Primary sclerosing cholangitis

Directory of Open Access Journals (Sweden)

Marina G Silveira

2008-01-01

Full Text Available Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a ‘beaded’ appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

Directory of Open Access Journals (Sweden)

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.

Sclerosing cholangitis with ulcerative colitis in a Nigerian woman ...

African Journals Online (AJOL)

Primary Sclerosing Cholangitis (PSC) is a relatively rare cause of chronic liver disease worldwide. It presents as chronic cholestasis associated with jaundice and pruritus. We report a middle aged Nigerian woman who presented with cholestatic jaundice and diagnosed with PSC with concurrent ulcerative colitis based on ...

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

International Nuclear Information System (INIS)

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)

MMP-2 is a disease-modifying gene in primary sclerosing cholangitis

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Korkmaz, Kerem Sebib; de Rooij, Bert-Jan F.; van Hoek, Bart; Janse, Marcel; Coenraad, Minneke J.; van der Reijden, Johan J.; Weersma, Rinse K.; Porte, Robert J.; Voorneveld, Philip W.; Baranski, Andrzej G.; Verspaget, Hein W.

BackgroundPrimary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the bile ducts, frequently necessitating orthotopic liver transplantation (OLT), often accompanied by inflammatory bowel disease (IBD). Matrix metalloproteinases (MMPs) are associated with fibrotic diseases caused by

Primary sclerosing cholangitis and disease distribution in inflammatory bowel disease.

LENUS (Irish Health Repository)

O'Toole, Aoibhlinn

2012-04-01

The relationship between site of intestinal inflammation and primary sclerosing cholangitis (PSC) development in inflammatory bowel disease (IBD) has not been studied extensively, but may be important in understanding the pathogenesis of PSC. We aimed to determine patterns of disease distribution in IBD patients with and without PSC.

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

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.

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

 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.

Radiation diagnosis of patients with primary sclerosing cholangitis

International Nuclear Information System (INIS)

Sharipov, V.Sh.

2001-01-01

Results of combined examination of patients for the purpose of diagnosis of primary sclerosing cholangitis (PSC) are presented. Combined examination consisted of the following techniques: ultrasonography, routine X-ray contrast study of upper section of digestive system, relaxation duodenography, percutaneous transhepatic cholangiography, computerized tomography, endoscopic retrograde pancreatocholangiography. Peculiarities in X-ray PSC semiotics were revealed. It is shown that the combined examination and X-ray semiotics of the disease is of great significance for PSC preoperational diagnosis [ru

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

Applicability and prognostic value of histologic scoring systems in primary sclerosing cholangitis

NARCIS (Netherlands)

de Vries, Elisabeth M. G.; Verheij, Joanne; Hubscher, Stefan G.; Leeflang, Mariska M. G.; Boonstra, Kirsten; Beuers, Ulrich; Ponsioen, Cyriel Y.

2015-01-01

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. At present, there is no appropriate histologic scoring system available for PSC, evaluating both degree of necroinflammatory activity (grade) and fibrosis (stage). The aim of this study was to assess if three scoring

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

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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.

Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI

International Nuclear Information System (INIS)

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

2013-01-01

Background: It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. Purpose: To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Material and Methods: Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. Results: On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P = 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P = 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. Conclusion: The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC

Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI

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Kim; Jin Hee; Byun, Jae Ho; Kim, So Yeon; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon-Gyu [Dept. of Radiology and Research Inst. of Radiology, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)], e-mail: jhbyun@amc.seoul.kr; Kim, Myung-Hwan [Dept. of Internal Medicine, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)

2013-07-15

Background: It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. Purpose: To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Material and Methods: Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. Results: On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P = 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P = 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. Conclusion: The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC.

Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.

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Moon, Sung-Hoon; Kim, Myung-Hwan; Lee, Jong Kyun; Baek, Seunghee; Woo, Young Sik; Cho, Dong Hui; Oh, Dongwook; Song, Tae Jun; Park, Do Hyun; Lee, Sang Soo; Seo, Dong Wan; Lee, Sung Koo

2017-04-01

Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features. A scoring system was developed on the basis of these variables, and its performance was internally validated using a bootstrapping-based method. The scoring system in the final model included age (IgG4-SC. The discrimination between IgG4-SC and PSC using the scoring system was excellent (area under the receiver operating characteristic curve, 0.986). A reliable differentiation of IgG4-SC from PSC can be made using the scoring system presented here. We suggest the diagnosis of IgG4-SC at a cutoff of 7 points or higher and the indication of diagnostic steroid trial at 5 or 6 points. External validation of our scoring system is warranted.

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

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.

Science.gov (United States)

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.

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Development and validation of a primary sclerosing cholangitis-specific patient-reported outcomes instrument: The PSC PRO.

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Younossi, Zobair M; Afendy, Arian; Stepanova, Maria; Racila, Andrei; Nader, Fatema; Gomel, Rachel; Safer, Ricky; Lenderking, William R; Skalicky, Anne; Kleinman, Leah; Myers, Robert P; Subramanian, G Mani; McHutchison, John G; Levy, Cynthia; Bowlus, Christopher L; Kowdley, Kris; Muir, Andrew J

2017-11-20

Primary sclerosing cholangitis (PSC) is a chronic liver disease associated with inflammation and biliary fibrosis that leads to cholangitis, cirrhosis, and impaired quality of life. Our objective was to develop and validate a PSC-specific patient-reported outcome (PRO) instrument. We developed a 42-item PSC PRO instrument that contains two modules (Symptoms and Impact of Symptoms) and conducted an external validation. Reliability and validity were evaluated using clinical data and a battery of other validated instruments. Test-retest reliability was assessed in a subgroup of patients who repeated the PSC PRO after the first administration. One hundred two PSC subjects (44 ± 13 years; 32% male, 74% employed, 39% with cirrhosis, 14% with a history of decompensated cirrhosis, 38% history of depression, and 68% with inflammatory bowel disease [IBD]) completed PSC PRO and other PRO instruments (Short Form 36 V2 [SF-36], Chronic Liver Disease Questionnaire [CLDQ], Primary Biliary Cholangitis - 40 [PBC-40], and five dimensions [5-D Itch]). PSC PRO demonstrated excellent internal consistency (Cronbach alphas, 0.84-0.94) and discriminant validity (41 of 42 items had the highest correlations with their own domains). There were good correlations between PSC PRO domains and relevant domains of SF-36, CLDQ, and PBC-40 (R = 0.69-0.90; all P 0.05). Test-retest reliability was assessed in 53 subjects who repeated PSC PRO within a median (interquartile range) of 37 (27-47) days. There was excellent reliability for most domains with intraclass correlations (0.71-0.88; all P < 0.001). PSC PRO is a self-administered disease-specific instrument developed according to U.S. Food and Drug Administration guidelines. This preliminary validation study suggests good psychometric properties. Further validation of the instrument in a larger and more diverse sample of PSC patients is needed. (Hepatology 2017). © 2017 by the American Association for the Study of Liver Diseases.

