagnostic marker for CC in patients with and without PSC. Material and Methods: The institutional review board approved the study, and all patients gave informed consent. Bile from 49 patients was sampled and investigated using 1 H-MRS. MR spectra of bile samples from 45 patients (18 female; age range 22-87 years, mean age 57 years) were analyzed both conventionally and using computerized multivariate analysis. Sixteen of the patients had CC, 18 had PSC, and 11 had other benign findings. Results: The spectra of bile from CC patients differed from the benign group in the levels of phosphatidylcholine, bile acids, lipid, and cholesterol. It was possible to distinguish CC from benign conditions in all patients with malignancy. Two benign non-PSC patients were misclassified as malignant. The sensitivity, specificity, and accuracy were 88.9%, 87.1%, and 87.8%, respectively. Conclusion: With 1 H-MRS of bile, cholangiocarcinoma could be discriminated from benign biliary conditions with or without PSC

The effect of silver nitrate, chloroformic garlic extract and normal saline in induction of sclerosing cholangitis in rabbits

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Hosseni, Seyed V.; Mohebzadeh, J.; Mehrabani, D.; Amini, M.; Kumar, Perikala V.; Bagheri, Mohammad H.; Sadjjadi, Seyed M.; Amini, A.

2008-01-01

Objective was to the effects of 0.5% silver nitrate, 20% chloroformic garlic extract and 0.9% normal saline in induction of sclerosing cholangitis in the bile ducts of rabbits. During a 6-months period from April to September 2006 in Shiraz University Laboratory Animal Research Center, we selected 3 equal groups of rabbits. We injected 0.5% silver nitrate, 20% chloroformic garlic extract and 0.9% normal saline into the bile ducts of each group. The animals were euthanized and autopsied after 4 months and the liver and bile ducts were removed and studied histopathologically. Cholangiography was undertaken to evaluate the presence and extent of any sclerosing cholangitis. Animals showed sclerosing cholangitis in silver nitrate group (7 [58%]), one (8%) in chloroformic garlic extract group and one (7%) in normal saline group. The difference between silver nitrate and chloroformic garlic extract groups were statistically significant and similar results were noticed between chloroformic garlic extract and normal saline groups. Twenty percent of chloroformic garlic extract had fewer complications such as sclerosing cholangitis, compared to other materials. (author)

MR imaging of primary sclerosing cholangitis - Additional value of diffusion-weighted imaging and ADC measurement

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Djokicc Kovac, Jelena [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia)], e-mail: jelenadjokic2003@yahoo.co.uk; Maksimovic, Ruzica [Center for Radiology and Magnetic Resonance Imaging, Clinical Center Serbia, Belgrade (Serbia); Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Jesic, Rada [Clinic for Gastroenterohepatology, Clinical Center Serbia, Belgrade (Serbia); Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Stanisavljevic, Dejana [Inst. for Statistics, Faculty of Medicine, Univ. of Belgrade, Belgrade (Serbia); Kovac, Bojan [Military Medical Academy, Belgrade (Serbia)

2013-04-15

Background: Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with chronic inflammation and progressive destruction of biliary tree. Magnetic resonance (MR) examination with diffusion-weighted imaging (DWI) allows analysis of morphological liver parenchymal changes and non-invasive assessment of liver fibrosis. Moreover, MR cholangiopancreatography (MRCP), as a part of standard MR protocol, provides insight into bile duct irregularities. Purpose: To evaluate MR and MRCP findings in patients with primary sclerosing cholangitis and to determine the value of DWI in the assessment of liver fibrosis. Material and Methods: The following MR findings were reviewed in 38 patients: abnormalities in liver parenchyma signal intensity, changes in liver morphology, lymphadenopathy, signs of portal hypertension, and irregularities of intra- and extrahepatic bile ducts. Apparent diffusion coefficient (ADC) was calculated for six locations in the liver for b = 800 s/mm{sup 2}. Results: T2-weighted hyperintensity was seen as peripheral wedge-shaped areas in 42.1% and as periportal edema in 28.9% of patients. Increased enhancement of liver parenchyma on arterial-phase imaging was observed in six (15.8%) patients. Caudate lobe hypertrophy was present in 10 (26.3%), while spherical liver shape was noted in 7.9% of patients. Liver cirrhosis was seen in 34.2% of patients; the most common pattern was micronodular cirrhosis (61.5%). Other findings included lymphadenopathy (28.9%), signs of portal hypertension (36.7%), and bile duct irregularities (78.9%). The mean ADCs (x10{sup -3} mm{sup 2}/s) were significantly different at stage I vs. stages III and IV, and stage II vs. stage IV. No significant difference was found between stages II and III. For prediction of stage {>=}II and stage {>=}III, areas under receiver-operating characteristic curves were 0.891 and 0.887, respectively. Conclusion: MR with MRCP is a necessary diagnostic procedure for diagnosis of PSC and

Surveillance of primary sclerosing cholangitis with ERC and brush cytology: risk factors for cholangiocarcinoma.

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Boyd, Sonja; Mustonen, Harri; Tenca, Andrea; Jokelainen, Kalle; Arola, Johanna; Färkkilä, Martti A

2017-02-01

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease leading to bile duct strictures and fibrosis, and predisposing to cholangiocarcinoma (CCA). Biliary dysplasia is a known precursor of CCA. In our unit, PSC patients undergo regular surveillance with ERC and brush cytology (BC), and liver transplantation is an option in case with biliary dysplasia. We evaluated the risk factors for biliary dysplasia and CCA based on ERC imaging, BC and liver function tests. Seven hundred and eighty-eight ERCs were performed with BC for 447 PSC patients. ERC images were evaluated using the modified Amsterdam score, neutrophilic inflammation was assessed in BC, and liver function tests were collected. Ploidy analysis with DNA flow cytometry was performed in cases with advanced PSC or previous suspicious BC/aneuploidy. The endpoint was either a benign disease course (follow-up for ≥2.4 years after the latest ERC), benign histology, biliary dysplasia or CCA. Benign disease course was seen in 424/447 (including 23 cases with biliary dysplasia), and CCA in 17 (3.8%) patients. Gallbladder carcinoma/carcinoma in situ was diagnosed in three patients. Advanced ERC findings, male gender, suspicious BC, aneuploidy in flow cytometry, inflammation, and elevation of ALP, bilirubin, ALT, AST, GGT, CEA and CA19-9 represented significant risk factors for CCA in univariate analysis. PSC patients with advanced bile duct disease and elevated liver enzymes, CEA or CA19-9, inflammation or suspicious BC are most likely to develop CCA. These patients may benefit from surveillance with BC if early liver transplantation is possible.

Immunosuppressive Agents for the Treatment of Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis.

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Peng, Xia; Luo, Xin; Hou, Jing-Ying; Wu, Shu-Yun; Li, Liang-Zong; Zheng, Ming-Hua; Wang, Ling-Yun

2017-01-01

Currently, there are no effective therapeutic agents for patients with primary sclerosing cholangitis (PSC). This study aimed to evaluate the safety and efficiency of immunosuppressive agents (IAs) for the treatment of PSC. The literatures were searched using the following keywords singly or in combination: PSC, treatments, IAs. The primary outcome was defined as the need for liver transplantation or mortality. Two hundred sixty six patients from 7 eligible studies were analyzed. IAs had no remarkable effects on the rate of mortality or liver transplantation (relative risk, RR 1.02, 95% CI 0.58-1.62, p = 0.92). Subgroup analyses showed no significant effect of IAs co-administration therapy (IAs co-administered with ursodeoxycholic acid, IA co-administered with IA; RR 1.41, 95% CI 0.40-4.95, p = 0.60). IAs caused adverse events (AEs) such as diarrhea, abdominal pain, and pruritus (RR 1.81, 95% CI 1.07-3.07, p = 0.03). IAs therapy did not significantly improve markers of liver function except for aspartate transaminase (weighted mean difference -9.76, 95% CI -12.92 to -6.6, p IAs administrated as either monotherapy or combination therapy do not reduce the risk of mortality or liver transplantation. IAs monotherapy is associated with AEs. © 2017 S. Karger AG, Basel.

Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings

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Itoh, S., E-mail: shigekimiyo@luck.ocn.ne.j [Department of Technical Radiology, Nagoya University School of Health Sciences, Nagoya (Japan); Nagasaka, T. [Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya (Japan); Suzuki, K.; Satake, H.; Ota, T.; Naganawa, S. [Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya (Japan)

2009-11-15

Aim: To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis. Materials and methods: Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining. Results: The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients. Conclusion: Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.

Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings

International Nuclear Information System (INIS)

Itoh, S.; Nagasaka, T.; Suzuki, K.; Satake, H.; Ota, T.; Naganawa, S.

2009-01-01

Aim: To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis. Materials and methods: Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining. Results: The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients. Conclusion: Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.

 Alkaline phosphatase normalization is a biomarker of improved survival in primary sclerosing cholangitis.

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Hilscher, Moira; Enders, Felicity B; Carey, Elizabeth J; Lindor, Keith D; Tabibian, James H

2016-01-01

 Introduction. Recent studies suggest that serum alkaline phosphatase may represent a prognostic biomarker in patients with primary sclerosing cholangitis. However, this association remains poorly understood. Therefore, the aim of this study was to investigate the prognostic significance and clinical correlates of alkaline phosphatase normalization in primary sclerosing cholangitis. This was a retrospective cohort study of patients with a new diagnosis of primary sclerosing cholangitis made at an academic medical center. The primary endpoint was time to hepatobiliaryneoplasia, liver transplantation, or liver-related death. Secondary endpoints included occurrence of and time to alkaline phosphatase normalization. Patients who did and did not achieve normalization were compared with respect to clinical characteristics and endpoint-free survival, and the association between normalization and the primary endpoint was assessed with univariate and multivariate Cox proportional-hazards analyses. Eighty six patients were included in the study, with a total of 755 patient-years of follow-up. Thirty-eight patients (44%) experienced alkaline phosphatase normalization within 12 months of diagnosis. Alkaline phosphatase normalization was associated with longer primary endpoint-free survival (p = 0.0032) and decreased risk of requiring liver transplantation (p = 0.033). Persistent normalization was associated with even fewer adverse endpoints as well as longer survival. In multivariate analyses, alkaline phosphatase normalization (adjusted hazard ratio 0.21, p = 0.012) and baseline bilirubin (adjusted hazard ratio 4.87, p = 0.029) were the only significant predictors of primary endpoint-free survival. Alkaline phosphatase normalization, particularly if persistent, represents a robust biomarker of improved long-term survival and decreased risk of requiring liver transplantation in patients with primary sclerosing cholangitis.

Individuals with Primary Sclerosing Cholangitis Have Elevated Levels of Biomarkers for Apoptosis but Not Necrosis.

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Masuoka, Howard C; Vuppalanchi, Raj; Deppe, Ross; Bybee, Phelan; Comerford, Megan; Liangpunsakul, Suthat; Ghabril, Marwan; Chalasani, Naga

2015-12-01

Hepatocyte apoptosis or necrosis from accumulation of bile salts may play an important role in the disease progression of primary sclerosing cholangitis (PSC). The aim of the current study was to measure serum markers of hepatocyte apoptosis (cytokeratin-18 fragments--K18) and necrosis (high-mobility group protein B1--HMGB1) in adults with PSC and examine the relationship with disease severity. We measured serum levels of K18 and HMGB1 in well-phenotyped PSC (N = 37) and 39 control subjects (N = 39). Severity of PSC was assessed biochemically, histologically, and PSC Mayo risk score. Quantification of hepatocyte apoptosis was performed using TUNEL assay. The mean age of the study cohort was 49.7 ± 13.3 years and comprised of 67% men and 93% Caucasian. Serum K18 levels were significantly higher in the PSC patients compared to control (217.4 ± 78.1 vs. 157.0 ± 58.2 U/L, p = 0.001). However, HMGB1 levels were not different between the two groups (5.38 ± 2.99 vs. 6.28 ± 2.85 ng/mL, p = 0.15). Within the PSC group, K18 levels significantly correlated with AST (r = 0.5, p = 0.002), alkaline phosphatase (r = 0.5, p = 0.001), total bilirubin (r = 0.61, p ≤ 0.001), and albumin (r = -0.4, p = 0.02). Serum K18 levels also correlated with the level of apoptosis present on the liver biopsy (r = 0.8, p ≤ 0.001) and Mayo risk score (r = 0.4, p = 0.015). Serum K18 but not HMGB1 levels were increased in PSC and associated with severity of underlying liver disease and the degree of hepatocyte apoptosis.

Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis.

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Wunsch, Ewa; Trottier, Jocelyn; Milkiewicz, Malgorzata; Raszeja-Wyszomirska, Joanna; Hirschfield, Gideon M; Barbier, Olivier; Milkiewicz, Piotr

2014-09-01

Ursodeoxycholic acid (UDCA) is no longer recommended for management of adult patients with primary sclerosing cholangitis (PSC). We undertook a prospective evaluation of UDCA withdrawal in a group of consecutive patients with PSC. Twenty six patients, all treated with UDCA (dose range: 10-15 mg/kg/day) were included. Paired blood samples for liver biochemistry, bile acids, and fibroblast growth factor 19 (FGF19) were collected before UDCA withdrawal and 3 months later. Liquid chromatography/tandem mass spectrometry was used for quantification of 29 plasma bile acid metabolites. Pruritus and health-related quality of life (HRQoL) were assessed with a 10-point numeric rating scale, the Medical Outcomes Study Short Form-36 (SF-36), and PBC-40 questionnaires. UDCA withdrawal resulted in a significant deterioration in liver biochemistry (increase of alkaline phosphatase of 75.6%; Pacid analysis revealed a significant decrease in lithocholic acid and its derivatives after UDCA withdrawal, but no effect on concentrations of primary bile acids aside from an increased accumulation of their taurine conjugates. After UDCA removal cholestatic parameters, taurine species of cholic acid and chenodeoxycholic acid correlated with serum FGF19 levels. No significant effect on HRQoL after UDCA withdrawal was observed; however, 42% of patients reported a deterioration in their pruritus. At 3 months, discontinuation of UDCA in patients with PSC causes significant deterioration in liver biochemistry and influences concentrations of bile acid metabolites. A proportion of patients report increased pruritus, but other short-term markers of quality of life are unaffected. © 2014 by the American Association for the Study of Liver Diseases.

Deterioration of cholestasis after endoscopic retrograde cholangiography in advanced primary sclerosing cholangitis

NARCIS (Netherlands)

Beuers, U.; Spengler, U.; Sackmann, M.; Paumgartner, G.; Sauerbruch, T.

1992-01-01

Complications of endoscopic retrograde cholangiography specific to patients with primary sclerosing cholangitis have not yet been reported. We observed transient rises of serum bilirubin after diagnostic endoscopic retrograde cholangiography in five of 15 patients and persistent rises in three of 15

Ursodeoxycholic acid in patients with ulcerative colitis and primary sclerosing cholangitis for prevention of colon cancer: a meta-analysis.

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Ashraf, Imran; Choudhary, Abhishek; Arif, Murtaza; Matteson, Michelle L; Hammad, Hazem T; Puli, Srinivas R; Bechtold, Matthew L

2012-04-01

Colon cancer risk is high in patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). Ursodeoxycholic acid has been shown to have some promise as a chemopreventive agent. A meta-analysis was performed to compare the efficacy of ursodeoxycholic acid in the prevention of colonic neoplasia in patients with UC and PSC. Multiple databases were searched (January 2011). Studies examining the use of ursodeoxycholic acid vs. no ursodeoxycholic acid or placebo in adult patients with UC and PSC were included. Data were extracted in standard forms by two independent reviewers. Meta-analysis for the effect of ursodeoxycholic acid was performed by calculating pooled estimates of adenoma or colon cancer formation by odds ratio (OR) with random effects model. Heterogeneity was assessed by calculating the I (2) measure of inconsistency. RevMan 5 was utilized for statistical analysis. Four studies (n = 281) met the inclusion criteria. The studies were of adequate quality. Ursodeoxycholic acid demonstrated no overall improvement in adenoma (OR 0.53; 95 % CI: 0.19-1.48, p = 0.23) or colon cancer occurrence (OR 0.50; 95 % CI: 0.18-1.43, p = 0.20) as compared to no ursodeoxycholic acid or placebo in patients with UC and PSC. Ursodeoxycholic acid use in patients with UC and PSC does not appear to decrease the risk of adenomas or colon cancer.

Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome

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Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Eurich, Dennis; Faber, Wladimir; Neuhaus, Peter; Seehofer, Daniel

2015-01-01

Abstract Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an important differential diagnosis in patients presenting with cholestasis and PSC-like cholangiographic changes in endoscopic retrograde cholangiography (ERC). As a relatively newly described entity, SSC-CIP is still underdiagnosed, and the diagnosis is often delayed. The present study aims to improve the early detection of SSC-CIP and the identification of its complications. A total of 2633 records of patients who underwent or were listed for orthotopic liver transplantation at the University Hospital Charité, Berlin, were analyzed retrospectively. The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed. Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis. Hypercholesterolemia occurred in at least 75% of the patients. SSC-CIP provoked a profound weight loss (mean 18 kg) in 94% of our patients. SSC-CIP was diagnosed by ERC in all patients. The 3 different cholangiographic features detected correspond roughly to the following stages: (I) evidence of biliary casts, (II) progressive destruction of intrahepatic bile ducts, and (III) picture of pruned tree. Biliary cast formation is a hallmark of SSC-CIP and was seen in 87% of our cases. In 75% of the patients, the clinical course was complicated by cholangiosepsis, cholangitic liver abscesses, acalculous cholecystitis, or gallbladder perforation. SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean). Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other types of sclerosing cholangitis. Specific characteristics enable identification of SSC-CIP. Early cooperation with a transplant center and special attention to biliary complications are required after diagnosis of SSC-CIP. PMID:26656347

High-Dose Ursodeoxycholic Acid Is Associated With the Development of Colorectal Neoplasia in Patients With Ulcerative Colitis and Primary Sclerosing Cholangitis

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Eaton, John E.; Silveira, Marina G.; Pardi, Darrell S.; Sinakos, Emmanouil; Kowdley, Kris V.; Luketic, Velimir A.C.; Harrison, M. Edwyn; McCashland, Timothy; Befeler, Alex S.; Harnois, Denise; Jorgensen, Roberta; Petz, Jan; Lindor, Keith D.

2011-01-01

OBJECTIVES Some studies have suggested that ursodeoxycholic acid (UDCA) may have a chemopreventive effect on the development of colorectal neoplasia in patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). We examined the effects of high-dose (28–30 mg/kg/day) UDCA on the development of colorectal neoplasia in patients with UC and PSC. METHODS Patients with UC and PSC enrolled in a prior, multicenter randomized placebo-controlled trial of high-dose UDCA were evaluated for the development of colorectal neoplasia. Patients with UC and PSC who received UDCA were compared with those who received placebo. We reviewed the pathology and colonoscopy reports for the development of low-grade or high-grade dysplasia or colorectal cancer. RESULTS Fifty-six subjects were followed for a total of 235 patient years. Baseline characteristics (including duration of PSC and UC, medications, patient age, family history of colorectal cancer, and smoking status) were similar for both the groups. Patients who received high-dose UDCA had a significantly higher risk of developing colorectal neoplasia (dysplasia and cancer) during the study compared with those who received placebo (hazard ratio: 4.44, 95% confidence interval: 1.30–20.10, P=0.02). CONCLUSIONS Long-term use of high-dose UDCA is associated with an increased risk of colorectal neoplasia in patients with UC and PSC. PMID:21556038

Isolated nail lichen planus with primary sclerosing cholangitis in a child

International Nuclear Information System (INIS)

Al-Ajroush, N.; Al-Khenaizan, S.

2007-01-01

Lichen planus (LP) is an uncommon, inflammatory dermatosis with characteristic lesions affecting the skin, nails and the mucous membranes. It is rare in childhood. Although nail abnormalities have been reported in 1-10% of patients with LP, the prevalence of nail involvement in affected children is unknown. Here we report a 2-year-old child with isolated nail LP, in association with primary sclerosing cholangitis. (author)

Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study.

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Gregorio, G V; Portmann, B; Karani, J; Harrison, P; Donaldson, P T; Vergani, D; Mieli-Vergani, G

2001-03-01

To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of autoimmune hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children with clinical and/or biochemical evidence of liver disease and circulating antinuclear (ANA), anti-smooth muscle (SMA), and/or liver-kidney-microsomal type 1 (LKM1) autoantibodies. They underwent liver biopsy, direct cholangiography, sigmoidoscopy, and rectal biopsy at presentation. Twenty-eight were diagnosed as AIH in the absence and 27 autoimmune sclerosing cholangitis (ASC) in the presence of radiological features of cholangiopathy. Twenty-six ASC and 20 AIH had ANA and/or SMA; 1 ASC and 8 AIH LKM1 autoantibody. Similarities between the 2 conditions included most clinical and biochemical parameters and a lower frequency of HLA DR4. Inflammatory bowel disease and histological biliary changes were more common in ASC; coagulopathy, hypoalbuminemia, lymphocytic periportal hepatitis, and HLA DR3 were more common in AIH. Histological biliary changes were observed in 65% of ASC and 31% of AIH patients. Eighty-nine percent responded to immunosuppression. Follow-up liver biopsies from 17 ASC and 18 AIH patients had similarly reduced inflammatory activity and no progression to cirrhosis. Sixteen follow-up cholangiograms from AIH patients and 9 from ASC patients were unchanged, while 8 ASC patients showed a progressive cholangiopathy. One child with AIH and ulcerative colitis developed sclerosing cholangitis 8 years after presentation. At 2 to 16 years (median, 7 years) from presentation, all patients are alive, including 4 ASC patients who underwent liver transplantation. In conclusion, ASC and AIH are similarly prevalent in childhood; cholangiography is often needed to distinguish between these 2 entities, which are likely to lie within the same disease process.

Prospective evaluation of PBC-specific health-related quality of life questionnaires in patients with primary sclerosing cholangitis.

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Raszeja-Wyszomirska, Joanna; Wunsch, Ewa; Krawczyk, Marek; Rigopoulou, Eirini I; Bogdanos, Dimitrios; Milkiewicz, Piotr

2015-06-01

Primary biliary cirrhosis and Primary sclerosing cholangitis are autoimmune cholestatic liver diseases sharing a lot in common, including a significant impairment of patients' health-related quality of life HRQoL HRQoL in PBC is assessed with disease-specific PBC-40 and PBC-27 questionnaires. A PSC-specific questionnaire has not been developed. Neither PBC-40 nor PBC-27s applicability for PSC has been evaluated. We applied these three questionnaires for HRQoL assessment in a large homogenous cohort of PSC patients. This cross-sectional study enrolled 102 Caucasian PSCs and 53 matched healthy controls and measured HRQoL using generic SF-36, and disease-specific (PBC-40/PBC-27) questionnaires. (i) SF-36. Most SF-36 domains were significantly lower in PSCs than controls. Physical Functioning and Mental Component Summary scores were significantly lower in female patients and correlated negatively with age but not with concurrent inflammatory bowel disease. Cirrhosis was associated with lower Physical Functioning, Role Physical, General Health, Vitality and Physical Component Summary. (ii) PBC-40 and PBC-27. Both tools showed similar HRQoL impairment scoring. Fatigue and Cognitive were impaired in female patients. Several correlations existed between HRQoL and laboratory parameters, including cholestatic tests and Itch. Cirrhosis correlated with Other symptoms and Fatigue PBC-40. (iii) PBC-40 vs PBC-27. Strong correlations among most domains of both questionnaires were seen, as well as between (iv) SF-36 vs PBC-40 or SF-36 vs PBC-27. This is the first study directly comparing PBC-40, PBC-27 and SF-36 in PSC. PSC patients, especially females, show HRQoL impairment. PBC-40 and PBC-27 questionnaires could be of potential use for HRQoL assessment in PSC. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Case report: A female case of isolated IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.

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Xiao, Jianchun; Li, Guanqiao; Yang, Gang; Jia, Congwei; Li, Binglu

2017-04-01

IgG4-related disease is a newly recognized fibroinflammatory disorder, characterized by tumefactive lesions, storiform fibrosis and IgG4-positive plasma cells infiltration. IgG4-related sclerosing cholangitis (IgG4-SC) is the most common extrapancreatic manifestation of IgG4-related disease, but it is frequently associated with autoimmune pancreatitis(AIP). Only few case was reported to be diagnosed with IgG4-SC in the absence of AIP, with a striking male preponderance. Here we report a female case of isolated IgG4 related sclerosing cholangitis mimicking cholangiocarcinoma. A 58-year-old woman complaint of one-month history of jaundice and right upper quadrant discomfort, and the biliary reconstruction showed full-length wall thickening and segmental stenosis. Cholangiocarcinoma was then diagnosed. Choledochoplasty was performed, followed by Roux-en-Y anastomosis. However, pathological examination revealed IgG4-related sclerosing cholangitis (IgG4-SC) and the retrospective measurement of serum IgG4 was 346 mg/dL post-operatively. The patient was followed for another nine monthswithout recurrence. The differential diagnosis between cholangiocarcinoma and IgG4-SC is challenging due to significant overlap of clinical manifestations, lab tests and imaging characteristics. However, as an afterthought of this case, typical cholangiocarcinoma rarely presents full-length wall thickening. What the case taught us was pre-operative IgG4 measurement for patients with long bile duct involvement was highly recommended in order to rule out IgG4-SC.

Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: relation to disease progression

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Sinakos, Emmanouil; Marschall, Hanns-Ulrich; Kowdley, Kris V.; Befeler, Alex; Keach, Jill; Lindor, Keith

2010-01-01

High-dose (28-30mg/kg/day) ursodeoxycholic acid (UDCA) treatment improves serum liver tests in patients with primary sclerosing cholangitis (PSC) but does not improve survival and is associated with increased rates of serious adverse events. The mechanism for the latter undesired effect remains unclear. High-dose UDCA could result in the production of hepatotoxic bile acids, such as lithocholic acid (LCA), due to limited small bowel absorption of UDCA and conversion of UDCA by bacteria in the colon. We determined the serum bile acid composition in 56 patients with PSC previously enrolled in a randomized, double-blind controlled trial of high dose UDCA versus placebo. Samples for analysis were obtained at baseline and at the end of treatment. The mean changes in UDCA (16.86 vs 0.05 μmol/L) and total bile acid (17.21 vs −0.55 μmol/L) levels were significantly higher in the UDCA group (n=29) compared to placebo (n=27) when pretreatment levels were compared (pacid (CA), deoxycholic acid (DCA) and chenodeoxycholic acid (CDCA). Patients (n=9) in the UDCA group who reached clinical endpoints of disease progression (development of cirrhosis, varices, liver transplantation or death) tend to have greater increase in their post-treatment total bile acid levels (34.99 vs 9.21 μmol/L) (pacid pool including lithocholic acid. PMID:20564380

Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma.

Science.gov (United States)

Tabata, Taku; Kamisawa, Terumi; Hara, Seiichi; Kuruma, Sawako; Chiba, Kazuro; Kuwata, Go; Fujiwara, Takashi; Egashira, Hideto; Koizumi, Koichi; Fujiwara, Junko; Arakawa, Takeo; Momma, Kumiko; Kurata, Masanao; Honda, Goro; Tsuruta, Koji; Itoi, Takao

2013-03-01

Few studies have differentiated immunoglobulin G (IgG) 4-related sclerosing cholangitis (IgG4-SC) from hilar cholangiocarcinoma (CC). Thus, we sought to investigate useful features for differentiating IgG4-SC from hilar CC. We retrospectively compared clinical, serological, imaging, and histological features of six patients with IgG4-SC and 42 patients with hilar CC. In patients with hilar CC, obstructive jaundice was more frequent (philar CC patients (philar or hepatic duct was completely obstructed in 83% of hilar CC patients (philar bile duct stenosis, was more frequent in IgG4-SC patients (philar CC.

Secondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome: A Series of 16 Cases.

Science.gov (United States)

Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Eurich, Dennis; Faber, Wladimir; Neuhaus, Peter; Seehofer, Daniel

2015-12-01

Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is an important differential diagnosis in patients presenting with cholestasis and PSC-like cholangiographic changes in endoscopic retrograde cholangiography (ERC). As a relatively newly described entity, SSC-CIP is still underdiagnosed, and the diagnosis is often delayed. The present study aims to improve the early detection of SSC-CIP and the identification of its complications.A total of 2633 records of patients who underwent or were listed for orthotopic liver transplantation at the University Hospital Charité, Berlin, were analyzed retrospectively. The clinical presentation and outcome (mean follow-up 62.7 months) of the 16 identified SSC-CIP cases were reviewed.Cholestasis was the first sign of SSC-CIP. GGT was the predominant enzyme of cholestasis. Hypercholesterolemia occurred in at least 75% of the patients. SSC-CIP provoked a profound weight loss (mean 18 kg) in 94% of our patients. SSC-CIP was diagnosed by ERC in all patients. The 3 different cholangiographic features detected correspond roughly to the following stages: (I) evidence of biliary casts, (II) progressive destruction of intrahepatic bile ducts, and (III) picture of pruned tree. Biliary cast formation is a hallmark of SSC-CIP and was seen in 87% of our cases. In 75% of the patients, the clinical course was complicated by cholangiosepsis, cholangitic liver abscesses, acalculous cholecystitis, or gallbladder perforation. SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean).Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other types of sclerosing cholangitis. Specific characteristics enable identification of SSC-CIP. Early cooperation with a transplant center and special attention to biliary complications are required after diagnosis of SSC-CIP.

Newly Diagnosed Colonic Adenocarcinoma: The Presenting Sign in a Young Woman with Undiagnosed Crohn’s Disease in the Absence of Primary Sclerosing Cholangitis and a Normal Microsatellite Instability Profile

Directory of Open Access Journals (Sweden)

Brett Matthew Lowenthal

2017-01-01

Full Text Available Ulcerative colitis has long been linked with an increased risk for colonic adenocarcinoma, whereas Crohn’s disease (CD has recently been reported to pose a similar increased risk. We report a 33-year-old healthy female with no family history who presented with abdominal pain and a colon mass. Histopathology revealed a moderately differentiated adenocarcinoma extending through the muscularis propria with metastatic lymph nodes and intact mismatch repair proteins by immunohistochemical expression and gene sequencing. The nonneoplastic grossly uninvolved background mucosa showed marked crypt distortion, crypt abscesses, CD-like lymphoid hyperplasia, transmural inflammation, and reactive epithelial atypia. Additional patient questioning revealed frequent loose stools since she was a teenager leading to diagnosis of a previously undiagnosed CD without primary sclerosing cholangitis (PSC. The adenocarcinoma is suspected to be related to the underlying CD. Newly diagnosed adenocarcinoma in a young female as the presenting sign for CD in the absence of PSC is extremely rare.

Trigger mechanisms of secondary sclerosing cholangitis in critically ill patients.

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Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Hetzer, Roland; Schaffartzik, Walter; Tryba, Michael; Neuhaus, Peter; Seehofer, Daniel

2015-03-31

In recent years the development of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) has increasingly been perceived as a separate disease entity. About possible trigger mechanisms of SSC-CIP has been speculated, systematic investigations on this issue are still lacking. The purpose of this study was to evaluate the prevalence and influence of promoting factors. Temporality, consistency and biological plausibility are essential prerequisites for causality. In this study, we investigated the temporality and consistency of possible triggers of SSC-CIP in a large case series. Biological plausibility of the individual triggers is discussed in a scientific context. SSC-CIP cases were recruited retrospectively from 2633 patients who underwent or were scheduled for liver transplantation at the University Hospital Charité, Berlin. All patients who developed secondary sclerosing cholangitis in association with intensive care treatment were included. Possible trigger factors during the course of the initial intensive care treatment were recorded. Sixteen patients (68% males, mean age 45.87 ± 14.64 years) with a confirmed diagnosis of SSC-CIP were identified. Of the 19 risk factors investigated, particularly severe hypotension with a prolonged decrease in mean arterial blood pressure (MAP) to <65 mmHg and systemic inflammatory response syndrome (SIRS) were established as possible triggers of SSC-CIP. The occurrence of severe hypotension appears to be the first and most significant step in the pathogenesis. It seems that severe hypotension has a critical effect on the blood supply of bile ducts when it occurs together with additional microcirculatory disturbances. In critically ill patients with newly acquired cholestasis the differential diagnosis of SSC-CIP should be considered when they have had an episode of haemodynamic instability with a prolonged decrease in MAP, initial need for large amounts of blood transfusions or colloids, and early

IgG4-associated sclerosing cholangitis masquerading as hilar cholangiocarcinoma.

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Yadav, Kamal Sunder; Sali, Priyanka Akhilesh; Mansukhani, Verushka M; Shah, Rajiv; Jagannath, P

2016-07-01

IgG4-sclerosing cholangitis (IgG4-SC) commonly presents with type 1 autoimmune pancreatitis. Isolated IgG4-SC is rare. Differentiating IgG4-SC from cholangiocarcinoma preoperatively is challenging due to overlapping radio-clinical manifestations and difficult preoperative histology. We present three cases preoperatively diagnosed and surgically treated as hilar cholangiocarcinoma. First and second cases presented with cholangiocarcinoma with portal vein involvement and third with a malignant-appearing hilar stricture. On histopathology, IgG4-SC was diagnosed in the first two cases. Third patient had raised serum IgG4, and histopathology was inconclusive for IgG4-SC and negative for malignancy. However, she responded to steroid therapy.

Analysis of U2 small nuclear RNA fragments in the bile differentiates cholangiocarcinoma from primary sclerosing cholangitis and other benign biliary disorders.

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Baraniskin, Alexander; Nöpel-Dünnebacke, Stefanie; Schumacher, Brigitte; Gerges, Christian; Bracht, Thilo; Sitek, Barbara; Meyer, Helmut E; Gerken, Guido; Dechene, Alexander; Schlaak, Jörg F; Schroers, Roland; Pox, Christian; Schmiegel, Wolff; Hahn, Stephan A

2014-07-01

Up to now the diagnosis of early stage cholangiocarcinoma (CC) has remained difficult, with low sensitivities reported for current diagnostic methods. Based on recent promising findings about circulating U2 small nuclear RNA fragments (RNU2-1f) as novel blood-based biomarkers for pancreatic and colorectal adenocarcinoma, we studied the utility of RNU2-1f as a diagnostic marker of CC in bile fluid. Bile fluid was collected from patients with CC (n = 12), controls (patients with choledocholithiasis) (n = 11) and with primary sclerosing cholangitis (PSC; n = 11). RNU2-1f levels were measured by real-time polymerase chain reaction normalized to cel-54. Measurement of RNU2-1f levels in bile fluids enabled the differentiation of patients with CC from controls in all cases. Furthermore, RNU2-1f levels in bile fluids of patients with CC were significantly higher than in patients with PSC, resulting in a receiver-operating characteristic curve area of 0.856, with sensitivity of 67 % and specificity of 91 %. Our data suggest that the measurement of RNU2-1 fragments detected in the bile fluid can be used as a diagnostic marker for CC and should be included in future prospective diagnostic studies for this disease entity.

Combination Therapy of All-Trans Retinoic Acid With Ursodeoxycholic Acid in Patients With Primary Sclerosing Cholangitis: A Human Pilot Study.

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Assis, David N; Abdelghany, Osama; Cai, Shi-Ying; Gossard, Andrea A; Eaton, John E; Keach, Jill C; Deng, Yanhong; Setchell, Kenneth D R; Ciarleglio, Maria; Lindor, Keith D; Boyer, James L

2017-02-01

To perform an exploratory pilot study of all-trans retinoic acid (ATRA) combined with ursodeoxycholic acid (UDCA) in patients with primary sclerosing cholangitis (PSC). PSC is a progressive disorder for which there is no accepted therapy. Studies in human hepatocyte cultures and in animal models of cholestasis indicate that ATRA might have beneficial effects in cholestatic disorders. ATRA (45 mg/m/d, divided and given twice daily) was combined with moderate-dose UDCA in patients with PSC who had incomplete response to UDCA monotherapy. The combination was administered for 12 weeks, followed by a 12-week washout in which patients returned to UDCA monotherapy. We measured alkaline phosphatase (ALP), alanine aminotransferase (ALT), bilirubin, cholesterol, bile acids, and the bile acid intermediate 7α-hydroxy-4-cholesten-3-one (C4) at baseline, week 12, and after washout. Fifteen patients completed 12 weeks of therapy. The addition of ATRA to UDCA reduced the median serum ALP levels (277±211 to 243±225 U/L, P=0.09) although this, the primary endpoint, did not reach significance. In contrast, median serum ALT (76±55 to 46±32 U/L, P=0.001) and C4 (9.8±19 to 7.9±11 ng/mL, P=0.03) levels significantly decreased. After washout, ALP and C4 levels nonsignificantly increased, whereas ALT levels significantly increased (46±32 to 74±74, P=0.0006), returning to baseline. In this human pilot study, the combination of ATRA and UDCA did not achieve the primary endpoint (ALP); however, it significantly reduced ALT and the bile acid intermediate C4. ATRA appears to inhibit bile acid synthesis and reduce markers of inflammation, making it a potential candidate for further study in PSC (NCT 01456468).