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

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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.

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.

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

Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma.

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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.

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

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

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

Trigger mechanisms of secondary sclerosing cholangitis in critically ill patients.

Science.gov (United States)

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.

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

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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.

Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis: IgG4-related SC, PSC/AIH and PSC alone.

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Lian, Min; Li, Bo; Xiao, Xiao; Yang, Yue; Jiang, Pan; Yan, Li; Sun, Chunyan; Zhang, Jun; Wei, Yiran; Li, Yanmei; Chen, Weihua; Jiang, Xiang; Miao, Qi; Chen, Xiaoyu; Qiu, Dekai; Sheng, Li; Hua, Jing; Tang, Ruqi; Wang, Qixia; Eric Gershwin, M; Ma, Xiong

2017-08-01

There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65% of IgG4-SC patients with an 86% sensitivity and 98% specificity for diagnosis. IgG4-SC patients had a better treatment response at 6-month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4≧1.25 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease. Copyright © 2017 Elsevier B.V. All

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

Variations in primary sclerosing cholangitis across the age spectrum.

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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.

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

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

Science.gov (United States)

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.

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

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.

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

Science.gov (United States)

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.

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

International Nuclear Information System (INIS)

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

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

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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.

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.

Papillary bile duct dysplasia in primary sclerosing cholangitis.

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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.

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

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

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.

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.

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

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

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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.

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

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.

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.

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

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

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.

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

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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.

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

International Nuclear Information System (INIS)

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.

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

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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.

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.

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

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.

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.

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...

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/.

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

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.

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.

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.

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.

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.

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

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

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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.

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.

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

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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

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.

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

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

Energy Technology Data Exchange (ETDEWEB)

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.

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

Science.gov (United States)

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).

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.

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 ...

Hepatic Encephalopathy

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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 ...

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

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.

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...

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.

Science.gov (United States)

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.

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.

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 ...

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.

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.

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.

Primary Sclerosing Cholangitis (PSC)

Science.gov (United States)

... Liver Function Tests Clinical Trials Liver Transplant FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related ... the Liver The Progression of Liver Disease FAQs Medical Terminology HOW YOU CAN HELP Sponsorship Ways to Give ...

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...

Autoimmune liver serology: current diagnostic and clinical challenges.

Science.gov (United States)

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.

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.)

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.

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

Directory of Open Access Journals (Sweden)

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.

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

Sclerosing Stromal Tumor of Ovary: A Case Report

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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.

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

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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.

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

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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

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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...

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.

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

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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.

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.

Hepatic Encephalopathy

Medline Plus

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 ...

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

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

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

Genetic Contribution to the Pathogenesis of Primary Biliary Cholangitis

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Satoru Joshita

2017-01-01

Full Text Available Formerly termed primary biliary cirrhosis, primary biliary cholangitis (PBC is a chronic and progressive cholestatic liver disease characterized by the presence of antimitochondrial antibodies. Ursodeoxycholic acid (UDCA therapy is the most effective and approved treatment for PBC and leads to a favorable outcome in the vast majority of cases. Although the etiology of PBC has not yet been elucidated, human leukocyte antigen (HLA class II alleles have been consistently associated with disease onset for decades. Individuals in different geographic regions of the world may have varying susceptibility alleles that reflect indigenous triggering antigens. In this review, we describe the influence of HLA alleles and other gene polymorphisms on PBC along with the results of genome-wide association studies (GWAS on this disease.

Intraductal location of the sclerosing adenosis of the breast.

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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.

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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.

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-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.

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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

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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

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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

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

NARCIS (Netherlands)

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.)

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

NARCIS (Netherlands)

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.

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

Hepatobiliary fascioliasis: a case with unusual radiological features.

Science.gov (United States)

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.

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

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

Directory of Open Access Journals (Sweden)

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

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

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.

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

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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

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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.

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

NARCIS (Netherlands)

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

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.

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

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.

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.

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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).

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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.

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

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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

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)

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

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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.

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

MRI in subacute sclerosing panencephalitis

Energy Technology Data Exchange (ETDEWEB)

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

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].

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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.

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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.

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

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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

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

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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.

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

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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.

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

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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.

Obeticholic acid for the treatment of primary biliary cholangitis in adult patients: clinical utility and patient selection

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Bowlus CL

2016-09-01

Full Text Available Christopher L Bowlus Division of Gastroenterology and Hepatology, University of California Davis, Davis, CA, USA Abstract: Primary biliary cholangitis (PBC, previously known as primary biliary “cirrhosis”, is a rare autoimmune liver disease characterized by the hallmark autoantibodies to mitochondrial antigens and immune-mediated destruction of small bile duct epithelial cells leading to cholestasis and cirrhosis. Surprisingly, while immune modulators have not been effective in the treatment of PBC, supplementation with the hydrophilic bile acid (BA ursodeoxycholic acid (UDCA has been demonstrated to slow the disease progression. However, a significant minority of PBC patients do not have a complete response to UDCA and remain at risk of continued disease progression. Although the mechanisms of action are not well understood, UDCA provided proof of concept for BA therapy in PBC. Obeticholic acid (OCA, a novel derivative of the human BA chenodeoxycholic acid, is a potent agonist of the nuclear hormone receptor farnesoid X receptor, which regulates BA synthesis and transport. A series of clinical trials of OCA in PBC, primarily in combination with UDCA, have established that OCA leads to significant reductions in serum alkaline phosphatase that are predicted to lead to improved clinical outcomes, while dose-dependent pruritus has been the most common adverse effect. On the basis of these studies, OCA was given conditional approval by the US Food and Drug Administration with plans to establish the long-term clinical efficacy of OCA in patients with advanced PBC. Keywords: primary biliary cholangitis, nuclear receptors, farnesoid X receptor, bile acid, obeticholic acid, ursodeoxycholic acid

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)

MDM2 Amplification and PI3KCA Mutation in a Case of Sclerosing Rhabdomyosarcoma

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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.

What Comes after Ursodeoxycholic Acid in Primary Biliary Cholangitis?