Gut barrier failure biomarkers are associated with poor disease outcome in patients with primary sclerosing cholangitis

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Tornai, Tamas; Palyu, Eszter; Vitalis, Zsuzsanna; Tornai, Istvan; Tornai, David; Antal-Szalmas, Peter; Norman, Gary L; Shums, Zakera; Veres, Gabor; Dezsofi, Antal; Par, Gabriella; Par, Alajos; Orosz, Peter; Szalay, Ferenc; Lakatos, Peter Laszlo; Papp, Maria

2017-01-01

AIM To assess the prevalence of a panel of serologic markers that reflect gut barrier dysfunction in a mixed cohort of pediatric and adult primary sclerosing cholangitis (PSC) patients. METHODS Sera of 67 PSC patients [median age (range): 32 (5-79) years, concomitant IBD: 67% and cirrhosis: 20%] were assayed for the presence of antibodies against to F-actin (AAA IgA/IgG) and gliadin (AGA IgA/IgG)] and for serum level of intestinal fatty acid-binding protein (I-FABP) by ELISA. Markers of lipopolysaccharide (LPS) exposure [LPS binding protein (LBP)] and various anti-microbial antibodies [anti-OMP Plus IgA and endotoxin core IgA antibody (EndoCAb)] were also determined. Poor disease outcome was defined as orthotopic liver transplantation and/or liver-related death during the follow-up [median: 99 (14-106) mo]. One hundred and fifty-three healthy subjects (HCONT) and 172 ulcerative colitis (UC) patients were the controls. RESULTS A total of 28.4%, 28.0%, 9% and 20.9% of PSC patients were positive for AAA IgA, AAA IgG, AGA IgA and AGA IgG, respectively. Frequencies of AAA IgA and AAA IgG (P < 0.001, for both) and AGA IgG (P = 0.01, for both) but not AGA IgA were significantly higher compared to both of the HCONT and the UC groups. In survival analysis, AAA IgA-positivity was revealed as an independent predictor of poor disease outcome after adjusting either for the presence of cirrhosis [HR = 5.15 (1.27-20.86), P = 0.022 or for the Mayo risk score (HR = 4.24 (0.99-18.21), P = 0.052]. AAA IgA-positivity was significantly associated with higher frequency of anti-microbial antibodies (P < 0.001 for EndoCab IgA and P = 0.012 for anti-OMP Plus IgA) and higher level of the enterocyte damage marker (median I-FABPAAA IgA pos vs neg: 365 vs 166 pg/mL, P = 0.011), but not with serum LBP level. CONCLUSION Presence of IgA type AAA identified PSC patients with progressive disease. Moreover, it is associated with enhanced mucosal immune response to various microbial antigens and

MR elastography in primary sclerosing cholangitis: correlating liver stiffness with bile duct strictures and parenchymal changes.

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Bookwalter, Candice A; Venkatesh, Sudhakar K; Eaton, John E; Smyrk, Thomas D; Ehman, Richard L

2018-04-07

To determine correlation of liver stiffness measured by MR Elastography (MRE) with biliary abnormalities on MR Cholangiopancreatography (MRCP) and MRI parenchymal features in patients with primary sclerosing cholangitis (PSC). Fifty-five patients with PSC who underwent MRI of the liver with MRCP and MRE were retrospectively evaluated. Two board-certified abdominal radiologists in agreement reviewed the MRI, MRCP, and MRE images. The biliary tree was evaluated for stricture, dilatation, wall enhancement, and thickening at segmental duct, right main duct, left main duct, and common bile duct levels. Liver parenchyma features including signal intensity on T2W and DWI, and hyperenhancement in arterial, portal venous, and delayed phase were evaluated in nine Couinaud liver segments. Atrophy or hypertrophy of segments, cirrhotic morphology, varices, and splenomegaly were scored as present or absent. Regions of interest were placed in each of the nine segments on stiffness maps wherever available and liver stiffness (LS) was recorded. Mean segmental LS, right lobar (V-VIII), left lobar (I-III, and IVA, IVB), and global LS (average of all segments) were calculated. Spearman rank correlation analysis was performed for significant correlation. Features with significant correlation were then analyzed for significant differences in mean LS. Multiple regression analysis of MRI and MRCP features was performed for significant correlation with elevated LS. A total of 439/495 segments were evaluated and 56 segments not included in MRE slices were excluded for correlation analysis. Mean segmental LS correlated with the presence of strictures (r = 0.18, p duct strictures. Segments with increased LS show T2 hyperintensity, DWI hyperintensity, and post-contrast hyperenhancement. Global liver stiffness shows a moderate correlation with number of segmental strictures and significantly correlates with spleen stiffness, splenomegaly, and varices.

Ursodeoxycholic acid therapy for primary sclerosing cholangitis: results of a 2-year randomized controlled trial to evaluate single versus multiple daily doses

NARCIS (Netherlands)

van Hoogstraten, H. J.; Wolfhagen, F. H.; van de Meeberg, P. C.; Kuiper, H.; Nix, G. A.; Becx, M. C.; Hoek, A. C.; van Houte, D. P.; Rijk, M. C.; Salemans, J. M.; Scherpenisse, J.; Schrijver, M.; Smit, A. M.; Spoelstra, P.; Stadhouders, P. H.; Tan, T. G.; Hop, W. C.; ten Kate, F. J.; vanBerge-Henegouwen, G. P.; Schalm, S. W.; van Buuren, H. R.

1998-01-01

Ursodeoxycholic acid has been reported to be of potential benefit for primary sclerosing cholangitis but little is known about the long-term biochemical, histological and radiological efficacy or the optimum frequency of ursodeoxycholic acid administration. A 2-year multicentre randomised controlled

Celiac Disease, Enteropathy-Associated T-Cell Lymphoma, and Primary Sclerosing Cholangitis in One Patient: A Very Rare Association and Review of the Literature

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N. Majid

2013-01-01

Full Text Available Enteropathy-associated T-cell lymphoma (EATL is a very rare peripheral T-cell lymphoma which is mostly associated with celiac disease. However, the association of primary sclerosing cholangitis and enteropathy-associated T-cell lymphoma is uncommon. Herein we report and discuss the first case of patient who presented simultaneously with these two rare diseases. It is a 54-year-old man who stopped gluten-free diet after 15 years history of celiac disease. The diagnosis was based on the histological examination of duodenal biopsy and the diagnosis of primary sclerosing cholangitis was made on liver biopsy, as well as the magnetic resonance cholangiogram. The treatment of EATL is mainly based on chemotherapy in addition to the optimal management of complications and adverse events that impact on the response to treatment and clinical outcomes, although the prognosis remains remarkably very poor.

Leukemoid reaction, a rare manifestation of autoimmune hemolytic anemia in a case of small duct primary sclerosing cholangitis.

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Salagre, Kaustubh D; Sahay, Ravindra Nath; Patil, Anuja; Pati, Anuja; Joshi, Amita; Shukla, Akash

2013-10-01

A 48 year old lady presented with jaundice and exertional breathlesness. Her laboratory reports showed anaemia, reticulocytosis, leucocytosis, elevated Lactate Dehydrogenase (LDH), alkaline phosphatase levels, hyperbillirubinemia and positive direct Coomb's test. After ruling out all the other causes of autoimmunity and hemolytic anemia, she was diagnosed as leukemoid reaction due to autoimmune hemolytic anemia with primary sclerosing cholangitis. Patient showed immediate improvement after corticosteroids.

Pruritus is associated with severely impaired quality of life in patients with primary sclerosing cholangitis.

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Gotthardt, Daniel Nils; Rupp, Christian; Bruhin, Miriam; Schellberg, Dieter; Weiss, Karl H; Stefan, Reinhard; Donnerstag, Nadine; Stremmel, Wolfgang; Löwe, Bernd; Juenger, Jana; Sauer, Peter

2014-12-01

Quality of life, fundamental to the individual patient, has shown a lack of correlation with severity in research on several diseases. Thus, we aimed to identify factors associated with quality of life in patients with primary sclerosing cholangitis. The Short Form Health Survey and the Patient Health Questionnaire were used to assess quality of life and depression. Complete data sets of 113 patients were analyzed for correlation with sex, age, presence of concomitant inflammatory bowel disease and dominant stenosis, frequency of pruritus, and Mayo Risk Score. Physical functioning decreased with age (P<0.001). Further, women experienced more prominent role limitations because of physical (P<0.03) and emotional (P<0.01) problems. Although patients' quality of life and depression scores were only slightly lower than normal, more frequent pruritus was associated with a considerable reduction in quality of life in terms of physical and social functioning, general and mental health, bodily pain, vitality, and roles (because of physical problems) (P<0.01). It did not differ significantly according to the Mayo Risk Score or the presence of dominant stenoses. Depression scores were only significantly affected in patients with more frequent pruritus. Pruritus severely affects quality of life in patients with primary sclerosing cholangitis and is associated with depression to varying extents, although the most commonly used parameters of disease severity do not correspond to quality of life in these patients. These findings need to be considered with respect to treatment outcomes and indications for liver transplantation.

Vascular adhesion protein-1 is elevated in primary sclerosing cholangitis, is predictive of clinical outcome and facilitates recruitment of gut-tropic lymphocytes to liver in a substrate-dependent manner.

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Trivedi, Palak J; Tickle, Joseph; Vesterhus, Mette Nåmdal; Eddowes, Peter J; Bruns, Tony; Vainio, Jani; Parker, Richard; Smith, David; Liaskou, Evaggelia; Thorbjørnsen, Liv Wenche; Hirschfield, Gideon M; Auvinen, Kaisa; Hubscher, Stefan G; Salmi, Marko; Adams, David H; Weston, Chris J

2018-06-01

Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of IBD. This clinical association is linked pathologically to the recruitment of mucosal T cells to the liver, via vascular adhesion protein (VAP)-1-dependent enzyme activity. Our aim was to examine the expression, function and enzymatic activation of the ectoenzyme VAP-1 in patients with PSC. We examined VAP-1 expression in patients with PSC, correlated levels with clinical characteristics and determined the functional consequences of enzyme activation by specific enzyme substrates on hepatic endothelium. The intrahepatic enzyme activity of VAP-1 was elevated in PSC versus immune-mediated disease controls and non-diseased liver (pgut-tropic α4β7 + lymphocytes to hepatic endothelial cells in vitro under flow was attenuated by 50% following administration of the VAP-1 inhibitor semicarbazide (pgut bacteria-was the most efficient (yielded the highest enzymatic rate) and efficacious in its ability to induce expression of functional mucosal addressin cell adhesion molecule-1 on hepatic endothelium. In a prospectively evaluated patient cohort with PSC, elevated serum soluble (s)VAP-1 levels predicted poorer transplant-free survival for patients, independently (HR: 3.85, p=0.003) and additively (HR: 2.02, p=0.012) of the presence of liver cirrhosis. VAP-1 expression is increased in PSC, facilitates adhesion of gut-tropic lymphocytes to liver endothelium in a substrate-dependent manner, and elevated levels of its circulating form predict clinical outcome in patients. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Een kind met primaire scleroserende cholangitis

NARCIS (Netherlands)

Rademaker, A. A. E. M.; Benninga, M. A.; Beuers, U. H. W.; Plötz, F. B.

2015-01-01

Primary sclerosing cholangitis is a rare liver disease which is mainly diagnosed in adults. This chronic progressive disease, characterised by inflammation, fibrosis and strictures of the intra- and extrahepatic bile ducts, leads to cirrhosis. There is a strong association between primary sclerosing

Primary Sclerosing Cholangitis (PSC)

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CD8 T cells primed in the gut-associated lymphoid tissue induce immune-mediated cholangitis in mice.

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Seidel, Daniel; Eickmeier, Ira; Kühl, Anja A; Hamann, Alf; Loddenkemper, Christoph; Schott, Eckart

2014-02-01

The pathogenesis of primary sclerosing cholangitis (PSC) remains poorly understood. Since PSC predominantly occurs in patients with inflammatory bowel disease, autoimmunity triggered by activated T cells migrating from the gut to the liver is a possible mechanism. We hypothesized that T cells primed in the gut-associated lymphoid tissue (GALT) by a specific antigen migrate to the liver and cause cholangitis when they recognize the same antigen on cholangiocytes. We induced ovalbumin-dependent colitis in mice that express ovalbumin in biliary epithelia (ASBT-OVA mice) and crossed ASBT-OVA mice with mice that express ovalbumin in enterocytes (iFABP-OVA mice). We analyzed T-cell activation in the GALT and crossreactivity to the same antigen in the liver as well as the effects of colitis per se on antigen-presentation and T-cell activation in the liver. Intrarectal application of ovalbumin followed by transfer of CD8 OT-I T cells led to antigen-dependent colitis. CD8 T cells primed in the GALT acquired effector function and the capability to migrate to the liver, where they caused cholangitis in a strictly antigen-dependent manner. Likewise, cholangitis developed in mice expressing ovalbumin simultaneously in biliary epithelia and enterocytes after transfer of OT-I T cells. Dextran sodium sulfate colitis led to increased levels of inflammatory cytokines in the portal venous blood, induced activation of resident liver dendritic cells, and promoted the induction of T-cell-dependent cholangitis. Our data strengthen the notion that immune-mediated cholangitis is caused by T cells primed in the GALT and provide the first link between colitis and cholangitis in an antigen-dependent mouse model. © 2013 by the American Association for the Study of Liver Diseases.

IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis.

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Detlefsen, Sönke; Klöppel, Günter

2018-04-01

In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts. The pancreatic and biliary alterations have been described under the terms autoimmune pancreatitis (AIP) and sclerosing cholangitis, respectively. These diseases are currently more precisely called IgG4-related pancreatitis (or type 1 AIP to distinguish it from type 2 AIP that is unrelated to IgG4-RD) and IgG4-related sclerosing cholangitis (IgG4-related SC). Clinically and grossly, both diseases commonly imitate pancreatic and biliary adenocarcinoma, tumors that are well known for their dismal prognosis. As IgG4-RD responds to steroid treatment, making a resection of a suspected tumor unnecessary, a biopsy is often required to establish the preoperative diagnosis. This review discusses the morphologic spectrum of IgG4-related pancreatitis and IgG4-related SC and focuses on the biopsy relevant histologic features for the diagnosis and differential diagnosis of these diseases.

IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis. A recurrent case after a 5-year history of spontaneous remission.

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Miura, Hideaki; Miyachi, Yasutaka

2009-07-06

A new clinicopathological concept of IgG4-related sclerosing disease affecting various organs has recently been proposed in relation to autoimmune pancreatitis. This report describes the case of IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis, which recurred after a long period of spontaneous remission. An 80-year-old Japanese man presented with obstructive jaundice owing to a hepatic hilum bile duct stricture. Coincidentally, a soft tissue mass surrounding the abdominal aorta, suggesting retroperitoneal fibrosis, was identified. Unexpectedly, spontaneous regression of obstructive jaundice together with retroperitoneal fibrosis occurred. The presence of high serum IgG4 concentrations measured later led us to consider a possible association with autoimmune pancreatitis; however, there were no clinical features confirming autoimmune pancreatitis. After a 5-year history of spontaneous clinical remission, there was an elevation of serum IgG4 levels and renal dysfunction owing to bilateral hydronephrosis caused by a reemergence of the retroperitoneal mass. Evaluation by endoscopic retrograde cholangiopancreatography revealed a biliary stricture, suggesting sclerosing cholangitis which was observed without the presence of any pancreatic duct abnormality. The subsequent excellent results obtained using steroid therapy, namely the decrease in serum IgG4 levels and the regression of the retroperitoneal mass, strongly suggested that the present case was an IgG4-related sclerosing disease. Aside from high serum IgG4 concentrations, markedly elevated levels of serum IgE was found retrospectively, although the clinical significance remains unknown. When we encounter fibrotic diseases of unknown etiology, we should measure serum IgG4 concentrations and monitor the disease activity over long periods even after achieving clinical remission.

Celiac disease (CD, ulcerative colitis (UC, and primary sclerosing cholangitis (PSC in one patient: a family study Enfermedad celiaca (EC, colitis ulcerosa (CU y colangitis esclerosante primaria (CEP asociadas en el mismo paciente: estudio familiar

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V. Cadahía

2005-12-01

Full Text Available We discuss the case of a 17-year-old male who at the age of 7 was diagnosed with celiac disease (CD together with ulcerative colitis (UC and primary sclerosing cholangitis (PSC. The patient was treated with gluten-free diet and immunosuppressive drugs (azathioprine, and currently remains asymptomatic. The patient's younger, 12-year-old sister was diagnosed with CD when she was 1.5 years old, and at 7 years she developed type-I diabetes mellitus, which was difficult to control. A family study was made, and both parents were found to be affected with silent CD. All were DQ2 (+. In relation to the case and family study, we provide a series of comments related to CD and its complications.Presentamos el caso de un varón de 17 años, que a la edad de 7 años fue diagnosticado de enfermedad celiaca (EC junto con una colitis ulcerosa (CU y una colangitis esclerosante primaria (CEP asociadas. Fue tratado con DSG e inmuno-supresores tipo azatioprina y se encuentra asintomático en la actualidad. Su hermana menor de 12 años, fue diagnosticada de EC cuando tenía 1,5 años y a los 7 años desarrolló una DM tipo 1 de difícil control. Se realizó un estudio familiar y ambos padres están afectos de una EC silente. Todos ellos son DQ2 (+. A propósito del caso y estudio familiar, se hacen una serie de consideraciones sobre la enfermedad celiaca y el desarrollo de complicaciones.

Contrast enhanced liver MRI in patients with primary sclerosing cholangitis: inverse appearance of focal confluent fibrosis on delayed phase MR images with hepatocyte specific versus extracellular gadolinium based contrast agents.

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Husarik, Daniela B; Gupta, Rajan T; Ringe, Kristina I; Boll, Daniel T; Merkle, Elmar M

2011-12-01

To assess the enhancement pattern of focal confluent fibrosis (FCF) on contrast-enhanced hepatic magnetic resonance imaging (MRI) using hepatocyte-specific (Gd-EOB-DTPA) and extracellular (ECA) gadolinium-based contrast agents in patients with primary sclerosing cholangitis (PSC). After institutional review board approval, 10 patients with PSC (6 male, 4 female; 33-61 years) with 13 FCF were included in this retrospective study. All patients had a Gd-EOB-DTPA-enhanced liver MRI exam, and a comparison ECA-enhanced MRI. On each T1-weighted dynamic dataset, the signal intensity (SI) of FCF and the surrounding liver as well as the paraspinal muscle (M) were measured. In the Gd-EOB-DTPA group, hepatocyte phase images were also included. SI FCF/SI M, SI liver/SI M, and [(SI liver - SI FCF)/SI liver] were compared between the different contrast agents for each dynamic phase using the paired Student's t-test. There was no significant difference in SI FCF/SI M in all imaging phases. SI liver/SI M was significantly higher for the Gd-EOB-DTPA group in the delayed phase (P DTPA group, mean [(SI liver - SI FCF)/SI liver] were as follows (values for ECA group in parentheses): unenhanced phase: 0.26 (0.26); arterial phase: 0.01 (-0.31); portal venous phase (PVP): -0.05 (-0.26); delayed phase (DP): 0.14 (-0.54); and hepatocyte phase: 0.26. Differences were significant for the DP (P DTPA-enhanced images. Copyright © 2011 AUR. Published by Elsevier Inc. All rights reserved.

Efficacy and safety of vedolizumab as a treatment option for moderate to severe refractory ulcerative colitis in two patients after liver transplant due to primary sclerosing cholangitis

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Raúl Vicente Olmedo-Martín

Full Text Available Vedolizumab is a humanized IgG1 monoclonal antibody that selectively blocks the lymphocyte integrin α4β7 and prevents its interaction with endothelial adhesion molecules and subsequent transmigration to the gastrointestinal tract. The drug was approved in 2014 for the induction and maintenance treatment of ulcerative colitis and moderate to severe Crohn's disease that is refractory or intolerant to conventional treatment with corticoids and immunosuppressants and/or anti-TNFα drugs. However, inflammatory bowel disease has a variable behavior following liver transplant. One third of patients with ulcerative colitis associated with primary sclerosing cholangitis are expected to deteriorate despite receiving immunosuppression to prevent rejection. There is limited experience with anti-TNFα agents in patients with inflammatory bowel disease in the setting of liver transplantation and the studies to date involve a limited number of cases. The efficacy and safety data of vedolizumab in this situation are unreliable and very preliminary. We present two cases with the aim to present the efficacy and safety of vedolizumab after one year of treatment in two patients who underwent a transplant due to primary sclerosing cholangitis. One case had de novo post-transplant ulcerative colitis refractory to two anti-TNFα drugs (golimumab and infliximab. The other patient had a colostomy due to fulminant colitis and developed severe ulcerative proctitis refractory to infliximab after reconstruction with an ileorectal anastomosis.

Hepatic Encephalopathy

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Full Text Available ... Biliary Cirrhosis (PBC) and Primary Sclerosing Cholangitis (PSC) Metabolic diseases such as Hemochromatosis, Wilson disease and Alpha- ... FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related Liver Disease Alpha-1 Antitrypsin Deficiency ...

Water-soluble C60 fullerenes reduce manifestations of acute cholangitis in rats

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Kuznietsova, H. M.; Lynchak, O. V.; Dziubenko, N. V.; Osetskyi, V. L.; Ogloblya, O. V.; Prylutskyy, Yu I.; Rybalchenko, V. K.; Ritter, U.; Scharff, P.

2018-03-01

Sclerosing cholangitis is the liver disease of uncertain etiology, extremely unfavorable prognosis and lack of effective medication therapy. Therefore, the effect of water-soluble biocompatible C60 fullerenes (C60FAS) on the liver functional state on rat acute-cholangitis model was aimed to be discovered. Acute cholangitis was simulated by single α-naphthyl isothiocyanate (ANIT, 100 mg/kg) per os administration; C60FAS (0.5 mg/kg) was administered either per os or intraperitoneally in 24 and 48 h after ANIT ingestion, and in 72 h the animals were sacrificed. The activities of alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), lactate dehydrogenase (LDH), the total and direct bilirubin, creatinine and urea in the blood serum were determined, and the liver morphological state was assessed. In animals experienced ANIT-induced acute cholangitis, the total and direct bilirubin, creatinine, ALT, AST, ALP and LDH 1.5-4-fold increase were observed, indicating cytolysis of hepatocytes, cholestasis, and renal dysfunction. The features of periductal fibrosis, biliary epithelium atrophy, and portal-portal linking septa formation were detected, confirming the sclerosing cholangitis development. C60FAS promoted to the normalization of direct and total bilirubin levels, the ALT activity and diminution of fibrotic features. In addition, C60FAS intraperitoneal administration also normalized the ALP activity, indicating the attenuation of disease symptoms. However, the AST activity and creatinine level remained unchanged, and the LDH activity even increased, manifesting the partial persistence of cholestasis and renal dysfunction. Thus, the therapeutic application of C60FAS promotes a partial protection of liver against cholangitis.

Inhibition of intestinal bile acid absorption improves cholestatic liver and bile duct injury in a mouse model of sclerosing cholangitis.

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Baghdasaryan, Anna; Fuchs, Claudia D; Österreicher, Christoph H; Lemberger, Ursula J; Halilbasic, Emina; Påhlman, Ingrid; Graffner, Hans; Krones, Elisabeth; Fickert, Peter; Wahlström, Annika; Ståhlman, Marcus; Paumgartner, Gustav; Marschall, Hanns-Ulrich; Trauner, Michael

2016-03-01

Approximately 95% of bile acids (BAs) excreted into bile are reabsorbed in the gut and circulate back to the liver for further biliary secretion. Therefore, pharmacological inhibition of the ileal apical sodium-dependent BA transporter (ASBT/SLC10A2) may protect against BA-mediated cholestatic liver and bile duct injury. Eight week old Mdr2(-/-) (Abcb4(-/-)) mice (model of cholestatic liver injury and sclerosing cholangitis) received either a diet supplemented with A4250 (0.01% w/w) - a highly potent and selective ASBT inhibitor - or a chow diet. Liver injury was assessed biochemically and histologically after 4weeks of A4250 treatment. Expression profiles of genes involved in BA homeostasis, inflammation and fibrosis were assessed via RT-PCR from liver and ileum homogenates. Intestinal inflammation was assessed by RNA expression profiling and immunohistochemistry. Bile flow and composition, as well as biliary and fecal BA profiles were analyzed after 1week of ASBT inhibitor feeding. A4250 improved sclerosing cholangitis in Mdr2(-/-) mice and significantly reduced serum alanine aminotransferase, alkaline phosphatase and BAs levels, hepatic expression of pro-inflammatory (Tnf-α, Vcam1, Mcp-1) and pro-fibrogenic (Col1a1, Col1a2) genes and bile duct proliferation (mRNA and immunohistochemistry for cytokeratin 19 (CK19)). Furthermore, A4250 significantly reduced bile flow and biliary BA output, which correlated with reduced Bsep transcription, while Ntcp and Cyp7a1 were induced. Importantly A4250 significantly reduced biliary BA secretion but preserved HCO3(-) and biliary phospholipid secretion resulting in an increased HCO3(-)/BA and PL/BA ratio. In addition, A4250 profoundly increased fecal BA excretion without causing diarrhea and altered BA pool composition, resulting in diminished concentrations of primary BAs tauro-β-muricholic acid and taurocholic acid. Pharmacological ASBT inhibition attenuates cholestatic liver and bile duct injury by reducing biliary BA

Inflammatory bowel disease after liver transplantation : Risk factors for recurrence and De novo disease

NARCIS (Netherlands)

Verdonka, RC; Dijkstra, G; Haagsma, EB; Shostrom, VK; Van den Berg, AP; Kleibeuker, JH

Inflammatory bowel disease (IBD) is associated with primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) and can recur or develop de novo after orthotopic liver transplantation (OLT). The aim of this study was to investigate the incidence and severity of IBD after liver

IgG4-Associated Cholangitis--A Mimic of PSC

NARCIS (Netherlands)

Beuers, Ulrich; Hubers, Lowiek M.; Doorenspleet, Marieke; Maillette de Buy Wenniger, Lucas; Klarenbeek, Paul L.; Boonstra, Kirsten; Ponsioen, Cyriel; Rauws, Erik; de Vries, Niek

2015-01-01

IgG4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract representing a major manifestation of IgG4-related disease (IgG4-RD) often with elevation of serum IgG4 levels, infiltration of IgG4+ plasma cells in the affected tissue and good response to immunosuppressive

IgG4-related sclerosing cholangitis overlapping with autoimmune hepatitis: Report of a case.

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Li, Hongyan; Sun, Li; Brigstock, David R; Qi, Lina; Gao, Runping

2017-05-01

IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IgG4-RD) but the presence of IgG4-SC in the porta hepatis is difficult to differentiate from hilar cholangiocarcinoma (HCCA). IgG4-related autoimmune hepatitis (IgG4-related AIH) is extremely rare and it is not fully clear whether IgG4-related AIH is a hepatic manifestation of IgG4-RD or a subtype of AIH. We present a rare case of a 52-year-old male who was admitted with obstructive jaundice and itchy skin. He primarily presented a severe bile duct stricture in the porta hepatis and an elevated serum level of carbohydrate antigen 19-9 (CA19-9) mimicking HCCA. The patient underwent a surgical resection of the left hepatic lobular and cholecyst as well as common bile duct with a right hepatico-jejunostomy. He was finally diagnosed as IgG4-SC accompanied with IgG4-related AIH by immunohistochemistry, but he lacked conventional autoantibodies. The patient responded well to steroid therapy and remains healthy with no signs of recurrence at six-month follow-up. This is the first case report that hepatic portal IgG4-SC overlapping with IgG4-related AIH without the presence of conventional autoantibodies. Additionally, we suggest that IgG4-RD should be always considered in case of a bile duct stricture in the porta hepatis to avoid unnecessary surgical operation. Copyright © 2017 Elsevier GmbH. All rights reserved.

Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation

DEFF Research Database (Denmark)

Lindström, Lina; Jørgensen, Kristin K; Boberg, Kirsten M

2018-01-01

PSC for prognosis. MATERIALS AND METHODS: All liver transplanted PSC patients in the Nordic countries between 1984 and 2007 (n = 440), identified by the Nordic Liver Transplant Registry, were studied. Data were retrieved from patients' chart reviews. Multivariable Cox regression models were used to calculate risk...

Autoimmune liver serology: current diagnostic and clinical challenges.

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Bogdanos, Dimitrios-P; Invernizzi, Pietro; Mackay, Ian-R; Vergani, Diego

2008-06-07

Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseases (AiLD), namely autoimmune hepatitis types 1 and 2 (AIH-1 and 2), primary biliary cirrhosis (PBC), and the sclerosing cholangitis variants in adults and children. AIH-1 is specified by anti-nuclear antibody (ANA) and smooth muscle antibody (SMA). AIH-2 is specified by antibody to liver kidney microsomal antigen type-1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1). SMA, ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation. PBC is specified by antimitochondrial antibodies (AMA) reacting with enzymes of the 2-oxo-acid dehydrogenase complexes (chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly reacting with nuclear pore gp210 and nuclear body sp100. Sclerosing cholangitis presents as at least two variants, first the classical primary sclerosing cholangitis (PSC) mostly affecting adult men wherein the only (and non-specific) reactivity is an atypical perinuclear antineutrophil cytoplasmic antibody (p-ANCA), also termed perinuclear anti-neutrophil nuclear antibodies (p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis (ASC) with serological features resembling those of type 1 AIH. Liver diagnostic serology is a fast-expanding area of investigation as new purified and recombinant autoantigens, and automated technologies such as ELISAs and bead assays, become available to complement (or even compete with) traditional immunofluorescence procedures. We survey for the first time global trends in quality assurance impacting as it does on (1) manufacturers/purveyors of kits and reagents, (2) diagnostic service laboratories that fulfill clinicians' requirements, and (3) the end-user, the physician providing patient care, who must properly interpret test results in the overall clinical context.

Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis

DEFF Research Database (Denmark)

de Vries, Elisabeth M G; de Krijger, Manon; Färkkilä, Martti

2017-01-01

across a multicenter PSC cohort. Liver biopsies from PSC patients were collected from seven European institutions. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems. Biopsies were independently scored by six liver pathologists for interobserver agreement.......19-5.80] for endpoint 2 and HR, 2.06 [95% CI, 1.09-3.89] for endpoint 3). Only the Nakanuma staging system was independently associated with endpoint 1: HR, 2.14 (95% CI, 1.22-3.77). Interobserver agreement was moderate for Nakanuma stage (κ = 0.56) and substantial for Nakanuma component fibrosis (κ = 0.67), Ishak...

A New Mutation Causing Progressive Familiar Intrahepatic Cholestasis Type 3 in Association with Autoimmune Hepatitis

Directory of Open Access Journals (Sweden)

Hugo M Oliveira

2017-03-01

Full Text Available Background: Some patients exhibit features of both autoimmune hepatitis (AIH and primary sclerosing cholangitis (PSC. Similarly, patients with progressive familial intrahepatic cholestasis type 3 (PFIC3 may share histological features with PSC. Case report: We report the case of a 22-year-old man who, since he was 5 years of age, has presented with pruritus, an approximately ninefold elevation of aminotransferases, and γ-glutamyl transferase levels ~10 times the upper limit. Initially he was diagnosed with an overlap syndrome of small duct PSC plus AIH. However, fluctuations in liver enzymes were observed over the following years. Analysis of the ABCB4 gene indicated the diagnosis of PFIC3, revealing a mutation not previously reported. Conclusion: With this case report we aim to describe a new mutation, raise awareness of this rare pathology and highlight the importance of genetic testing of the ABCB4 gene in patients with autoimmune liver disease (mainly small duct PSC with incomplete response to immunosuppressive treatment.

Discrimination of suppurative cholangitis from nonsuppurative cholangitis with computed tomography (CT)

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Lee, Nam Kyung [Department of Diagnostic Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of); Kim, Suk [Department of Diagnostic Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of)], E-mail: kimsuk@medigate.net; Lee, Jun Woo; Kim, Chang Won [Department of Diagnostic Radiology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of); Kim, Gwang Ha; Kang, Dae Hwan [Department of Gastrointestinal Internal Medicine, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of); Jo, Hong Jae [Department of Surgery, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Pusan National University, Busan 602-739 (Korea, Republic of)

2009-03-15

Purpose: Suppurative cholangitis is characterized by obstruction, inflammation, and pyogenic infection of the biliary tract. This disease represents a true emergency. The purpose of this study was to compare the computed tomography (CT) findings between acute calculous suppurative and nonsuppurative cholangitis and to determine if there are findings that assist in the differential diagnosis. Materials and methods: Fifteen patients with acute suppurative cholangitis were enrolled in this study. Findings at endoscopic retrograde cholangiopancreaticography (ERCP) were the standard of reference for suppurative cholangitis. To compare the findings of suppurative cholangitis with those of nonsuppurative cholangitis, 35 patients with nonsuppurative cholangitis were randomly selected. The following findings were evaluated: the presence of papillitis, the presence of stones in the ampulla, the presence of intrahepatic stones, the presence of early inhomogeneous enhancement of the liver, the degree of bile duct dilatation, the degree of bile duct wall thickening and presence of cholecystitis. Sensitivity and specificity for each of the individual findings were calculated. Statistical analyses were performed the Pearson {chi}{sup 2} test, Fisher's exact test and the Mann-Whitney U test. Results: Papillitis showed the highest specificity 86% with 60% sensitivity. Marked inhomogeneous enhancement of the liver during the arterial phase showed 80% specificity with 60% sensitivity. In multivariate logistic analysis, papillitis and marked early inhomogeneous enhancement of the liver were the most significant predictors of acute suppurative cholangitis. The combination of these two CT findings improved specificity (97% specificity) for the diagnosis of suppurative cholangitis. Conclusion: Papillitis and marked early inhomogeneous enhancement of the liver were found to be the most discriminative CT findings for the diagnosis of acute suppurative cholangitis and the

Autoimmune pancreatitis in Japan: overview and perspective.

Science.gov (United States)

Shimosegawa, Tooru; Kanno, Atsushi

2009-01-01

Since the rediscovery and definition of autoimmune pancreatitis (AIP) by Yoshida et al. in 1995, the disease has been attracting attention because of its unique clinical features and practical issues. This disease shows very impressive imaging findings, serological changes, and characteristic histopathology. It occurs most commonly in elderly males with painless jaundice or mild abdominal pain; resemblance in imaging findings between AIP and pancreatobiliary cancers poses an important practical issue of differentiation. With increasing recognition of AIP and accumulation of cases, another important feature of this disease has been revealed, i.e., association of extrapancreatic organ involvements. Initially misunderstood because it can be accompanied by other autoimmune disorders, such as Sjögren's syndrome or primary sclerosing cholangitis (PSC), AIP is now known to be associated with unique types of sialadenitis and cholangitis distinct from Sjögren's syndrome or PSC. Now the concept of "IgG4-related sclerosing disease" has become widely accepted and the list of organs involved continues to increase. With worldwide recognition, an emerging issue is the clinical definition of other possible types of autoimmune-related pancreatitis called "idiopathic duct-centric chronic pancreatitis (IDCP)" and "AIP with granulocyte epithelial lesion (GEL)" and their relation to AIP with lymphoplasmacytic sclerosing pancreatitis (LPSP). The time has arrived to establish clinical diagnostic criteria of AIP based on international consensus and to discuss regional and racial differences in the clinicopathological features of AIP. Consensus guidelines are also required for the ideal use of steroids in the treatment of AIP to suppress recurrence efficiently with minimal side effects. There are many issues to be settled in AIP; international collaboration of experts in the pancreas field is necessary to clarify the entire picture of this unique and important disease.

Autoimmune pancreatitis in Japan. Overview and perspective

International Nuclear Information System (INIS)

Shimosegawa, Tooru; Kanno, Atsushi

2009-01-01

Since the rediscovery and definition of autoimmune pancreatitis (AIP) by Yoshida et al. in 1995, the disease has been attracting attention because of its unique clinical features and practical issues. This disease shows very impressive imaging findings, serological changes, and characteristic histopathology. It occurs most commonly in elderly males with painless jaundice or mild abdominal pain; resemblance in imaging findings between AIP and pancreatobiliary cancers poses an important practical issue of differentiation. With increasing recognition of AIP and accumulation of cases, another important feature of this disease has been revealed, id est (i.e.), association of extrapancreatic organ involvements. Initially misunderstood because it can be accompanied by other autoimmune disorders, such as Sjogren's syndrome or primary sclerosing cholangitis (PSC), AIP is now known to be associated with unique types of sialadenitis and cholangitis distinct from Sjogren's syndrome or PSC. Now the concept of 'IgG4-related sclerosing disease' has become widely accepted and the list of organs involved continues to increase. With worldwide recognition, an emerging issue is the clinical definition of other possible types of autoimmune-related pancreatitis called 'idiopathic duct-centric chronic pancreatitis (IDCP)' and AIP with granulocyte epithelial lesion (GEL)' and their relation to AIP with lymphoplasmacytic sclerosing pancreatitis (LPSP). The time has arrived to establish clinical diagnostic criteria of AIP based on international consensus and to discuss regional and racial differences in the clinicopathological features of AIP. Consensus guidelines are also required for the ideal use of steroids in the treatment of AIP to suppress recurrence efficiently with minimal side effects. There are many issues to be settled in AIP; international collaboration of experts in the pancreas field is necessary to clarify the entire picture of this unique and important disease. (author)

Differential diagnosis of sclerosing cholangitis with autoimmune pancreatitis and periductal infiltrating cancer in the common bile duct at dynamic CT, endoscopic retrograde cholangiography and MR cholangiography

International Nuclear Information System (INIS)

Kim, Jin Hee; Byun, Jae Ho; Lee, So Jung; Park, Seong Ho; Kim, Hyoung Jung; Lee, Seung Soo; Lee, Moon-Gyu; Kim, Myung-Hwan; Kim, Jihun

2012-01-01

To compare findings at dynamic computed tomography (CT), endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) in patients with sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and periductal infiltrating cancer in the common bile duct (CBD), and to evaluate the diagnostic performance of ERC and MRC in differentiating between the two diseases. Bile duct changes at dynamic CT, ERC and MRC were compared in 58 patients with SC-AIP and CBD involvement and 93 patients with periductal infiltrating CBD cancer. Two radiologists rated their confidence in differentiating between the two diseases and the diagnostic performances of ERC and MRC were compared. At CT, SC-AIP was more frequently associated with intrapancreatic CBD involvement, thinner CBD walls, concentric wall thickening, smooth outer margins, and lower degrees of upstream ductal dilatation and contrast enhancement (P ≤ 0.05) than CBD cancer. At ERC and MRC, SC-AIP was more frequently associated with smooth margins, gradual and symmetric narrowing, multifocal involvement and hourglass appearance (P ≤ 0.027) than CBD cancer. MRC showed good diagnostic performance comparable to ERC. Dynamic CT, ERC and MRC can be helpful in distinguishing SC-AIP from periductal infiltrating CBD cancer. MRC may be a useful diagnostic alternative to ERC in differentiating between the two diseases. (orig.)

Differential diagnosis of sclerosing cholangitis with autoimmune pancreatitis and periductal infiltrating cancer in the common bile duct at dynamic CT, endoscopic retrograde cholangiography and MR cholangiography

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Kim, Jin Hee; Byun, Jae Ho; Lee, So Jung; Park, Seong Ho; Kim, Hyoung Jung; Lee, Seung Soo; Lee, Moon-Gyu [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Asanbyeongwon-gil 86, Songpa-Gu, Seoul (Korea, Republic of); Kim, Myung-Hwan [University of Ulsan College of Medicine, Asan Medical Center, Department of Internal Medicine, Asanbyeongwon-gil 86, Songpa-Gu, Seoul (Korea, Republic of); Kim, Jihun [University of Ulsan College of Medicine, Asan Medical Center, Department of Diagnostic Pathology, Asanbyeongwon-gil 86, Songpa-Gu, Seoul (Korea, Republic of)

2012-11-15

To compare findings at dynamic computed tomography (CT), endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) in patients with sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) and periductal infiltrating cancer in the common bile duct (CBD), and to evaluate the diagnostic performance of ERC and MRC in differentiating between the two diseases. Bile duct changes at dynamic CT, ERC and MRC were compared in 58 patients with SC-AIP and CBD involvement and 93 patients with periductal infiltrating CBD cancer. Two radiologists rated their confidence in differentiating between the two diseases and the diagnostic performances of ERC and MRC were compared. At CT, SC-AIP was more frequently associated with intrapancreatic CBD involvement, thinner CBD walls, concentric wall thickening, smooth outer margins, and lower degrees of upstream ductal dilatation and contrast enhancement (P {<=} 0.05) than CBD cancer. At ERC and MRC, SC-AIP was more frequently associated with smooth margins, gradual and symmetric narrowing, multifocal involvement and hourglass appearance (P {<=} 0.027) than CBD cancer. MRC showed good diagnostic performance comparable to ERC. Dynamic CT, ERC and MRC can be helpful in distinguishing SC-AIP from periductal infiltrating CBD cancer. MRC may be a useful diagnostic alternative to ERC in differentiating between the two diseases. (orig.)

Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma

International Nuclear Information System (INIS)

Yata, M.; Suzuki, K.; Furuhashi, N.; Kawakami, K.; Kawai, Y.; Naganawa, S.

2016-01-01

Aim: To compare the multidetector-row computed tomography (MDCT) findings of IgG4-related sclerosing cholangitis (IgG4-SC) and extrahepatic cholangiocarcinoma (EH-CCA). Materials and methods: Two radiologists who had no knowledge of the patients' clinical information retrospectively evaluated the CT findings of patients with IgG4-SC (n=33) and EH-CCA (n=39) on a consensus basis. Another radiologist measured the biliary lesions. IgG4-SC was diagnosed using the Japan Biliary Association criteria (2012) or the Mayo Clinic's HISORt criteria. EH-CCA was diagnosed based on surgical findings. Results: Compared with EH-CCA, IgG4-SC exhibited the following findings significantly more frequently: (a) wall thickening alone, (b) concentric wall thickening, (c) smooth inner margins, (d) homogeneous attenuation in the arterial phase, (e) a lesion involving the intrapancreatic bile duct, (f) smooth outer margins, (g) fully visible lumen, (h) a funnel-shaped proximal bile duct, (i) skip lesions, and (j) abnormal pancreatic findings. Conversely, (k) dual-layered attenuation in all phases was significantly more common in EH-CCA. The specificity values of parameters (e–k) were >80%. Regarding dimensions, (l) the biliary lesions were longer in IgG4-SC than in EH-CCA. (m) The diameters of the dilated proximal common bile duct and (n) the dilated proximal intrahepatic bile duct were smaller in IgG4-SC than in EH-CCA. Conclusion: A number of CT findings are useful for differentiating between IgG4-SC and EH-CCA. CT findings (e–k) are particularly useful for this purpose. - Highlights: • Some CT findings are useful for differentiating between IgG4-SC and EH-CCA. • Homogeneous attenuation in all phases was more common in IgG4-SC than in EH-CCA. • Abnormal pancreatic findings showed high sensitivity and specificity values. • Dual-layered attenuation in all phases was more common in EH-CCA than in IgG4-SC.

Hepcidin is an antibacterial, stress-inducible peptide of the biliary system.

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Pavel Strnad

Full Text Available BACKGROUND/AIMS: Hepcidin (gene name HAMP, an IL-6-inducible acute phase peptide with antimicrobial properties, is the key negative regulator of iron metabolism. Liver is the primary source of HAMP synthesis, but it is also produced by other tissues such as kidney or heart and is found in body fluids such as urine or cerebrospinal fluid. While the role of hepcidin in biliary system is unknown, a recent study demonstrated that conditional gp130-knockout mice display diminished hepcidin levels and increased rate of biliary infections. METHODS: Expression and localization of HAMP in biliary system was analyzed by real time RT-PCR, in-situ hybridization, immunostaining and -blotting, while prohepcidin levels in human bile were determined by ELISA. RESULTS: Hepcidin was detected in mouse/human gallbladder and bile duct epithelia. Biliary HAMP is stress-inducible, in that it is increased in biliary cell lines upon IL-6 stimulation and in gallbladder mucosa of patients with acute cholecystitis. Hepcidin is also present in the bile and elevated prohepcidin levels were observed in bile of primary sclerosing cholangitis (PSC patients with concurrent bacterial cholangitis compared to PSC subjects without bacterial infection (median values 22.3 vs. 8.9; p = 0.03. In PSC-cholangitis subjects, bile prohepcidin levels positively correlated with C-reactive protein and bilirubin levels (r = 0.48 and r = 0.71, respectively. In vitro, hepcidin enhanced the antimicrobial capacity of human bile (p<0.05. CONCLUSION: Hepcidin is a stress-inducible peptide of the biliary epithelia and a potential marker of biliary stress. In the bile, hepcidin may serve local functions such as protection from bacterial infections.

Sclerosing cholangitis

Science.gov (United States)

... gallstones in the bile duct) Infections in the liver, gallbladder, and bile ducts Symptoms The first symptoms are usually: Fatigue Itching ... varices (enlarged veins) Biliary cirrhosis (inflammation of the bile ducts) Liver failure Persistent jaundice Some people develop infections of ...

Autoimmune diseases of the liver and biliary tract and overlap syndromes in childhood.

Science.gov (United States)

Maggiore, G; Riva, S; Sciveres, M

2009-03-01

Autoimmune liver diseases in childhood includes Autoimmune Hepatitis (AIH) and Primary (Autoimmune) Sclerosing Cholangitis (P(A)SC). Both diseases are characterized by a chronic, immune-mediated liver inflammation involving mainly hepatocytes in AIH and bile ducts in PSC. Both diseases, if untreated, lead to liver cirrhosis. AIH could be classified, according to the autoantibodies pattern, into two subtypes: AIH type 1 presents at any age as a chronic liver disease with recurrent flares occasionally leading to liver cirrhosis and liver failure. Characterizing autoantibodies are anti-nuclear (ANA) and anti-smooth muscle (SMA), usually at high titer (>1:100). These autoantibodies are not specific and probably do not play a pathogenic role. AIH type 2 shows a peak of incidence in younger children, however with a fluctuating course. The onset is often as an acute liver failure. Anti-liver kidney microsome autoantibodies type 1 (LKM1) and/or anti-liver cytosol autoantibody (LC1) are typically found in AIH type 2 and these autoantibodies are accounted to have a potential pathogenic role. Diagnosis of AIH is supported by the histological finding of interface hepatitis with massive portal infiltration of mononuclear cells and plasmocytes. Inflammatory bile duct lesions are not unusual and may suggest features of ''overlap'' with P(A)SC. A diagnostic scoring system has been developed mainly for scientific purposes, but his diagnostic role in pediatric age is debated. Conventional treatment with steroids and azathioprine is the milestone of therapy and it is proved effective. Treatment withdrawal however should be attempted only after several years. Cyclosporin A is the alternative drug currently used for AIH and it is effective as steroids. P(A)SC exhibit a peak of incidence in the older child, typically in pre-pubertal age with a slight predominance of male gender. Small bile ducts are always concerned and the histological picture shows either acute cholangitis (bile duct

Type 1 Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis Is Associated With Extrapancreatic Organ Failure, Malignancy, and Mortality in a Prospective UK Cohort

Science.gov (United States)

Kumar, M.; Hurst, J.M.; Rodriguez-Justo, M.; Chapman, M.H.; Johnson, G.J.; Pereira, S.P.; Chapman, R.W.

2015-01-01

OBJECTIVES Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers. METHODS Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1–107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics. RESULTS Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio = 2.25, CI = 1.12–3.94, P = 0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio = 2.07, CI = 1.07–3.55, P = 0.02). CONCLUSIONS Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up. PMID:25155229

Significant influence of the primary liver disease on the outcomes of hepatic retransplantation.

LENUS (Irish Health Repository)

Qasim, A

2012-02-01

BACKGROUND: There are many indications for hepatic retransplantation. AIM: To identify factors influencing retransplantation needs and outcomes. PATIENTS AND METHODS: Retransplantation records from January 1993 to March 2005 were analysed. Patient and disease characteristics and survival outcomes for retransplantation were compared between various groups. RESULTS: Totally, 286 primary and 42 hepatic retransplantations were performed. Retransplantation indications included primary sclerosing cholangitis (PSC), primary biliary cirrhosis, chronic hepatitis C (HCV), chronic active hepatitis (CAH), and alcohol-related disease. Mean follow-up post-retransplantation was 31 +\\/- 9 months. Actuarial patient survival at 3 months, 1 year, 3 years, 5 years, and at the end of study was 71.4, 69, 59.5, 54.7, and 50%, respectively. Early and late retransplantation had 1-year survival of 73 and 68.5%, respectively. Retransplantation need was significantly higher for PSC, HCV, and CAH. CONCLUSIONS: Hepatic retransplantation remains a successful salvage option for transplant complications; however, its need is significantly influenced by the primary liver disease.

Hepatic Encephalopathy

Medline Plus

Full Text Available ... Non-Alcoholic Fatty Liver Disease Primary Biliary Cholangitis Primary Sclerosing Cholangitis What Is Reye’s Syndrome? Wilson’s Disease Liver Disease Resources OVERVIEW Find a ...

[Autoimmune hepatitis: Immunological diagnosis].

Science.gov (United States)

Brahim, Imane; Brahim, Ikram; Hazime, Raja; Admou, Brahim

2017-11-01

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. The diagnosis of AIHT is based on the presence of hyperglobulinemia, cytolysis, cholestasis, typical even specific circulating auto-antibodies, distinctive of AIH or PBC, and histological abnormalities as well as necrosis and inflammation. Anti-F actin, anti-LKM1, anti-LC1 antibodies permit to distinguish between AIH type 1 and AIH type 2. Anti-SLA/LP antibodies are rather associated to more severe hepatitis, and particularly useful for the diagnosis of seronegative AIH for other the antibodies. Due to the relevant diagnostic value of anti-M2, anti-Sp100, and anti-gp210 antibodies, the diagnosis of PBC is more affordable than that of PSC and AIC. Based on clinical data, the immunological diagnosis of AIHT takes advantage of the various specialized laboratory techniques including immunofluorescence, immunodot or blot, and the Elisa systems, provided of a closer collaboration between the biologist and the physician. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Hepatic Encephalopathy

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Full Text Available ... Jaundice In Newborns Diseases of the Liver Lysosomal Acid Lipase Deficiency Liver Cancer Liver Cysts Non-Alcoholic Fatty Liver Disease Primary Biliary Cholangitis Primary Sclerosing Cholangitis ...

Cholangitis following percutaneous biliary drainage

International Nuclear Information System (INIS)

Audisio, R.A.; Bozzetti, F.; Cozzi, G.; Severini, A.; Belloni, M.; Friggerio, L.F.

1989-01-01

The binomial PTBD-cholangitis often stands under different and sometimes even opposite relations. Among its indications the procedure lists, the treatment of cholangitis which, on the other hand, may be itself a complication of biliary drainage. The present work proposes a critical review of cholangitis-PTBD correlations, from an ordinary clinical-radiological point of view. Different pathogenetic hypothesis of cholangitis (inflammation, cholestasis, surgical manipulation) are discussed together with risk factors (impaired macrophagic-phagocytic system, immunosuppresion, wide neoplastic liver involvement, multiple intrahepatic ductal obstructions, chronic liver diseases, aged patients, etc.). The authors also report about prevention and treatment of septic complications which must be carried out following technical and therapeutic strategies, such as chemoprophylaxis and focused antibiotic therapy according to coltural samples, slow injection of small amounts of contrast medium, peripheral branches approach, gentle handling of catheters and guidewires, flushing with saline solutions and brushing of the catheter itself, and finally use of large gauge catheters in the presence of bile sludge

Creation of a Fistula Between the Hepatic Duct and the Gastric Lumen by way of Percutaneous Transhepatic Cholangiography: A Case Report

DEFF Research Database (Denmark)

Lauridsen, Mette Cathrine; Mortensen, Frank Viborg; Nielsen, Dennis Tønner

2012-01-01

Introduction: Cholangiocellular carcinoma accounts for 3% of gastrointestinal tumors. It is the second most common primary hepatic malignancy and is associated with primary sclerosing cholangitis. Case description: We report a patient with primary sclerosing cholangitis and cholangiocellular...... carcinoma who underwent partial hepatectomy and postoperatively suffered life-threatening biliary stasis with cholascos and peritonitis. The patient had cholangiocellular carcinoma recurrence at the resection margins and local lymph node metastases, but chemotherapy was not possible because of elevated...

Clinicomicrobiological analysis of patients with cholangitis

Directory of Open Access Journals (Sweden)

S M Shenoy

2014-01-01

Full Text Available Acute cholangitis is inflammation of biliary ductal system from infection with an associated biliary obstruction. This retrospective study was done to determine the factors responsible for cholangitis and the microbiological profile of the bile in patients with cholangitis. In the study involving 348 patients, 36.4% had associated malignancy. A total of 54% of the bile samples were positive for aerobic culture. Nearly 66-73% of the Escherichia coli and Klebsiella isolates were Extended spectrum beta lactamases (ESBL producers. Two isolates of Candida spps were also obtained. Polymicrobial infection was seen in 31.5% of the culture positive cases. Ideal antibiotics in case of cholangitis would be those which are excreted in the bile such as third-generation cephalosporins, ureidopenicillins, carbapenems and fluoroquinolones to combat resistance and polymicrobial aetiology. Anti-fungal drugs may also be necessary if the patient is not responding to biliary decompression and antibacterial agents to prevent fungaemia.

Nor-Ursodeoxycholic Acid as a Novel Therapeutic Approach for Cholestatic and Metabolic Liver Diseases.

Science.gov (United States)

Halilbasic, Emina; Steinacher, Daniel; Trauner, Michael

2017-01-01

Norursodeoxycholic acid (norUDCA) is a side-chain-shortened derivative of ursodeoxycholic acid with relative resistance to amidation, which enables its cholehepatic shunting. Based on its specific pharmacologic properties, norUDCA is a promising drug for a range of cholestatic liver and bile duct disorders. Recently, norUDCA has been successfully tested clinically in patients with primary sclerosing cholangitis (PSC) as first application in patients. Moreover, hepatic enrichment of norUDCA facilitates direct therapeutic effects on both parenchymal and non-parenchymal liver cells, thereby counteracting cholestasis, steatosis, hepatic inflammation and fibrosis, inhibiting hepatocellular proliferation, and promoting autophagy. This may open its therapeutic use to other non-cholestatic and metabolic liver diseases. This review article is a summary of a lecture given at the XXIV International Bile Acid Meeting (Falk Symposium 203) on "Bile Acids in Health and Disease" held in Düsseldorf, on June 17-18, 2016 and summarizes the recent progress of norUDCA as novel therapeutic approach in cholestatic and metabolic liver disorders with a specific focus on PSC. © 2017 S. Karger AG, Basel.

Hepatic Encephalopathy

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Full Text Available ... Lipase Deficiency Liver Cancer Liver Cysts Non-Alcoholic Fatty Liver Disease Primary Biliary Cholangitis Primary Sclerosing Cholangitis What ... B & C Alcohol-related Liver Disease Non-alcoholic Fatty Liver Disease (NAFLD) & Non-alcoholic Steatohepatitis (NASH) Autoimmune Hepatitis ...

Mapping chronic liver disease questionnaire scores onto SF-6D utility values in patients with primary sclerosing cholangitis

DEFF Research Database (Denmark)

Kalaitzakis, Evangelos; Benito de Valle, Maria; Rahman, Monira

2016-01-01

regression analyses were employed to devise a mapping function predicting utilities. This was validated in three random subsamples of the cohort and in a separate sample of PSC patients following liver transplantation. Adjusted R2 and root-mean-square error (RMSE) as well as Pearson’s r coefficients and mean...

CT findings in recurrent pyogenic cholangitis

International Nuclear Information System (INIS)

Jung, Seung Hye; Lim, Jae Hoon; Ko, Young Tae; Lee, Dong Ho

1991-01-01

Recurrent pyogenic cholangitis is characterized clinically by recurrent attacks of right upper abdominal pain, fever and jaundice, and pathologically by chronic inflammation of the bile ducts with or without pigment bile duct stones. We analyzed the CT findings of 33 cases with recurrent pyogenic cholangitis. Twenty-four cases were confirmed by operation, and 9 cases were diagnosed clinically and cholangiographically. The CT findings of recurrent pyogenic cholangitis were dilatation of the intrahepatic ducts (n = 30), dilatation of the extrahepatic ducts (n = 24) intrahepatic stones (n = 16), extrahepatic stones (n = 12), stricture of the bile ducts (n = 10), wall enhancement of the bile ducts (n = 8), gallstones (n = 8), segmental atrophy of the liver (n = 7), pneumobilia (n = 4), abscess (n = 3), and segmental enhancement (n = 1) of the liver. A CT is considered helpful when sectional imaging is needed, but sonographic findings are equivocal or not confirmative; space-occupying lesions complicated with recurrent pyogenic cholangitis: hepatic resection is planned; and imaging guidance is needed for complex drainage procedures

What an endoscopist should know about immunoglobulin-G4-associated disease of the pancreas and biliary tree

NARCIS (Netherlands)

Maillette de Buy Wenniger, L.; Rauws, E. A.; Beuers, U.

2012-01-01

Autoimmune pancreatitis (AIP) and IgG4-associated cholangitis (IAC) are the recently recognized pancreatobiliary manifestations of IgG4-associated systemic disease (ISD). Clinically, ISD of the pancreas and/or biliary tree may mimic pancreatic cancer, sclerosing cholangitis, or cholangiocarcinoma.

Autoimmune liver disease 2007.

Science.gov (United States)

Muratori, Paolo; Granito, Alessandro; Pappas, Georgios; Muratori, Luigi; Lenzi, Marco; Bianchi, Francesco B

2008-01-01

Autoimmune liver disease (ALD) includes a spectrum of diseases which comprises both cholestatic and hepatitic forms: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and the so called "overlap" syndromes where hepatitic and cholestatic damage coexists. All these diseases are characterized by an extremely high heterogeneity of presentation, varying from asymptomatic, acute (as in a subset of AIH) or chronic (with aspecific symptoms such as fatigue and myalgia in AIH or fatigue and pruritus in PBC and PSC). The detection and characterization of non organ specific autoantibodies plays a major role in the diagnostic approach of autoimmune liver disease; anti nuclear reactivities (ANA) and anti smooth muscle antibodies (SMA) mark type 1 AIH, liver kidney microsomal antibody type 1 (LKM1) and liver cytosol type 1 (LC1) are the serological markers of type 2 AIH; antimitochondrial antibodies (AMA) are associated with PBC, while no specific marker is found in PSC, since anticytoplasmic neutrophil antibodies with perinuclear pattern (atypical p-ANCA or p-ANNA) are also detected in a substantial proportion of type 1 AIH cases. Treatment options rely on immunosoppressive therapy (steroids and azathioprine) in AIH and on ursodeoxycholic acid in cholestatic conditions; in all these diseases liver transplantation remains the only therapeutical approach for the end stage of liver disease.

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Lifescience Database Archive (English)

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Lifescience Database Archive (English)

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Lifescience Database Archive (English)

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Lifescience Database Archive (English)

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IgG4-Associated Cholangitis Can Mimic Hilar Cholangiocarcinoma.

Science.gov (United States)

Zaydfudim, Victor M; Wang, Andrew Y; de Lange, Eduard E; Zhao, Zimin; Moskaluk, Christopher A; Bauer, Todd W; Adams, Reid B

2015-07-01

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.

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Lifescience Database Archive (English)

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Prophylaxis of post-ERC infectious complications in patients with biliary obstruction by adding antimicrobial agents into ERC contrast media- a single center retrospective study.

Science.gov (United States)

Wobser, Hella; Gunesch, Agnetha; Klebl, Frank

2017-01-13

Patients with biliary obstruction are at high risk to develop septic complications after endoscopic retrograde cholangiography (ERC). We evaluated the benefits of local application of antimicrobial agents into ERC contrast media in preventing post-ERC infectious complications in a high-risk study population. Patients undergoing ERC at our tertiary referral center were retrospectively included. Addition of vancomycin, gentamicin and fluconazol into ERC contrast media was evaluated in a case-control design. Outcomes comprised infectious complications within 3 days after ERC. In total, 84 ERC cases were analyzed. Primarily indications for ERC were sclerosing cholangitis (75%) and malignant stenosis (9.5%). Microbial testing of collected bile fluid in the treatment group was positive in 91.4%. Detected organisms were sensitive to the administered antimicrobials in 93%. The use of antimicrobials in contrast media was associated with a significant decrease in post-ERC infectious complications compared to non-use (14.3% vs. 33.3%; odds ratio [OR]: 0.33, 95% confidence interval [CI]: 0.114-0.978). After adjusting for the variables acute cholangitis prior to ERC and incomplete biliary drainage, the beneficial effect of intraductal antibiotic prophylaxis was even more evident (OR = 0.153; 95% CI: 0.039-0.598, p = 0.007). Patients profiting most obviously from intraductal antimicrobials were those with secondary sclerosing cholangitis. Local application of a combination of antibiotic and antimycotic agents to ERC contrast media efficiently reduced post-ERC infectious events in patients with biliary obstruction. This is the first study that evaluates ERC-related infectious complications in patients with secondary sclerosing cholangitis. Our first clinical results should now be prospectively evaluated in a larger patient cohort to improve the safety of ERC, especially in patients with secondary sclerosing cholangitis.

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Recombinant PrPSc shares structural features with brain-derived PrPSc: Insights from limited proteolysis.

Science.gov (United States)

Sevillano, Alejandro M; Fernández-Borges, Natalia; Younas, Neelam; Wang, Fei; R Elezgarai, Saioa; Bravo, Susana; Vázquez-Fernández, Ester; Rosa, Isaac; Eraña, Hasier; Gil, David; Veiga, Sonia; Vidal, Enric; Erickson-Beltran, Melissa L; Guitián, Esteban; Silva, Christopher J; Nonno, Romolo; Ma, Jiyan; Castilla, Joaquín; R Requena, Jesús

2018-01-01

Very solid evidence suggests that the core of full length PrPSc is a 4-rung β-solenoid, and that individual PrPSc subunits stack to form amyloid fibers. We recently used limited proteolysis to map the β-strands and connecting loops that make up the PrPSc solenoid. Using high resolution SDS-PAGE followed by epitope analysis, and mass spectrometry, we identified positions ~116/118, 133-134, 141, 152-153, 162, 169 and 179 (murine numbering) as Proteinase K (PK) cleavage sites in PrPSc. Such sites likely define loops and/or borders of β-strands, helping us to predict the threading of the β-solenoid. We have now extended this approach to recombinant PrPSc (recPrPSc). The term recPrPSc refers to bona fide recombinant prions prepared by PMCA, exhibiting infectivity with attack rates of ~100%. Limited proteolysis of mouse and bank vole recPrPSc species yielded N-terminally truncated PK-resistant fragments similar to those seen in brain-derived PrPSc, albeit with varying relative yields. Along with these fragments, doubly N- and C-terminally truncated fragments, in particular ~89/97-152, were detected in some recPrPSc preparations; similar fragments are characteristic of atypical strains of brain-derived PrPSc. Our results suggest a shared architecture of recPrPSc and brain PrPSc prions. The observed differences, in particular the distinct yields of specific PK-resistant fragments, are likely due to differences in threading which result in the specific biochemical characteristics of recPrPSc. Furthermore, recombinant PrPSc offers exciting opportunities for structural studies unachievable with brain-derived PrPSc.

Degenrative Fibroid and Sclerosing Peritonitis

Directory of Open Access Journals (Sweden)

Michael Critchley

2012-01-01

Full Text Available Sclerosing peritonitis is a rare condition characterised by ascites, peritoneal and bowel wall thickening. Causes reported in the literature include luteal ovarian the comas, peritoneal dialysis, peritoneal chemotherapy and liver cirrhosis. We report an interesting case of a woman presenting with diarrhoea, abdominal distension, ascites and pleural effusion. She was subsequently diagnosed with Sclerosing Peritonitis caused by a degenerating fibroid which was successfully treated by Total Abdominal Hysterectomy and Bilateral Salpingoophrectomy.

Successful treatment of recurrent cholangitis with antibiotic maintenance therapy

NARCIS (Netherlands)

van den Hazel, S. J.; Speelman, P.; Tytgat, G. N.; van Leeuwen, D. J.

1994-01-01

The impact of antibiotic maintenance therapy on the incidence of biliary tract infection was evaluated in patients with recurrent cholangitis after resection of a malignancy at the hepatic confluence. Thirty-eight of 54 patients (70%) experienced episodes of cholangitis. In 14 of the 38 patients

Extraintestinal manifestations of inflammatory bowel disease: epidemiology, diagnosis, and management

DEFF Research Database (Denmark)

Larsen, Signe; Bendtzen, Klaus; Nielsen, Ole Haagen

2010-01-01

', 'bronchiectasis', 'bronchitis', 'cutaneous manifestations', 'erythema nodosum', 'extraintestinal manifestations', 'hyperhomocysteinemia', 'infliximab', 'iridocyclitis', 'lung disease', 'ocular manifestations', 'osteomalacia', 'pancreatitis', 'primary sclerosing cholangitis', 'renal stones', 'sulfasalazine...

Sclerosing encapsulating peritonitis: a case report

Energy Technology Data Exchange (ETDEWEB)

Candido, Paula de Castro Menezes; Werner, Andrea de Freitas; Pereira, Izabela Machado Flores; Matos, Breno Assuncao; Pfeilsticker, Rudolf Moreira; Silva Filho, Raul, E-mail: paulacmcandido@yahoo.com.br [Hospital Felicio Rocho, Belo Horizonte, MG (Brazil)

2015-01-15

Sclerosing encapsulating peritonitis, a rare cause of bowel obstruction, was described as a complication associated with peritoneal dialysis which is much feared because of its severity. The authors report a case where radiological findings in association with clinical symptoms have allowed for a noninvasive diagnosis of sclerosing encapsulating peritonitis, emphasizing the high sensitivity and specificity of computed tomography to demonstrate the characteristic findings of such a condition. (author)

Complementary role of helical CT cholangiography to MR cholangiography in the evaluation of biliary function and kinetics

International Nuclear Information System (INIS)

Eracleous, Eleni; Genagritis, Marios; Kontou, Allayioti Maria; Papanikolaou, Nicos; Prassopoullos, P.; Chrysikopoulos, Haris; Gourtsoyiannis, Nicholas; Allan, Paul

2005-01-01

To explore the potential role of computed tomographic cholangiography (CTC) in relation to magnetic resonance cholangiography (MRC) in cases in which knowledge of biliary kinetics and functional information are important for therapeutic decisions, 31 patients (14 men and 17 women) underwent MRC followed by CTC. We examined nine post-cholecystectomy cases with right upper quadrant abdominal pain, six cases with a previous biliary-enteric anastomosis and clinical evidence of cholangitis, eight biliary strictures with pain or symptoms of cholangitis, four cases with strong clinical evidence of sclerosing cholangitis, three cases with suspected post-laparoscopic cholecystectomy bile leakage, and one case with chronic pancreatitis and a common bile duct stent associated with cholangitis. In relation to MRC, CTC provided additional biliary functional information as follows: abnormal biliary drainage through the ampulla in 7/9 cholecystectomy cases, impaired drainage in 3/6 biliary-enteric anastomoses, and complete obstruction in 2/8 biliary strictures. CTC diagnosed early sclerosing cholangitis in 4/4 cases and confirmed suspected bile leakage in 1/3 post-laparoscopic cholecystectomy patients, and the patency of the biliary stent in the patient with chronic pancreatitis. Thus, CTC provides clinically important information about the function and kinetics of bile and complements findings obtained by MRC. (orig.)

Complementary role of helical CT cholangiography to MR cholangiography in the evaluation of biliary function and kinetics

Energy Technology Data Exchange (ETDEWEB)

Eracleous, Eleni; Genagritis, Marios; Kontou, Allayioti Maria [Diagnostic Center of Ayios Therissos, Department of Radiology, Nicosia (Cyprus); Papanikolaou, Nicos; Prassopoullos, P.; Chrysikopoulos, Haris; Gourtsoyiannis, Nicholas [University of Crete, Department of Radiology, Heraklion (Greece); Allan, Paul [Royal Infirmary of Edinburgh, Department of Radiology, Edinburgh (United Kingdom)

2005-10-01

To explore the potential role of computed tomographic cholangiography (CTC) in relation to magnetic resonance cholangiography (MRC) in cases in which knowledge of biliary kinetics and functional information are important for therapeutic decisions, 31 patients (14 men and 17 women) underwent MRC followed by CTC. We examined nine post-cholecystectomy cases with right upper quadrant abdominal pain, six cases with a previous biliary-enteric anastomosis and clinical evidence of cholangitis, eight biliary strictures with pain or symptoms of cholangitis, four cases with strong clinical evidence of sclerosing cholangitis, three cases with suspected post-laparoscopic cholecystectomy bile leakage, and one case with chronic pancreatitis and a common bile duct stent associated with cholangitis. In relation to MRC, CTC provided additional biliary functional information as follows: abnormal biliary drainage through the ampulla in 7/9 cholecystectomy cases, impaired drainage in 3/6 biliary-enteric anastomoses, and complete obstruction in 2/8 biliary strictures. CTC diagnosed early sclerosing cholangitis in 4/4 cases and confirmed suspected bile leakage in 1/3 post-laparoscopic cholecystectomy patients, and the patency of the biliary stent in the patient with chronic pancreatitis. Thus, CTC provides clinically important information about the function and kinetics of bile and complements findings obtained by MRC. (orig.)

Sclerosing Stromal Tumor of Ovary: A Case Report

Directory of Open Access Journals (Sweden)

Menka Khanna

2012-01-01

Full Text Available Sclerosing stromal tumor (SST is an extremely rare and distinctive sex cord stromal tumor which occurs predominantly in the second and third decades of life. We report a case of a 32-year-old woman who developed a sclerosing stromal tumor of ovary and presented with irregular menstruation and pelvic pain. Her hormonal status was normal but CA-125 was raised. She was suspected to have a malignant tumor on computed tomography and underwent bilateral salpingo-oopherectomy. It is therefore necessary to keep in mind the possibility of sclerosing stromal tumor in a young woman.

Adult bile duct strictures: differentiating benign biliary stenosis from cholangiocarcinoma.

Science.gov (United States)

Nguyen Canh, Hiep; Harada, Kenichi

2016-12-01

Biliary epithelial cells preferentially respond to various insults under chronic pathological conditions leading to reactively atypical changes, hyperplasia, or the development of biliary neoplasms (such as biliary intraepithelial neoplasia, intraductal papillary neoplasm of the bile duct, and cholangiocarcinoma). Moreover, benign biliary strictures can be caused by a variety of disorders (such as IgG4-related sclerosing cholangitis, eosinophilic cholangitis, and follicular cholangitis) and often mimic malignancies, despite their benign nature. In addition, primary sclerosing cholangitis is a well-characterized precursor lesion of cholangiocarcinoma and many other chronic inflammatory disorders increase the risk of malignancies. Because of these factors and the changes in biliary epithelial cells, biliary strictures frequently pose a diagnostic challenge. Although the ability to differentiate neoplastic from non-neoplastic biliary strictures has markedly progressed with the advance in radiological modalities, brush cytology and bile duct biopsy examination remains effective. However, no single modality is adequate to diagnose benign biliary strictures because of the low sensitivity. Therefore, understanding the underlying causes by compiling the entire clinical, laboratory, and imaging data; considering the under-recognized causes; and collaborating between experts in various fields including cytopathologists with multiple approaches is necessary to achieve an accurate diagnosis.

Multifocal fibrosclerosis: a new case report and review of the literature

Energy Technology Data Exchange (ETDEWEB)

Oguz, Kader Karli; Oguz, Oguzhan; Cila, Aysenur; Oto, Aytekin [Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara (Turkey); Kiratli, Hayyam [Department of Ophthalmology, Faculty of Medicine, Hacettepe University, Ankara (Turkey); Gokoz, Aytac [Department of Pathology, Faculty of Medicine, Hacettepe University, Ankara (Turkey)

2002-05-01

A case of multifocal fibrosclerosis is presented with MR images. Bilateral sclerosing orbital pseudotumor invading cavernous sinuses were the presenting disorder. Magnetic resonance imaging showed involvement of paranasal sinuses bilaterally and multiple supratentorial dural masses. Retroperitoneal fibrosis was associated with the condition. Multifocal involvement should be considered in patients with sclerosing orbital pseudotumor and an imaging approach should be performed on the appropriate clinical condition to document possible coexistence of other disorders including retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, and Riedel's thyroiditis (orig.)

Correlation of Imaging Findings with Pathologic Findings of Sclerosing Adenosis

International Nuclear Information System (INIS)

Choi, Bo Bae; Shu, Kwang Sun

2012-01-01

The purpose of this study was to evaluate the mammographic and sonographic findings of pure sclerosing adenosis. We retrospectively reviewed the mammographic and sonographic findings in 40 cases of pure sclerosing adenosis confirmed by core needle biopsy (n = 23), vacuum-assisted biopsy (n = 7), excision biopsy (n = 9), and lumpectomy (n = 1) from January 2002 to March 2010. All imaging findings were analyzed according to the American College of Radiology (ACR) breast imaging reporting and data system (BI-RADS). Radiologic features were correlated with pathologic findings. Although most mammograms showed negative findings (57%), calcification was the most common abnormal finding of sclerosing adenosis. On sonography, the most common finding was a circumscribed oval hypoechoic mass without posterior features (78%). Most masses showed BI-RADS category 3, (75%, 27/36). Five cases showed categories 4 or 5 (14%, 5/36). Most mammographic and sonographic findings of sclerosing adenosis are non-specific and non-pathognomonic, even though sometimes sclerosing adenosis can be radiologically or histopathologically confused with malignancy

[Sanation of biliary system using antiseptic decasan in complex treatment of cholangitis].

Science.gov (United States)

Aripova, N U; Magzumov, I Kh

2014-02-01

Experience of treatment of 17 patients, suffering cholangitis of various genesis, using antiseptic Decasan, is presented. Clinical efficacy of the preparation in complex treatment of cholangitis, confirmed by results of the bile bacteriological investigation, was noted.

Analysis list: Psc [Chip-atlas[Archive

Lifescience Database Archive (English)

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Sclerosing lobular hyperplasia of breast: cytomorphologic and histomorphologic features: a case report

Directory of Open Access Journals (Sweden)

Kapur Payal

2006-04-01

Full Text Available Abstract Background Mammary sclerosing lobular hyperplasia is an uncommon benign lesion of adolescent and young women. Fine-needle aspiration cytology of mammary sclerosing lobular hyperplasia is said to show characteristic features that include an absence of stromal fragments. Case presentation In this article, we describe a case of sclerosing lobular hyperplasia that occurred in the right breast of a 12-year-old girl. Fine-needle aspiration cytology showed some fibroadenoma-like features including the presence of stromal fragments, while branched tubular fragments were not seen. The diagnosis of sclerosing lobular hyperplasia was made on histologic examination that showed preserved acinar architecture with lobular hyperplasia and sclerosis of intralobular and interlobular stroma. Conclusion Fine-needle aspiration cytology features of mammary sclerosing lobular hyperplasia are not diagnostic and overlap with those of fibroadenoma; however, a distinction between the two benign entities is of no clinical significance. The definitive diagnosis of sclerosing lobular hyperplasia requires histopathologic evaluation.

cholangitis associated bowel disease in 1981

African Journals Online (AJOL)

1982-08-19

Aug 19, 1982 ... revealed the presence of PSC in 2 (1,2%) and chronic active hepatitis ... Departments of Medicine, Surgery, Radiology and Patbo- logy, University ... A pancreatic carcinoma ..... Treatment of PSC is chiefly symptomatic. As with ...

Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome.

Science.gov (United States)

Wu, E T; Chen, H L; Ni, Y H; Lee, P I; Hsu, H Y; Lai, H S; Chang, M H

2001-07-01

Bacterial cholangitis (BC) is a common complication in patients with biliary atresia (BA) and is characterized by fever, acholic stools and positive blood cultures. The diagnosis is often empirical because the yield of blood cultures is low. It is difficult to differentiate BC from other febrile episodes. In order to characterize the clinical and laboratory features of BC in patients with BA, identify risk factors, and correlate cholangitis with outcome, 37 patients with BA from 1993 to 1998 who underwent a Kasai operation in our hospital were studied. The follow-up period ranged from 6 to 59 months. A total of 107 febrile episodes were documented in these patients. The diagnostic criteria for cholangitis were fever, increased jaundice, or acholic stools. The clinical features, laboratory data, results of bacterial cultures, and outcomes were analyzed retrospectively. A total of 107 febrile episodes, including 78 bouts of cholangitis and 29 non-cholangitis infections, were found in 34 patients. Patients with BC had higher postoperative bilirubin levels (P = 0.02) and less frequent use of prophylactic antibiotics (P = 0.05) than those with non-cholangitis infections. Abnormal white blood cell counts (> 12,000 or Acinetobacter baumanni, and Salmonella typhi. The sensitivity tests justified empirical therapy with ceftriaxone. The effectiveness of prophylactic trimethoprim-sulfamethoxazole or neomycin warrants further studies. BC was a highly prevalent postoperative complication in patients with BA, especially those with inadequate bile drainage. It significantly affected early mortality. Aggressive and complete treatment with empirical ceftriaxone was appropriate.