Science.gov (United States)

Wong, Lin Lee; Hegade, Vinod S; Jones, David E J

2017-01-01

Primary biliary cholangitis (PBC) is a rare autoimmune liver disease characterized by chronic cholestasis. Treatment with the accepted primary therapy ursodeoxycholic acid (UDCA) has been shown to be associated with delayed disease progression probably through reduced impact of cholestatic injury on the target biliary epithelial cells. Patients with inadequate response to UDCA (which can be identified through validated biochemical criteria) are at increased risk of disease progression, need for liver transplantation, and death. Obeticholic acid (OCA) is a farnesoid X receptor (FXR) agonist which has been evaluated as a second-line therapy in PBC and has been recently licensed by the Food and Drug Administration and European Medicines Agency for use in patients showing an inadequate response to UDCA or who are unable to tolerate it. Although evidence for biochemical improvement by OCA is compelling, there is, as yet, no evidence that OCA improves hard clinical outcomes or quality of life. In addition, OCA may not be suitable for PBC patients with pruritus as it can worsen the symptom. Other novel agents currently in clinical development may have better side-effect profile. Fibrates have the potential but currently lack high quality evidence to support their routine clinical use in PBC. Symptom management of PBC is challenging and ASBT inhibitors and rituximab are being evaluated for pruritus and fatigue, respectively. © 2017 S. Karger AG, Basel.

Sclerosing Encapsulating Peritonitis; Review

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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

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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.

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

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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

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)

Toward precision medicine in primary biliary cholangitis.

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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.

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)

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.

Subareolar Sclerosing Ductal Hyperplasia.

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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.

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.

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.

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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.

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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.

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

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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?

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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.

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

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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.

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

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?

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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.

Successful treatment of recurrent cholangitis by constructing a hepaticojejunostomy with long Roux-en-Y limb in a long-term surviving patient after a Whipple procedure for pancreatic adenocarcinoma.

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Tsalis, Konstantinos; Antoniou, Nikolaos; Koukouritaki, Zambia; Patridas, Dimitrios; Sakkas, Leonidas; Kyziridis, Dimitrios; Lazaridis, Charalampos

2014-08-20

Female, 74. Recurrent cholangitis. -. -. -. Gastroenterology and Hepatology. Unusual clinical course. Cholangitis may result from biliary obstruction (e.g., biliary or anastomotic stenosis, or foreign bodies) or occur in the presence of normal biliary drainage. Although reflux of intestinal contents into the biliary tree after hepaticojejunostomy appears to be a rare complication, it is important to emphasize that there are few available surgical therapeutic techniques. A 74-year-old woman presented to our hospital after 17 years of episodes of cholangitis. The patient had undergone a pancreatoduodenectomy (Whipple procedure) 18 years earlier due to pancreatic adenocarcinoma. The reconstruction was achieved through the sequential placement of pancreatic, biliary, and retrocolic gastric anastomosis into the same jejunal loop. The postoperative course was uneventful and the patient received adjuvant chemotherapy. Approximately 6 months after the initial operation, the patient started having episodes of cholangitis. Over the next 17 years she experienced several febrile episodes presumed to be secondary to cholangitis. A computing tomography (CT) scan of the abdomen revealed intrahepatic bile ducts partially filled with orally administered contrast material (Gastrografin). Magnetic resonance cholangiopancreatography (MRCP) showed dilatation of the left intrahepatic bile ducts. A percutaneous transhepatic cholangiography showed that the bilioenteric anastomosis was normal, without stenosis. Based on these findings, a diagnosis of a short loop between the hepaticojejunostomy and the gastrojejunostomy permitting the reflux of intestinal juice into the biliary tree was made. During the re-operation, a new hepaticojejunal anastomosis in a 100-cm long Roux-en-Y loop was performed to prevent the reflux of the intestinal fluid into the biliary tree. The patient was discharged on postoperative day 10. One year after the second procedure, the patient enjoys good health and has

Case report 834: Chronic sclerosing osteomyelitis of the mandible with long bone periostitis.

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Stewart, A; Carneiro, R; Pollock, L; Shaw, D

1994-04-01

We present the case of a patient with primary chronic osteomyelitis of the mandible, the radiological appearance of which is compatible with a diagnosis of chronic sclerosing osteomyelitis. The accompanying femoral and tibial periosteal reactive new bone formation and the benign clinical course suggest that this presentation may represent a form of chronic multifocal osteomyelitis.

Effect of deferred or no treatment with ursodeoxycholic acid in patients with early primary biliary cholangitis.

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Tanaka, Atsushi; Hirohara, Junko; Nakano, Toshiaki; Yagi, Minami; Namisaki, Tadashi; Yoshiji, Hitoshi; Nakanuma, Yasuni; Takikawa, Hajime

2018-02-06

As primary biliary cholangitis (PBC) is a heterogeneous disease, we hypothesized that there is a population of patients with early PBC who do not require prompt treatment with ursodeoxycholic acid (UDCA). In this study, we analyzed data from a large-scale PBC cohort in Japan, and retrospectively investigated whether outcomes of early PBC patients were affected with prompt or deferred/no UDCA treatment. We defined early PBC as asymptomatic, serum alkaline phosphatase early PBC patients between the treatment regimens; prompt treatment group (UDCA was initiated within 1 year after diagnosis) and deferred/no treatment group (UDCA initiated >1 year after diagnosis or never initiated). Furthermore, we examined the outcomes of early PBC patients alternatively defined only with symptomatology and biochemistry. We identified 562 early PBC patients (prompt: n = 509; deferred/no treatment: n = 53). Incidence rates (per 1000 patient-years) for liver-related mortality or liver transplantation and decompensating events were 0.5 and 5.4, respectively, in the prompt treatment group, and 0 and 8.7, respectively, in the deferred/no treatment group. Multivariate analyses showed that age and bilirubin were significantly associated with developing decompensating events, whereas the prompt and deferred/no treatments were not. We obtained similar results in early PBC patients defined without histological examination. We showed that deferred/no treatment for early PBC patients did not affect the outcomes. This study provides a rationale for a future prospective, randomized study. © 2018 The Japan Society of Hepatology.

A case of autoimmune cholangitis misdiagnosed for cholangiocarcinoma: How to avoid unnecessary surgical intervention?

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Ignjatović Igor I.

2015-01-01

Full Text Available Introduction. Autoimmune cholangitis or immunoglobulin G4-associated cholangitis (IAC has been recently regarded as a new clinical and histopathological entity and is a part of a complex autoimmune disorder - IgG4-related systemic disease (ISD. ISD is an autoimmune disease with multi-organic involvement, characterized with IgG4-positive plasmocytic infiltration of various tissues and organs with a consequent sclerosis, which responds well to steroid therapy. Most commonly affected organs are the pancreas (autoimmune pancreatitis, [AIP] and the common bile duct (IAC. IAC and cholangiocarcinoma (CCA share many clinical, laboratory and imaging findings. Case Outline. We present a case of a 60-year-old male with a biliary stricture of a common bile duct, which was clinically considered as a bile duct carcinoma and treated surgically. Definite histopathological findings and immunohistochemistry revealed profound chronic inflammation, showing lymphoplasmacytic IgG-positive infiltration of a resected part of a common bile duct, highly suggestive for the diagnosis of IAC. In addition, postoperative IgG4 serum levels were also increased. Conclusion. It is of primary clinical importance to make a difference between IAC and CCA, in order to avoid unnecessary surgical intervention. Therefore, IAC should be considered in differential diagnosis in similar cases.

Pelvic pain in a young patient: Sclerosing stromal tumor

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Huriye Ayşe Parlakgümüş

2013-03-01

Full Text Available Introduction: Sclerosing stromal tumors are rare, benign sex chord stromal tumors. They are usually unilateral and are seen in second or third decades. The complaint at admission may be menstrual irregularity, pelvic pain, palpable pelvic mass, precocious puberty and postmenopausal bleeding. Because the complaint at admission and radiological findings are not specific to SSTs preoperative diagnosis is challenging. Herein we present the sonographical, intraoperative and histopathological findings of a SST diagnosed during laparoscopy in a patient who admitted with chronic pelvic pain and received pelvic inflammatory disease and endometriosis treatment and differential diagnosis of SSTs with the other ovarian tumors. Case report: 24 years old nulliparous patient first admitted to the gynecology department with the complaint of foul smelling vaginal discharge and pelvic pain. The diagnosis was pelvic inflammatory disease and the patient received antibiotics. The pelvic examination was normal except the mass in the right ovary which had similar echogenity to the ovary. Because of the pelvic pain the mass was assumed to be an endometrioma and the patient was prescribed an oral contraceptive treatment for 3 months. Because of the persistent pelvic pain a diagnostic laparoscopy was performed which revealed a 2 cm, pinkish- white, exophytic lesion originating from the right ovary. Pathological examination reported the mass to be a sclerosing stromal tumor. After the treatment the patient no longer complained of vaginal discharge but pelvic pain still persisted. After the operation the patient no longer complained of pelvic pain. Conclusion: Although SSTs are rare, they should be kept in mind when a young patient admits with menstrual irregularity, pelvic pain and hirsutism, particularly if the pain is refractory to treatment.

An experimental study for efficacy of acetic acid as a sclerosing agent

International Nuclear Information System (INIS)

Kim, Young Chan; Oh, Ju Hyung; Yoon, Yup; Ko, Young Tae; Choi, Woo Suk; Kim, Eui Jong; Lim, Joo Won

1997-01-01

To evaluate the efficacy of acetic acid as a sclerosing agent by observation of histologic change in urinary bladder epithelium after the instillation of acetic acid. Urinary bladder of the rabbit was catheterized with a Foley catheter, and acetic acid of 10%, 20%, 30%, 40% and 50% concentration was instilled for 5 minutes. After evacuation of the acid, the bladder was irrigated three times with normal saline. After two days, gross and histologic examinations of the bladder were performed. A bladder into which 10% acetic acid had been instilled revealed a nearly normal epithelium without denudation. In two cases, 20% acetic acid was instilled;one revealed partial denudation of the epithelium and the other revealed complete denudation. Mild to moderate interstitial edema and vascular congestion of the bladder wall were evident in all cases in which acid at a concentration of 30% or more had been instilled. In all cases in which the concentration of acid was greater than 30%, the epithelium was completely denuded. An acetic acid concentration of 40% or more is sufficient to completely destroy the epithelium of rabbit urinary bladder, and may be effective as a new sclerosing agent in cases of renal or hepatic cyst

Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid: A cytological dilemma

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Chayanika Pantola

2016-01-01

Full Text Available Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE of the thyroid is a rare primary thyroid tumor arising in a background of Hashimoto′s/lymphocytic thyroiditis and has been recently introduced in the World Health Organization (WHO classification of thyroid tumors. It is characterized by extensive sclerosis, squamous and glandular differentiation, and inflammatory infiltrate rich in eosinophil. Here, we are discussing the cytological features of this rare case in a 35-year-old female presented with thyroid swelling and lymph-node enlargement.

Differentiation of benign and malignant hilar bile duct stenosis.

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Liu, Xiaolei; Yang, Zhiying; Tan, Haidong; Shao, Chen; Liu, Liguo; Si, Shuang; Xu, Li; Sun, Yongliang

2016-06-15

Failure to differentiate benign and malignant hilar bile duct stenosis may lead to inappropriate treatment. We retrospectively analyzed the methods for differentiation. A total of 53 patients with hilar bile duct stenosis were included, comprising 41 malignant cases (hilar cholangiocarcinoma) and 12 benign cases (six primary sclerosing cholangitis and six IgG4-associated sclerosing cholangitis). Data of clinical histories, laboratory tests, imaging studies, and liver pathologies were collected, and comparison was made between benign and malignant groups. Compared with malignant group, patients in the benign group were more likely to have multiorgan involvement of clinical histories (P < 0.001). There was no difference on bilirubin, liver enzyme, and serum tumor marker between the two groups, whereas serum IgG4 levels were higher in the benign group (P = 0.003). Patients in the benign group were more likely to have pancreatic changes (P < 0.001) and multiple-segmental bile duct stenosis (P < 0.001) on imaging. Compared with the malignant group, patients in the benign group were more likely to show severe periportal inflammation in noninvolved liver (P < 0.001), fibrosis around intrahepatic bile duct (P < 0.001), and more IgG4-positive plasma cells (P < 0.001) on liver pathology. Benign lesion should be considered for patients with history of multiorgan involvement, pancreas changes, or multiple-segmental bile duct stenosis on imaging. Liver biopsy could be helpful for differential diagnosis before surgery. Copyright © 2016 Elsevier Inc. All rights reserved.

Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease.

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Nakanuma, Yasuni; Ishizu, Yoji; Zen, Yoh; Harada, Kenichi; Umemura, Takeji

2016-08-01

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system. Two types of hepatic parenchymal involvement have been reported in IgG4-RD: IgG4-related autoimmune hepatitis (AIH) and IgG4-hepatopathy. Moreover, only three cases of IgG4-related AIH have been reported. Immunoglobulin G4-related AIH is clinicopathologically similar to AIH, except for an elevated serum IgG4 level and heavy infiltration of IgG4-positive plasma cells in the liver tissue. Interestingly, IgG4-related AIH can be complicated by well-known IgG4-RD(s). Immunoglobulin G4-hepatopathy, which includes various histopathological lesions encountered in the liver of patients with type I autoimmune pancreatitis, is classified into five histological categories: portal inflammation, large bile duct damage, portal sclerosis, lobular hepatitis, and cholestasis. Immunoglobulin G4-hepatopathy is currently a collective term covering hepatic lesions primarily or secondarily related to IgG4-related sclerosing cholangitis and type 1 autoimmune pancreatitis. In conclusion, the liver is not immune to IgG4-RD, and at least two types of hepatic involvement in IgG4-RD have been reported: IgG4-related AIH and IgG4-hepatopathy. Additional studies are required to clarify their precise clinical significance with respect to IgG4-RD and inherent liver diseases. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Review article: the gut microbiome as a therapeutic target in the pathogenesis and treatment of chronic liver disease.