Modern imaging in patients with obstructive jaundice

African Journals Online (AJOL)

2007-07-19

Jul 19, 2007 ... chronic pancreatitis, sclerosing cholangitis, benign bile duct strictures or ... ducts are obstructed, surgery or therapeutic endoscopy may be required, but ... expensive and invasive procedures, is now available for evaluating.

A case of immunoglobulin G-4 related sclerosing disease mimicking lung cancer

International Nuclear Information System (INIS)

Kwon, Soo Hee; Lee, Young Kyung; Shim, Mi Suk; Lee, Hyang Im

2013-01-01

Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease.

Ultrasonographic findings of sclerosing encapsulating peritonitis

Energy Technology Data Exchange (ETDEWEB)

Han, Jong Kyu; Lee, Hae Kyung; Moon, Chul; Hong, Hyun Sook; Kwon, Kwi Hyang; Choi, Deuk Lin [Soonchunhyangi University College of Medicine, Seoul (Korea, Republic of)

2001-03-15

To evaluate the ultrasonographic findings of the patients with sclerosing encapsulating peritonitis (SEP). Thirteen patients with surgically confirmed sclerosing encapsulating peritonitis were involved in this study. Because of intestinal obstruction, all patients had received operations. Among 13 patients, 12 cases had continuous ambulatory peritoneal dialysis (CAPD) for 2 months-12 years and 4 months from (mean; 6 years and 10 months), owing to chronic renal failure and one patient had an operation due to variceal bleeding caused by liver cirrhosis. On ultrasonographic examination, all patients showed loculated ascites which were large (n=7) or small (n=6) in amount with multiple separations. The small bowel loops were tethered posteriorly perisaltic movement and covered with the thick membrane. The ultrasonographic of findings of sclerosing encapsulating peritonitis were posteriorly tethered small bowels covered with a thick membrane and loculated ascites with multiple septa. Ultrasonographic examination can detect the thin membrane covering the small bowel loops in the early phase of the disease, therefore ultrasonography would be a helpful modality to diagnose SEP early.

Severe acute cholangitis after endoscopic sphincterotomy induced by barium examination: A case report.

Science.gov (United States)

Zhang, Zhen-Hai; Wu, Ya-Guang; Qin, Cheng-Kun; Su, Zhong-Xue; Xu, Jian; Xian, Guo-Zhe; Wu, Shuo-Dong

2012-10-21

Endoscopic sphincterotomy (EST) is considered as a possible etiological factor for severe cholangitis. We herein report a case of severe cholangitis after endoscopic sphincterotomy induced by barium examination. An adult male patient presented with epigastric pain was diagnosed as having choledocholithiasis by ultrasonography. EST was performed and the stone was completely cleaned. Barium examination was done 3 d after EST and severe cholangitis appeared 4 h later. The patient was recovered after treated with tienam for 4 d. Barium examination may induce severe cholangitis in patients after EST, although rare, barium examination should be chosen cautiously. Cautions should be also used when EST is performed in patients younger than 50 years to avoid the damage to the sphincter of Oddi.

Risk factors for percutaneous transhepatic biliary drainage-related cholangitis in patients with malignant obstructive jaundice: a prospective study

International Nuclear Information System (INIS)

Niu Hongtao; Zhai Renyou; Wang Jianfeng; Huang Qiang; Yu Ping; Dai Dingke

2011-01-01

Objective: To investigate the risk factors for percutaneous transhepatic biliary drainage (PTBD) related cholangitis in patients with malignant obstructive jaundice. Methods: One hundred and fifty-four consecutive patients with malignant obstructive jaundice and without leukocytosis, fever and other manifestations of biliary tract infection received initial PTBD drainage. They were enrolled in this study. An uncontrolled prospective study was conducted of cholangitis occurrence within 30 days after PTBD. Twenty potential preoperative risk factors were assessed by univariate and multivariate analysis. Results: Fifty-five patients (55/154, 35.7%) developed PTBD-related cholangitis, which composed of cholangitis group. Other patients composed of non-cholangitis group (99/154). The cholangitis-related mortality rate was 2.6% (4/154). Intraoperative bile culture were performed for 131 patients (131/154), including 45 in cholangitis group and 86 in non-cholangitis group. Positive result occurred in 26 patients (26/45) in cholangitis group and 17 patients (17/86) in non-cholangitis group. There was statistical significant difference between these two groups (χ 2 =19.357, P 2 = 10.470, P 2 =36.324, P 2 =9.540, P 2 =9.856, P 2 =14.196, P 2 =6.190, P 2 =5.439, P<0.05) were significantly different between cholangitis group and non-cholangitis group. By multivariate analysis, diabetes (OR=5.093, P<0.01), Child-Pugh C grade (OR=13.412, P<0.01), undrained biliary duct (OR=3.348, P<0.05), external-internal drainage (OR=3.168, P<0.05) and history of ERCP or cholangiojejunostomy (OR=8.330, P<0.01) remained significant difference. Conclusions: PTBD is an effective and safe palliative treatment for patients with malignant obstructive jaundice. Sufficient preoperative preparation and effective control of risk factors may reduce the incidence of cholangitis after PTCD. (authors)

Edwardsiella tarda-associated cholangitis associated with Lemmel syndrome

Directory of Open Access Journals (Sweden)

Shinji Miyajima

2018-01-01

Full Text Available Edwardsiella tarda is an unusual human pathogen. Gastroenteritis is the most frequently reported manifestation of E.tarda infection and extraintestinal infection including cholangitis has rarely been reported. The overall mortality rate for E.tarda bacteremia is, however, reported to be up to 50% (Janda and Abbott, 1993. We describe a 80-year-old diabetic woman with cholangitis and E.tarda bacteremia with a biliary obstruction associated with a large juxtapapillary duodenal diverticulum (Lemmel syndrome in the setting of past partial hepatectomy and cholecystectomy. She was successfully treated with endoscopic biliary drainage and antibacterials.

Sclerosing stromal tumor of the ovary: A case report

Directory of Open Access Journals (Sweden)

Navjot Kaur

2014-01-01

Full Text Available Sclerosing stromal tumors are benign ovarian neoplasms of the sex cord-stromal category, occurring predominantly in the second and third decades of life. Herein, we report a 23-year-old female who presented with pelvic pain, irregular menses but normal hormonal status and was diagnosed as having a right ovarian tumor. A right oophorectomy was performed, and microscopic examination revealed a sclerosing stromal tumor of the right ovary. We stress the importance of being familiar with sclerosing stromal tumors when evaluating ovarian neoplasms in young women, in order to contribute to the appropriate clinical management, preventing extensive and unnecessary surgery, and preserving fertility.

Celiac disease in autoimmune cholestatic liver disorders.

Science.gov (United States)

Volta, Umberto; Rodrigo, Luis; Granito, Alessandro; Petrolini, Nunzio; Muratori, Paolo; Muratori, Luigi; Linares, Antonio; Veronesi, Lorenza; Fuentes, Dolores; Zauli, Daniela; Bianchi, Francesco B

2002-10-01

In this study, serological screening for celiac disease (CD) was performed in patients with autoimmune cholestasis to define the prevalence of such an association and to evaluate the impact of gluten withdrawal on liver disease associated with gluten sensitive enteropathy. Immunoglobulin A endomysial, human and guinea pig tissue transglutaminase antibodies, and immunoglobulin A and G gliadin antibodies were sought in 255 patients with primary biliary cirrhosis, autoimmune cholangitis, and primary sclerosing cholangitis. Immunoglobulin A endomysial and human tissue transglutaminase antibodies were positive in nine patients (seven primary biliary cirrhosis, one autoimmune cholangitis, and one primary sclerosing cholangitis), whose duodenal biopsy results showed villous atrophy consistent with CD. Two of these patients had a malabsorption syndrome, and one had iron-deficiency anemia. Clinical and biochemical signs of cholestasis did not improve after gluten withdrawal in the three patients with severe liver disease. A longer follow-up of the six celiac patients with mild liver damage is needed to clarify whether gluten restriction can contribute to slow down the progression of liver disease. The high prevalence of CD (3.5%) in autoimmune cholestasis suggests that serological screening for CD should be routinely performed in such patients by immunoglobulin A endomysial or human tissue transglutaminase antibodies.

Colitis ulcerosa

DEFF Research Database (Denmark)

Nielsen, Ole Haagen; Jess, Tine; Bjerrum, Jacob Tveiten

2013-01-01

Ulcerative colitis (UC) is a prevalent inflammatory bowel disease of the colonic mucosa affecting approximately 20,000-25,000 Danes. Apart from subgroups with early onset, extensive and long-standing inflammation, or primary sclerosing cholangitis the risk of developing colorectal cancer is of th......Ulcerative colitis (UC) is a prevalent inflammatory bowel disease of the colonic mucosa affecting approximately 20,000-25,000 Danes. Apart from subgroups with early onset, extensive and long-standing inflammation, or primary sclerosing cholangitis the risk of developing colorectal cancer...... is of the same magnitude as in the background population. The symptoms are usually diarrhoea including bloody stools, rectal tenesmi, anaemia, and fatigue. This review is an update on diagnostics and treatment strategies of relevance for clinicians, and as UC often affects patients during their peak reproductive...

Human induced pluripotent stem cell (hiPSC)-derived neurons respond to convulsant drugs when co-cultured with hiPSC-derived astrocytes.

Science.gov (United States)

Ishii, Misawa Niki; Yamamoto, Koji; Shoji, Masanobu; Asami, Asano; Kawamata, Yuji

2017-08-15

Accurate risk assessment for drug-induced seizure is expected to be performed before entering clinical studies because of its severity and fatal damage to drug development. Induced pluripotent stem cell (iPSC) technology has allowed the use of human neurons and glial cells in toxicology studies. Recently, several studies showed the advantage of co-culture system of human iPSC (hiPSC)-derived neurons with rodent/human primary astrocytes regarding neuronal functions. However, the application of hiPSC-derived neurons for seizure risk assessment has not yet been fully addressed, and not at all when co-cultured with hiPSC-derived astrocytes. Here, we characterized hiPSC-derived neurons co-cultured with hiPSC-derived astrocytes to discuss how hiPSC-derived neurons are useful to assess seizure risk of drugs. First, we detected the frequency of spikes and synchronized bursts hiPSC-derived neurons when co-cultured with hiPSC-derived astrocytes for 8 weeks. This synchronized burst was suppressed by the treatment with 6-cyano-7-nitroquinoxaline-2,3-dione, α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) receptor antagonist, and D-(-)-2-amino-5-phosphonopentanoic acid, an N-Methyl-d-aspartate (NMDA) receptor antagonist. These data suggested that co-cultured hiPSC-derived neurons formed synaptic connections mediated by AMPA and NMDA receptors. We also demonstrated that co-cultured hiPSC-derived neurons showed epileptiform activity upon treatment with gabazine or kaliotoxin. Finally, we performed single-cell transcriptome analysis in hiPSC-derived neurons and found that hiPSC-derived astrocytes activated the pathways involved in the activities of AMPA and NMDA receptor functions, neuronal polarity, and axon guidance in hiPSC-derived neurons. These data suggested that hiPSC-derived astrocytes promoted the development of action potential, synaptic functions, and neuronal networks in hiPSC-derived neurons, and then these functional alterations result in the epileptiform

Cytogenetic study of a pulmonary sclerosing hemangioma.

Science.gov (United States)

Pareja, María J; Vargas, María T; Sánchez, Ana; Ibáñez, José; González-Cámpora, Ricardo

2009-11-01

Pulmonary sclerosing hemangioma (PSH) is an uncommon benign tumor that presents as a solitary asymptomatic and slow-growing nodule. It occurs in both young and old persons; peak incidence is in the fifth decade. Both sexes are affected by this tumor, but women more frequently than men. On histological examination, PSH shows prominent sclerotization and vascularization of the tissue. Recent studies conclude that PSH derives from type II pneumocytes, but the potential for progression and histogenesis remains controversial. We report a case of pulmonary sclerosing hemangioma in a 61-year-old woman with a neoplastic node 1 cm in diameter. The karyotype was 46,XX,t(8;18),der(14;15),+14 in all the cells analyzed. PTEN (10q23) and IgH (14q32) probes were analyzed in interphase nuclei and paraffin-embedded tissues of tumor cells. These chromosome abnormalities could provide information about the relationship of genetic changes to the biological properties of sclerosing hemangioma tumors.

Destructive Cholangitis in an Adult Jack Russell Terrier

Directory of Open Access Journals (Sweden)

Atsushi Kodama

2012-01-01

Full Text Available A 4-year-old female Jack Russell terrier dog exhibited vomiting and severe jaundice of the visible mucous membranes and skin. Ultrasonography revealed diffuse areas of high echogenicity and focal areas of low echogenicity in the left lobe of the liver. On macroscopic observation of the biopsied liver specimen, many scattered irregularly shaped red spots were observed on the liver surface and on the cut surface. Histopathologically, there was loss of the interlobular bile duct and cholangitis accompanied by infiltration of pigment-laden macrophages in the Glisson’s capsule. Therefore, in the present case the dog was diagnosed with destructive cholangitis.

Deposition of C3, the terminal complement complex and vitronectin in primary biliary cirrhosis and primary sclerosing cholangitis

DEFF Research Database (Denmark)

Garred, P; Lyon, H; Christoffersen, P

1993-01-01

-dependent cytotoxic mechanisms in the pathogenesis. Therefore, we investigated liver biopsy specimens from 21 patients with PBC, six patients with PSC and six controls for complement deposits by immunohistochemistry using polyclonal and monoclonal antibodies against C3d, the terminal complement complex (TCC......) and vitronectin (S-protein). We found C3d, TCC and vitronectin deposits only in the portal tracts. C3d and TCC were present in the walls of the hepatic arteries and in the connective tissue stroma but never around the bile ducts. We found vitronectin deposits throughout the connective tissue, often independent...... of the TCC deposits. When vitronectin and TCC were co-localized, the staining patterns were inverse; that is, intense staining for TCC accompanied weak staining for vitronectin and vice versa. Occasionally complete dissociation between TCC and vitronectin staining was observed. Deposits of TCC...

Bone mineral density before and after OLT: long-term follow-up and predictive factors.

Science.gov (United States)

Guichelaar, Maureen M J; Kendall, Rebecca; Malinchoc, Michael; Hay, J Eileen

2006-09-01

Fracturing after liver transplantation (OLT) occurs due to the combination of preexisting low bone mineral density (BMD) and early posttransplant bone loss, the risk factors for which are poorly defined. The prevalence and predictive factors for hepatic osteopenia and osteoporosis, posttransplant bone loss, and subsequent bone gain were studied by the long-term posttransplant follow-up of 360 consecutive adult patients with end-stage primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Only 20% of patients with advanced PBC or PSC have normal bone mass. Risk factors for low spinal BMD are low body mass index, older age, postmenopausal status, muscle wasting, high alkaline phosphatase and low serum albumin. A high rate of spinal bone loss occurred in the first 4 posttransplant months (annual rate of 16%) especially in those with younger age, PSC, higher pretransplant bone density, no inflammatory bowel disease, shorter duration of liver disease, current smoking, and ongoing cholestasis at 4 months. Factors favoring spinal bone gain from 4 to 24 months after transplantation were lower baseline and/or 4-month bone density, premenopausal status, lower cumulative glucocorticoids, no ongoing cholestasis, and higher levels of vitamin D and parathyroid hormone. Bone mass therefore improves most in patients with lowest pretransplant BMD who undergo successful transplantation with normal hepatic function and improved gonadal and nutritional status. Patients transplanted most recently have improved bone mass before OLT, and although bone loss still occurs early after OLT, these patients also have a greater recovery in BMD over the years following OLT.

Analysis of five chronic inflammatory diseases identifies 27 new associations and highlights disease-specific patterns at shared loci

DEFF Research Database (Denmark)

Ellinghaus, David; Jostins, Luke; Spain, Sarah L

2016-01-01

We simultaneously investigated the genetic landscape of ankylosing spondylitis, Crohn's disease, psoriasis, primary sclerosing cholangitis and ulcerative colitis to investigate pleiotropy and the relationship between these clinically related diseases. Using high-density genotype data from more th...

MR cholangiopancreatography diagnosis for cholangitis caused by clonorchis sinensis

International Nuclear Information System (INIS)

Cui Bing; Hu Qiugen; Wang Yan

2003-01-01

Objective: To evaluate the MR cholangiopancreatography (MRCP) diagnosis for cholangitis caused by clonorchis sinensis. Methods: Fifty-four cases with cholangitis caused by clonorchis sinensis were examined by MRCP (3D FASE-Heavy T 2 WI sequence). The results of MRCP were compared with that of ERCP, laparoscopy, and pathology. Results: The diagnostic accuracy for the cause of the disease was 88.9%. Main findings on MRCP included slight dilation of the intra-hepatic biliary duct (n=46), small cystiform dilation of peripheral biliary ending (n=43), extra-hepatic biliary dilations (n=15) and strictures (n=19), and low signal intensity filling defect in the common bile duct and gallbladder (n=6). Conclusion: MRCP of biliary tree images can be obtained with 3D FASE Heavy T 2 WI sequence in considerable details. The characteristic of the cholangitis caused by clonorchis sinensis on MRCP was the slight dilation or stricture of extensive intra-hepatic biliary duct, combined with small cystiform dilation of peripheral biliary ending. MRCP was an ideal technique in diagnosing the disease

Cholangiohydatidosis: an Infrequent Cause of Obstructive Jaundice and Acute Cholangitis.

Science.gov (United States)

Manterola, Carlos; Otzen, Tamara

One of the evolutionary complications of hepatic echinococcosis (HE) is cholangiohydatidosis, a rare cause of obstructive jaundice and cholangitis. The aim of this study was to describe the results of surgical treatment on a group of patients with cholangiohydatidosis and secondary cholangitis in terms of post-operative morbidity (POM). Case series of patients operated on for cholangiohydatidosis and cholangitis in the Department at Surgery of the Universidad de La Frontera and the Clínica Mayor in Temuco, Chile between 2004 and 2014. The minimum follow-up time was six months. The principal outcome variable was the development of POM. Other variables of interest were age, sex, cyst diameter, hematocrit, leukocytes, total bilirubin, alkaline phosphatase and transaminases, type of surgery, existence of concomitant evolutionary complications in the cyst, length of hospital stay, need for surgical re-intervention and mortality. Descriptive statistics were calculated. A total of 20 patients were studied characterized by a median age of 53 years, 50.0% female and 20.0% having two or more cysts with a mean diameter of 13.3 ± 6.3 cm. A median hospital stay of six days and follow-up of 34 months was recorded. POM was 30.0%, re-intervention rate was 10.0% and mortality rate was 5.0%. Cholangiohydatidosis is a rare cause of obstructive jaundice and cholangitis associated with significant rates of POM and mortality.

Role of antibiotics in the treatment and prevention of acute and recurrent cholangitis

NARCIS (Netherlands)

van den Hazel, S. J.; Speelman, P.; Tytgat, G. N.; Dankert, J.; van Leeuwen, D. J.

1994-01-01

Cholangitis is usually the consequence of a combination of factors: impairment of the flow of bile and bacterial colonization of the biliary tract. Although reestablishing biliary drainage is the mainstay of treatment, antibiotics play an important role in the management of cholangitis. In this

The clinical extremes of autoimmune cholangitis

Directory of Open Access Journals (Sweden)

Sara Campos

Full Text Available Autoimmune cholangitis (AIC was first described in 1987 as immunocholangitis in three women who presented with signs and symptoms of primary biliary cholangitis (PBC, but who were antimitochondrial (AMA negative and antinuclear antibodies (ANA positive, and responded to immunosuppressive therapy with azathioprine and prednisolone (1. AIC is a rare chronic cholestatic inflammatory disease characterized by the presence of high ANA or smooth muscle antibodies (SMA but AMA seronegativity. Histologically, AIC exhibits bile duct injury (2. In terms of therapeutics, in addition to response to ursodeoxycholic acid, a prompt response to corticosteroids has also been reported in earlier stages, distinguishing it from PBC. Herein the authors describe two cases with mixed signs of PBC and autoimmune hepatitis (AIH. The diagnostic differentiation between these diseases (AIC, PBC and AIH is essential because of the different therapeutic strategies. Our cases highlight the importance of clinician awareness of the autoimmune spectrum of liver diseases.

Diagnosis and Management of Pediatric Autoimmune Liver Disease : ESPGHAN Hepatology Committee Position Statement

NARCIS (Netherlands)

Mieli-Vergani, Giorgina; Vergani, Diego; Baumann, Ulrich; Czubkowski, Piotr; Debray, Dominique; Dezsofi, Antal; Fischler, Björn; Gupte, Girish; Hierro, Loreto; Indolfi, Giuseppe; Jahnel, Jörg; Smets, Françoise; Verkade, Henkjan J; Hadžić, Nedim

Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis

[The most common rheumatic diseases in patients with autoimmune liver disease in the Hospital Arzobispo Loayza from 2008-2013, Lima, Peru].

Science.gov (United States)

Paredes Millán, Mileydy; Chirinos Montes, Nataly Juliana; Martinez Apaza, Anthony; Lozano, Adelina

2014-01-01

To identify the most common autoimmune rheumatic diseases in patients with autoimmune liver disease in the Hospital Arzobispo Loayza (HAL) from 2008 -2013. This is a transversal and descriptive study, we analyzed 125 medical records, only 86 patients fulfill the diagnostic criteria for autoimmune liver disease, of whom 46 had diagnosis of autoimmune hepatitis(AIH), 39 primary biliary cirrhosis(PBC) and just 1 primary sclerosing cholangitis (PSC). In our study group we looked for the clinical and laboratory characteristics most common and the frequency of cases in the HAL. Of the 46 patients with AIH, 16 (34.78%) were diagnosed with autoimmune rheumatic disease concurrence. Of these, 7 (15.22%) patients had Sjogren ́s Disease (SD), 6 (13.04%) had systemic lupus erythematosus (SLE) and 3 (6.52%) had rheumatoid arthritis (RA). We found 39 patients with PBC, 18 (46.15%) had other associated extrahepatic autoimmune disease, of whom 12 (30.77%) had SD, 3 (7.69%) SLE and 3 (7.69%) RA. One patient had the diagnosis of PSC, a sixty year old woman that had no concurrence with rheumatic disease. In our study was found that SD is the most common rheumatic disease in patients with AIH and PBC, followed by SLE and RA, with autoimmune liver disease with rheumatic symptoms and vice versa.

Intraductal location of the sclerosing adenosis of the breast.

Science.gov (United States)

Unal, Bulent; Gur, A Serhat; Bhargava, Rohit; Edington, Howard; Ahrendt, Gretchen; Soran, Atilla

2009-01-01

Sclerosing adenosis is a benign breast disease with non-specific images on ultrasound or mammogram. It can mimic infiltrating carcinoma when the above mentioned imaging techniques are used. Herein we present a patient with breast cancer who received neoadjuvant chemotherapy and subsequently underwent mastectomy. Ductoscopy was performed to the mastectomised breast specimen as per the ductoscopy research protocol. Ductoscopy revealed several nodular lesions in the duct with no additional demonstrable intraductal pathology. The lesions were reported as sclerosing adenosis by pathologist. As to our knowledge, this is the first case in literature that demonstrates the use of ductoscopy in diagnosing the sclerosing adenosis in the breast tissue. Ductoscopy and development of ductoscopy guided biopsy techniques may be used as an early diagnostic method for the ductal breast lesions (Fig. 2, Ref. 10). Full Text (Free, PDF) www.bmj.sk.

Generation of sclerosant foams by mechanical methods increases the foam temperature.

Science.gov (United States)

Tan, Lulu; Wong, Kaichung; Connor, David; Fakhim, Babak; Behnia, Masud; Parsi, Kurosh

2017-08-01

Objective To investigate the effect of agitation on foam temperature. Methods Sodium tetradecyl sulphate and polidocanol were used. Prior to foam generation, the sclerosant and all constituent equipment were cooled to 4-25℃ and compared with cooling the sclerosant only. Foam was generated using a modified Tessari method. During foam agitation, the temperature change was measured using a thermocouple for 120 s. Results Pre-cooling all the constituent equipment resulted in a cooler foam in comparison with only cooling the sclerosant. A starting temperature of 4℃ produced average foam temperatures of 12.5 and 13.2℃ for sodium tetradecyl sulphate and polidocanol, respectively. It was also found that only cooling the liquid sclerosant provided minimal cooling to the final foam temperature, with the temperature 20 and 20.5℃ for sodium tetradecyl sulphate and polidocanol, respectively. Conclusion The foam generation process has a noticeable impact on final foam temperature and needs to be taken into consideration when creating foam.

IgG4-related disease

DEFF Research Database (Denmark)

Detlefsen, Sönke; Klöppel, Günter

2018-01-01

disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts. The pancreatic and biliary...... alterations have been described under the terms autoimmune pancreatitis (AIP) and sclerosing cholangitis, respectively. These diseases are currently more precisely called IgG4-related pancreatitis (or type 1 AIP to distinguish it from type 2 AIP that is unrelated to IgG4-RD) and IgG4-related sclerosing...... cholangitis (IgG4-related SC). Clinically and grossly, both diseases commonly imitate pancreatic and biliary adenocarcinoma, tumors that are well known for their dismal prognosis. As IgG4-RD responds to steroid treatment, making a resection of a suspected tumor unnecessary, a biopsy is often required...

Sclerosing polycystic adenosis of the salivary gland: a report of 16 cases.

NARCIS (Netherlands)

Gnepp, D.R.; Wang, L.J.; Brandwein-Gensler, M.; Slootweg, P.J.; Gill, M.; Hille, J.

2006-01-01

Sclerosing polycystic adenosis is a recently described, extremely rare, reactive, sclerosing, inflammatory process somewhat similar to fibrocystic changes and adenosis tumor of the breast. To date, there have been 22 cases described in the literature. Because of the infrequency of this lesion, we

Clinical significance of autoantibodies in autoimmune hepatitis.

Science.gov (United States)

Liberal, Rodrigo; Mieli-Vergani, Giorgina; Vergani, Diego

2013-10-01

The accurate diagnosis and classification of autoimmune hepatitis (AIH) rely upon the detection of characteristic autoantibodies. Positivity for anti-nuclear (ANA) and/or anti-smooth muscle (SMA) autoantibodies defines AIH type 1 (AIH-1), whereas anti-liver kidney microsomal type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) define AIH type 2 (AIH-2). ANA and SMA, and less commonly anti-LKM1, have also been detected in de-novo autoimmune hepatitis developing after liver transplantation, a condition that may affect patients transplanted for non-autoimmune liver disease. The diagnostic autoantibodies associated with AIH-1 are also detected in the paediatric AIH/sclerosing cholangitis overlap syndrome, referred to as autoimmune sclerosing cholangitis (ASC). ASC, like adult primary sclerosing cholangitis, is often associated with atypical perinuclear anti-neutrophil cytoplasmic autoantibodies (p-ANCA), although p-ANCA are also detected in other autoimmune liver diseases. These associations highlight the necessity for simple and prompt diagnostic autoantibody testing, and the requirement for the accurate interpretation of the results of the tests in the clinical context. Fine-mapping of antigenic autoantibody targets has facilitated the development of rapid molecular assays that have the potential to revolutionise the field if properly standardised and when used in combination with classical immunofluorescence. Despite their diagnostic significance, the pathogenic role of the various autoantibodies and the mechanisms by which they can potentially inflict damage onto the liver cell remain a topic for further research. Copyright © 2013 Elsevier Ltd. All rights reserved.

Subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Modi, G.; Bill, P.; Campbell, H.

1989-01-01

A 19-year-old female patient presented in an acute state of akinetic mutism. Serological analysis of serum and cerebrospinal fluid demonstrated the presence of antibodies to measles virus. CT scan carried out during this acute phase of relapse demonstrated white matter enhancement affecting the cortical white matter of the frontal lobes and corpus callosum. These features indicate that active demyelination occurs during acute relapse in subacute sclerosing panencephalitis (SSPE) and suggest that immunotherapy should be considered during this acute phase. (orig.)

Clinical Investigation Program. Annual Progress Report. Volume 1

Science.gov (United States)

1994-01-20

Components Surgical Day, Bethesda, MD, in April 1993. 2. Planned for FY-94 The Department of Clinical Investigation hopes to maintain the essence of the...sclerosing cholangitis after choledochojejun- ostomy : radiographic and biochemical improvement with antibiotic therapN. Am J Gastroenterol 1993;88:1635

Percutaneous biliary drainage in acute suppurative cholangitis with biliary sepsis

International Nuclear Information System (INIS)

Kim, Hyung Lyul; Cho, June Sik; Kwon, Soon Tae; Lee, Sang Jin; Rhee, Byung Chull

1993-01-01

Acute suppurative cholangitis is a severe inflammatory process of the bile duct occurred as result of partial or complete obstruction of the bile duct, and may manifest clinically severe form of disease, rapidly deteriorating to life-threatening condition. We analyzed emergency percutaneous transhepatic biliary drainage in 20 patients of acute suppurative cholangitis with biliary sepsis to evaluate the therapeutic effect and complication of the procedure. The underlying cause were 12 benign disease(stones) and eight malignant tumors and among eight malignant tumors, bile duct stones(n=4) and clonorchiasis(n=1) were combined. Percutaneous transhepatic biliary drainage was performed successfully in 17 of 20 patients resulting in improvement of general condition and failed in three patients. The procedure were preterminated due to the patient's condition in two and biliary-proto fistula was developed in one. After biliary decompression by percutaneous transhepatic biliary drainage, effective and successful elective surgery was performed in nine cases, which were seven biliary stones and two biliary cancer with stones. Our experience suggest that emergency percutaneous transhepatic biliary drainage is an initial and effective treatment of choice for acute suppurative cholangitis with sepsis and a safe alternative for nonsurgical treatment

iPSC Core

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Federal Laboratory Consortium — The induced Pluripotent Stem Cells (iPSC) Core was created in 2011 to accelerate stem cell research in the NHLBI by providing investigators consultation, technical...

Pathogenic aspects of acute cholangitis

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V. Borisenko

2014-10-01

Full Text Available The research is aimed at the study of dynamic pathomorphological changes of choledoch and acute cholangitis development factors determined during the experiment. 36 rats of Wistar line were under trial. The main group consisted of 30 animals undergoing the open laparotomy, choledoch ligation and puncture modeling of acute cholangitis by E. coli culture in 1 х 108 CFU/ml concentration under general anesthesia. 6 healthy rats were included in the control group. Samples of general biliary duct under autopsy for pathomorphological study were taken on the 3rd, 7th, 14th, 21st and 30th day. In panoramic samples colored by hematoxilin and eozin the degree of dystrophic, necrobiotic, hemodynamic, inflammatory and atrophic manifestations’ changes were studied. Average depth of choledoch wall and height of its epithelial lining were morphometrically estimated. Collagen of the IV type as well as expressing receptors to CD34 were defined with the help of monoclonal antibodies in choledoch epithelial cells of basal membranes and choledoch vessels endotheliocytes. In choledoch, enhancement of edema and inflammatory infiltration by lymphoplasmocytic elements with the admixture of neutrofils with granulation tissue was detected from the 3rd up to the 30th day of the experiment. From the 14th day formation of bile clots of blood was detected in choledoch clearance, part of which was locked to its de-epitheliolized internal surface. According to morphometrical study data, choledoch wall depth increased from 261.1 ± 3.13 µm on the 3rd day to 572.5 ± 3.42 µm on the 30th day of the experiment. Mucosa membrane has lost its folding on the 14th day, epitheliocytes flattening was replaced by their destruction with fragments rejection into the duct lumen by the 30th day of the experiment. The epithelium height index decreased from 14.8 ± 0.09 µm on the 3rd day to 11.7 ± 0.15 µm on the 30 day of the experiment. Collagen of the IV type fluorescence intensity of

A Recurrence of Bilateral Diffuse Sclerosing Lobular Hyperplasia of Breast: A Case Report.

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Elfituri, Osama; Sonawane, Snehal; Xu, Haoliang; Warso, Michael A; Wiley, Elizabeth

2017-12-01

Mammary sclerosing lobular hyperplasia is an uncommon benign fibroproliferative lesion of adolescent and young women, often of African American heritage with an incidence of ~3%. Patients generally complain of a palpable, painless, or slightly tender and well-defined lump in breast. Very rarely, this lesion may be bilateral and diffuse. The definitive diagnosis of sclerosing lobular hyperplasia requires histopathologic evaluation. Here, we describe a case of diffuse sclerosing lobular hyperplasia in a 29-year-old African American woman that required bilateral mastectomy and recurred bilaterally requiring second resections. This appears to be the first report of this phenomenon.

On the role of IgG4 in inflammatory conditions: lessons for IgG4-related disease

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Trampert, David C.; Hubers, Lowiek M.; van de Graaf, Stan F. J.; Beuers, Ulrich

2017-01-01

The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic,

A new lethal sclerosing bone dysplasia

International Nuclear Information System (INIS)

Kingston, H.M.; Freeman, J.S.; Hall, C.M.

1991-01-01

A neonate is described with a lethal sclerosing bone dysplasia associated with prenatal fractures and craniofacial abnormalities including microcephaly, exophthalmos, hypoplastic nose and mid-face, small jaw and nodular hyperplasia of the gums. Parental consanguinity suggests that an autosomal recessive mutation is the likely aetiology. (orig.)

A national standard for psychosocial safety climate (PSC): PSC 41 as the benchmark for low risk of job strain and depressive symptoms.

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Bailey, Tessa S; Dollard, Maureen F; Richards, Penny A M

2015-01-01

Despite decades of research from around the world now permeating occupational health and safety (OHS) legislation and guidelines, there remains a lack of tools to guide practice. Our main goal was to establish benchmark levels of psychosocial safety climate (PSC) that would signify risk of job strain (jobs with high demands and low control) and depression in organizations. First, to justify our focus on PSC, using interview data from Australian employees matched at 2 time points 12 months apart (n = 1081), we verified PSC as a significant leading predictor of job strain and in turn depression. Next, using 2 additional data sets (n = 2097 and n = 1043) we determined benchmarks of organizational PSC (range 12-60) for low-risk (PSC at 41 or above) and high-risk (PSC at 37 or below) of employee job strain and depressive symptoms. Finally, using the newly created benchmarks we estimated the population attributable risk (PAR) and found that improving PSC in organizations to above 37 could reduce 14% of job strain and 16% of depressive symptoms in the working population. The results provide national standards that organizations and regulatory agencies can utilize to promote safer working environments and lower the risk of harm to employee mental health. PsycINFO Database Record (c) 2014 APA, all rights reserved.

Diffuse sclerosing variant of papillary thyroid carcinoma: case report

International Nuclear Information System (INIS)

Lee, Seung Chan; Kim, Dong Wook

2006-01-01

Diffuse sclerosing papillary carcinoma (DSPC) is a variant of papillary thyroid carcinoma (PTC), but it shows more aggressive clinical course and a poorer prognosis than the other types of PTC. Most PTCs show a focal nodular pattern in the thyroid on the imaging modalities, but DSPC reveals a diffuse infiltrating configuration in the thyroid without any focal nodular lesion. To our knowledge, there are scant radiological reports of diffuse sclerosing variant of papillary thyroid carcinoma. In this report, we present the case of a patient with DSPC who showed the characteristic findings on sonography and computed tomography

Autoimmune liver disease in children.

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Mieli-Vergani, G; Vergani, D

2003-03-01

Autoimmune liver disorders are characterised by an inflammatory liver histology, circulating non-organ specific autoantibodies and increased levels of immunoglobulin G (IgG) in the absence of a known aetiology. They respond to immunosuppressive treatment, which should be instituted as soon as diagnosis is made. Liver disorders with a likely autoimmune pathogenesis include autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC). Two types of AIH are recognised according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, type 1) or liver kidney microsomal antibody (LKM1, type 2). There is a female predominance in both. LKM1-positive patients tend to present more acutely, at a younger age, and commonly have immunoglobulin A (IgA) deficiency, while duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment and long-term prognosis are similar in both groups. The most common type of paediatric sclerosing cholangitis is ASC. The clinical, biochemical, immunological and histological presentation of ASC is often indistinguishable from that of AIH. In both, there are high IgG, non-organ specific autoantibodies and interface hepatitis. Diagnosis is made by cholangiography. Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates, times to normalisation of biochemical parameters and decreased inflammatory activity on follow-up liver biopsies. However, the cholangiopathy can progress and there may be an evolution from AIH to ASC over the years, despite treatment. Whether the juvenile autoimmune form of sclerosing cholangitis and AIH are 2 distinct entities, or different aspects of the same condition, remains to be elucidated.

Less promising results with sclerosing ethoxysclerol injections for midportion achilles tendinopathy: a retrospective study

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van Sterkenburg, Maayke N.; de Jonge, Milko C.; Sierevelt, Inger N.; van Dijk, C. Niek

2010-01-01

BACKGROUND: Local injections of the sclerosing substance polidocanol (Ethoxysclerol) have shown good clinical results in patients with chronic midportion Achilles tendinopathy. After training by the inventors of the technique, sclerosing Ethoxysclerol injections were applied on a group of patients

Diagnostic Dilemma in a Patient with Jaundice: How to Differentiate between Autoimmune Pancreatitis, Primary Sclerosing Cholangitis and Pancreas Carcinoma

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Matthias Buechter

2012-04-01

Full Text Available A 68-year-old male patient was referred to our institution in May 2011 for a suspected tumor in the pancreatic head with consecutive jaundice. Using magnetic resonance imaging, further differentiation between chronic inflammation and a malignant process was not possible with certainty. Apart from cholestasis, laboratory studies showed increased values for CA 19-9 to 532 U/ml (normal <37 U/ml and hypergammaglobulinemia (immunoglobulin G, IgG of 19.3% (normal 8.0–15.8% with an elevation of the IgG4 subtype to 2,350 mg/l (normal 52–1,250 mg/l. Endoscopic retrograde cholangiopancreatography revealed a prominent stenosis of the distal ductus hepaticus communis caused by pancreatic head swelling and also a bihilar stenosis of the main hepatic bile ducts. Cytology demonstrated inflammatory cells without evidence of malignancy. Under suspicion of autoimmune pancreatitis with IgG4-associated cholangitis, immunosuppressive therapy with steroids and azathioprine was started. Follow-up endoscopic retrograde cholangiopancreatography after 3 months displayed regressive development of the diverse stenoses. Jaundice had disappeared and blood values had returned to normal ranges. Moreover, no tumor of the pancreatic head was present in the magnetic resonance control images. Due to clinical and radiological similarities but a consecutive completely different prognosis and therapy, it is of fundamental importance to differentiate between pancreatic cancer and autoimmune pancreatitis. Especially, determination of serum IgG4 levels and associated bile duct lesions induced by inflammation should clarify the diagnosis of autoimmune pancreatitis and legitimate immunosuppressive therapy.

Grey-Turner's sign in sclerosing peritonitis

NARCIS (Netherlands)

Stouthard, J. M.; Krediet, R. T.; Arisz, L.

1989-01-01

A 41-year-old CAPD patient developed Grey-Turner's sign during the course of bacterial peritonitis due to Pseudomonas aeruginosa. At the same time a diagnosis of sclerosing peritonitis was made by CT-scanning of the abdomen. We think that Grey-Turner's flank staining could either have been caused by

The CT appearances of sclerosing mesenteritis and associated diseases

International Nuclear Information System (INIS)

Wat, S.Y.J.; Harish, S.; Winterbottom, A.; Choudhary, A.K.; Freeman, A.H.

2006-01-01

Sclerosing mesenteritis is characterized by non-specific inflammation of the mesenteric fat associated with variable amount of fibrosis. The aetiology is unclear; the pathogenesis is obscure, and even its nomenclature remains variable. It is a rare condition with imaging features that can be mistaken either for a mesenteric neoplasm or for a wide variety of non-neoplastic inflammatory conditions. Knowledge of the imaging features of this condition may prevent unwarranted aggressive therapy. This review discusses the pathogenesis, clinical manifestations of this condition, as well as illustrating the characteristic computed tomography (CT) features of sclerosing mesenteritis. A rational approach to the differential diagnosis is discussed

Ischemic Cholangitis Caused by Transcatheter Hepatic Arterial Chemoembolization 10 Months After Resection of the Extrahepatic Bile Duct

International Nuclear Information System (INIS)

Hasegawa, Kiyoshi; Kubota, Keiichi; Aoki, Taku; Hirai, Ichiro; Miyazawa, Masashi; Ohtomo, Kuni; Makuuchi, Masatoshi

2000-01-01

We report a case of ischemic cholangitis that occurred after transcatheter hepatic arterial chemoembolization (TAE). Ten months prior to TAE the patient had undergone central bisegmentectomy for hepatocellular carcinoma with resection of the extrahepatic bile duct. Eleven days after TAE, he developed suppurative cholangitis and multiple organ failure. Prior surgical ligation of the peribiliary arteries around the extrahepatic bile duct followed by TAE was considered to have played a crucial role in the development of ischemic cholangitis. This case demonstrates the importance of blood flow from the peribiliary arteries for the survival of the biliary epithelium

Benign Biliary Strictures and Leaks.

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Devière, Jacques

2015-10-01

The major causes of benign biliary strictures include surgery, chronic pancreatitis, primary sclerosing cholangitis, and autoimmune cholangitis. Biliary leaks mainly occur after surgery and, rarely, abdominal trauma. These conditions may benefit from a nonsurgical approach in which endoscopic retrograde cholangiopancreatography (ERCP) plays a pivotal role in association with other minimally invasive approaches. This approach should be evaluated for any injury before deciding about the method for repair. ERCP, associated with peroral cholangioscopy, plays a growing role in characterizing undeterminate strictures, avoiding both unuseful major surgeries and palliative options that might compromise any further management. Copyright © 2015 Elsevier Inc. All rights reserved.

Diagnostic accuracy of atypical p-ANCA in autoimmune hepatitis using ROC- and multivariate regression analysis.

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Terjung, B; Bogsch, F; Klein, R; Söhne, J; Reichel, C; Wasmuth, J-C; Beuers, U; Sauerbruch, T; Spengler, U

2004-09-29

Antineutrophil cytoplasmic antibodies (atypical p-ANCA) are detected at high prevalence in sera from patients with autoimmune hepatitis (AIH), but their diagnostic relevance for AIH has not been systematically evaluated so far. Here, we studied sera from 357 patients with autoimmune (autoimmune hepatitis n=175, primary sclerosing cholangitis (PSC) n=35, primary biliary cirrhosis n=45), non-autoimmune chronic liver disease (alcoholic liver cirrhosis n=62; chronic hepatitis C virus infection (HCV) n=21) or healthy controls (n=19) for the presence of various non-organ specific autoantibodies. Atypical p-ANCA, antinuclear antibodies (ANA), antibodies against smooth muscles (SMA), antibodies against liver/kidney microsomes (anti-Lkm1) and antimitochondrial antibodies (AMA) were detected by indirect immunofluorescence microscopy, antibodies against the M2 antigen (anti-M2), antibodies against soluble liver antigen (anti-SLA/LP) and anti-Lkm1 by using enzyme linked immunosorbent assays. To define the diagnostic precision of the autoantibodies, results of autoantibody testing were analyzed by receiver operating characteristics (ROC) and forward conditional logistic regression analysis. Atypical p-ANCA were detected at high prevalence in sera from patients with AIH (81%) and PSC (94%). ROC- and logistic regression analysis revealed atypical p-ANCA and SMA, but not ANA as significant diagnostic seromarkers for AIH (atypical p-ANCA: AUC 0.754+/-0.026, odds ratio [OR] 3.4; SMA: 0.652+/-0.028, OR 4.1). Atypical p-ANCA also emerged as the only diagnostically relevant seromarker for PSC (AUC 0.690+/-0.04, OR 3.4). None of the tested antibodies yielded a significant diagnostic accuracy for patients with alcoholic liver cirrhosis, HCV or healthy controls. Atypical p-ANCA along with SMA represent a seromarker with high diagnostic accuracy for AIH and should be explicitly considered in a revised version of the diagnostic score for AIH.

Sclerosing encapsulating peritonitis in chronic ambulatory peritoneal dialysis;preoperative catheter drainage : a case report

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Kim, Tae Hoon [Dankook Univ. Hospital, Seoul (Korea, Republic of)

1996-10-01

Sclerosing encapsulating peritonitis is a well recognized, but uncommon, complication of chronic ambulatory peritoneal dialysis. I report a case of sclerosing encapsulating peritonitis in which percutaneous catheter drainage was performed preoperatively. Ultrasonography(US) and computed tomography(CT) showed a large multi-septated cystic mass which occupied nearly all the peritoneal cavity. Percutaneous drainage with two 8.5 French catheters was preoperatively performed under fluoroscopy and about 2100 ml of bloody fluid was drained for 20 days. On follow-up CT, the size of the cyst had significantly decreased and anoperation was performed. It is considered that percutaneous catheter drainage is useful in the preoperative decompression of sclerosing encapsulating peritonitis.

Sclerosing encapsulating peritonitis in chronic ambulatory peritoneal dialysis;preoperative catheter drainage : a case report

International Nuclear Information System (INIS)

Kim, Tae Hoon

1996-01-01

Sclerosing encapsulating peritonitis is a well recognized, but uncommon, complication of chronic ambulatory peritoneal dialysis. I report a case of sclerosing encapsulating peritonitis in which percutaneous catheter drainage was performed preoperatively. Ultrasonography(US) and computed tomography(CT) showed a large multi-septated cystic mass which occupied nearly all the peritoneal cavity. Percutaneous drainage with two 8.5 French catheters was preoperatively performed under fluoroscopy and about 2100 ml of bloody fluid was drained for 20 days. On follow-up CT, the size of the cyst had significantly decreased and anoperation was performed. It is considered that percutaneous catheter drainage is useful in the preoperative decompression of sclerosing encapsulating peritonitis

Bedside Endoscopic Retrograde Cholangiopancreatography Using Portable X-Ray in Acute Severe Cholangitis

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Rushikesh Shah

2018-01-01

Full Text Available Patients with acute cholangitis require emergent biliary decompression. Those who are hemodynamically unstable on vasopressor support and mechanical ventilation are too critically ill to move outside of the intensive care unit. This prohibits performing Endoscopic Retrograde Cholangiopancreatography (ERCP in the endoscopy unit. Fluoroscopic guidance is required to confirm deep biliary cannulation during ERCP. There are a few reported cases of bedside ERCP using portable C-arm fluoroscopy unit or ultrasound guided cannulation. We present a unique case of life-saving emergent bedside ERCP in a severely ill patient with cholangitis and septic shock, using simple portable X-ray to confirm biliary cannulation.

Diffuse sclerosing variant of thyroid carcinoma presenting as Hashimoto thyroiditis: a case report.

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Vukasović, Anamarija; Kuna, Sanja Kusacić; Ostović, Karmen Trutin; Prgomet, Drago; Banek, Tomislav

2012-11-01

The aim of report is to present a case of a rare diffuse sclerosing variant of a papillary thyroid carcinoma. A 15-year old girl referred for ultrasound examination because of painless thyroid swelling lasting 10 days before. An ultrasound of the neck showed diffusely changed thyroid parenchyma, without nodes, looking as lymphocytic thyroiditis Hashimoto at first, but with snow-storm appearance, predominantly in the right lobe. Positive thyroid peroxidase antibodies (TPO-AT) also suggested Hashimoto thyroiditis. Repeated US-FNAB (fine needle-aspiration biopsy) of the right lobe revealed diffuse sclerosing variant of papillary thyroid carcinoma and patient underwent total thyreoidectomy. Patohistologic finding confirmed diffuse sclerosing variant of a papillary thyroid carcinoma in the both thyroid lobes and several metastatic lymph nodes. Two months later patient recived radioablative therapy with 3700 MBq (100 mCi) of 1-131 followed by levothyroxine replacement. At the moment, patient is without evidence of local or distant metastases and next regular control is scheduled in 6 months. In conclusion, a diffuse sclerosing variant is rare form of papillary thyroid carcinoma that echographically looks similar to Hashimoto thyroiditis and sometimes could be easily overlooked.

Role of endoscopic retrograde cholangiopancreatography in the management of benign biliary strictures: What’s new?

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Ferreira, Rosa; Loureiro, Rui; Nunes, Nuno; Santos, António Alberto; Maio, Rui; Cravo, Marília; Duarte, Maria Antónia

2016-01-01

Benign biliary strictures comprise a heterogeneous group of diseases. The most common strictures amenable to endoscopic treatment are post-cholecystectomy, post-liver transplantation, related to primary sclerosing cholangitis and to chronic pancreatitis. Endoscopic treatment of benign biliary strictures is widely used as first line therapy, since it is effective, safe, noninvasive and repeatable. Endoscopic techniques currently used are dilation, multiple plastic stents insertion and fully covered self-expandable metal stents. The main indication for dilation alone is primary sclerosing cholangitis related strictures. In the vast majority of the remaining cases, temporary placement of multiple plastic stents with/without dilation is considered the treatment of choice. Although this approach is effective, it requires multiple endoscopic sessions due to the short duration of stent patency. Fully covered self-expandable metal stents appear as a good alternative to plastic stents, since they have an increased radial diameter, longer stent patency, easier insertion technique and similar efficacy. Recent advances in endoscopic technique and various devices have allowed successful treatment in most cases. The development of novel endoscopic techniques and devices is still ongoing. PMID:26962404

Ursodeoxycholic Acid Influences the Expression of p27kip1 but Not FoxO1 in Patients with Non-Cirrhotic Primary Biliary Cirrhosis

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Malgorzata Milkiewicz

2014-01-01

Full Text Available Background. Enhanced expression of cell cycle inhibitor p27kip1 suppresses cell proliferation. Ursodeoxycholic acid (UDCA delays progression of primary biliary cirrhosis (PBC but its effect on p27kip1 expression is uncertain. Aims. To analyze the expression of p27kip1 and its transcription modulator FoxO1 in patients with PBC, and to assess the impact of UDCA on this pathway. Materials and Methods. The examined human tissue included explanted livers from patients with cirrhotic PBC (n=23, primary sclerosing cholangitis (PSC; n=9, alcoholic liver disease (ALD; n=9, and routine liver biopsies from patients with non-cirrhotic PBC (n=26. Healthy liver samples served as controls (n=19. Livers of FoxO-deficient mice were also studied. mRNA and protein expressions were analyzed by real-time PCR and Western blot. Results. p27kip1 expression was increased in cirrhotic and non-cirrhotic PBC. FoxO1 mRNA levels were increased in PBC (8.5-fold increase versus controls. FoxO1 protein expression in PBC was comparable to controls, but it was decreased in patients with PSC and ALD (63% and 70% reduction, respectively; both P<0.05 versus control. UDCA-treated non-cirrhotic patients with PBC showed decreased expression of p27kip1 mRNA. Conclusion. PBC progression is characterized by a FoxO1-independent increase of p27kip1 expression. In early PBC, UDCA may enhance liver regeneration via p27kip1-dependent mechanism.

Frequency and predictive factors for overlap syndrome between autoimmune hepatitis and primary cholestatic liver disease.

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Gheorghe, Liana; Iacob, Speranta; Gheorghe, Cristian; Iacob, Razvan; Simionov, Iulia; Vadan, Roxana; Becheanu, Gabriel; Parvulescu, Iuliana; Toader, Cristina

2004-06-01

To evaluate the frequency of cholestatic pattern in patients with autoimmune hepatitis (AIH) and to identify predictive factors associated with the development of the overlap syndrome. Eighty-two consecutive patients diagnosed with AIH at the referral centre between January 1998 and June 2002 were included in the study. The new scoring system modified by the International Autoimmune Hepatitis Group was used to classify patients as definite/probable. Overlap syndrome was considered when the patient had clinical, serological and histological characteristics of two conditions: AIH and primary biliary cirrhosis (PBC) or AIH and primary sclerosing cholangitis (PSC). From the 82 AIH patients (76 female and six male), 84.1% presented definite AIH (> 15 points) and 15.9% probable AIH (10 - 15 points). The frequency of the overlap syndrome was 20%: 13% with PBC and 7% with PSC. In the univariate analysis the overlap syndrome was associated with male gender (P = 0.01), age < 35 years (P < 0.0001), histopathological aspect of cholestasis (P < 0.0001), suboptimal response to treatment (P < 0.0001) and probable AIH (P < 0.0001). Age < 35 years, probable AIH and the absence of anti-nuclear antibody (ANA) have been identified as independent indicators of the overlap diagnosis by the logistic regression analysis. Patients with overlap syndrome between AIH and primary cholestatic liver disease are frequently diagnosed in clinical practice, representing 20% of AIH cases in our study. The independent predictive factors associated with the diagnosis of overlap syndrome are young age, ANA(-) profile, and probable diagnosis according with the scoring system for AIH.

Hepatobiliary fascioliasis: a case with unusual radiological features.

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Yeşildağ, Ahmet; Senol, Altuğ; Köroğlu, Mert; Koçkar, Cem; Oyar, Orhan; Işler, Mehmet

2010-12-01

We report a case of hepatobiliary fascioliasis presenting with unusual radiological findings that have not been reported previously. Imaging studies revealed hepatic cystic pouches communicating with intrahepatic bile ducts. Snail-like, oval shaped and conglomerated echogenic particles with no acoustic shadowing, suggesting F. hepatica, were detected in these cystic pouches. In addition, secondary sclerosing cholangitis developed after fascioliasis.

The new energy management policy: Indonesian PSC-gross-split applied on steam flooding project

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Irham, S.; Julyus, P.

2018-01-01

“SIPY” oil field has been producing oil using steam flooding technology since 1992 under the PSC-Cost-Recovery policy. In 2021, the contract will be finished, and a new agreement must be submitted to the Indonesian government. There are two applied fiscal policies on oil and gas management: PSC-Cost-Recovery and PSC-Gross-Split (introduced in 2017 as the new energy management plan). The contractor must choose between PSC-Cost-Recovery and PSC-Gross-split which makes more profit. The aim of this research is to determine the best oil and gas contract policy for the contractor. The methods are calculating contractor cash flow and comparing the Profitability Indexes. The results of this study are (1) Net Present Values for the PSC-Cost-Recovery and the PSC-Gross-Split are 15 MMUS and 61 MMUS, respectively; and (2) Internal Rate of Return values for the PSC-Cost-Recovery and PSC-Gross-Split are 10% and 11%, respectively. The conclusion is that the Net Present Value and Internal Rate of Return of PSC-Gross-Split are greater than those of PSC-Cost-Recovery, but in Pay Out Time of PSC-Gross-split is longer than Pay Out Time in PSC-Cost-Recovery. Thus, the new energy management policy will be more attractive than PSC-Cost-Recovery.

Tokyo Guidelines 2018: antimicrobial therapy for acute cholangitis and cholecystitis

NARCIS (Netherlands)

Gomi, Harumi; Solomkin, Joseph S.; Schlossberg, David; Okamoto, Kohji; Takada, Tadahiro; Strasberg, Steven M.; Ukai, Tomohiko; Endo, Itaru; Iwashita, Yukio; Hibi, Taizo; Pitt, Henry A.; Matsunaga, Naohisa; Takamori, Yoriyuki; Umezawa, Akiko; Asai, Koji; Suzuki, Kenji; Han, Ho-Seong; Hwang, Tsann-Long; Mori, Yasuhisa; Yoon, Yoo-Seok; Huang, Wayne Shih-Wei; Belli, Giulio; Dervenis, Christos; Yokoe, Masamichi; Kiriyama, Seiki; Itoi, Takao; Jagannath, Palepu; Garden, O. James; Miura, Fumihiko; de Santibañes, Eduardo; Shikata, Satoru; Noguchi, Yoshinori; Wada, Keita; Honda, Goro; Supe, Avinash Nivritti; Yoshida, Masahiro; Mayumi, Toshihiko; Gouma, Dirk J.; Deziel, Daniel J.; Liau, Kui-Hin; Chen, Miin-Fu; Liu, Keng-Hao; Su, Cheng-Hsi; Chan, Angus C. W.; Yoon, Dong-Sup; Choi, In-Seok; Jonas, Eduard; Chen, Xiao-Ping; Fan, Sheung Tat; Ker, Chen-Guo; Giménez, Mariano Eduardo; Kitano, Seigo; Inomata, Masafumi; Mukai, Shuntaro; Higuchi, Ryota; Hirata, Koichi; Inui, Kazuo; Sumiyama, Yoshinobu; Yamamoto, Masakazu

2018-01-01

Antimicrobial therapy is a mainstay of the management for patients with acute cholangitis and/or cholecystitis. The Tokyo Guidelines 2018 (TG18) provides recommendations for the appropriate use of antimicrobials for community-acquired and healthcare-associated infections. The listed agents are for

Generation of iPSC line iPSC-FH2.1 in hypoxic conditions from human foreskin fibroblasts

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María Questa

2016-03-01

Full Text Available Human foreskin fibroblasts were used to generate the iPSC line iPSC-FH2.1 using the EF1a-hSTEMCCA-loxP vector expressing OCT4, SOX2, c-MYC and KLF4, in 5% O2 culture conditions. Stemness was confirmed, as was pluripotency both in vivo and in vitro, in normoxia and hypoxia. Human Embryonic Stem Cell (hESC line WA-09 and reprogrammed fibroblast primary culture HFF-FM were used as controls.

A case of cholestatic hepatitis associated with histologic features of acute cholangitis

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Takeuchi H

2011-11-01

Full Text Available Hajime Takeuchi1, Toru Kaneko1, Toshikazu Otsuka1, Kumiko Tahara1, Tadashi Motoori2, Makoto Ohbu3, Masaya Oda4, Hiroaki Yokomori11Department of Internal Medicine; 2Division of Pathology, Kitasato Medical Center Hospital, Kitasato University, Saitama; 3Department of Pathology, School of Allied Health Sciences, Kitasato University, Sagamihara, Kanagawa; 4Department of Internal Medicine, Saitama Social Insurance Hospital, Saitama; 5Organized Center of Clinical Medicine, International University of Health and Welfare, Sanno Hospital, Tokyo, JapanAbstract: This report describes a case showing histologic features of acute cholangitis with an over-the-counter drug. A 48-year-old woman was diagnosed with general malaise and progressive jaundice. A thorough review of her medical history revealed that the patient had taken an over-the-counter drug, Pabron Gold®, which she had used previously, that may have caused liver injury. Laboratory investigations revealed jaundice and liver dysfunction. Endoscopic retrograde cholangiography detected no extrahepatic biliary duct dilatation or stones. Liver biopsy indicated acute cholangitis involving neutrophils and eosinophils. Electron microscopy revealed fragmented nuclei, indicating that the degenerative bile duct-related epithelial cells were in an apoptotic process.Keywords: liver injury, over-the-counter drug, histologic features, acute cholangitis, electron microscopy, Pabron Gold

Radiologic Findings of Immunoglobulin G4 Related Sclerosing Esophagitis: A Case Report

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Kim, Mi Sun; Kim, Su Young; Lee, Byung Hoon; Hwang, Yoon Joon; Han, Yoon Hee [Dept. of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, Koyang (Korea, Republic of)

2012-02-15

We describe a case of immunoglobulin G4 (IgG4)-related sclerosing esophagitis occurring in a 63-year-old man with progressive dysphagia and 10-kg weight loss over 9 months. An esophagoscopy revealed significant stricture with diffuse mucosal friability and ulceration at mid esophagus level. Barium esophagogram showed diffuse stenosis at the mid and lower esophagus levels with ulcerations and irregularity of the mucosa. Multidetector computed tomography revealed diffuse edematous and circumferential thickening of the submucosa and muscle layer of this esophageal segment. Fluorine 18 fluorodeoxyglucose positron emission tomography (FDG PET) revealed diffuse mild FDG uptake in mid to lower esophagus. Although benign inflammatory lesion was suspected based on the imaging findings, the patient underwent surgery for worsening esophageal stricture and the esophageal lesion was pathologically confirmed as IgG4-related sclerosing esophagitis. Radiologic benignancy and high clinical suspicion for IgG4-related sclerosing disease may help making a proper decision and avoiding unnecessary operation.

Radiologic Findings of Immunoglobulin G4 Related Sclerosing Esophagitis: A Case Report

International Nuclear Information System (INIS)

Kim, Mi Sun; Kim, Su Young; Lee, Byung Hoon; Hwang, Yoon Joon; Han, Yoon Hee

2012-01-01

We describe a case of immunoglobulin G4 (IgG4)-related sclerosing esophagitis occurring in a 63-year-old man with progressive dysphagia and 10-kg weight loss over 9 months. An esophagoscopy revealed significant stricture with diffuse mucosal friability and ulceration at mid esophagus level. Barium esophagogram showed diffuse stenosis at the mid and lower esophagus levels with ulcerations and irregularity of the mucosa. Multidetector computed tomography revealed diffuse edematous and circumferential thickening of the submucosa and muscle layer of this esophageal segment. Fluorine 18 fluorodeoxyglucose positron emission tomography (FDG PET) revealed diffuse mild FDG uptake in mid to lower esophagus. Although benign inflammatory lesion was suspected based on the imaging findings, the patient underwent surgery for worsening esophageal stricture and the esophageal lesion was pathologically confirmed as IgG4-related sclerosing esophagitis. Radiologic benignancy and high clinical suspicion for IgG4-related sclerosing disease may help making a proper decision and avoiding unnecessary operation.

Potential of iPSC-Derived Mesenchymal Stromal Cells for Treating Periodontal Disease

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K. Hynes

2018-01-01

Full Text Available Mesenchymal stromal cell-like populations have been derived from mouse-induced pluripotent stem cells (miPSC-MSC with the capability for tissue regeneration. In this study, murine iPSC underwent differentiation towards an MSC-like immunophenotype. Stable miPSC-MSC cultures expressed the MSC-associated markers, CD73, CD105, and Sca-1, but lacked expression of the pluripotency marker, SSEA1, and hematopoietic markers, CD34 and CD45. Functionally, miPSC-MSC exhibited the potential for trilineage differentiation into osteoblasts, adipocytes, and chondrocytes and the capacity to suppress the proliferation of mitogen-activated splenocytes. The efficacy of miPSC-MSC was assessed in an acute inflammation model following systemic or local delivery into mice with subcutaneous implants containing heat-inactivated P. gingivalis. Histological analysis revealed less inflammatory cellular infiltrate within the sponges in mice treated with miPSC-MSC cells delivered locally rather than systemically. Assessment of proinflammatory cytokines in mouse spleens found that CXCL1 transcripts and protein were reduced in mice treated with miPSC-MSC. In a periodontitis model, mice subjected to oral inoculation with P. gingivalis revealed less bone tissue destruction and inflammation within the jaws when treated with miPSC-MSC compared to PBS alone. Our results demonstrated that miPSC-MSC derived from iPSC have the capacity to control acute and chronic inflammatory responses associated with the destruction of periodontal tissue. Therefore, miPSC-MSC present a promising novel source of stromal cells which could be used in the treatment of periodontal disease and other inflammatory systemic diseases such as rheumatoid arthritis.

Mesenteric ossification in CT indicates sclerosing peritonitis in chronic bacterial infection and pancreatitis

International Nuclear Information System (INIS)

Kirchner, J.; Kirchner, E.M.; Kickuth, R.; Stein, A.

2004-01-01

Sclerosing peritonitis already has been described as a serious complication of the continuous ambulatory peritoneal dialysis. But different other affections of the pertioneum such as chronic bacterial peritonitis and pancreatitis may result in sclerosing peritonitis, too. The symptom is characterised by thickened small bowel walls and periotoneal membranes as well as peritoneal calcifications which can be shown in computed tomography. We demonstrate two cases of peritoneal ossifications due to peritonitis and pancreatitis. (orig.) [de

The Emerging Role of Soluble Adenylyl Cyclase in Primary Biliary Cholangitis

NARCIS (Netherlands)

Chang, Jung-Chin; Beuers, Ulrich; Oude Elferink, Ronald P. J.

2017-01-01

Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic fibrosing cholangiopathy with the signature of an autoimmune disease and features of intrahepatic cholestasis. Immunosuppressing treatments are largely unsuccessful. Responsiveness to ursodeoxycholic

Genetics Home Reference: polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

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... feelings of intense happiness (euphoria), a loss of inhibition, and poor concentration. These neurologic changes cause significant ... with sclerosing leukoencephalopathy Orphanet: Nasu-Hakola disease Patient Support and Advocacy Resources (3 links) Alzheimer's Association Family ...

Frequency and importance of radiologically visible coronary scleroses

International Nuclear Information System (INIS)

Hoyer, B.

1981-01-01

The importance of radiologically visible coronary sceleroses for an early diagnosing of coronary heart disease was investigated. In 3 mixed collectives of patients with and without coronary heart diseases who had had a coronary angiography were examined retrospectively for coronary sclerosis (group I: standard films, group II: old X-ray findings, group III: fluoroscopy before beginning coronary angiography). In the retrospective evaluation, the sensitivity to the recognition of coronary heart disease was low. With prospective examination by means of fluoroscopy, coronary calcification could be proven in 37.66% of the patients with coronary diseases (sensitivity); the specificity (no coronary calcification in persons without coronary disease) was 99%. The duration and extent of a calcification do not show a definite influence on the severity of the disease. Several coronary scleroses in one or several vessels mostly indicate a vascular disease. Coronary scleroses are not necessarily located in the same point as coronary stenoses: in 85.5% of the patients with coronary sclerosis of the left truncus, haemodynamically important stenoses were found only in following vascular regions. Considering the high specificity of coronary scleroses in the fluoroscopic picture and the high sensitivity under favourable conditions, this method seems to be suitable as a screening method for early recognition of coronary heart diseases in the asymptomatic stage. A proven coronary sclerosis should in any case be the cause for continuing the search for a coronary heart disease. The radiation exposure during fluoroscopy of coronary sclerosis is low if the adjustment is correct, it takes little time, the patient is not put to discomfort. (orig./MG) [de

Sclerosing stromal tumor of the ovary: a case report

Energy Technology Data Exchange (ETDEWEB)

Kang, Hyun Koo; Koh, Byung Hee; Rhim, Hyun Chul; Cho, On Koo; Kim, Yong Soo; Hahm, Chang Kok [School of Medicine, Hanyang Univ., Seoul (Korea, Republic of)

2002-07-01

Sclerosing stromal tumor of the ovary is a rare benign neoplasm, with distinctive clinical and pathologic features. It occurs predominantly in females during the second and third decades of life. Histologically, it is composed of cellular and acellular collagenized areas, and edematous stromal areas, and at ultrasonography and computed tomography is seen as a distinctive mixed solid and cystic mass lesion. We report a case of sclerosing stromal tumor of the ovary in a 15-year-old girl with a history of menorrhagia since menarche. Ultrasonography revealed the tumor as a well-defined, lobulated, heterogenous echogenic pelvic mass, while at CT, a huge pelvic mass 9 x 9 x 10 cm in size, was seen. This comprised a well-enhanced internal solid portion, a capsule, septa, and a non-enhanced cystic portion.

Sclerosing stromal tumor of the ovary in a premenarchal female

International Nuclear Information System (INIS)

Fefferman, Nancy R.; Pinkney, Lynne P.; Rivera, Rafael; Popiolek, Dorota; Hummel-Levine, Pascale; Cosme, Jaqueline

2003-01-01

Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm of stromal origin with less than 100 cases reported in the literature. Unlike the other stromal tumors, thecomas and fibromas, which tend to occur in the fifth and sixth decades, sclerosing stromal tumors predominantly affect females in the second and third decades. Computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound findings have been described, but have not been reported previously in the pediatric literature. We present a case of SST of the ovary in a 10-year-old premenarchal female, the youngest patient to our knowledge reported in the literature, and describe the ultrasound and CT findings with pathologic correlation. (orig.)

Sclerosing peritonitis with gross calcification: case report

Energy Technology Data Exchange (ETDEWEB)

Kim, Cheung Sook; Kim, Young Jae; Min, Seon Jeong; Cho, Seong Whi; Lee, Gyung Kyu; Lee, Eil Seong; Kang, Ik Won [Hallym University College of Medicine, Seoul (Korea, Republic of)

2003-09-01

Sclerosing peritonitis is an uncommon complication of continuous ambulatory peritoneal dialysis (CAPD) and can lead to small bowel dysfunction involving abdominal pain, progressive loss of ultrafiltration, and small intestinal obstruction. Peritoneal thickening, in which calcification can develop, often starts as al small plaque which gradually becomes larger. We report a case of CAPD-related calcifying peritonitis.

The new management policy: Indonesian PSC-Gross split applied on CO2 flooding project

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Irham, S.; Sibuea, S. N.; Danu, A.

2018-01-01

“SIAD” oil field will be developed by CO2 flooding. CO2, a famous pollutant gas, is injected into the oil reservoir to optimize the oil recovery. This technique should be conducted economically according to the energy management policy in Indonesia. In general, Indonesia has two policy contracts on oil and gas: the old one is PSC-Cost-Recovery, and the new one is PSC-Gross-Split (introduced in 2017 as the new energy management plan). The contractor must choose between PSC-Cost-Recovery and PSC-Gross-Split which makes more profit. The aim of this paper is to show the best oil and gas contract policy for the contractor. The methods are calculating and comparing the economic indicators. The result of this study are (1) NPV for the PSC-Cost-Recovery is -46 MUS, while for the PSC-Gross-Split is 73 MUS, and (2) IRR for the PSC-Cost-Recovery is 9%, whereas for the PSC-Gross-Split is 11%. The conclusion is that the NPV and IRR for PSC-Gross-Split are greater than the NPV and IRR of PSC-Cost-Recovery, but POT in PSC-Gross-split is longer than POT in PSC-Cost-Recovery. Thus, in this case, the new energy policy contract can be applied for CO2 flooding technology since it yields higher economic indicators than its antecendent.

Neonatal Transplantation Confers Maturation of PSC-Derived Cardiomyocytes Conducive to Modeling Cardiomyopathy

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Gun-Sik Cho

2017-01-01

Full Text Available Summary: Pluripotent stem cells (PSCs offer unprecedented opportunities for disease modeling and personalized medicine. However, PSC-derived cells exhibit fetal-like characteristics and remain immature in a dish. This has emerged as a major obstacle for their application for late-onset diseases. We previously showed that there is a neonatal arrest of long-term cultured PSC-derived cardiomyocytes (PSC-CMs. Here, we demonstrate that PSC-CMs mature into adult CMs when transplanted into neonatal hearts. PSC-CMs became similar to adult CMs in morphology, structure, and function within a month of transplantation into rats. The similarity was further supported by single-cell RNA-sequencing analysis. Moreover, this in vivo maturation allowed patient-derived PSC-CMs to reveal the disease phenotype of arrhythmogenic right ventricular cardiomyopathy, which manifests predominantly in adults. This study lays a foundation for understanding human CM maturation and pathogenesis and can be instrumental in PSC-based modeling of adult heart diseases. : Pluripotent stem cell (PSC-derived cells remain fetal like, and this has become a major impediment to modeling adult diseases. Cho et al. find that PSC-derived cardiomyocytes mature into adult cardiomyocytes when transplanted into neonatal rat hearts. This method can serve as a tool to understand maturation and pathogenesis in human cardiomyocytes. Keywords: cardiomyocyte, maturation, iPS, cardiac progenitor, neonatal, disease modeling, cardiomyopathy, ARVC, T-tubule, calcium transient, sarcomere shortening

Neonatal Transplantation Confers Maturation of PSC-Derived Cardiomyocytes Conducive to Modeling Cardiomyopathy.

Science.gov (United States)

Cho, Gun-Sik; Lee, Dong I; Tampakakis, Emmanouil; Murphy, Sean; Andersen, Peter; Uosaki, Hideki; Chelko, Stephen; Chakir, Khalid; Hong, Ingie; Seo, Kinya; Chen, Huei-Sheng Vincent; Chen, Xiongwen; Basso, Cristina; Houser, Steven R; Tomaselli, Gordon F; O'Rourke, Brian; Judge, Daniel P; Kass, David A; Kwon, Chulan

2017-01-10

Pluripotent stem cells (PSCs) offer unprecedented opportunities for disease modeling and personalized medicine. However, PSC-derived cells exhibit fetal-like characteristics and remain immature in a dish. This has emerged as a major obstacle for their application for late-onset diseases. We previously showed that there is a neonatal arrest of long-term cultured PSC-derived cardiomyocytes (PSC-CMs). Here, we demonstrate that PSC-CMs mature into adult CMs when transplanted into neonatal hearts. PSC-CMs became similar to adult CMs in morphology, structure, and function within a month of transplantation into rats. The similarity was further supported by single-cell RNA-sequencing analysis. Moreover, this in vivo maturation allowed patient-derived PSC-CMs to reveal the disease phenotype of arrhythmogenic right ventricular cardiomyopathy, which manifests predominantly in adults. This study lays a foundation for understanding human CM maturation and pathogenesis and can be instrumental in PSC-based modeling of adult heart diseases. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis

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Nevens, Frederik; Andreone, Pietro; Mazzella, Giuseppe; Strasser, Simone I.; Bowlus, Christopher; Invernizzi, Pietro; Drenth, Joost P. H.; Pockros, Paul J.; Regula, Jaroslaw; Beuers, Ulrich; Trauner, Michael; Jones, David E.; Floreani, Annarosa; Hohenester, Simon; Luketic, Velimir; Shiffman, Mitchell; van Erpecum, Karel J.; Vargas, Victor; Vincent, Catherine; Hirschfield, Gideon M.; Shah, Hemant; Hansen, Bettina; Lindor, Keith D.; Marschall, Hanns-Ulrich; Kowdley, Kris V.; Hooshmand-Rad, Roya; Marmon, Tonya; Sheeron, Shawn; Pencek, Richard; MacConell, Leigh; Pruzanski, Mark; Shapiro, David; Angus, Peter; Roberts, Stuart; Vogel, Wolfgang; Graziadei, Ivo; de Lédinghen, Victor; Berg, Thomas; Gotthardt, Daniel; Hartmann, Heinz; Kremer, Andreas E.; Lammert, Frank; Manns, Michael P.; Rust, Christian; Schramm, Christoph; Trautwein, Christian; Zeuzem, Stefan; Carbone, Marco; van Nieuwkerk, Carin C. M. J.; Celinski, Krzysztof; Gonciarz, Maciej; Hartleb, Marek; Milkiewicz, Piotr; Parés, Albert; Bramley, Peter; Thorburn, Douglas; Mookerjee, Rajeshwar P.; Burroughs, Andrew; Chapman, Roger; Dillon, John F.; Greer, John A.; Tripathi, Dhiraj; McCune, Anne; Ryder, Stephen; Bacon, Bruce R.; Naik, Jahnavi; Wang, Lan Sun; Bodenheimer, Henry C.; Bowlus, Christopher L.; Chalasani, Naga; Forman, Lisa M.; Gordon, Stuart C.; Luketic, Velimir A.; Mayo, Marlyn; Muir, Andrew J.; Reddy, K. Gautham; Talwalker, Jayant T.; Vierling, John M.

2016-01-01

BACKGROUND Primary biliary cholangitis ( formerly called primary biliary cirrhosis) can progress to cirrhosis and death despite ursodiol therapy. Alkaline phosphatase and bilirubin levels correlate with the risk of liver transplantation or death. Obeticholic acid, a farnesoid X receptor agonist, has

Neonatal Transplantation Confers Maturation of PSC-Derived Cardiomyocytes Conducive to Modeling Cardiomyopathy

OpenAIRE

Cho, Gun-Sik; Lee, Dong I.; Tampakakis, Emmanouil; Murphy, Sean; Andersen, Peter; Uosaki, Hideki; Chelko, Stephen; Chakir, Khalid; Hong, Ingie; Seo, Kinya; Vincent Chen, Huei-Sheng; Chen, Xiongwen; Basso, Cristina; Houser, Steven R.; Tomaselli, Gordon F.

2017-01-01

Summary: Pluripotent stem cells (PSCs) offer unprecedented opportunities for disease modeling and personalized medicine. However, PSC-derived cells exhibit fetal-like characteristics and remain immature in a dish. This has emerged as a major obstacle for their application for late-onset diseases. We previously showed that there is a neonatal arrest of long-term cultured PSC-derived cardiomyocytes (PSC-CMs). Here, we demonstrate that PSC-CMs mature into adult CMs when transplanted into neonata...