Science.gov (United States)

Woodhouse, C A; Patel, V C; Singanayagam, A; Shawcross, D L

2018-01-01

Mortality from chronic liver disease is rising exponentially. The liver is intimately linked to the gut via the portal vein, and exposure to gut microbiota and their metabolites translocating across the gut lumen may impact upon both the healthy and diseased liver. Modulation of gut microbiota could prove to be a potential therapeutic target. To characterise the changes in the gut microbiome that occur in chronic liver disease and to assess the impact of manipulation of the microbiome on the liver. We conducted a PubMed search using search terms including 'microbiome', 'liver' and 'cirrhosis' as well as 'non-alcoholic fatty liver disease', 'steatohepatitis', 'alcohol' and 'primary sclerosing cholangitis'. Relevant articles were also selected from references of articles and review of the ClinicalTrials.gov website. Reduced bacterial diversity, alcohol sensitivity and the development of gut dysbiosis are seen in several chronic liver diseases, including non-alcoholic fatty liver disease, alcohol-related liver disease and primary sclerosing cholangitis. Perturbations in gut commensals could lead to deficient priming of the immune system predisposing the development of immune-mediated diseases. Furthermore, transfer of stool from an animal with the metabolic syndrome may induce steatosis in a healthy counterpart. Patients with cirrhosis develop dysbiosis, small bowel bacterial overgrowth and increased gut wall permeability, allowing bacterial translocation and uptake of endotoxin inducing hepatic and systemic inflammation. Manipulation of the gut microbiota with diet, probiotics or faecal microbiota transplantation to promote the growth of "healthy" bacteria may ameliorate the dysbiosis and alter prognosis. © 2017 John Wiley & Sons Ltd.

Immunoglobulin G4-Related Sclerosing Disease Involving the Urethra: Case Report

International Nuclear Information System (INIS)

Choi, Jin Woo; KIm, Sang Youn; Cho, Jeong Yeon; Kim, Seung Hyup; Moon, Kyung Chul

2012-01-01

Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.

Differences in long-term survival among liver transplant recipients and the general population

DEFF Research Database (Denmark)

Åberg, Fredrik; Gissler, Mika; Karlsen, Tom H

2015-01-01

) (SMR change 23.1-9.2), hepatocellular carcinoma (HCC) (SMR 38.4-18.8), and primary sclerosing cholangitis (SMR 11.0-4.2), and deterioration in alcoholic liver disease (8.3-24.0) and acute liver failure (ALF) (5.9-7.6). SMRs for cancer and liver disease (recurrent or transplant-unrelated disease) were...... elevated in all indications except primary biliary cirrhosis (PBC). Absolute mortality rates underestimated the elevated premature mortality from infections (SMR 22-693) and kidney disease (SMR 13-45) across all indications, and from suicide in HCV and ALF. SMR for cardiovascular disease was significant...

Hepatic manifestations of celiac disease

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Hugh James Freeman

2010-05-01

Full Text Available Hugh James FreemanDepartment of Medicine (Gastroenterology, University of British Columbia, Vancouver, British Columbia, CanadaAbstract: Different hepatic and biliary tract disorders may occur with celiac disease. Some have been hypothesized to share genetic or immunopathogenetic factors, such as primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis. Other hepatic changes in celiac disease may occur with malnutrition resulting from impaired nutrient absorption, including hepatic steatosis. In addition, celiac disease may be associated with rare hepatic complications, such as hepatic T-cell lymphoma.Keywords: celiac disease, autoimmune liver disease, primary biliary cirrhosis, fatty liver, gluten-free diet

Chitosan–Sodium Tetradecyl Sulfate Hydrogel: Characterization and Preclinical Evaluation of a Novel Sclerosing Embolizing Agent for the Treatment of Endoleaks

Energy Technology Data Exchange (ETDEWEB)

Zehtabi, Fatemeh [École de technologie supérieure, Department of Mechanical Engineering (Canada); Dumont-Mackay, Vincent [Centre hospitalier de l’Université de Montréal, Département de pathologie (Canada); Fatimi, Ahmed [École de technologie supérieure, Department of Mechanical Engineering (Canada); Bertrand-Grenier, Antony; Héon, Hélène; Soulez, Gilles [Centre Hospitalier de l’Université de Montréal (CRCHUM), Research Centre (Canada); Lerouge, Sophie, E-mail: Sophie.lerouge@etsmtl.ca [École de technologie supérieure, Department of Mechanical Engineering (Canada)

2017-04-15

PurposeTo compare the efficacy of an embolization agent with sclerosing properties (made of chitosan and sodium tetradecyl sulfate, CH–STS) with a similar embolization agent but without sclerosing properties (made of chitosan, CH) in treating endoleaks in a canine endovascular aneurysm repair model.MethodsTwo chitosan-based radiopaque hydrogels were prepared, one with STS and one without STS. Their rheological, injectability, and embolizing properties were assessed in vitro; afterwards, their efficacy in occluding endoleaks was compared in a canine bilateral aneurysm model reproducing type I endoleaks (n = 9 each). The primary endpoint was endoleak persistence at 3 or 6 months, assessed on a CT scan and macroscopic examination. Secondary endpoints were the occurrence of stent-graft (SG) thrombosis, the evolution of the aneurysm mean diameter, as well as aneurysm healing and inflammation scores in pathology examinations.ResultsIn vitro experiments showed that both products gelled rapidly and presented initial storage moduli greater than 800 Pa, which increased with time. Both gels were compatible with microcatheter injection and occlude flow up to physiological pressure in vitro. In a type I endoleak model, the injection of CH–STS sclerosing gel tended to reduce the risk of occurrence of endoleaks, compared to CH non-sclerosing agent (2/9 vs. 6/9, p = 0.069). No case of SG thrombosis was observed. Moderate inflammation was found around both gels, with a comparable intensity score in both CH and CH–STS groups (2.6 ± 0.9 and 2.7 ± 0.9, respectively; p = 0.789).ConclusionsFlow occlusion combined with chemical endothelial denudation appears promising for the treatment of endoleaks.Level of EvidenceN/A.

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

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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.