Subacute sclerosing panencephalitis presenting as mania

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Aggarwal Ashish

2011-01-01

Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

Engineering-derived approaches for iPSC preparation, expansion, differentiation and applications.

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Li, Yang; Li, Ling; Chen, Zhi-Nan; Gao, Ge; Yao, Rui; Sun, Wei

2017-07-31

Remarkable achievements have been made since induced pluripotent stem cells (iPSCs) were first introduced in 2006. Compared with non-pluripotent stem cells, iPSC research faces several additional complexities, such as the choice of extracellular matrix proteins, growth and differentiation factors, as well as technical challenges related to self-renewal and directed differentiation. Overcoming these challenges requires the integration of knowledge and technologies from multiple fields including cell biology, biomaterial science, engineering, physics and medicine. Here, engineering-derived iPSC approaches are reviewed according to three aspects of iPSC studies: preparation, expansion, differentiation and applications. Engineering strategies, such as 3D systems establishment, cell-matrix mechanics and the regulation of biophysical and biochemical cues, together with engineering techniques, such as 3D scaffolds, cell microspheres and bioreactors, have been applied to iPSC studies and have generated insightful results and even mini-organs such as retinas, livers and intestines. Specific results are given to demonstrate how these approaches impact iPSC behavior, and related mechanisms are discussed. In addition, cell printing technologies are presented as an advanced engineering-derived approach since they have been applied in both iPSC studies and the construction of diverse tissues and organs. Further development and possible innovations of cell printing technologies are presented in terms of creating complex and functional iPSC-derived living tissues and organs.

Lymphocytic cholangitis in cats: a microbiological, histological and clinical approach

NARCIS (Netherlands)

Otte, C.M.A.

2017-01-01

In this thesis, a general overview is given of the healthy feline liver and feline diseases of the gall bladder and biliary tree. Lymphocytic cholangitis (LC) is one of the most common inflammatory hepatic diseases in cats. It is a chronic disease that affects the biliary tree and progresses slowly

9+ Years of CALIOP PSC Data: An Evolving Climatology

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Pitts, Michael C.; Poole, Lamont R.

2015-01-01

Polar stratospheric clouds (PSCs) play key roles in the springtime chemical depletion of ozone at high latitudes. PSC particles provide sites for heterogeneous chemical reactions that transform stable chlorine and bromine reservoir species into highly reactive ozone-destructive forms. Furthermore, large nitric acid trihydrate (NAT) PSC particles can irreversibly redistribute odd nitrogen through gravitational sedimentation, which prolongs the ozone depletion process by slowing the reformation of the stable chlorine reservoirs. However, there are still significant gaps in our understanding of PSC processes, particularly concerning the details of NAT particle formation. Spaceborne observations from the CALIOP (Cloud-Aerosol Lidar with Orthogonal Polarization) lidar on the CALIPSO (Cloud-Aerosol Lidar and Infrared Pathfinder Satellite Observations) satellite are providing a rich new dataset for studying PSCs on unprecedented vortex-wide scales. In this paper, we examine the vertical and spatial distribution of PSCs in the Antarctic and Arctic on vortex-wide scales for entire PSC seasons over the more than nine-year data record.

Sclerosing Variant of the Bronchioloalveolar Carcinoma: Imaging Findings in an Atypical Case

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Carolina Lamas Constantino

2010-01-01

Full Text Available Bronchioloalveolar carcinoma remains one of the most enigmatic lung cancers, demonstrating varied growth patterns, mixed histological features, and confusing clinical manifestations. This paper reports a case of an unusual form of presentation: a sclerosing type associated with desmoplastic reaction and cicatrization. A 75-year-old woman was admitted with persistent dry cough and progressive dyspnea. Physical examination showed bilateral inspiratory crackles. A chest radiograph and high-resolution computed tomography demonstrated confluent airspace nodules, forming areas of consolidation in both lungs, with signs of architectural distortion. The lung biopsy revealed a nonmucinous sclerosing bronchioloalveolar carcinoma.

Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation.

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Lien, Tien-Hau; Bu, Ling-Nan; Wu, Jia-Feng; Chen, Huey-Ling; Chen, An-Chyi; Lai, Ming-Wei; Shih, Hsiang-Hung; Lee, I-Hsien; Hsu, Hong-Yuan; Ni, Yen-Hsuan; Chang, Mei-Hwei

2015-05-01

Recurrent cholangitis may aggravate cholestatic liver cirrhosis in biliary atresia (BA) after the Kasai operation. This pilot study aimed to investigate whether Lactobacillus casei rhamnosus has the prophylactic efficacy for recurrent cholangitis in comparison with the conventional neomycin prophylaxis. Twenty jaundice-free patients with BA ages 0 to 3 years who underwent a Kasai operation were enrolled and randomized into 2 groups with 10 patients each: neomycin (25 mg · kg · day for 4 days/wk) and L casei rhamnosus (8 × 10 colony-forming unit per day) groups. The treatment duration was 6 months. Bacterial stool cultures were performed before treatment and 1, 3, and 6 months after starting treatment. In addition, 10 patients with BA with similar status but without prophylaxis served as the historical control group. In the Lactobacillus group, 2 patients (20%, mean 0.03 ± 0.07 episodes per month) developed cholangitis during the study period, with the same frequency as in the neomycin group and significantly lower than that in the control group (80%, P = 0.005, mean 0.22 ± 0.16 episodes per month). The mean change in body weight z score during the 6 months in the Lactobacillus group was 0.97 ± 0.59, which was significantly better than that in the control group (-0.01 ± 0.79, P = 0.006). In bacterial stool cultures, the Lactobacillus and Escherichia coli populations significantly increased and decreased, respectively, in the Lactobacillus group. The use of L casei rhamnosus was as effective as neomycin in preventing cholangitis in patients with BA who underwent Kasai operation, and therefore could be considered as a potential alternative prophylactic regimen.

Can gravity waves significantly impact PSC occurrence in the Antarctic?

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R. M. Woollands

2009-11-01

Full Text Available A combination of POAM III aerosol extinction and CHAMP RO temperature measurements are used to examine the role of atmospheric gravity waves in the formation of Antarctic Polar Stratospheric Clouds (PSCs. POAM III aerosol extinction observations and quality flag information are used to identify Polar Stratospheric Clouds using an unsupervised clustering algorithm.

A PSC proxy, derived by thresholding Met Office temperature analyses with the PSC Type Ia formation temperature (T_NAT, shows general agreement with the results of the POAM III analysis. However, in June the POAM III observations of PSC are more abundant than expected from temperature threshold crossings in five out of the eight years examined. In addition, September and October PSC identified using temperature thresholding is often significantly higher than that derived from POAM III; this observation probably being due to dehydration and denitrification. Comparison of the Met Office temperature analyses with corresponding CHAMP observations also suggests a small warm bias in the Met Office data in June. However, this bias cannot fully explain the differences observed.

Analysis of CHAMP data indicates that temperature perturbations associated with gravity waves may partially explain the enhanced PSC incidence observed in June (relative to the Met Office analyses. For this month, approximately 40% of the temperature threshold crossings observed using CHAMP RO data are associated with small-scale perturbations. Examination of the distribution of temperatures relative to T_NAT shows a large proportion of June data to be close to this threshold, potentially enhancing the importance of gravity wave induced temperature perturbations. Inspection of the longitudinal structure of PSC occurrence in June 2005 also shows that regions of enhancement are geographically associated with the Antarctic Peninsula; a known mountain wave "hotspot". The

Recurrent acute pancreatitis and cholangitis in a patient with autosomal dominant polycystic kidney disease

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Kambiz Yazdanpanah

2013-01-01

Full Text Available Autosomal dominant polycystic kidney disease (ADPKD is an inherited disorder associated with multiple cyst formation in the different organs. Development of pancreatic cyst in ADPKD is often asymptomatic and is associated with no complication. A 38-year-old man with ADPKD was presented with six episodes of acute pancreatitis and two episodes of cholangitis in a period of 12 months. Various imaging studies revealed multiple renal, hepatic and pancreatic cysts, mild ectasia of pancreatic duct, dilation of biliary system and absence of biliary stone. He was managed with conservative treatment for each attack. ADPKD should be considered as a potential risk factor for recurrent acute and/or chronic pancreatitis and cholangitis.

Urgent endoscopic retrograde cholangiopancreatography is not superior to early ERCP in acute biliary pancreatitis with biliary obstruction without cholangitis.

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Lee, Hee Seung; Chung, Moon Jae; Park, Jeong Youp; Bang, Seungmin; Park, Seung Woo; Song, Si Young; Chung, Jae Bock

2018-01-01

Acute pancreatitis is a common diagnosis worldwide, with gallstone disease being the most prevalent cause (50%). The American College of Gastroenterology recommends urgent endoscopic retrograde cholangiopancreatography (ERCP) (within 24 h) for patients with biliary pancreatitis accompanied by cholangitis. Most international guidelines recommend that ERCP be performed within 72 h in patients with biliary pancreatitis and a bile duct obstruction without cholangitis, but the optimal timing for endoscopy is controversial. We investigated the optimal timing for ERCP in patients with biliary pancreatitis and a bile duct obstruction without cholangitis, and whether performing endoscopy within 24 h is superior to performing it after 24 h. We analyzed the clinical data of 505 patients with newly diagnosed acute pancreatitis, from January 1, 2005 to December 31, 2014. We divided the patients into two groups according to the timing of ERCP: pancreatitis and a bile duct obstruction without cholangitis. The mean age of the patients was 55 years (range: 26-90 years). Bile duct stones and biliary sludge were identified on endoscopy in 45 (61.6%) and 11 (15.0%) patients, respectively. The timing of ERCP within 72 h was not associated with ERCP-related complications (P = 0.113), and the total length of hospital stay was not different between urgent and early ERCP (5.9 vs. 5.7 days, P = 0.174). No significant differences were found in total length of hospitalization or procedural-related complications, in patients with biliary pancreatitis and a bile duct obstruction without cholangitis, according to the timing of ERCP (< 24 h vs. 24-72 h).

New developments in the treatment of primary biliary cholangitis – role of obeticholic acid

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Jhaveri MA

2017-08-01

Full Text Available Manan A Jhaveri, Kris V Kowdley Liver Care Network, Swedish Medical Center, Seattle, WA, USA Abstract: Primary biliary cholangitis (PBC is a chronic autoimmune cholestatic liver disease that predominantly affects women in early to middle age. It is typically associated with autoantibodies to mitochondrial antigens and results in immune-mediated destruction of small and medium-sized intrahepatic bile ducts leading to cholestasis, hepatic fibrosis and may progress to cirrhosis or hepatic failure and, in some cases, hepatocellular carcinoma. The clinical presentation and the natural history of PBC have improved over the years due to recognition of earlier widespread use of ursodeoxycholic acid (UDCA; about one-third of patients show suboptimal biochemical response to UDCA with poor prognosis. Until recently, UDCA was the only US Food and Drug Administration approved agent for this disease for more than two decades; obeticholic acid was approved in 2016 for treatment of patients with PBC with a suboptimal response or intolerance to UDCA. Currently, liver transplantation is the most effective treatment modality for PBC patients with end-stage liver disease. This review will focus on the recent advances in therapy of primary biliary cholangitis, with emphasis on obeticholic acid. Keywords: primary biliary cholangitis, obeticholic acid, ursodeoxycholic acid

Management of acute cholangitis as a result of occlusion from a self-expandable metallic stent in patients with malignant distal and hilar biliary obstructions.

Science.gov (United States)

Shiomi, Hideyuki; Matsumoto, Kazuya; Isayama, Hiroyuki

2017-04-01

Acute cholangitis as a result of common bile duct stones can be managed; however, cholangitis caused by occlusion with a biliary self-expandable metallic stent (SEMS) in patients with an unresectable malignant biliary obstruction has not been fully discussed. The acute cholangitis clinical guidelines (Tokyo Guidelines 2013) recommend following the same procedure as that used for cholangitis; however, the patient's condition, including performance status, tumor extension or staging, and prognosis must be considered. Most physicians manage cholangitis from a SEMS occlusion using a two-step procedure. They insert endoscopic drainage with a plastic stent or insert a nasobiliary drainage tube, which does not exacerbate sepsis. Addition or replacement of a biliary SEMS is required in many cases depending on the cause of the occlusion. Tumor ingrowth through the stent mesh is common in uncovered SEMS and requires placement of another stent in an in-stent method. However, covered SEMS tends to be occluded by sludge, so it must be replaced because of the bacterial biofilm that forms on the covering membrane. The location of the biliary stricture (hilar or distal) should also be considered. Strategies for managing cholangitis as a result of occlusion by a biliary SEMS remain controversial, so prospective clinical trials are needed. © 2017 The Authors. Digestive Endoscopy © 2017 Japan Gastroenterological Endoscopy Society.

Falciform ligament abscess from left sided portal pyaemia following malignant obstructive cholangitis

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Warren Leigh R

2012-12-01

Full Text Available Abstract Abscess formation of the falciform ligament is incredibly rare and perplexing when encountered for the first time. It is reported to occur in the setting of cholecystitis and cholangitis, but the pathophysiology is poorly understood. In this case report, we present a 73-year-old man with falciform ligament abscess following cholangitis from an obstructive ampullary carcinoma. The patient was referred to the Royal Adelaide Hospital from a country hospital, with progressive jaundice, anorexia and nausea. Prior to transfer, he deteriorated with cholangitis, dehydration and renal failure. On arrival, his abdomen was exquisitely tender along the course of the falciform ligament. His blood tests revealed an elevated white cell count of 14.9 x 103/μl, bilirubin of 291μmol/l and creatinine of 347 μmol/l. His CA 19-9 was markedly elevated at 35,000 kU/l. A non-contrast computed tomography (CT demonstrated gross biliary dilatation and a collection tracking along the path of the falciform ligament to the umbilicus. The patient was commenced on intravenous antibiotics and underwent an urgent endoscopic retrograde cholangiopancreatogram (ERCP with sphincterotomy and biliary stent drainage. Cholangiogram revealed a grossly dilated biliary tree, with abrupt transition at the ampulla, which on biopsy confirmed an obstructing ampullary carcinoma. Following ERCP, his jaundice and abdominal tenderness resolved. He was optimized over 4 weeks for an elective pancreaticoduodenectomy. At operation, we found abscess transformation of the falciform ligament. Copious amounts of pus and necrotic material was drained. Part of the round ligament was resected along the undersurface of the liver. Histology showed that there was prominent histiocytic inflammation with granular acellular eosinophilic components. The patient recovered slowly but uneventfully. A contrast CT scan undertaken 2 weeks post-operatively (approximately 7 weeks after the initial CT revealed

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

Science.gov (United States)

Serafimidis, Costas; Katsarolis, Ioannis; Vernadakis, Spyros; Rallis, George; Giannopoulos, George; Legakis, Nikolaos; Peros, George

2006-02-13

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon) is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

File list: Unc.PSC.50.Unclassified.AllCell [Chip-atlas[Archive

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Full Text Available Unc.PSC.50.Unclassified.AllCell mm9 Unclassified Unclassified Pluripotent stem cell...73,SRX355578 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.50.Unclassified.AllCell.bed ...

File list: Unc.PSC.05.Unclassified.AllCell [Chip-atlas[Archive

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Full Text Available Unc.PSC.05.Unclassified.AllCell mm9 Unclassified Unclassified Pluripotent stem cell...8,SRX1034724 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.05.Unclassified.AllCell.bed ...

Air contamination in the sclerosing foam for the treatment of varicose veins.

Science.gov (United States)

de Franciscis, S; Nobile, Cga; Larosa, E; Montemurro, R; Serra, R

2016-03-01

Fluids and drugs formulated for intravenous infusion may potentially promote the growth of microorganisms that can cause infections. The aim of this study is to test the sterility of sclerosing foam. Polidocanol was used for the production of the foam. The Tessari method was used in order to generate the foam. The preparation was carried out both in the operating theater and in an outpatient room. A validation test with microorganisms was also performed. The measurements showed no evident growth of microorganisms and in the validation tests the foam appeared to even display bacteriostatic and/or bactericide properties. Sclerosing foam seems to be safe from a microbiological point of view. © The Author(s) 2014.

Effects of Melittin Treatment in Cholangitis and Biliary Fibrosis in a Model of Xenobiotic-Induced Cholestasis in Mice

Directory of Open Access Journals (Sweden)

Kyung-Hyun Kim

2015-08-01

Full Text Available Cholangiopathy is a chronic immune-mediated disease of the liver, which is characterized by cholangitis, ductular reaction and biliary-type hepatic fibrosis. There is no proven medical therapy that changes the course of the disease. In previous studies, melittin was known for attenuation of hepatic injury, inflammation and hepatic fibrosis. This study investigated whether melittin provides inhibition on cholangitis and biliary fibrosis in vivo. Feeding 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC to mice is a well-established animal model to study cholangitis and biliary fibrosis. To investigate the effects of melittin on cholangiopathy, mice were fed with a 0.1% DDC-containing diet with or without melittin treatment for four weeks. Liver morphology, serum markers of liver injury, cholestasis markers for inflammation of liver, the degree of ductular reaction and the degree of liver fibrosis were compared between with or without melittin treatment DDC-fed mice. DDC feeding led to increased serum markers of hepatic injury, ductular reaction, induction of pro-inflammatory cytokines and biliary fibrosis. Interestingly, melittin treatment attenuated hepatic function markers, ductular reaction, the reactive phenotype of cholangiocytes and cholangitis and biliary fibrosis. Our data suggest that melittin treatment can be protective against chronic cholestatic disease in DDC-fed mice. Further studies on the anti-inflammatory capacity of melittin are warranted for targeted therapy in cholangiopathy.

Sclerosing adenosis: mammographic and ultrasonographic findings with clinical and histopathological correlation

International Nuclear Information System (INIS)

Guenhan-Bilgen, Isil; Memis, Aysenur; Uestuen, Esin Emin; Oezdemir, Necmettin; Erhan, Yildiz

2002-01-01

Objective: To evaluate the mammographic and ultrasonographic findings of sclerosing adenosis, a relatively uncommon entity which may sometimes mimic carcinoma. Materials and methods: A retrospective review of the records of 33700 women, who have undergone mammographic examination at our institution between January 1985 and July 2001 revealed 43 histopathologically proven sclerosing adenosis. The history, physical examination, mammographic and ultrasonographic findings were analyzed in all patients. In 30 patients, the nonpalpable lesions were preoperatively localized by the needle-hookwire system under the guidance of mammography (n=22) or ultrasonography (US) (n=8). Radiological features were correlated with histopathological findings. Results: The age of the patients varied between 32 and 55 years (mean, 43.7 years). Only two patients had a family history of breast cancer. In six patients, the presenting complaint was mastalgia. A palpable mass was present in 13 cases. The mammographic findings were; microcalcifications in 24 (55.8%) (clustered in 22, diffuse in two), mass in five (11.6%), asymmetric focal density in three (6.9%), and focal architectural distortion in three (6.9%) patients. Four of the masses were irregularly contoured, while one was well-circumscribed. On US, focal acoustic shadowing without a mass configuration was noted in the three patients who showed asymmetrical focal density on mammography. In eight patients, who showed normal mammograms, a solid mass was detected on US. Two masses had discrete well-circumscribed oval or lobulated contours, while six showed microlobulation and irregularity. In one case, the irregularly contoured mass had marked posterior acoustic shadowing. Two of the three patients, who had focal architectural distortion on mammograms, had an irregularly contoured solid mass, while the third presented as focal acoustic shadowing without a mass configuration. Conclusion: Sclerosing adenosis mostly presents as a nonpalpable

Solid Organ Transplantation in Patients with Inflammatory Bowel Diseases (IBD: Analysis of Transplantation Outcome and IBD Activity in a Large Single Center Cohort.

Directory of Open Access Journals (Sweden)

Fabian Schnitzler

Full Text Available Currently, limited data of the outcome of inflammatory bowel disease (IBD in patients after solid organ transplantation (SOT are available. We aimed to analyze effects of SOT on the IBD course in a large IBD patient cohort.Clinical data from 1537 IBD patients were analyzed for patients who underwent SOT (n = 31 between July 2002 and May 2014. Sub-analyses included SOT outcome parameters, IBD activity before and after SOT, and efficacy of IBD treatment.4.74% of patients with ulcerative colitis (UC and 0.84% of patients with Crohn's disease (CD underwent SOT (p = 2.69 x 10(-6, UC vs. CD. 77.4% of patients with SOT underwent liver transplantation (LTx with tacrolimus-based immunosuppressive therapy after SOT. All LTx were due to primary sclerosing cholangitis (PSC or PSC overlap syndromes. Six patients (19.4% required renal transplantation and one patient (3.2% heart transplantation. A survival rate of 83.9% after a median follow-up period of 103 months was observed. Before SOT, 65.0% of patients were in clinical remission and 5 patients received immunosuppressive therapy (16.1%. After SOT, 61.0% of patients were in remission (p = 1.00 vs. before SOT and 29.0% required IBD-specific immunosuppressive or anti-TNF therapy (p = 0.54 vs. before SOT. 42.9% of patients with worsening of IBD after SOT were at higher risk of needing steroid therapy for increased IBD activity (p = 0.03; relative risk (RR: 10.29; 95% CI 1.26-84.06. Four patients (13.0% needed anti-TNF therapy after SOT (response rate 75%.SOT was more common in UC patients due to the higher prevalence of PSC-related liver cirrhosis in UC. Despite mainly tacrolimus-based immunosuppressive regimens, outcome of SOT and IBD was excellent in this cohort. In this SOT cohort, concomitant immunosuppressive therapy due to IBD was well tolerated.

Sexually localized expression of pseudo-self compatibility (PSC) in Petunia X hybrida Hort : 2. Stylar inactivation.

Science.gov (United States)

Dana, M N; Ascher, P D

1986-01-01

A previously identified S-linked stylar-inactivation PSC factor (Flaschenriem and Ascher 1979b) was studied for its location relative to S. Plants exhibiting complete stylar-inactivation PSC were those with higher multigenic PSC background level than plants with only S-linked partial stylar-inactivation PSC. A pollen-mediated pseudo-self compatibility (PMPSC) adjustment factor was offered as a device to focus on stylar-inactivation PSC by removing some male origin, multigenic PSC. The stylar inactivation factor was not tightly linked to S but affected expression of only the allele to which it was linked. A three part interacting association of genetic material governing self incompatibility (SI) is proposed. The parts of S are the SI identity gene, S-specific PSC genes and, finally, PSC genes which are not S-specific in action. The complete association is termed the SI-complex.

Less promising results with sclerosing ethoxysclerol injections for midportion achilles tendinopathy: a retrospective study.

Science.gov (United States)

van Sterkenburg, Maayke N; de Jonge, Milko C; Sierevelt, Inger N; van Dijk, C Niek

2010-11-01

Local injections of the sclerosing substance polidocanol (Ethoxysclerol) have shown good clinical results in patients with chronic midportion Achilles tendinopathy. After training by the inventors of the technique, sclerosing Ethoxysclerol injections were applied on a group of patients in our center. Sclerosing Ethoxysclerol injections will yield good results in the majority of patients. Case series; Level of evidence, 4. In 113 patients (140 tendons) with Achilles tendinopathy, we identified 62 patients (70 tendons) showing neovascularization on color Doppler ultrasound. Fifty-three Achilles tendons (48 patients) were treated with sclerosing Ethoxysclerol injections, with intervals of 6 weeks and a maximum of 5 sessions. Treatment was completed when neovascularization or pain had disappeared, or when there was no positive treatment effect after 3 to 4 sessions. Forty-eight patients (20 women and 28 men) with a median age of 45 years, (range, 33-68 years) were treated. Median symptom duration was 23 months (range, 3-300 months). Fifty-three tendons were treated with a median of 3 sessions of Ethoxysclerol injections. Six weeks after the last injection, 35% of patients had no complaints, 9% had minimal symptoms, 42% were the same, and 14% had more complaints. Women were 3.8 times (95% confidence interval: 1.1-13.8) more likely to have unsatisfactory outcome than men. Pain correlated positively with neovessels on ultrasound (P < .01). At 2.7 to 5.1 year follow-up, 53% had received additional (surgical/conservative) treatment; 3 of these patients (7.5%) still had complaints of Achilles tendinopathy. In 6 patients, complaints that were still present 6 weeks after treatment had resolved spontaneously by final follow-up. Our study did not confirm the high beneficial value of sclerosing neovascularization in patients with midportion Achilles tendinopathy. Despite the retrospective design of our study, we consider it important to stress that injection of Ethoxysclerol may

CT guided puncture aspiration and sclerosing treatment of ovary cyst

International Nuclear Information System (INIS)

Peng Yongjun; Du Xiumei; Yuan Jinrong; Chen Chanqing

2007-01-01

Objective: To analyze the method and the curative effect with CT guided percutaneous puncture aspiration and sclerosing treatment of ovary cyst. Method: 22 ovary cysts in 22 patients were treated with percutaneous puncture aspiration and underwent repeated sclerotherapy with 99.7% ethanol injection. Among the 22 patients, 18 patients had solitary ovary cyst and was aspirated with an 18-22G gauge aspiration needle. The amount of aspirated fluid varied from 30ml-500ml and 25%-30% cyst volume was replaced by appropriate ethanol Post treatment follow-up were achieved every 3 months. Results All the Punctures were successfully completed. During the 3 months to one year follow-up, 16 ovary cyst disappeared, 6 cysts were small over 50%, without main complication. Conclusion CT guided percutaneous puncture aspiration and sclerosing treatment of ovary cyst is a treatment of choice because of its safety, low complication, and high curative effect. (authors)

Development of Embedded EM Sensors for Estimating Tensile Forces of PSC Girder Bridges

Science.gov (United States)

Kim, Ju-Won; Lee, Chaggil; Park, Seunghee

2017-01-01

The tensile force of pre-stressed concrete (PSC) girders is the most important factor for managing the stability of PSC bridges. The tensile force is induced using pre-stressing (PS) tendons of a PSC girder. Because the PS tendons are located inside of the PSC girder, the tensile force cannot be measured after construction using conventional NDT (non-destructive testing) methods. To monitor the induced tensile force of a PSC girder, an embedded EM (elasto-magnetic) sensor was proposed in this study. The PS tendons are made of carbon steel, a ferromagnetic material. The magnetic properties of the ferromagnetic specimen are changed according to the induced magnetic field, temperature, and induced stress. Thus, the tensile force of PS tendons can be estimated by measuring their magnetic properties. The EM sensor can measure the magnetic properties of ferromagnetic materials in the form of a B (magnetic density)-H (magnetic force) loop. To measure the B-H loop of a PS tendon in a PSC girder, the EM sensor should be embedded into the PSC girder. The proposed embedded EM sensor can be embedded into a PSC girder as a sheath joint by designing screw threads to connect with the sheath. To confirm the proposed embedded EM sensors, the experimental study was performed using a down-scaled PSC girder model. Two specimens were constructed with embedded EM sensors, and three sensors were installed in each specimen. The embedded EM sensor could measure the B-H loop of PS tendons even if it was located inside concrete, and the area of the B-H loop was proportionally decreased according to the increase in tensile force. According to the results, the proposed method can be used to estimate the tensile force of unrevealed PS tendons. PMID:28867790

Cholangiographic evaluation of bile duct carcinoma

International Nuclear Information System (INIS)

Nichols, D.A.; MacCarty, R.L.; Gaffey, T.A.

1983-01-01

Cholangiograms and clinical histories of 82 patients with biopsy-proved bile duct carcinoma were reviewed. The carcinomas were classified according to morphologic findings and clinical outcome. Ulcerative colitis and antecedent inflammatory disease of the biliary tree, particularly primary sclerosing cholangitis, seem to predispose to the development of bile duct carcinoma. Focal stenotic lesions were the most common morphologic type (62/82). Polypoid carcinomas and diffuse sclerosing carcinomas were less common and of about equal frequency. Prognosis was best for patients with polypoid carcinomas and worst for those with diffuse sclerosing carcinomas. In 69 cases (84%), the tumors involved the intrahepatic or proximal extrahepatic ducts, makin curative resection difficult or impossible. Patients with carcinomas limited to the more distal extrahepatic bile ducts had a longer average survival and a higher probability of surgical cure. Proper management of patients with bile duct carcinoma requires a complete and accurate cholangiographic evaluation of the morphology, location, and extent of the disease

Recurrent pyogenic cholangitis in Asian immigrants: use of ultrasonography, computed tomography, and cholangiography

International Nuclear Information System (INIS)

Federle, M.P.; Cello, J.P.; Laing, F.C.; Jeffery, R.B. Jr.

1982-01-01

Five cases of recurrent pyogenic cholangitis (RPC) were studied by ultrasonography, computed tomography (CT), and cholangiography. All patients were recent immigrants from the Orient or Indonesia and had had recurrent attacks of cholangitis for many years. The bile was infected by E. coli and the biliary ducts were dilated; in addition, extrahepatic bile-pigment calculi we represent in all 5 and intrahepatic calculi in 4. Abdominal ultrasound usually failed to demonstrate duct calculi and extrahepatic dilatation due to the soft, mud-like consistency of the stones. CT was successful in showing the calculi and the full extent of dilatation. The authors conclude that preoperative diagnosis of RPC is best achieved by awareness of the characteristic clinical presentation and the findings on abdominal CT. Preoperative cholangiography provides excellent detail, but poses the danger of biliary sepsis requiring antibiotics

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon

Directory of Open Access Journals (Sweden)

Legakis Nikolaos

2006-02-01

Full Text Available Abstract Background Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. Case presentation A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Conclusion Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

File list: Oth.PSC.05.Myc.AllCell [Chip-atlas[Archive

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MiRNA-506 promotes primary biliary cholangitis-like features in cholangiocytes and immune activation

NARCIS (Netherlands)

Erice, Oihane; Munoz-Garrido, Patricia; Vaquero, Javier; Perugorria, Maria J.; Fernandez-Barrena, Maite G.; Saez, Elena; Santos-Laso, Alvaro; Arbelaiz, Ander; Jimenez-Agüero, Raul; Fernandez-Irigoyen, Joaquin; Santamaria, Enrique; Torrano, Verónica; Carracedo, Arkaitz; Ananthanarayanan, Meenakshisundaram; Marzioni, Marco; Prieto, Jesus; Beuers, Ulrich; Oude Elferink, Ronald P.; LaRusso, Nicholas F.; Bujanda, Luis; Marin, Jose J. G.; Banales, Jesus M.

2017-01-01

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

PSC/PSI power supply control prototype based on RTEMS

International Nuclear Information System (INIS)

Shi Haoli; Wang Chunhong; Tang Jingyu

2010-01-01

A PSC/PSI power supply control prototype was developed by using an open-source real-time operating system RTEMS and PSC/PSI power supply controller developed by BNL. The structure of the prototype, development procedures as well as testing result with a power supply of a corrector magnet were described. It can switch on/off the power supply, ramp up/down the current, and monitor the real-time states of the power supply. (authors)

CHANDRA X-RAY DETECTION OF THE ENIGMATIC FIELD STAR BP Psc

International Nuclear Information System (INIS)

Kastner, Joel H.; Montez, Rodolfo; Rodriguez, David; Zuckerman, B.; Perrin, Marshall D.; Grosso, Nicolas; Forveille, Thierry; Graham, James R.

2010-01-01

BP Psc is a remarkable emission-line field star that is orbited by a dusty disk and drives a parsec-scale system of jets. We report the detection by the Chandra X-ray Observatory of a weak X-ray point source coincident with the centroids of optical/IR and submillimeter continuum emission at BP Psc. As the star's photosphere is obscured throughout the visible and near-infrared, the Chandra X-ray source likely represents the first detection of BP Psc itself. The X-rays most likely originate with magnetic activity at BP Psc and hence can be attributed either to a stellar corona or to star-disk interactions. The log of the ratio of X-ray to bolometric luminosity, log(L X /L bol ), lies in the range -5.8 to -4.2. This is smaller than log(L X /L bol ) ratios typical of low-mass, pre-main sequence stars, but is well within the log(L X /L bol ) range observed for rapidly rotating (FK Com-type) G giant stars. Hence, the Chandra results favor an exotic model wherein the disk/jet system of BP Psc is the result of its very recently engulfing a companion star or a giant planet, as the primary star ascended the giant branch.

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MRI in subacute sclerosing panencephalitis

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Tuncay, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Akman-Demir, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Goekyigit, A. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Eraksoy, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Barlas, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Tolun, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Guersoy, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey)

1996-10-01

Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease. (orig.). With 4 figs., 1 tab.

MRI in subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Tuncay, R.; Akman-Demir, G.; Goekyigit, A.; Eraksoy, M.; Barlas, M.; Tolun, R.; Guersoy, G.

1996-01-01

Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease. (orig.). With 4 figs., 1 tab

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis: A retrospective cohort study of 39 IAC patients.

Science.gov (United States)

Xiao, Jianchun; Xu, Peiran; Li, Binglu; Hong, Tao; Liu, Wei; He, Xiaodong; Zheng, Chaoji; Zhao, Yupei

2018-02-01

Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC. The majority of patients were men (82%). Clinical features on presentation included obstructive jaundice in 26 patients (67%) and abdominal pain in 20 (51%). Positive IgG4 immunostaining was seen in 27 patients. The median serum IgG4 level before treatment was 769.4 mg/dL (range, 309.1-1229.7 mg/dL). After the steroid therapy, the median serum IgG4 level in 23 patients was 247.0 mg/dL (range, 139.0-355.0 mg/dL). Cholangiograms were available in 36 (92%) patients. Stenosis of the lower part of the common bile duct was found in 26 of 39 patients. Stenosis was diffusely distributed in the intra- and extrahepatic bile ducts in 14 of 39 patients. Additionally, strictures of the bile duct were detected in the hilar hepatic lesions in 27 of 39 patients. AIP was the most frequent comorbidity (35/39 in this study) of IAC. Other affected organs included eyes (n = 6), salivary glands (sialadenitis, n = 10), lymph nodes (mediastinal and axillary, n = 3), kidneys (n = 2), and the retroperitoneum (retroperitoneal fibrosis, n = 2).Regarding treatment, 29 patients were treated with steroids, of whom one underwent pancreatoduodenectomy, and one underwent choledochojejunostomy. Eight patients were treated with biliary stents. The remaining 19 patients took prednisolone alone. Eight patients achieved spontaneous resolution. Four patients with suspected pancreatic cancer or cholangiocarcinoma underwent surgery, including 2

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An immunoglobulin G-4 related sclerosing disease of the small bowel: CT and small bowel series findings

International Nuclear Information System (INIS)

Ko, Young Hwan; Hwang, Dae Hyun; Min, Seon Jeong; Woo, Ji Young; Kim, Jeong Won; Hong, Hye Sook; Yang, Ik; Lee, Yul

2013-01-01

Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.

[Treatment of autoimmune hepatic diseases].

Science.gov (United States)

Bueverov, A O

2004-01-01

The immunosuppresive drugs, primarily glucocorticosteroids, serve as the basis for the pathogenetic treatment of autoimmune diseases of the liver. In autoimmune hepatitis, immunosuppressive therapy induces and maintains persistent remission in most patients while in primary biliary cirrhosis and primary sclerosing cholangitis, its capacities are substantially limited. Ursodeoxycholic acid is used as the basic drug in predominantly occurring intrahepatic cholestasis. The treatment of cross autoimmune syndromes generally requires the choice of a combination of drugs.

An 111In-Pentetreotide Positive Sclerosing Pneumocytoma.

Science.gov (United States)

Savelli, Giordano; Bnà, Claudio; Zambelli, Claudia; Illuminati, Sonia; Bonello, Luke

2017-04-01

A 43-year-old woman had an incidental lung mass identified on shoulder x-ray performed for pain. Contrast-enhanced CT showed a 38-mm mass in the medial segment of the right middle lobe, with features suggestive of carcinoid tumor. A In-pentetreotide scan showed intense uptake; furthermore, fine needle aspiration biopsy yielded neuroendocrine cells confirming the carcinoid hypothesis. However, definitive surgical histology showed a sclerosing pneumocytoma. This could potentially suggest that such rare tumors, with metastatic potential, could respond to somatostatin analogue treatment.

File list: Unc.PSC.20.AllAg.ZHBTc4 [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Unc.PSC.20.AllAg.ZHBTc4 mm9 Unclassified Pluripotent stem cell ZHBTc4 SRX567344,SRX...567345 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.20.AllAg.ZHBTc4.bed ...

Myosin light chain 2-based selection of human iPSC-derived early ventricular cardiac myocytes.

Science.gov (United States)

Bizy, Alexandra; Guerrero-Serna, Guadalupe; Hu, Bin; Ponce-Balbuena, Daniela; Willis, B Cicero; Zarzoso, Manuel; Ramirez, Rafael J; Sener, Michelle F; Mundada, Lakshmi V; Klos, Matthew; Devaney, Eric J; Vikstrom, Karen L; Herron, Todd J; Jalife, José

2013-11-01

Applications of human induced pluripotent stem cell derived-cardiac myocytes (hiPSC-CMs) would be strengthened by the ability to generate specific cardiac myocyte (CM) lineages. However, purification of lineage-specific hiPSC-CMs is limited by the lack of cell marking techniques. Here, we have developed an iPSC-CM marking system using recombinant adenoviral reporter constructs with atrial- or ventricular-specific myosin light chain-2 (MLC-2) promoters. MLC-2a and MLC-2v selected hiPSC-CMs were purified by fluorescence-activated cell sorting and their biochemical and electrophysiological phenotypes analyzed. We demonstrate that the phenotype of both populations remained stable in culture and they expressed the expected sarcomeric proteins, gap junction proteins and chamber-specific transcription factors. Compared to MLC-2a cells, MLC-2v selected CMs had larger action potential amplitudes and durations. In addition, by immunofluorescence, we showed that MLC-2 isoform expression can be used to enrich hiPSC-CM consistent with early atrial and ventricular myocyte lineages. However, only the ventricular myosin light chain-2 promoter was able to purify a highly homogeneous population of iPSC-CMs. Using this approach, it is now possible to develop ventricular-specific disease models using iPSC-CMs while atrial-specific iPSC-CM cultures may require additional chamber-specific markers. © 2013.