Diagnosis of sclerosing hemangioma of lung: Don′t rely on fine-needle aspiration cytology diagnosis alone

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Kaushik Saha

2013-01-01

Full Text Available Sclerosing hemangioma is a rare variety of benign pulmonary neoplasm. It usually presents as asymptomatic, solitary, peripheral, circumscribed lesions in middle-aged women. Here, we describe a 46-year-old woman presenting to us for evaluation of right parahilar lung mass. Previous chest radiography done 10 years back showed a lung mass of almost similar size. Computed Tomography (CT-guided fine-needle aspiration cytology (FNAC was suggestive of adenocarcinoma of lung. A well-circumscribed, capsulated, ovoid mass measuring 5.6 cm × 4 cm × 3 cm, adjacent to the transverse fissure of the right lung was excised by lateral thoracotomy. Histopathological examination along with immunohistochemistry was suggestive of sclerosing hemangioma of lung. A pathologist must consider the clinicoradiological features before coming to a final diagnosis of lung malignancy from FNAC. Whenever there is any confusion regarding lung mass, thoracotomy must be done for arriving at an exact diagnosis from histopathology.

Echocardiographic evaluation of patients with subacute sclerosing panencephalitis

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Derya Çimen

2014-03-01

Full Text Available Objective: Subacute sclerosing panencephalitis is a slowly progressive, inflammatory and neurodegenerative disease caused by virus infection in the central nervous system. Since there are a limited number of studies in the literature evaluating the cardiovascular functions of patients with SSPE, the present study evaluates the patients with SSPE using tissue Doppler echocardiography and compares them between the control group in order to shed some light on the subject. Methods: The study is a prospective observational study. 49 patients (17 female, 32 male with SSPE were included in the study. Patients were divided into two groups: Stage 2 (n=29 and Stage 3 (n=20. Echocardiographic data were compared with a control group of 26 which is the same average age. All children underwent a detailed echocardiography, which contained an M-mode, pulse Doppler and tissue Doppler imaging. Results: Sinus tachycardia ( >100 beats/min in children was detected in nineteen (38.7% patients. There were not significant differences between parameters of systolic and diastolic function of the heart. Stage 2 group, EF: 69.9±6.4; SF: 39.2±5.58; and MPI (mitral: 0.38±0.03 and MPI (tricuspid: 0.39±0.10. And in the Stage 3 group, EF: 68.5±7.0, SF: 37.8±5.34, MPI (mitral: 0.37±0.09 and MPI (tricuspid: 0.38±0.12. In the control group EF:70.96±5.54; SF:39.96±5.05 and MPI(mitral: 0.35±0.06 MPI (tricuspid:0.36±0.04 and statistically meaningful differences were not found between patients and control groups (p >0.05. Conclusion: Cardiac functions may be preserved and cardiac functions constitute no significant risks of mortality in the advanced stages of patients with Subacute sclerosing panencephalitis, which is a group of chronic and bedridden patients.

Segmentectomy for giant pulmonary sclerosing haemangiomas with high serum KL-6 levels

OpenAIRE

Kuroda, Hiroaki; Mun, Mingyon; Okumura, Sakae; Nakagawa, Ken

2012-01-01

We describe a 61-year old female patient with a giant pulmonary sclerosing haemangioma (PSH) and an extremely high preoperative serum KL-6 level. During an annual health screening, the patient showed a posterior mediastinal mass on chest radiography. Chest computed tomography and magnetic resonance imaging revealed a well-circumscribed 60 mm diameter nodule with a marked contrast enhancement in the left lower lobe. The preoperative serum KL-6 level was elevated to 8204 U/ml. We performed a fo...

Is patient-reported outcome improved by nalfurafine hydrochloride in patients with primary biliary cholangitis and refractory pruritus? A post-marketing, single-arm, prospective study.

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Yagi, Minami; Tanaka, Atsushi; Namisaki, Tadashi; Takahashi, Atsushi; Abe, Masanori; Honda, Akira; Matsuzaki, Yasushi; Ohira, Hiromasa; Yoshiji, Hitoshi; Takikawa, Hajime

2018-04-16

Patients with primary biliary cholangitis (PBC) frequently suffer from pruritus, which can severely impair their health-related quality of life (HRQOL). Nalfurafine hydrochloride, a selective κ-opioid receptor agonist, was recently approved in Japan for refractory pruritus in patients with chronic liver diseases, but it still remains unclear whether this treatment improves the patient-reported outcome (PRO) in PBC patients with refractory pruritus. Herein, we conducted a multicenter, post-marketing, single-arm prospective study to investigate the efficacy of nalfurafine in terms of PRO, and the associations of the efficacy with any clinical characteristics. After screening for pruritus in 496 patients with PBC using PBC-40 and the visual analog scale (VAS), we identified 141 patients with moderate to severe pruritus; these were invited to participate in the study. The participants received 2.5 μg nalfurafine once daily for 12 weeks, and pruritus and HRQOL were assessed in week 12 of this treatment. Generic HRQOL, short form 36, blood chemistries, and serum autotaxin levels were also measured at baseline and at week 12. Forty-four patients participated in this study. The mean PBC-40 itch domain scores and VAS declined during the study period, from 8.56 to 7.63 (P = 0.041) and from 42.9 to 29.3 (P = 0.001) at baseline and at week 12, respectively, indicating a significant effect of nalfurafine. The other domains of PBC-40 and all domains of SF-36 were not significantly altered by this treatment. We failed to find any association between the change in VAS and PBC-40 itch scores and any clinical variable. Serum autotaxin levels were significantly increased during the study period. This study demonstrated that nalfurafine improved pruritus in patients with PBC, independent of their clinical characteristics, but had a limited effect on the PRO.

Platynosomum fastosum-induced chronic intrahepatic cholangitis and Spirometra spp. infections in feral cats from Grand Cayman.

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Headley, S A; Gillen, M A; Sanches, A W D; Satti, M Z

2012-06-01

The occurrence of platynosomiasis and intestinal sparganosis is described in feral cats from Grand Cayman, Cayman Islands. Spirometra spp. was observed within the intestine of 18.18% (10/55) of cats; 1.18% (1/55) of cats demonstrated gross and histological manifestation of parasitism by Platynosomum fastosum, but 14.5% (8/55) of cats had the characteristic pathological manifestations of P. fastosum-induced intrahepatic cholangitis without the concomitant presence of the intraductal trematode. Combined parasitism (Spirometra spp. and P. fastosum) was observed in 9.09% (5/55) of feral cats. Significant pathological findings were only associated with the hepatic fluke, P. fastosum, and were grossly characterized by moderate hepatomegaly with enlarged and dilated bile ducts. Examples of cestodes with morphological features characteristic of Spirometra spp. were observed within the small intestine without any associated pathological lesion. The histopathological evaluation of liver fragments revealed chronic intrahepatic cholangitis with and without the associated intraductal trematode, and was characterized by marked periductal fibrosis, adenomatous proliferation of bile duct epithelium, dilation of intrahepatic bile ducts and portal accumulations of inflammatory cells. The occurrence of the cestode in feral cats coupled with factors that are unique to Grand Cayman makes this island the ideal location for sporadic cases of human sparganosis.

Recurrent cholangitis in the tropics: Worm or cast?

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Jain P

2010-01-01

Full Text Available The development of biliary casts is very rare, especially in non-liver transplant patients. The etiology of these casts is uncertain but several factors have been proposed which lead to bile stasis and/or gallbladder hypo-contractility and promote cast formation. Here, we report a 54-year-old male, with diabetes and ischemic heart disease, who presented with recurrent attacks of cholangitis. Magnetic resonance cholangiopancreatography revealed linear T1 hyperintense and T2 hypointense filling defects in the right and left hepatic ducts extending into the common hepatic duct, and a calculus in the lower common bile duct, raising a suspicion of worm in the biliary tree. In view of failed attempts at extraction on endoscopy, patient underwent surgery. At exploration, biliary casts and stones were extracted from the proximal and the second order bile ducts, with the help of intraoperative choledochoscopy and a bilio-enteric anastomosis was accomplished. Although endoscopic retrieval of the biliary cast can be employed as first-line management, surgery should be considered in case it fails.

Case report. Sclerosing peritoneal mesothelioma in a dog: histopathological, histochemical and immunohistochemical investigations

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Anna Rita D'Angelo

2014-12-01

Full Text Available Mesotheliomas are rare neoplasm affecting on rare occasions both animals and humans and which arise from the mesothelial cells lining the coelomic cavities. We report herein the histopathological, histochemical and immunohistochemical findings in a dog affected by sclerosing peritoneal mesothelioma, a rare variant of canine mesothelioma, and submitted to laparotomy in December 2012 (Teramo, Italy. Our data confirm that mesothelioma still represents a diagnostic challenge and that immunohistochemistry can be extremely useful as supportive diagnostic technique.

Optimising the clinical strategy for autoimmune liver diseases: Principles of value-based medicine.

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Carbone, Marco; Cristoferi, Laura; Cortesi, Paolo Angelo; Rota, Matteo; Ciaccio, Antonio; Okolicsanyi, Stefano; Gemma, Marta; Scalone, Luciana; Cesana, Giancarlo; Fabris, Luca; Colledan, Michele; Fagiuoli, Stefano; Ideo, Gaetano; Belli, Luca Saverio; Munari, Luca Maria; Mantovani, Lorenzo; Strazzabosco, Mario

2018-04-01

Autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis represent the three major autoimmune liver diseases (AILDs). Their management is highly specialized, requires a multidisciplinary approach and often relies on expensive, orphan drugs. Unfortunately, their treatment is often unsatisfactory, and the care pathway heterogeneous across different centers. Disease-specific clinical outcome indicators (COIs) able to evaluate the whole cycle of care are needed to assist both clinicians and administrators in improving quality and value of care. Aim of our study was to generate a set of COIs for the three AILDs. We then prospectively validated these indicators based on a series of consecutive patients recruited at three tertiary clinical centers in Lombardy, Italy. In phase I using a Delphi method and a RAND 9-point appropriateness scale a set of COIs was generated. In phase II the indicators were applied in a real-life dataset. Two-hundred fourteen patients were enrolled and followed-up for a median time of 54months and the above COIs were recorded using a web-based electronic medical record program. The COIs were easy to collect in the clinical practice environment and their values compared well with the available natural history studies. We have generated a comprehensive set of COIs which sequentially capture different clinical outcome of the three AILDs explored. These indicators represent a critical tool to implement a value-based approach to patients with these conditions, to monitor, compare and improve quality through benchmarking of clinical performance and to assess the significance of novel drugs and technologies. This article is part of a Special Issue entitled: Cholangiocytes in Health and Diseaseedited by Jesus Banales, Marco Marzioni, Nicholas LaRusso and Peter Jansen. Copyright © 2017. Published by Elsevier B.V.

A case of nivolumab-related cholangitis and literature review: how to look for the right tools for a correct diagnosis of this rare immune-related adverse event.

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Gelsomino, Francesco; Vitale, Giovanni; Ardizzoni, Andrea

2018-02-01

Anti-programmed cell death-1 (PD-1) monoclonal antibodies, such as nivolumab, used for the treatment of several tumors, can trigger effector T-cells against tumor- and self-antigens, leading to the occurrence of different immune-related adverse events. Among them, liver injuries are rare and usually transient. To date, only four cases of immune-related cholangitis in non-small cell lung cancer (NSCLC) patients have been described during nivolumab treatment. Here, we describe laboratory tests, imaging and liver biopsy features that confirm this diagnosis as opposed to other forms of autoimmune liver disease; nevertheless, we also provide evidence of the presence of different clinical-pathological patterns of immune-related cholangitis.

Autoimmune liver disease and therapy in childhood

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Matjaž Homan

2013-10-01

Full Text Available Autoimmune hepatitis is a chronic immune-mediated disease of the liver. In childhood, autoimmune liver disorders include autoimmune hepatitis type I and II, autoimmune sclerosing cholangitis, Coombs-positive giant cell hepatitis, and de novo autoimmune hepatitis after liver transplantation. Autoimmune liver disease has a more aggressive course in children, especially autoimmune hepatitis type II. Standard therapy is a combination of corticosteroids and azathioprine. Around 80 % of children with autoimmune liver disease show a rapid response to combination therapy. The non-responders are treated with more potent drugs, otherwise autoimmune disease progresses to cirrhosis of the liver and the child needs liver transplantation as rescue therapy.

Comparison of two editions of Tokyo guidelines for the management of acute cholangitis.

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Sun, Gang; Han, Lu; Yang, Yunsheng; Linghu, Enqiang; Li, Wen; Cai, Fengchun; Kong, Jinyan; Wang, Xiangdong; Meng, Jiangyun; Du, Hong; Wang, Hongbin; Huang, Qiyang; Hyder, Quratulain; Zhang, Xiuli

2014-02-01

The Tokyo guidelines from 2007 (TG07) and 2013 (TG13) were compared for the management of acute cholangitis (AC). We reviewed patients with clinically-proven AC by detecting purulent biles during biliary drainage. TG07 and TG13 were compared regarding diagnosis, severity grading and prognostic values. New risk factors for 30-day mortality were investigated. Definite diagnosis for 120 eligible patients was made in 104 (86.7%) and 101 (84.2%) cases by TG07 and TG13, respectively (P = 0.36), higher than 61 (50.8%) by Charcot's triad (P < 0.001). Diagnostic overlap and concordance (κ) are 90.8% (109/120) and 0.63 (P < 0.0001). Patients classified into mild and moderate grades by TG07 and TG13 differed significantly (P = 0.043). Both guidelines could not predict clinical outcomes except the needs for multi ERCP session by TG13. Intrahepatic obstruction (OR = 11.2, 95% CI: 1.55-226.9) and hypoalbuminemia (≤ 25.0 g/l; OR = 17.3, 95% CI: 3.5-313.6) were independent risk factors for 30-day mortality in multivariate model. Two guidelines are reproducible and reliable in AC diagnosis but different in severity grading. TG13 are more practical for immediate severity grading, enabling planning treatment upon admission. Intrahepatic obstruction is a new candidate predictor of 30-day mortality for further assessment. © 2013 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