Primary (Poorly Differentiated Sclerosing Liposarcoma of Temporal Region. An Uncommon Tumor in a Rare Site: A Case Report

Directory of Open Access Journals (Sweden)

Anuradha CK Rao

2015-02-01

Full Text Available Liposarcoma (LS in the head and neck region is a rare tumor. The sclerosing variant of LS is a subtype of well-differentiated LS characterized by areas of conventional LS admixed with hypocellular areas of stromal sclerosis that show atypical lipomatous cells. The (poorly differentiated sclerosing LS, on the other hand, is more cellular with atypical, pleomorphic and often bizarre giant tumor cells admixed with atypical lipoblasts. We report a case of poorly differentiated sclerosing LS of temporal region in a 49-year-old man. Radiologically, the tumor was dumbbell shaped with intra and extra cranial extension. In this case, we discuss the clinico-radiological and pathological findings of an unusual tumor in a rare location. [J Interdiscipl Histopathol 2015; 3(1.000: 33-35

File list: Oth.PSC.05.Biotin.AllCell [Chip-atlas[Archive

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File list: Oth.PSC.20.Biotin.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.20.Biotin.AllCell mm9 TFs and others Biotin Pluripotent stem cell SRX477548...44,SRX115145,SRX984568,SRX172568,SRX218274,SRX327702,SRX312228,SRX213794,SRX327701 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.20.Biotin.AllCell.bed ...

File list: Oth.PSC.50.Biotin.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.10.Biotin.AllCell mm9 TFs and others Biotin Pluripotent stem cell SRX218273...69,SRX984573,SRX115147,SRX327702,SRX984572,SRX984568,SRX115145,SRX172568,SRX218274 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.10.Biotin.AllCell.bed ...

File list: Unc.PSC.20.AllAg.EpiLC [Chip-atlas[Archive

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File list: Unc.PSC.50.AllAg.EpiLC [Chip-atlas[Archive

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Full Text Available Unc.PSC.10.AllAg.EpiLC mm9 Unclassified Pluripotent stem cell EpiLC SRX1074910,SRX1...074907 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.10.AllAg.EpiLC.bed ...

File list: Unc.PSC.05.AllAg.EpiLC [Chip-atlas[Archive

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Full Text Available Unc.PSC.05.AllAg.EpiLC mm9 Unclassified Pluripotent stem cell EpiLC SRX1074910,SRX1...074907 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.05.AllAg.EpiLC.bed ...

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File list: Oth.PSC.10.Etv2.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.10.Etv2.AllCell mm9 TFs and others Etv2 Pluripotent stem cell SRX652259,SRX...652260 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.10.Etv2.AllCell.bed ...

Elevation of serum IgG4 in Western patients with autoimmune sclerosing pancreatocholangitis: a word of caution.

Science.gov (United States)

Hochwald, Steven N; Hemming, Alan W; Draganov, Peter; Vogel, Stephen B; Dixon, Lisa R; Grobmyer, Stephen R

2008-04-01

Autoimmune pancreatocholangitis is characterized by sclerosing inflammation of the biliary tree or pancreatic duct and can mimic pancreaticobiliary malignancy. Serum immunoglobin (Ig) G4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from pancreatic malignancy in the Japanese population; however, its significance in the Western population has not been well studied. We report a retrospective analysis of 7 consecutive patients with autoimmune pancreatocholangitis and compare them to 23 patients with pancreatic malignancy. Clinical presentation, diagnostic tests, and preoperative IgG4 levels were reviewed in all patients. Presence of autoimmune pancreatocholangitis or pancreatic malignancy was determined by pathologic analysis in all patients and reviewed by a single pathologist. In all patients, autoimmune pancreatocholangitis manifested in a similar fashion to pancreatic malignancy. Median IgG4 levels were far lower in pancreatic cancer patients with localized, resectable disease (24 mg/dL), locally advanced disease (24 mg/dL), and metastatic disease (28 mg/dL) as compared with patients with autoimmune pancreatocholangitis (142 mg/dL, P 100 mg/dL. In contrast, all patients with autoimmune pancreatitis or cholangitis had levels >100 mg/dL. However, in five of these seven patients, IgG4 levels were below the upper limits of normal. Autoimmune pancreatocholangitis mimics pancreatobiliary malignancy. Serum IgG4 values seem to be helpful in distinguishing autoimmune pancreatocholangitis from malignancy in the Western population. However, absolute values seem to be lower in the United States compared with Japan. The upper limit of normal as reported in laboratories in the United States may not be useful in identifying abnormally high IgG4 values. A new upper limit of normal may need to be defined because IgG subclass determinations are being used more frequently in Western patients with biliary obstruction.

File list: Unc.PSC.50.AllAg.EpiSC [Chip-atlas[Archive

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Full Text Available Unc.PSC.50.AllAg.EpiSC mm9 Unclassified Pluripotent stem cell EpiSC SRX1074917,SRX1...074905,SRX1074908 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.50.AllAg.EpiSC.bed ...

File list: Unc.PSC.05.AllAg.EpiSC [Chip-atlas[Archive

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Full Text Available Unc.PSC.05.AllAg.EpiSC mm9 Unclassified Pluripotent stem cell EpiSC SRX1074917,SRX1...074908,SRX1074905 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.05.AllAg.EpiSC.bed ...

File list: Unc.PSC.20.AllAg.EpiSC [Chip-atlas[Archive

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Full Text Available Unc.PSC.20.AllAg.EpiSC mm9 Unclassified Pluripotent stem cell EpiSC SRX1074917,SRX1...074908,SRX1074905 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.20.AllAg.EpiSC.bed ...

File list: Oth.PSC.05.TEAD4.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.05.TEAD4.AllCell hg19 TFs and others TEAD4 Pluripotent stem cell SRX190301,...SRX378124,SRX378125 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Oth.PSC.05.TEAD4.AllCell.bed ...

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Full Text Available Oth.PSC.10.TEAD4.AllCell hg19 TFs and others TEAD4 Pluripotent stem cell SRX190301,...SRX378124,SRX378125 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Oth.PSC.10.TEAD4.AllCell.bed ...

File list: Unc.PSC.50.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available Unc.PSC.50.AllAg.Embryoid_Bodies mm9 Unclassified Pluripotent stem cell Embryoid Bodie...353849,SRX353851,SRX353852,SRX353850 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.50.AllAg.Embryoid_Bodies.bed ...

File list: His.PSC.50.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available His.PSC.50.AllAg.Embryoid_Bodies mm9 Histone Pluripotent stem cell Embryoid Bodies ...SRX385956,SRX385955,SRX385958,SRX385957 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/His.PSC.50.AllAg.Embryoid_Bodies.bed ...

File list: His.PSC.05.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available His.PSC.05.AllAg.Embryoid_Bodies mm9 Histone Pluripotent stem cell Embryoid Bodies ...SRX385956,SRX385955,SRX385958,SRX385957 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/His.PSC.05.AllAg.Embryoid_Bodies.bed ...

File list: His.PSC.20.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available His.PSC.20.AllAg.Embryoid_Bodies mm9 Histone Pluripotent stem cell Embryoid Bodies ...SRX385955,SRX385956,SRX385958,SRX385957 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/His.PSC.20.AllAg.Embryoid_Bodies.bed ...

File list: Unc.PSC.10.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available Unc.PSC.10.AllAg.Embryoid_Bodies mm9 Unclassified Pluripotent stem cell Embryoid Bodie...203366,SRX203359,SRX353849,SRX353851 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.10.AllAg.Embryoid_Bodies.bed ...

File list: Unc.PSC.20.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available Unc.PSC.20.AllAg.Embryoid_Bodies mm9 Unclassified Pluripotent stem cell Embryoid Bodie...353851,SRX353849,SRX353852,SRX353850 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.20.AllAg.Embryoid_Bodies.bed ...

File list: Unc.PSC.05.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available Unc.PSC.05.AllAg.Embryoid_Bodies mm9 Unclassified Pluripotent stem cell Embryoid Bodie...353852,SRX353850,SRX203366,SRX203357 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Unc.PSC.05.AllAg.Embryoid_Bodies.bed ...

Sclerosing lymphangitis of the penis associated with marked penile oedema and skin erosions.

Science.gov (United States)

Karray, Mehdi; Litaiem, Noureddine; Jones, Mariem; Zeglaoui, Faten

2017-07-27

Sclerosing lymphangitis of the penis is a benign, under-reported condition consisting of a asymptomatic firm cord-like swelling around the coronal sulcus of the penis usually affecting men in the second or third decade of life. Penile oedema and erosions are rarely reported. Clinical signs may be remarkable contrasting with the self-limited character of the disease. We report a new case of sclerosing lymphangitis of the penis occurring in a 59-year-old patient marked by penile swelling and several overlying skin erosions, and discuss the clinical features and the pathogenesis aspects of the disease. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Computed tomography appearances of sclerosing encapsulating peritonitis

International Nuclear Information System (INIS)

George, C.; Al-Zwae, K.; Nair, S.; Cast, J.E.I.

2007-01-01

Sclerosing encapsulating peritonitis (SEP) is a serious complication of peritoneal dialysis (PD) characterized by thickened peritoneal membranes, which lead to decreased ultra-filtration and intestinal obstruction. Its early clinical features are nonspecific, and it is often diagnosed late following laparotomy and peritoneal biopsy, when the patient develops small bowel obstruction, which can be a life-threatening complication. However, this is changing with increasing awareness of computed tomography (CT) findings in SEP. CT can yield an early, non-invasive diagnosis that may improve patient outcome. We present a review of the CT appearances of SEP

Computed tomography appearances of sclerosing encapsulating peritonitis

Energy Technology Data Exchange (ETDEWEB)

George, C. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom)]. E-mail: cheriangeorge@hotmail.com; Al-Zwae, K. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom); Nair, S. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom); Cast, J.E.I. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom)

2007-08-15

Sclerosing encapsulating peritonitis (SEP) is a serious complication of peritoneal dialysis (PD) characterized by thickened peritoneal membranes, which lead to decreased ultra-filtration and intestinal obstruction. Its early clinical features are nonspecific, and it is often diagnosed late following laparotomy and peritoneal biopsy, when the patient develops small bowel obstruction, which can be a life-threatening complication. However, this is changing with increasing awareness of computed tomography (CT) findings in SEP. CT can yield an early, non-invasive diagnosis that may improve patient outcome. We present a review of the CT appearances of SEP.

File list: ALL.PSC.05.AllAg.Embryoid_Bodies [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available ALL.PSC.05.AllAg.Embryoid_Bodies mm9 All antigens Pluripotent stem cell Embryoid Bodie...X385956,SRX385955,SRX385958,SRX385957 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.PSC.05.AllAg.Embryoid_Bodies.bed ...

File list: ALL.PSC.10.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available ALL.PSC.10.AllAg.Embryoid_Bodies mm9 All antigens Pluripotent stem cell Embryoid Bodie...X385958,SRX385956,SRX385955,SRX385957 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.PSC.10.AllAg.Embryoid_Bodies.bed ...

File list: ALL.PSC.50.AllAg.Embryoid_Bodies [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available ALL.PSC.50.AllAg.Embryoid_Bodies mm9 All antigens Pluripotent stem cell Embryoid Bodie...X385956,SRX385955,SRX385958,SRX385957 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.PSC.50.AllAg.Embryoid_Bodies.bed ...

File list: ALL.PSC.20.AllAg.Embryoid_Bodies [Chip-atlas[Archive

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Full Text Available ALL.PSC.20.AllAg.Embryoid_Bodies mm9 All antigens Pluripotent stem cell Embryoid Bodie...X385956,SRX385958,SRX385957,SRX353850 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.PSC.20.AllAg.Embryoid_Bodies.bed ...

Biliary fascioliasis--an uncommon cause of recurrent biliary colics: report of a case and brief review.

Science.gov (United States)

Al Qurashi, Hesham; Masoodi, Ibrahim; Al Sofiyani, Mohammad; Al Musharaf, Hisham; Shaqhan, Mohammed; All, Gamal Nasr Ahmed Abdel

2012-01-01

Biliary parasitosis is one of the important causes of biliary obstruction in endemic areas, however due to migration and travel the disease is known to occur in non endemic zones as well. The spectrum of biliary fascioliasis ranges from recurrent biliary colics to acute cholangitis. The long term complications are gall stones, sclerosing cholangitis and biliary cirrhosis. We describe fascioliasis as a cause of recurrent biliary colics in a young male necessitating multiple hospitalizations over a period of four years. Investigative profile had been non-contributory every time he was hospitalized for his abdominal pain prior to the current presentation. He never had cholangitis due to the worm in the common bile duct. It was only at endoscopic retrograde cholangiopancreatography (ERCP) biliary fascioliasis was discovered to be the cause of his recurrent biliary colics. After removal of the live Fasciola hepatica from the common bile duct he became symptom free and is attending our clinic for last 11 months now. Clinical spectrum of biliary fascioliasis is discussed in this report.

File list: Oth.PSC.20.Epitope_tags.AllCell [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Oth.PSC.20.Epitope_tags.AllCell mm9 TFs and others Epitope tags Pluripotent stem ce...822,SRX266828,SRX352996,ERX320411,SRX204802 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.20.Epitope_tags.AllCell.bed ...

File list: Oth.PSC.05.Epitope_tags.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.05.Epitope_tags.AllCell mm9 TFs and others Epitope tags Pluripotent stem ce...821,ERX320410,SRX266822,SRX352996,ERX320411 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.05.Epitope_tags.AllCell.bed ...

File list: Oth.PSC.20.Smarca4.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.20.Smarca4.AllCell mm9 TFs and others Smarca4 Pluripotent stem cell SRX1300...3820,SRX823825,SRX823833,SRX823835 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.20.Smarca4.AllCell.bed ...

File list: Oth.PSC.10.Smarca4.AllCell [Chip-atlas[Archive

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Full Text Available Oth.PSC.10.Smarca4.AllCell mm9 TFs and others Smarca4 Pluripotent stem cell SRX1901...3830,SRX823825,SRX823820,SRX823835 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.10.Smarca4.AllCell.bed ...

The B-mode Sonography and Sonoelastographic Features of Sclerosing Adenosis of the Breast

International Nuclear Information System (INIS)

Myong, Joo Hwa; Kim, Sung Hun; Kang, Bong Joo; Ahn, Young I; Yoon, Soo Kyoung; Lee, A Won; Yim, Kwang Il; Kim, Tae Eun; Song, Byung Joo

2011-01-01

The purpose of this study was to evaluate the B-mode sonographic and sonoelastographic features of high risk lesions of the breast. From April 2009 to February 2010, 1390 patients with breast lesions underwent US-guided core-biopsy. Among them, 13 lesions were confirmed to be pure sclerosing adenosis by subsequent surgical excision or on imaging follow-up of more than 1 year. Two radiologists retrospectively analyzed the B-mode sonography according to the Breast Imaging Reporting and Data System classification. The sonoelastographic images were classified into 5 elasticity scores according to the Itoh classification and the strain ratio between the mass and the surrounding fat tissue was reviewed. We considered the sonoelastographic patterns to be suspicious for the case with a score of 4 and 5 and a strain ratio of more than a 2.24. The common B-mode sonographic features of sclerosing adenosis were an irregular shape (69.2%, 9 of 13), an indistinct margin (92.3%, 12 of 13), hypoechogenicity (76.9%, 10 of 13) and category 4A, a low suspicion of malignancy (61.5%, 8 of 13). The common sonoelastographic features were a score of 2 (42%, 6 of 13) and a strain ratio < 2.24 (69.2%, 9 of 13). Sclerosing adenosis showed suspicious B-mode sonographic findings, but it had benign sonolastographic features

The B-mode Sonography and Sonoelastographic Features of Sclerosing Adenosis of the Breast

Energy Technology Data Exchange (ETDEWEB)

Myong, Joo Hwa; Kim, Sung Hun; Kang, Bong Joo; Ahn, Young I; Yoon, Soo Kyoung; Lee, A Won; Yim, Kwang Il; Kim, Tae Eun; Song, Byung Joo [Seoul St. Mary' s Hospital, The Catholic University, Seoul (Korea, Republic of)

2011-06-15

The purpose of this study was to evaluate the B-mode sonographic and sonoelastographic features of high risk lesions of the breast. From April 2009 to February 2010, 1390 patients with breast lesions underwent US-guided core-biopsy. Among them, 13 lesions were confirmed to be pure sclerosing adenosis by subsequent surgical excision or on imaging follow-up of more than 1 year. Two radiologists retrospectively analyzed the B-mode sonography according to the Breast Imaging Reporting and Data System classification. The sonoelastographic images were classified into 5 elasticity scores according to the Itoh classification and the strain ratio between the mass and the surrounding fat tissue was reviewed. We considered the sonoelastographic patterns to be suspicious for the case with a score of 4 and 5 and a strain ratio of more than a 2.24. The common B-mode sonographic features of sclerosing adenosis were an irregular shape (69.2%, 9 of 13), an indistinct margin (92.3%, 12 of 13), hypoechogenicity (76.9%, 10 of 13) and category 4A, a low suspicion of malignancy (61.5%, 8 of 13). The common sonoelastographic features were a score of 2 (42%, 6 of 13) and a strain ratio < 2.24 (69.2%, 9 of 13). Sclerosing adenosis showed suspicious B-mode sonographic findings, but it had benign sonolastographic features

Brain CT and MRI findings of a long-term case of subacute sclerosing panencephalitis

Energy Technology Data Exchange (ETDEWEB)

Aoshiba, Kazunori; Ota, Kohei; Komatsuzaki, Satoshi; Kobayashi, Itsuro; Maruyama, Shoichi

1987-11-01

Our study involved a long-term case (ten years) of subacute sclerosing panencephalitis. The case began with a 23 year-old experiencing visual deterioration. During the course of his illness, amnesia, autism and abnormal behavior were observed without any myoclonus. On the electroencephalogram, periodic synclonous discharge was shown in the early stage of his illness and subsequently disappeared. The brain CT and the MRI disclosed diffuse lesions in both cortical and subcortical areas of the cerebral hemispheres. The location and spread of lesions were more clearly revealed by the MRI than the brain CT. These findings suggest that the MRI is more useful than the brain CT in the diagnosis of subacute sclerosing panencephalitis.

Brain CT and MRI findings of a long-term case of subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Aoshiba, Kazunori; Ota, Kohei; Komatsuzaki, Satoshi; Kobayashi, Itsuro; Maruyama, Shoichi

1987-01-01

Our study involved a long-term case (ten years) of subacute sclerosing panencephalitis. The case began with a 23 year-old experiencing visual deterioration. During the course of his illness, amnesia, autism and abnormal behavior were observed without any myoclonus. On the electroencephalogram, periodic synclonous discharge was shown in the early stage of his illness and subsequently disappeared. The brain CT and the MRI disclosed diffuse lesions in both cortical and subcortical areas of the cerebral hemispheres. The location and spread of lesions were more clearly revealed by the MRI than the brain CT. These findings suggest that the MRI is more useful than the brain CT in the diagnosis of subacute sclerosing panencephalitis. (author)

Methamphetamine increases Prion Protein and induces dopamine-dependent expression of protease resistant PrPsc.

Science.gov (United States)

Ferrucci, M; Ryskalin, L; Biagioni, F; Gambardella, S; Busceti, C L; Falleni, A; Lazzeri, G; Fornai, F

2017-07-01

The cellular prion protein (PrPc) is physiologically expressed within selective brain areas of mammals. Alterations in the secondary structure of this protein lead to scrapie-like prion protein (PrPsc), which precipitates in the cell. PrPsc has been detected in infectious, inherited or sporadic neurodegenerative disorders. Prion protein metabolism is dependent on autophagy and ubiquitin proteasome. Despite not being fully elucidated, the physiological role of prion protein relates to chaperones which rescue cells under stressful conditions.Methamphetamine (METH) is a widely abused drug which produces oxidative stress in various brain areas causing mitochondrial alterations and protein misfolding. These effects produce a compensatory increase of chaperones while clogging cell clearing pathways. In the present study, we explored whether METH administration modifies the amount of PrPc. Since high levels of PrPc when the clearing systems are clogged may lead to its misfolding into PrPsc, we further tested whether METH exposure triggers the appearance of PrPsc. We analysed the effects of METH and dopamine administration in PC12 and striatal cells by using SDS-PAGE Coomassie blue, immune- histochemistry and immune-gold electron microscopy. To analyze whether METH administration produces PrPsc aggregates we used antibodies directed against PrP following exposure to proteinase K or sarkosyl which digest folded PrPc but misfolded PrPsc. We fond that METH triggers PrPsc aggregates in DA-containing cells while METH is not effective in primary striatal neurons which do not produce DA. In the latter cells exogenous DA is needed to trigger PrPsc accumulation similarly to what happens in DA containing cells under the effects of METH. The present findings, while fostering novel molecular mechanisms involving prion proteins, indicate that, cell pathology similar to prion disorders can be mimicked via a DA-dependent mechanism by a drug of abuse.

MDM2 Amplification and PI3KCA Mutation in a Case of Sclerosing Rhabdomyosarcoma

Directory of Open Access Journals (Sweden)

Ken Kikuchi

2013-01-01

Full Text Available A rare sclerosing variant of rhabdomyosarcoma characterized by prominent hyalinization and pseudovascular pattern has recently been described as a subtype biologically distinct from embryonal, alveolar, and pleomorphic forms. We present cytogenetic and molecular findings as well as experimental studies of an unusual case of sclerosing rhabdomyosarcoma. The primary lesion arose within the plantar subcutaneous tissue of the left foot of an otherwise healthy 23-year-old male who eventually developed pulmonary nodules despite systemic chemotherapy. Two genetic abnormalities identified in surgical and/or autopsy samples of the tumor were introduced into 10T1/2 murine fibroblasts to determine whether these genetic changes cooperatively facilitated transformation and growth. Cytogenetic analysis revealed a complex abnormal hyperdiploid clone, and MDM2 gene amplification was confirmed by fluorescence in situ hybridization. Cancer gene mutation screening using a combination of multiplexed PCR and mass spectroscopy revealed a PIK3CA exon 20 H1047R mutation in the primary tumor, lung metastasis, and liver metastasis. However, this mutation was not cooperative with MDM2 overexpression in experimental assays for transformation or growth. Nevertheless, MDM2 and PIK3CA are genes worthy of further investigation in patients with sclerosing rhabdomyosarcoma and might be considered in the enrollment of these patients into clinical trials of targeted therapeutics.

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Sclerosing Encapsulating Peritonitis; Review

Directory of Open Access Journals (Sweden)

Norman O. Machado

2016-05-01

Full Text Available Sclerosing encapsulating peritonitis (SEP is a rare chronic inflammatory condition of the peritoneum with an unknown aetiology. Also known as abdominal cocoon, the condition occurs when loops of the bowel are encased within the peritoneal cavity by a membrane, leading to intestinal obstruction. Due to its rarity and nonspecific clinical features, it is often misdiagnosed. The condition presents with recurrent episodes of small bowel obstruction and can be idiopathic or secondary; the latter is associated with predisposing factors such as peritoneal dialysis or abdominal tuberculosis. In the early stages, patients can be managed conservatively; however, surgical intervention is necessary for those with advanced stage intestinal obstruction. A literature review revealed 118 cases of SEP; the mean age of these patients was 39 years and 68.0% were male. The predominant presentation was abdominal pain (72.0%, distension (44.9% or a mass (30.5%. Almost all of the patients underwent surgical excision (99.2% without postoperative complications (88.1%.

Lymphoplasmacytic Sclerosing Pancreatitis and Retroperitoneal Fibrosis

Directory of Open Access Journals (Sweden)

Nigel K. F. Koo Ng

2008-01-01

Full Text Available Although cases of lymphoplasmacytic sclerosing pancreatitis (LSP associated with idiopathic retroperitoneal fibrosis have been reported, the association is rare. We describe a 74-year-old man who presented with obstructive jaundice and weight loss. Nineteen months earlier, he had been diagnosed with idiopathic retroperitoneal fibrosis and treated with bilateral ureteric stents. Initial investigations were suggestive of a diagnosis of LSP, however, a malignant cause could not be ruled out. He underwent an exploratory laparotomy and frozen sections confirmed the diagnosis of LSP. An internal biliary bypass was performed using a Roux loop of jejunum, and the patient made an uneventful recovery. This case illustrates the difficulty in distinguishing LSP from pancreatic carcinoma preoperatively.

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Acute cholangitis due to afferent loop syndrome after a Whipple procedure: a case report.

Science.gov (United States)

Spiliotis, John; Karnabatidis, Demetrios; Vaxevanidou, Archodoula; Datsis, Anastasios C; Rogdakis, Athanasios; Zacharis, Georgios; Siamblis, Demetrios

2009-08-25

Patients with resection of stomach and especially with Billroth II reconstruction (gastro jejunal anastomosis), are more likely to develop afferent loop syndrome which is a rare complication. When the afferent part is obstructed, biliary and pancreatic secretions accumulate and cause the distention of this part. In the case of a complete obstruction (rare), there is a high risk developing necrosis and perforation. This complication has been reported once in the literature. A 54-year-old Greek male had undergone a pancreato-duodenectomy (Whipple procedure) one year earlier due to a pancreatic adenocarcinoma. Approximately 10 months after the initial operation, the patient started having episodes of cholangitis (fever, jaundice) and abdominal pain. This condition progressively worsened and the suspicion of local recurrence or stenosis of the biliary-jejunal anastomosis was discussed. A few days before his admission the patient developed signs of septic cholangitis. Our case demonstrates a rare complication with serious clinical manifestation of the afferent loop syndrome. This advanced form of afferent loop syndrome led to the development of huge enterobiliary reflux, which had a serious clinical manifestation as cholangitis and systemic sepsis, due to bacterial overgrowth, which usually present in the afferent loop. The diagnosis is difficult and the interventional radiology gives all the details to support the therapeutic decision making. A variety of factors can contribute to its development including adhesions, kinking and angulation of the loop, stenosis of gastro-jejunal anastomosis and internal herniation. In order to decompress the afferent loop dilatation due to adhesions, a lateral-lateral jejunal anastomosis was performed between the afferent loop and a small bowel loop.

How the concept of biochemical response influenced the management of primary biliary cholangitis over time

NARCIS (Netherlands)

Lammers, W. J.; Leeman, M.; Ponsioen, C. I. J.; Boonstra, K.; van Erpecum, K. J.; Wolfhagen, F. H. J.; Kuyvenhoven, J. Ph; Vrolijk, J. M.; Drenth, J. P. H.; Witteman, E. M.; van Nieuwkerk, C. M. J.; van der Spek, B. W.; Witteman, B. J. M.; Erkelens, G. W.; Verhagen, M. A. M. T.; van Tuyl, S. A. C.; Poen, A. C.; Brouwer, J. T.; ter Borg, F.; Koek, G. H.; van Ditzhuijsen, T. J. M.; Hansen, B. E.; van Buuren, H. R.

2016-01-01

Criteria assessing biochemical response to ursodeoxycholic acid (UDCA) are established risk stratification tools in primary biliary cholangitis (PBC). We aimed to evaluate to what extent liver tests influenced patient management during a three decade period, and whether this changed over time. 851

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Colour doppler ultrasonography and sclerosing therapy in diagnosis and treatment of tendinopathy in horses-a research model for human medicine

DEFF Research Database (Denmark)

Boesen, Morten Ilum; Nanni, Simone; Langberg, Henning

2007-01-01

Sclerosing therapy has in recent studies showed promising results in patients with clinically and ultrasonographically diagnosed tendinosis in Achilles and patellar tendons. The aim of this investigation was to study the presence of intratendinous colour Doppler (CD) flow in horses with clinically...... diagnosed chronic tendinopathy and to test if experience from human studies could be extrapolated to horses. Special interest was focused on the treatment with sclerosing therapy and whether we could obtain the same successful peroperative findings as in humans. Four horses with clinically diagnosed...... unilateral chronic tendinosis in the forelimbs were examinated with both grey-scale ultrasonography (US) and CD. The horses were to be euthanised according to standard procedure is such cases. The US findings were used for guidance of sclerosing therapy. All horses showed abnormal findings on US, especially...

Subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Inada, Hiroshi; Hattori, Hideji; Nakajima, Seijun; Iwamura, Chiyo; Tanaka, Akemi; Kim, Masayoshi; Matsuoka, Osamu; Murata, Ryosuke; Inoue, Yuichi

1986-01-01

We studied three children with subacute sclerosing panencephalitis (SSPE) who had been diagnosed between 1981 and 1983. They were treated with inosiplex and transfer factor, and one was given interferon. Clinical symptoms in all three patients sometimes improved for periods of several months. In two patients computed tomography (CT) first showed low density in the basal ganglia, which later improved and finally disappeared. In all three patients CT showed gradual enlargement of the ventricles and cerebral atrophy. Disappearance of the low-density areas may mean that some of the pathological changes of this disease, including inflammation, demyelination, and gliosis, are reversible. In two patients, we studied magnetic resonance imaging. The spin-echo images showed high intensity in the lateral portions of basal ganglia, in the parieto-occipital portions, and in the frontal portions. Inversion recovery images usually showed low intensity of the same lesions. We think that the MRI gave more useful detail than CT. We think that the improvement in the CT findings and clinical symptoms were due both to the treatment (inosiplex seemed to be especially helpful) and to the natural course of this disease. (author)

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Electrophysiological analysis of human pluripotent stem cell-derived cardiomyocytes (hPSC-CMs) using multi-electrode arrays (MEAs)

NARCIS (Netherlands)

Sala, Luca; Ward-van Oostwaard, Dorien; Tertoolen, Leon G.J.; Mummery, Christine L.; Bellin, Milena

2017-01-01

Cardiomyocytes can now be derived with high efficiency from both human embryonic and human induced-Pluripotent Stem Cells (hPSC). hPSC-derived cardiomyocytes (hPSC-CMs) are increasingly recognized as having great value for modeling cardiovascular diseases in humans, especially arrhythmia syndromes.

Toward precision medicine in primary biliary cholangitis.

Science.gov (United States)

Carbone, Marco; Ronca, Vincenzo; Bruno, Savino; Invernizzi, Pietro; Mells, George F

2016-08-01

Primary biliary cholangitis is a chronic, cholestatic liver disease characterized by a heterogeneous presentation, symptomatology, disease progression and response to therapy. In contrast, clinical management and treatment of PBC is homogeneous with a 'one size fits all' approach. The evolving research landscape, with the emergence of the -omics field and the availability of large patient cohorts are creating a unique opportunity of translational epidemiology. Furthermore, several novel disease and symptom-modifying agents for PBC are currently in development. The time is therefore ripe for precision medicine in PBC. In this manuscript we describe the concept of precision medicine; review current approaches to risk-stratification in PBC, and speculate how precision medicine in PBC might develop in the near future. Copyright © 2016 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

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File list: ALL.PSC.50.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available ALL.PSC.50.AllAg.iPS_cells hg19 All antigens Pluripotent stem cell iPS cells SRX088...16,SRX189400,SRX189399 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/ALL.PSC.50.AllAg.iPS_cells.bed ...

File list: ALL.PSC.20.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available ALL.PSC.20.AllAg.iPS_cells hg19 All antigens Pluripotent stem cell iPS cells SRX088...27,SRX189400,SRX189399 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/ALL.PSC.20.AllAg.iPS_cells.bed ...

File list: ALL.PSC.20.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available ALL.PSC.20.AllAg.iPS_cells mm9 All antigens Pluripotent stem cell iPS cells SRX9773...30,SRX146522,SRX146547 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.PSC.20.AllAg.iPS_cells.bed ...

File list: Pol.PSC.05.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Pol.PSC.05.AllAg.iPS_cells mm9 RNA polymerase Pluripotent stem cell iPS cells SRX97...7435,SRX027462,SRX977434 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Pol.PSC.05.AllAg.iPS_cells.bed ...

Structural Immaturity of Human iPSC-Derived Cardiomyocytes: In Silico Investigation of Effects on Function and Disease Modeling

Science.gov (United States)

Koivumäki, Jussi T.; Naumenko, Nikolay; Tuomainen, Tomi; Takalo, Jouni; Oksanen, Minna; Puttonen, Katja A.; Lehtonen, Šárka; Kuusisto, Johanna; Laakso, Markku; Koistinaho, Jari; Tavi, Pasi

2018-01-01

Background: Human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) have emerged as a promising experimental tool for translational heart research and drug development. However, their usability as a human adult cardiomyocyte model is limited by their functional immaturity. Our aim is to analyse quantitatively those characteristics and how they differ from adult CMs. Methods and Results: We have developed a novel in silico model with all essential functional electrophysiology and calcium handling features of hiPSC-CMs. Importantly, the virtual cell recapitulates the immature intracellular ion dynamics that are characteristic for hiPSC-CMs, as quantified based our in vitro imaging data. The strong “calcium clock” is a source for a dual function of excitation-contraction coupling in hiPSC-CMs: action potential and calcium transient morphology vary substantially depending on the activation sequence of underlying ionic currents and fluxes that is altered in spontaneous vs. paced mode. Furthermore, parallel simulations with hiPSC-CM and adult cardiomyocyte models demonstrate the central differences. Results indicate that hiPSC-CMs translate poorly the disease specific phenotypes of Brugada syndrome, long QT Syndrome and catecholaminergic polymorphic ventricular tachycardia, showing less robustness and greater tendency for arrhythmic events than adult CMs. Based on a comparative sensitivity analysis, hiPSC-CMs share some features with adult CMs, but are still functionally closer to prenatal CMs than adult CMs. A database analysis of 3000 hiPSC-CM model variants suggests that hiPSC-CMs recapitulate poorly fundamental physiological properties of adult CMs. Single modifications do not appear to solve this problem, which is mostly contributed by the immaturity of intracellular calcium handling. Conclusion: Our data indicates that translation of findings from hiPSC-CMs to human disease should be made with great caution. Furthermore, we established a

Modelling neurodegenerative diseases in vitro: Recent advances in 3D iPSC technologies

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Elodie J Siney

2018-03-01

Full Text Available The discovery of induced pluripotent stem cells (iPSC 12 years ago has fostered the development of innovative patient-derived in vitro models for better understanding of disease mechanisms. This is particularly relevant to neurodegenerative diseases, where availability of live human brain tissue for research is limited and post-mortem interval changes influence readouts from autopsy-derived human tissue. Hundreds of iPSC lines have now been prepared and banked, thanks to several large scale initiatives and cell banks. Patient- or engineered iPSC-derived neural models are now being used to recapitulate cellular and molecular aspects of a variety of neurodegenerative diseases, including early and pre-clinical disease stages. The broad relevance of these models derives from the availability of a variety of differentiation protocols to generate disease-specific cell types and the manipulation to either introduce or correct disease-relevant genetic modifications. Moreover, the use of chemical and physical three-dimensional (3D matrices improves control over the extracellular environment and cellular organization of the models. These iPSC-derived neural models can be utilised to identify target proteins and, importantly, provide high-throughput screening for drug discovery. Choosing Alzheimer’s disease (AD as an example, this review describes 3D iPSC-derived neural models and their advantages and limitations. There is now a requirement to fully characterise and validate these 3D iPSC-derived neural models as a viable research tool that is capable of complementing animal models of neurodegeneration and live human brain tissue. With further optimization of differentiation, maturation and aging protocols, as well as the 3D cellular organisation and extracellular matrix to recapitulate more closely, the molecular extracellular-environment of the human brain, 3D iPSC-derived models have the potential to deliver new knowledge, enable discovery of novel

Generation of iPSC lines from primary human chorionic villi cells

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Björn Lichtner

2015-11-01

Full Text Available Primary human chorionic villi (CV cells were used to generate the iPSC line by retroviral transduction of the four Yamanaka-factors OCT4, SOX2, KLF4 and c-MYC. Pluripotency was confirmed both in vivo and in vitro. The transcriptomes of the CV-derived iPSC lines and the human embryonic stem cell lines—H1 and H9 have a Pearson correlation of 0.929 and 0.943 respectively.

File list: ALL.PSC.10.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available ALL.PSC.10.AllAg.iPS_cells mm9 All antigens Pluripotent stem cell iPS cells SRX9774...30,SRX146524,SRX146547,SRX146522 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/ALL.PSC.10.AllAg.iPS_cells.bed ...

Repressed SIRT1/PGC-1α pathway and mitochondrial disintegration in iPSC-derived RPE disease model of age-related macular degeneration.

Science.gov (United States)

Golestaneh, Nady; Chu, Yi; Cheng, Shuk Kei; Cao, Hong; Poliakov, Eugenia; Berinstein, Daniel M

2016-12-20

Study of age related macular degeneration (AMD) has been hampered by lack of human models that represent the complexity of the disease. Here we have developed a human in vitro disease model of AMD to investigate the underlying AMD disease mechanisms. Generation of iPSCs from retinal pigment epithelium (RPE) of AMD donors, age-matched normal donors, skin fibroblasts of a dry AMD patient, and differentiation of iPSCs into RPE (AMD RPE-iPSC-RPE, normal RPE-iPSC-RPE and AMD Skin-iPSC-RPE, respectively). Immunostaining, cell viability assay and reactive oxygen species (ROS) production under oxidative stress conditions, electron microscopy (EM) imaging, ATP production and glycogen concentration assays, quantitative real time PCR, western blot, karyotyping. The AMD RPE-iPSC-RPE and AMD Skin-iPSC-RPE present functional impairment and exhibit distinct disease phenotypes compared to RPE-iPSC-RPE generated from normal donors (Normal RPE-iPSC-RPE). The AMD RPE-iPSC-RPE and AMD Skin-iPSC-RPE show increased susceptibility to oxidative stress and produced higher levels of reactive oxygen species (ROS) under stress in accordance with recent reports. The susceptibility to oxidative stress-induced cell death in AMD RPE-iPSC-RPE and Skin-iPSC-RPE was consistent with inability of the AMD RPE-iPSC-RPE and Skin-iPSC-RPE to increase SOD2 expression under oxidative stress. Phenotypic analysis revealed disintegrated mitochondria, accumulation of autophagosomes and lipid droplets in AMD RPE-iPSC-RPE and AMD Skin-iPSC-RPE. Mitochondrial activity was significantly lower in AMD RPE-iPSC-RPE and AMD Skin-iPSC-RPE compared to normal cells and glycogen concentration was significantly increased in the diseased cells. Furthermore, Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α), a regulator of mitochondrial biogenesis and function was repressed, and lower expression levels of NAD-dependent deacetylase sirtuin1 (SIRT1) were found in AMD RPE-iPSC-RPE and AMD Skin-iPSC

File list: InP.PSC.05.AllAg.Embryoid_Bodies [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.05.AllAg.Embryoid_Bodies mm9 Input control Pluripotent stem cell Embryoid Bodie...s SRX212083,SRX385954,SRX109458,SRX109456,SRX109460,SRX026526 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.05.AllAg.Embryoid_Bodies.bed ...

File list: InP.PSC.10.AllAg.Embryoid_Bodies [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.10.AllAg.Embryoid_Bodies mm9 Input control Pluripotent stem cell Embryoid Bodie...s SRX385954,SRX212083,SRX109456,SRX109460,SRX109458,SRX026526 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.10.AllAg.Embryoid_Bodies.bed ...

File list: InP.PSC.50.AllAg.Embryoid_Bodies [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.50.AllAg.Embryoid_Bodies mm9 Input control Pluripotent stem cell Embryoid Bodie...s SRX109460,SRX109458,SRX212083,SRX109456,SRX385954,SRX026526 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.50.AllAg.Embryoid_Bodies.bed ...

File list: Oth.PSC.20.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Oth.PSC.20.AllAg.mESCs,_differentiated mm9 TFs and others Pluripotent stem cell mESCs, differentia...4,SRX754570,SRX065538,SRX523453,SRX523451,SRX065537 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.20.AllAg.mESCs,_differentiated.bed ...

File list: Oth.PSC.50.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Oth.PSC.50.AllAg.mESCs,_differentiated mm9 TFs and others Pluripotent stem cell mESCs, differentia...8,SRX523453,SRX523451,SRX754570,SRX882858,SRX065537 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.50.AllAg.mESCs,_differentiated.bed ...

File list: Oth.PSC.10.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Oth.PSC.10.AllAg.mESCs,_differentiated mm9 TFs and others Pluripotent stem cell mESCs, differentia...2,SRX065538,SRX523453,SRX754570,SRX065537,SRX523451 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.10.AllAg.mESCs,_differentiated.bed ...

File list: Oth.PSC.05.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Oth.PSC.05.AllAg.mESCs,_differentiated mm9 TFs and others Pluripotent stem cell mESCs, differentia...8,SRX209322,SRX065538,SRX065537,SRX523453,SRX523451 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Oth.PSC.05.AllAg.mESCs,_differentiated.bed ...

File list: InP.PSC.50.AllAg.EpiLC [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.50.AllAg.EpiLC mm9 Input control Pluripotent stem cell EpiLC SRX823839,SRX5...5,SRX823838,SRX823831,SRX823826,SRX590324 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.50.AllAg.EpiLC.bed ...

File list: InP.PSC.20.AllAg.Embryoid_Bodies [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.20.AllAg.Embryoid_Bodies mm9 Input control Pluripotent stem cell Embryoid B...odies SRX109460,SRX109458,SRX212083,SRX109456,SRX385954,SRX026526 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.20.AllAg.Embryoid_Bodies.bed ...

From iPSC towards cardiac tissue-a road under construction.

Science.gov (United States)

Peischard, Stefan; Piccini, Ilaria; Strutz-Seebohm, Nathalie; Greber, Boris; Seebohm, Guiscard

2017-10-01

The possibility to generate induced pluripotent stem cells (iPSC) opens the way to generate virtually all cell types of our human body. In combination with modern gene editing techniques like CRISPR/CAS, a new set of powerful tools becomes available for life science. Scientific fields like genotype and cell type-specific pharmacology, disease modeling, stem cell biology, and developmental biology have been dramatically fostered and their faces have been changed. However, as golden as the age of iPSC-derived cells and their manipulation has started, the shine begins to tarnish. Researchers face more and more practical problems intrinsic to the system. These problems are related to the specific culturing conditions which are not yet sufficient to mimic the natural environment of native stem cells differentiating towards adult cells. However, researchers work hard to uncover these factors. Here, we review a common standard approach to generate iPSCs and transduce these to iPSC cardiomyocytes. Further, we review recent achievements and discuss their current limitations and future perspectives. We are on track, but the road is still under construction.

Molecular diagnostic testing for primary biliary cholangitis.

Science.gov (United States)

Gatselis, Nikolaos K; Dalekos, George N

2016-09-01

A reliable liver autoimmune serology for the diagnosis of primary biliary cholangitis (PBC) is of particular importance. Recognition of patients at early stages and prompt treatment initiation may alter the outcome, slow progression, delays liver failure, and improves survival. In this review, we summarize and discuss the published data obtained from literature searches from PubMed and The National Library of Medicine (USA) and our own experience on the current and potential molecular based approaches to the diagnosis of PBC. Expert commentary: Standardization of liver diagnostic serology and clinical governance are two major points as antimitochondrial antibodies are the diagnostic hallmark of the disease and PBC-specific antinuclear antibodies could assist in the diagnosis and estimation of prognosis. New biomarkers such as novel autoantibodies, genetic polymorphisms, metabolomic profiling, micro-RNA and epigenetics may assist to the understanding, diagnosis and management of the disease.

File list: NoD.PSC.05.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available NoD.PSC.05.AllAg.mESCs,_differentiated mm9 No description Pluripotent stem cell mESCs, differentia...ted http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/NoD.PSC.05.AllAg.mESCs,_differentiated.bed ...

File list: InP.PSC.20.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.20.AllAg.mESCs,_differentiated mm9 Input control Pluripotent stem cell mESCs, differentia...,SRX388447 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.20.AllAg.mESCs,_differentiated.bed ...

File list: NoD.PSC.20.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available NoD.PSC.20.AllAg.mESCs,_differentiated mm9 No description Pluripotent stem cell mESCs, differentia...ted http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/NoD.PSC.20.AllAg.mESCs,_differentiated.bed ...

File list: InP.PSC.50.AllAg.mESCs,_differentiated [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available InP.PSC.50.AllAg.mESCs,_differentiated mm9 Input control Pluripotent stem cell mESCs, differentia...,SRX388447 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/InP.PSC.50.AllAg.mESCs,_differentiated.bed ...

9+ Years of CALIPSO PSC Observations: An Evolving Climatology

Science.gov (United States)

Pitts, Michael C.; Poole, Lamont R.

2015-01-01

Polar stratospheric clouds (PSCs) play a crucial role in the springtime chemical depletion of ozone at high latitudes. PSC particles (primarily supercooled ternary solution, or STS droplets) provide sites for heterogeneous chemical reactions that transform stable chlorine and bromine reservoir species into highly reactive ozone-destructive forms. Furthermore, large nitric acid trihydrate (NAT) PSC particles can irreversibly redistribute odd nitrogen through gravitational sedimentation (a process commonly known as denitrification), which prolongs the ozone depletion process by slowing the reformation of the stable chlorine reservoirs. Spaceborne observations from the CALIOP (Cloud-Aerosol Lidar with Orthogonal Polarization) lidar on the CALIPSO (Cloud-Aerosol Lidar and Infrared Pathfinder Satellite Observations) satellite are providing a rich new dataset for studying PSCs. CALIPSO is an excellent platform for studying polar processes with CALIOP acquiring, on average, over 300,000 backscatter profiles daily at latitudes between 55o and 82o in both hemispheres. PSCs are detected in the CALIOP backscatter profiles using a successive horizontal averaging scheme that enables detection of strongly scattering PSCs (e.g., ice) at the finest possible spatial resolution (5 km), while enhancing the detection of very tenuous PSCs (e.g., low number density NAT) at larger spatial scales (up to 135 km). CALIOP PSCs are separated into composition classes (STS; liquid/NAT mixtures; and ice) based on the ensemble 532-nm scattering ratio (the ratio of total-to-molecular backscatter) and 532-nm particulate depolarization ratio (which is sensitive to the presence of non-spherical, i.e. NAT and ice particles). In this paper, we will provide an overview of the CALIOP PSC detection and composition classification algorithm and then examine the vertical and spatial distribution of PSCs in the Arctic and Antarctic on vortex-wide scales for entire PSC seasons over the more than nine-year data

PSC Matrix for Active GSA Schedules and GSA GWACs

Data.gov (United States)

General Services Administration — The Product Service Codes (PSC) and North American Industrial Classification Systems (NAICS) are the two methods the Federal government classifies contracts. They...

Antibacterial Therapy of Acute Cholecystitis and Cholangitis (According to Tokyo Guidelines2013

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Yu.M. Stepanov

2015-04-01

Antibiotics should be used wisely in antimicrobial therapy in each institution, region and country. The recent global spread of antibiotic resistance gives us a warning in the modern practice. Tokyo Guidelines 2013 provide practical guidance for physicians and surgeons involved in the treatment of community-acquired and hospital acute biliary infection. Much remains uncertain in this view. Continuous monitoring of local resistance to antibiotics and further studies in acute cholecystitis and cholangitis should be justified.

File list: DNS.PSC.50.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available DNS.PSC.50.AllAg.iPS_cells hg19 DNase-seq Pluripotent stem cell iPS cells SRX040379...,SRX040378,SRX135563,SRX040376,SRX040377,SRX189427,SRX189400,SRX189399 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/DNS.PSC.50.AllAg.iPS_cells.bed ...

File list: His.PSC.50.AllAg.iPS_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available His.PSC.50.AllAg.iPS_cells mm9 Histone Pluripotent stem cell iPS cells SRX977417,SR...RX127376,SRX146530,SRX146522,SRX146547,SRX333561,SRX035985,SRX1090869 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/His.PSC.50.AllAg.iPS_cells.bed ...

Subareolar Sclerosing Ductal Hyperplasia.

Science.gov (United States)

Cheng, Esther; D'Alfonso, Timothy M; Arafah, Maria; Marrero Rolon, Rebecca; Ginter, Paula S; Hoda, Syed A

2017-02-01

Subareolar sclerosing duct hyperplasia (SSDH) remains to be fully characterized nearly 20 years after initial description. Thirty-five SSDH cases diagnosed over a 16-year period (January 2000 to December 2015) were reviewed. All patients were female (mean age = 59 years, range = 18-80) who had presented with a unilateral solitary lesion (left 22, right 13) with a mean size of 1.3 cm (range = 0.4-3.0 cm), and showed florid and papillary epithelial hyperplasia with dense sclerosis without involvement of nipple or areolar epidermis. Significant lesions concurrent within SSDH included low-grade adenosquamous carcinoma (n = 1), ductal carcinoma in situ (DCIS; n = 1), lobular carcinoma in situ (LCIS; n = 1), and atypical ductal hyperplasia (ADH; n = 13). No case of SSDH recurred in a mean follow-up of 44 months (range = 6-189). Subsequent significant lesions occurred in 6 patients: DCIS (n = 3; ipsilateral 2, contralateral 1), ipsilateral ADH (n = 2), and ipsilateral atypical lobular hyperplasia (n = 1). Long-term follow-up for patients with SSDH is indicated as DCIS can occur subsequently in either breast.

Electrophysiological Characteristics of Human iPSC-Derived Cardiomyocytes for the Assessment of Drug-Induced Proarrhythmic Potential.

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Wataru Yamamoto

Full Text Available The aims of this study were to (1 characterize basic electrophysiological elements of human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs that correspond to clinical properties such as QT-RR relationship, (2 determine the applicability of QT correction and analysis methods, and (3 determine if and how these in-vitro parameters could be used in risk assessment for adverse drug-induced effects such as Torsades de pointes (TdP. Field potential recordings were obtained from commercially available hiPSC-CMs using multi-electrode array (MEA platform with and without ion channel antagonists in the recording solution. Under control conditions, MEA-measured interspike interval and field potential duration (FPD ranged widely from 1049 to 1635 ms and from 334 to 527 ms, respectively and provided positive linear regression coefficients similar to native QT-RR plots obtained from human electrocardiogram (ECG analyses in the ongoing cardiovascular-based Framingham Heart Study. Similar to minimizing the effect of heart rate on the QT interval, Fridericia's and Bazett's corrections reduced the influence of beat rate on hiPSC-CM FPD. In the presence of E-4031 and cisapride, inhibitors of the rapid delayed rectifier potassium current, hiPSC-CMs showed reverse use-dependent FPD prolongation. Categorical analysis, which is usually applied to clinical QT studies, was applicable to hiPSC-CMs for evaluating torsadogenic risks with FPD and/or corrected FPD. Together, this results of this study links hiPSC-CM electrophysiological endpoints to native ECG endpoints, demonstrates the appropriateness of clinical analytical practices as applied to hiPSC-CMs, and suggests that hiPSC-CMs are a reliable models for assessing the arrhythmogenic potential of drug candidates in human.

Human iPSC for Therapeutic Approaches to the Nervous System: Present and Future Applications

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Maria Giuseppina Cefalo

2016-01-01

Full Text Available Many central nervous system (CNS diseases including stroke, spinal cord injury (SCI, and brain tumors are a significant cause of worldwide morbidity/mortality and yet do not have satisfying treatments. Cell-based therapy to restore lost function or to carry new therapeutic genes is a promising new therapeutic approach, particularly after human iPSCs became available. However, efficient generation of footprint-free and xeno-free human iPSC is a prerequisite for their clinical use. In this paper, we will first summarize the current methodology to obtain footprint- and xeno-free human iPSC. We will then review the current iPSC applications in therapeutic approaches for CNS regeneration and their use as vectors to carry proapoptotic genes for brain tumors and review their applications for modelling of neurological diseases and formulating new therapeutic approaches. Available results will be summarized and compared. Finally, we will discuss current limitations precluding iPSC from being used on large scale for clinical applications and provide an overview of future areas of improvement. In conclusion, significant progress has occurred in deriving iPSC suitable for clinical use in the field of neurological diseases. Current efforts to overcome technical challenges, including reducing labour and cost, will hopefully expedite the integration of this technology in the clinical setting.

File list: NoD.PSC.20.AllAg.STAP_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available NoD.PSC.20.AllAg.STAP_cells mm9 No description Pluripotent stem cell STAP cells SRX...472660,SRX472654,SRX472663,SRX472661,SRX472656,SRX472665,SRX472662,SRX472655,SRX472664 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/NoD.PSC.20.AllAg.STAP_cells.bed ...

File list: NoD.PSC.05.AllAg.STAP_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available NoD.PSC.05.AllAg.STAP_cells mm9 No description Pluripotent stem cell STAP cells SRX...472660,SRX472663,SRX472654,SRX472665,SRX472656,SRX472662,SRX472661,SRX472664,SRX472655 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/NoD.PSC.05.AllAg.STAP_cells.bed ...

Pretreatment with a γ-Secretase Inhibitor Prevents Tumor-like Overgrowth in Human iPSC-Derived Transplants for Spinal Cord Injury

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Toshiki Okubo

2016-10-01

Full Text Available Neural stem/progenitor cells (NS/PCs derived from human induced pluripotent stem cells (hiPSCs are considered to be a promising cell source for cell-based interventions that target CNS disorders. We previously reported that transplanting certain hiPSC-NS/PCs in the spinal cord results in tumor-like overgrowth of hiPSC-NS/PCs and subsequent deterioration of motor function. Remnant immature cells should be removed or induced into more mature cell types to avoid adverse effects of hiPSC-NS/PC transplantation. Because Notch signaling plays a role in maintaining NS/PCs, we evaluated the effects of γ-secretase inhibitor (GSI and found that pretreating hiPSC-NS/PCs with GSI promoted neuronal differentiation and maturation in vitro, and GSI pretreatment also reduced the overgrowth of transplanted hiPSC-NS/PCs and inhibited the deterioration of motor function in vivo. These results indicate that pretreatment with hiPSC-NS/PCs decreases the proliferative capacity of transplanted hiPSC-NS/PCs, triggers neuronal commitment, and improves the safety of hiPSC-based approaches in regenerative medicine.

Co-existence of Distinct Prion Types Enables Conformational Evolution of Human PrPSc by Competitive Selection*

Science.gov (United States)

Haldiman, Tracy; Kim, Chae; Cohen, Yvonne; Chen, Wei; Blevins, Janis; Qing, Liuting; Cohen, Mark L.; Langeveld, Jan; Telling, Glenn C.; Kong, Qingzhong; Safar, Jiri G.

2013-01-01

The unique phenotypic characteristics of mammalian prions are thought to be encoded in the conformation of pathogenic prion proteins (PrPSc). The molecular mechanism responsible for the adaptation, mutation, and evolution of prions observed in cloned cells and upon crossing the species barrier remains unsolved. Using biophysical techniques and conformation-dependent immunoassays in tandem, we isolated two distinct populations of PrPSc particles with different conformational stabilities and aggregate sizes, which frequently co-exist in the most common human prion disease, sporadic Creutzfeldt-Jakob disease. The protein misfolding cyclic amplification replicates each of the PrPSc particle types independently and leads to the competitive selection of those with lower initial conformational stability. In serial propagation with a nonglycosylated mutant PrPC substrate, the dominant PrPSc conformers are subject to further evolution by natural selection of the subpopulation with the highest replication rate due to its lowest stability. Cumulatively, the data show that sporadic Creutzfeldt-Jakob disease PrPSc is not a single conformational entity but a dynamic collection of two distinct populations of particles. This implies the co-existence of different prions, whose adaptation and evolution are governed by the selection of progressively less stable, faster replicating PrPSc conformers. PMID:23974118

A Radial Sclerosing Lesion Mimicking Breast Cancer on Mammography in a Young Woman

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Masashi Furukawa

2012-02-01

Full Text Available A spiculated mass on a mammogram is highly suggestive of malignancy. We report the case of a 32-year-old woman with a radial sclerosing lesion that mimicked breast cancer on mammography. She visited her physician after palpating a lump in her left breast. Mammography showed architectural distortion in the upper inner quadrant of the left breast. Ultrasonography showed a low echoic area with an ambiguous boundary. Core needle biopsy was performed because of the suspicion of malignancy. Histological examination did not reveal any malignant cells. After 6 months, the breast lump became larger and the patient was referred to our hospital. Mammography performed in our hospital showed a spiculated mass, and therefore mammotome biopsy was performed. Histological examination revealed dense fibroelastic stroma with a wide variety of mastopathic changes, leading to a diagnosis of a radial sclerosing lesion. One year after the biopsy, the lump on her left breast had disappeared and mammography showed no spiculated mass.

Cadmium and lead accumulations and agronomic quality of a newly bred pollution-safe cultivar (PSC) of water spinach.

Science.gov (United States)

Huang, Ying-Ying; Mu, Yang-Xiu; He, Chun-Tao; Fu, Hui-Ling; Wang, Xue-Song; Gong, Fei-Yue; Yang, Zhong-Yi

2018-04-01

Breeding for pollution-safe cultivars (PSCs) can reduce pollutant accumulation in crops. However, the PSC breeding would face the risk of nutritional quality reduction, which is usually ignored in conventional breeding programs targeting to increase crop yield or nutritional quality. Thus, the doubt whether the risk would exist has to be clarified for supporting the PSC breeding. In the present study, a newly bred Cd/Pb-PSC of water spinach (Ipomoea aquatic Forsk.) and its parents (QLQ with low-Cd/Pb accumulation ability and T308 with high yield) of water spinach were employed to clarify the above-mentioned issue. Yields, and concentrations of Cd, Pb, nitrite, and organic and inorganic nutrients in shoots of the three experimental lines were determined. There were no significant differences in Cd/Pb concentration between the new PSC and QLQ, in nitrite content between the new PSC and its two parents and in yield between the new PSC and T308. It is decisively significant that shoot concentrations of organic and inorganic nutrients in the Cd/Pb-PSC were as high as those in one of its parents. It is affirmed that the breeding operations (crossing and consequently continuous selfing) for lowering Cd/Pb accumulation capacity of water spinach would not lower the nutritional values of the obtained Cd/Pb-PSCs from the breeding, which should be a pillar that supports the feasibility to minimize Cd/Pb pollution in vegetables using PSC-breeding method.

Bifocal sclerosing osteosarcoma: unusual presentation and course

International Nuclear Information System (INIS)

Abramovici, L.; Steiner, G.C.; Rosenberg, Z.; Kenan, S.

1998-01-01

Multifocal osteosarcoma is uncommon. Long-term survival of an incompletely treated case is exceptional. We report an unusual case of bifocal sclerosing osteosarcoma in a 38-year-old women that involved the left ilium and right proximal femur. The femoral lesion was resected. The tumor in the left ilium was not treated. She did not receive chemotherapy and has been free of metastases for 7 years. Recently, growth of the pelvic osteosarcoma has resulted in vascular compression and edema of the lower extremity. The patient's alkaline phosphatase has been elevated throughout. The tumor was HMB-45 positive, which has not been previously reported in osteosarcoma. The pathogenesis of multifocal osteosarcoma is discussed. (orig.)

Heart and bile acids - Clinical consequences of altered bile acid metabolism.

Science.gov (United States)

Vasavan, Tharni; Ferraro, Elisa; Ibrahim, Effendi; Dixon, Peter; Gorelik, Julia; Williamson, Catherine

2018-04-01

Cardiac dysfunction has an increased prevalence in diseases complicated by liver cirrhosis such as primary biliary cholangitis and primary sclerosing cholangitis. This observation has led to research into the association between abnormalities in bile acid metabolism and cardiac pathology. Approximately 50% of liver cirrhosis cases develop cirrhotic cardiomyopathy. Bile acids are directly implicated in this, causing QT interval prolongation, cardiac hypertrophy, cardiomyocyte apoptosis and abnormal haemodynamics of the heart. Elevated maternal serum bile acids in intrahepatic cholestasis of pregnancy, a disorder which causes an impaired feto-maternal bile acid gradient, have been associated with fatal fetal arrhythmias. The hydrophobicity of individual bile acids in the serum bile acid pool is of relevance, with relatively lipophilic bile acids having a more harmful effect on the heart. Ursodeoxycholic acid can reverse or protect against these detrimental cardiac effects of elevated bile acids. Copyright © 2018 Elsevier B.V. All rights reserved.

Sclerosing lipogranuloma of the eyelid: unusual complication following nasal packing in endoscopic sinus surgery.

Science.gov (United States)

Ramaswamy, Balakrishnan; Singh, Rohit; Manusrut, Manusrut; Hazarika, Manali

2015-03-06

An eyelid or orbital lipogranuloma can occur following nasal packing with liquid paraffin, petroleum jelly or an antibiotic-based cream. It usually presents a few weeks or months after the initial procedure. We present a report of three such cases of sclerosing lipogranuloma involving the eyelid, which occurred following a sinonasal surgery where nasal packing using petroleum jelly was performed. The typical clinical course and the classical histopathological features are highlighted. All cases were diagnosed by histopathological examination as sclerosing lipogranuloma. Complete surgical removal resulted in complete resolution on 1 month follow-up. The diagnosis is based on a high degree of suspicion following a detailed history of prior use of lipid-based products for nasal packing following endonasal surgery. Histopathology is diagnostic. Surgical excision is the treatment of choice, however, due to its infiltrative nature, it may be difficult to obtain a complete cure. 2015 BMJ Publishing Group Ltd.

Electrophysiological Analysis of human Pluripotent Stem Cell-derived Cardiomyocytes (hPSC-CMs) Using Multi-electrode Arrays (MEAs).

Science.gov (United States)

Sala, Luca; Ward-van Oostwaard, Dorien; Tertoolen, Leon G J; Mummery, Christine L; Bellin, Milena

2017-05-12

Cardiomyocytes can now be derived with high efficiency from both human embryonic and human induced-Pluripotent Stem Cells (hPSC). hPSC-derived cardiomyocytes (hPSC-CMs) are increasingly recognized as having great value for modeling cardiovascular diseases in humans, especially arrhythmia syndromes. They have also demonstrated relevance as in vitro systems for predicting drug responses, which makes them potentially useful for drug-screening and discovery, safety pharmacology and perhaps eventually for personalized medicine. This would be facilitated by deriving hPSC-CMs from patients or susceptible individuals as hiPSCs. For all applications, however, precise measurement and analysis of hPSC-CM electrical properties are essential for identifying changes due to cardiac ion channel mutations and/or drugs that target ion channels and can cause sudden cardiac death. Compared with manual patch-clamp, multi-electrode array (MEA) devices offer the advantage of allowing medium- to high-throughput recordings. This protocol describes how to dissociate 2D cell cultures of hPSC-CMs to small aggregates and single cells and plate them on MEAs to record their spontaneous electrical activity as field potential. Methods for analyzing the recorded data to extract specific parameters, such as the QT and the RR intervals, are also described here. Changes in these parameters would be expected in hPSC-CMs carrying mutations responsible for cardiac arrhythmias and following addition of specific drugs, allowing detection of those that carry a cardiotoxic risk.

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Phenotype and Clinical Course of Inflammatory Bowel Disease with Co-Existent Celiac Disease.

Science.gov (United States)

Tse, Chung Sang; Deepak, Parakkal; De La Fuente, Jaime; Bledsoe, Adam C; Larson, Joseph J; Murray, Joseph A; Papadakis, Konstantinos A

2018-05-07

Inflammatory bowel diseases, principally Crohn's disease and ulcerative colitis, and celiac disease are among the most common immune-mediated gastrointestinal diseases. We aim to elucidate the clinical course and outcomes of patients with concomitant inflammatory bowel disease and celiac disease, a unique population that remains scarcely studied to date. A retrospective matched case-control study of adults with coexistent inflammatory bowel disease and celiac disease was performed at a tertiary referral institution in North America. Logistic regression and Kaplan-Meier curves compared disease characteristics and clinical outcomes of the two groups. A total of 342 inflammatory bowel disease patients were included in this study, of which 114 had coexistent celiac disease and 228 did not. Patients with coexistent inflammatory bowel disease and celiac disease had higher rates of primary sclerosing cholangitis (19.3% vs 5.7%; odds ratio, 4.4; 95% confidence interval, 2.1-9.4; pceliac disease (10.5% vs 3.5%; odds ratio 3.2; 95% confidence interval 1.3-8.2; p=0.01), compared to patients without concomitant celiac disease. Patients with inflammatory bowel disease with concomitant celiac disease have unique phenotypic features compared to non-celiac inflammatory bowel disease, with higher risks for colitis-related hospitalizations, extensive colitis, and primary sclerosing cholangitis. Increased recognition of coexistent IBD and celiac disease can prompt clinicians to investigate for concomitant disease sooner, particularly in patients with seemingly refractory disease.

Molecular identification of protozoa causing AIDS-associated cholangiopathy in Buenos Aires, Argentina.

Science.gov (United States)

Nétor Velásquez, Jorge; Marta, Edgardo; Alicia di Risio, Cecilia; Etchart, Cristina; Gancedo, Elisa; Victor Chertcoff, Agustín; Bruno Malandrini, Jorge; Germán Astudillo, Osvaldo; Carnevale, Silvana

2012-12-01

Several species of microsporidia and coccidia are protozoa parasites responsible for cholan-giopathy disease in patients infected with human immunodeficiency virus (HIV). The goals of this work were to identift opportunistic protozoa by molecular methods and describe the clinical manifestations at the gastrointestinal tract and the biliary system in patients with AIDS-associated cholangiopathy from Buenos Aires, Argentina. This study included 11 adult HIV-infected individuals with diagnosis ofAIDS- associated cholangiopathy. An upper gastrointestinal endoscopy with biopsy specimen collection and a stool analysis for parasites were performed on each patient. The ultrasound analysis revealed bile ducts compromise. An endoscopic retrograde cholangiopancreatography and a magnetic resonance cholangiography were carried out. The identification to the species level was performed on biopsy specimens by molecular methods. Microorganisms were identified in 10 cases. The diagnosis in patients with sclerosing cholangitis was cryptosporidiosis in 3 cases, cystoisosporosis in 1 and microsporidiosis in 1. In patients with sclerosing cholangitis and papillary stenosis the diagnosis was microsporidiosis in 2 cases, cryptosporidiosis in 2 and cryptosporidiosis associated with microsporidiosis in 1. In 3 cases with cryptosporidiosis the species was Cryptosporidium hominis, 1 of them was associated with Enterocytozoon bieneusi, and the other 2 were coinfected with Cryptosporidium parvum. In the 4 cases with microsporidiosis the species was Enterocytozoon bieneusi. These results suggest that molecular methods may be useful tools to identify emerging protozoa in patients with AIDS-associated cholangiopathy.

Autoimmune hepatitis in children: what is different from adult AIH?

Science.gov (United States)

Mieli-Vergani, Giorgina; Vergani, Diego

2009-08-01

Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in the absence of a known etiology. Two types of childhood AIH are recognized according to seropositivity: smooth muscle antibody (SMA) and/or antinuclear antibody (ANA), which is AIH type 1; and antibodies to liver-kidney microsome type 1 (anti-LKM1), which is AIH type 2. There is a female predominance in both. Autoimmune hepatitis type 2 presents more acutely, at a younger age, and commonly with IgA deficiency; however, duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the two groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to nonadherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AIH type 1 patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. The differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis, liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress. Copyright Thieme Medical Publishers.

Contractile Defect Caused by Mutation in MYBPC3 Revealed under Conditions Optimized for Human PSC-Cardiomyocyte Function

Directory of Open Access Journals (Sweden)

Matthew J. Birket

2015-10-01

Full Text Available Maximizing baseline function of human pluripotent stem cell-derived cardiomyocytes (hPSC-CMs is essential for their effective application in models of cardiac toxicity and disease. Here, we aimed to identify factors that would promote an adequate level of function to permit robust single-cell contractility measurements in a human induced pluripotent stem cell (hiPSC model of hypertrophic cardiomyopathy (HCM. A simple screen revealed the collaborative effects of thyroid hormone, IGF-1 and the glucocorticoid analog dexamethasone on the electrophysiology, bioenergetics, and contractile force generation of hPSC-CMs. In this optimized condition, hiPSC-CMs with mutations in MYBPC3, a gene encoding myosin-binding protein C, which, when mutated, causes HCM, showed significantly lower contractile force generation than controls. This was recapitulated by direct knockdown of MYBPC3 in control hPSC-CMs, supporting a mechanism of haploinsufficiency. Modeling this disease in vitro using human cells is an important step toward identifying therapeutic interventions for HCM.

Emergency endoscopic needle-knife precut papillotomy in acute severe cholangitis resulting from impacted common bile duct stones at duodenal papilla.

Science.gov (United States)

Zheng, Mingwei; Liu, Xufeng; Li, Ning; Li, Wei-Zhi

2018-03-01

To evaluate the efficacy and safety of emergency endoscopic needle-knife precut papillotomy in acute severe cholangitis resulting from impacted common bile duct stones at duodenal papilla. Between January 2010 and January 2015, 118 cases of acute severe cholangitis with impacted common bile duct stones at the native papilla underwent emergency endoscopic retrograde cholangiopancreatography (ERCP) and early needle-knife precut papillotomy in a tertiary referral center. Precut techniques were performed according to the different locations of stones in the duodenal papilla. Clinical data about therapy and recovery of the 118 patients were recorded and analyzed. One hundred and eighteen patients underwent emergency ERCP within 24 h after hospitalization, with a total success rate of 100%. The mean operating time was 6.4 ± 4.1 min. Postoperative acute physiology and chronic health evaluation (APACHE) II scores, white blood cell count and liver function improved significantly. The complication rate was 4.2% (5/118); two with hemorrhage and three with acute pancreatitis. There was no procedure-related mortality. Emergency endoscopic needle-knife precut papillotomy is effective and safe for acute severe cholangitis resulting from impacted common bile duct stones at the duodenal papilla. Copyright © 2017 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

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How do PrPSc Prions Spread between Host Species, and within Hosts?

Directory of Open Access Journals (Sweden)

Neil A. Mabbott

2017-11-01

Full Text Available Prion diseases are sub-acute neurodegenerative diseases that affect humans and some domestic and free-ranging animals. Infectious prion agents are considered to comprise solely of abnormally folded isoforms of the cellular prion protein known as PrPSc. Pathology during prion disease is restricted to the central nervous system where it causes extensive neurodegeneration and ultimately leads to the death of the host. The first half of this review provides a thorough account of our understanding of the various ways in which PrPSc prions may spread between individuals within a population, both horizontally and vertically. Many natural prion diseases are acquired peripherally, such as by oral exposure, lesions to skin or mucous membranes, and possibly also via the nasal cavity. Following peripheral exposure, some prions accumulate to high levels within the secondary lymphoid organs as they make their journey from the site of infection to the brain, a process termed neuroinvasion. The replication of PrPSc prions within secondary lymphoid organs is important for their efficient spread to the brain. The second half of this review describes the key tissues, cells and molecules which are involved in the propagation of PrPSc prions from peripheral sites of exposure (such as the lumen of the intestine to the brain. This section also considers how additional factors such as inflammation and aging might influence prion disease susceptibility.

Ulcerative colitis and esclerosant cholangitis in children and adolescents; case report and literature revision

International Nuclear Information System (INIS)

Parra G, Giovanna S; Edelberto Mulett V; Mario Santacoloma O

2002-01-01

In spite of being considered rare in the pediatric practice, the intestinal inflammatory illness is recognized now with more frequency in children of all the ages. In fact, 25 to 30% of all the patients with Crohn illness, and 20% the patients with ulcerative colitis, they consult before the 20 years. A case is presented of patient of 15 years with primary esclerosant cholangitis

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File list: Pol.PSC.05.AllAg.hESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: Pol.PSC.10.AllAg.hESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Pol.PSC.10.AllAg.hESC_derived_neural_cells hg19 RNA polymerase Pluripotent stem cell hESC derived neural... cells SRX190259 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Pol.PSC.10.AllAg.hESC_derived_neural_cells.bed ...

File list: Oth.PSC.05.AllAg.iPS_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: Pol.PSC.50.AllAg.hESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Pol.PSC.50.AllAg.hESC_derived_neural_cells hg19 RNA polymerase Pluripotent stem cell hESC derived neural... cells SRX190259 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/Pol.PSC.50.AllAg.hESC_derived_neural_cells.bed ...

Computed tomographic findings of early subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Pedersen, H.; Wulff, C.H.; Rigshospitalet, Copenhagen

1982-01-01

Computed tomography of the brain (CT) was carried out at the early stages of subacute sclerosing panencephalitis (SSPE) in three children. The lateral ventricles were very small and the hemispheric sulci and interhemispheric fissures were not visible in all three patients in contrast to severe atrophy found at a later stage in one patient. The early CT abnormalities were revealed at the same time as the titres of measles antibodies in blood and cerebrospinal fluid were elevated, and the characteristic periodic complexes in the electroencephalogram established the diagnosis of SSPE. The CT changes indicating brain swelling reflect the reactive changes of this slow virus infection. (orig.)

Down's syndrome and inflammatory bowel disease: is there a real link?

Directory of Open Access Journals (Sweden)

Raquel Souto-Rodríguez

Full Text Available Down's syndrome (DS is a genetic disease that has been associated with several immune and autoimmune diseases, including digestive and liver diseases, like celiac disease, autoimmune chronic hepatitis and sclerosing cholangitis. Despite in inflammatory bowel disease (IBD pathogenesis, genetics and immune mechanism play an important role, the association among DS and IBD has been poorly studied. Data about IBD diagnosis in DS patients is very scarce with only some individual case-reports. We report three cases of DS patients diagnosed of IBD and we discuss the possible association of these two entities.

Human pancreatic islet-derived extracellular vesicles modulate insulin expression in 3D-differentiating iPSC clusters.

Directory of Open Access Journals (Sweden)

Diana Ribeiro

Full Text Available It has been suggested that extracellular vesicles (EVs can mediate crosstalk between hormones and metabolites within pancreatic tissue. However, the possible effect of pancreatic EVs on stem cell differentiation into pancreatic lineages remains unknown. Herein, human islet-derived EVs (h-Islet-EVs were isolated, characterized and subsequently added to human induced pluripotent stem cell (iPSC clusters during pancreatic differentiation. The h-islet-EVs had a mean size of 117±7 nm and showed positive expression of CD63 and CD81 EV markers as measured by ELISA. The presence of key pancreatic transcription factor mRNA, such as NGN3, MAFA and PDX1, and pancreatic hormone proteins such as C-peptide and glucagon, were confirmed in h-Islet-EVs. iPSC clusters were differentiated in suspension and at the end stages of the differentiation protocol, the mRNA expression of the main pancreatic transcription factors and pancreatic hormones was increased. H-Islet-EVs were supplemented to the iPSC clusters in the later stages of differentiation. It was observed that h-Islet-EVs were able to up-regulate the intracellular levels of C-peptide in iPSC clusters in a concentration-dependent manner. The effect of h-Islet-EVs on the differentiation of iPSC clusters cultured in 3D-collagen hydrogels was also assessed. Although increased mRNA expression for pancreatic markers was observed when culturing the iPSC clusters in 3D-collagen hydrogels, delivery of EVs did not affect the insulin or C-peptide intracellular content. Our results provide new information on the role of h-Islet-EVs in the regulation of insulin expression in differentiating iPSC clusters, and are highly relevant for pancreatic tissue engineering applications.

File list: His.PSC.10.H3K122ac.AllCell [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: His.PSC.05.H3K122ac.AllCell [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: His.PSC.50.H3K122ac.AllCell [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: DNS.PSC.50.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: His.PSC.50.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: His.PSC.05.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: His.PSC.10.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: DNS.PSC.10.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: DNS.PSC.20.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: DNS.PSC.05.AllAg.mESC_derived_pancreatic_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: Pol.PSC.20.AllAg.mESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: ALL.PSC.05.AllAg.hESC_derived_neural_crests [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: DNS.PSC.05.AllAg.hESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: Pol.PSC.05.AllAg.mESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Pol.PSC.05.AllAg.mESC_derived_neural_cells mm9 RNA polymerase Pluripotent stem cell mESC derived neural...RX213764,SRX213760 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Pol.PSC.05.AllAg.mESC_derived_neural_cells.bed ...

File list: DNS.PSC.50.AllAg.hESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: Pol.PSC.50.AllAg.mESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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File list: Pol.PSC.10.AllAg.mESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

Full Text Available Pol.PSC.10.AllAg.mESC_derived_neural_cells mm9 RNA polymerase Pluripotent stem cell mESC derived neural...RX213760,SRX213764 http://dbarchive.biosciencedbc.jp/kyushu-u/mm9/assembled/Pol.PSC.10.AllAg.mESC_derived_neural_cells.bed ...

File list: DNS.PSC.10.AllAg.hESC_derived_neural_cells [Chip-atlas[Archive

Lifescience Database Archive (English)

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