Timing and modality of the sclerosing agents binding to the human proteins: laboratory analysis and clinical evidences

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Lorenzo Tessari

2014-07-01

Full Text Available Sclerosing agents (SA are blood inactivated. Nevertheless, investigations concerning the interaction among SA and blood components have never been deeply investigated. Aim of the study is to precisely identify SA blood ligands, to determine their binding time and to highlight the clinical consequences. Thirty-one blood samples were collected from chronic venous disease patients and tested by capillary and agarose gel (AGE electrophoresis before and after adding polidocanol (POL and sodiumtetradecylsulphate (STS. The two different types of electrophoresis allowed an evaluation of the blood proteins binding with the sclerosing agents, with a reaction time lower than 8 seconds for the AGE. Subsequently six patients underwent foam sclerotherapy and then were subdivided in group A (4 patients and B (2 patients. In group A blood sample was obtained from the ipsilateral brachial vein immediately before (T0 and repeated 1, 3, 5, and 10 minutes after injection of STS 3% injection into the GSV. In group B, the same procedure was performed with the same timing from the ipsilateral femoral vein. Free STS (fSTS and total proteinbound STS (bSTS were measured. POL mainly binds to β-globulins (11%, while STS to albumin and α-globulins (62.6% and 30.7% on the protidogram, respectively. Both in the brachial and in the femoral vein, the average fSTS was always 0. STS binds to albumin (62.6% and α-globulins (30.7%, while POL is bound mainly by the b-globulins (11%. The present paper demonstrates how the vast majority of the sclerosing agent is bound to the blood proteins, suggesting the need to look for possible sclerotherapy complications factors also in the used gas and/or in the subsequent cathabolites release.

Raine Syndrome (OMIM #259775), Caused By FAM20C Mutation, Is Congenital Sclerosing Osteomalacia With Cerebral Calcification (OMIM 259660).

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Whyte, Michael P; McAlister, William H; Fallon, Michael D; Pierpont, Mary Ella; Bijanki, Vinieth N; Duan, Shenghui; Otaify, Ghada A; Sly, William S; Mumm, Steven

2017-04-01

In 1985, we briefly reported infant sisters with a unique, lethal, autosomal recessive disorder designated congenital sclerosing osteomalacia with cerebral calcification. In 1986, this condition was entered into Mendelian Inheritance In Man (MIM) as osteomalacia, sclerosing, with cerebral calcification (MIM 259660). However, no attestations followed. Instead, in 1989 Raine and colleagues published an affected neonate considering unprecedented the striking clinical and radiographic features. In 1992, "Raine syndrome" entered MIM formally as osteosclerotic bone dysplasia, lethal (MIM #259775). In 2007, the etiology emerged as loss-of-function mutation of FAM20C that encodes family with sequence similarity 20, member C. FAM20C is highly expressed in embryonic calcified tissues and encodes a kinase (dentin matrix protein 4) for most of the secreted phosphoproteome including FGF23, osteopontin, and other regulators of skeletal mineralization. Herein, we detail the clinical, radiological, biochemical, histopathological, and FAM20C findings of our patients. Following premortem tetracycline labeling, the proposita's non-decalcified skeletal histopathology after autopsy indicated no rickets but documented severe osteomalacia. Archival DNA revealed the sisters were compound heterozygotes for a unique missense mutation and a novel deletion in FAM20C. Individuals heterozygous for the missense mutation seemed to prematurely fuse their metopic suture and develop a metopic ridge sometimes including trigonocephaly. Our findings clarify FAM20C's role in hard tissue formation and mineralization, and show that Raine syndrome is congenital sclerosing osteomalacia with cerebral calcification. © 2016 American Society for Bone and Mineral Research. © 2016 American Society for Bone and Mineral Research.

Microbubbles in macrocysts - Contrast-enhanced ultrasound assisted sclerosant therapy of a congenital macrocystic lymphangioma: a case report.

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Menendez-Castro, Carlos; Zapke, Maren; Fahlbusch, Fabian; von Goessel, Heiko; Rascher, Wolfgang; Jüngert, Jörg

2017-07-06

Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application. Here we report the case of 2 month old male patient with a large thoracic congenital cystic lymphangioma. Preinterventional imaging of the malformation was performed by contrast-enhanced ultrasound after intracavitary application of SonoVue® immediately followed by a successful sclerotherapy with OK-432. Contrast agent-enhanced ultrasound imaging offers a valuable option to preinterventionally clarify the anatomic specifications of a congenital cystic lymphangioma in more detail than by single conventional sonography. By the exact knowledge about the composition and especially about the intercystic communications of the lymphangioma sclerosant therapy becomes safer and more efficient.

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...

An Optimized Set of Fluorescence In Situ Hybridization Probes for Detection of Pancreatobiliary Tract Cancer in Cytology Brush Samples.

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Barr Fritcher, Emily G; Voss, Jesse S; Brankley, Shannon M; Campion, Michael B; Jenkins, Sarah M; Keeney, Matthew E; Henry, Michael R; Kerr, Sarah M; Chaiteerakij, Roongruedee; Pestova, Ekaterina V; Clayton, Amy C; Zhang, Jun; Roberts, Lewis R; Gores, Gregory J; Halling, Kevin C; Kipp, Benjamin R

2015-12-01

Pancreatobiliary cancer is detected by fluorescence in situ hybridization (FISH) of pancreatobiliary brush samples with UroVysion probes, originally designed to detect bladder cancer. We designed a set of new probes to detect pancreatobiliary cancer and compared its performance with that of UroVysion and routine cytology analysis. We tested a set of FISH probes on tumor tissues (cholangiocarcinoma or pancreatic carcinoma) and non-tumor tissues from 29 patients. We identified 4 probes that had high specificity for tumor vs non-tumor tissues; we called this set of probes pancreatobiliary FISH. We performed a retrospective analysis of brush samples from 272 patients who underwent endoscopic retrograde cholangiopancreatography for evaluation of malignancy at the Mayo Clinic; results were available from routine cytology and FISH with UroVysion probes. Archived residual specimens were retrieved and used to evaluate the pancreatobiliary FISH probes. Cutoff values for FISH with the pancreatobiliary probes were determined using 89 samples and validated in the remaining 183 samples. Clinical and pathologic evidence of malignancy in the pancreatobiliary tract within 2 years of brush sample collection was used as the standard; samples from patients without malignancies were used as negative controls. The validation cohort included 85 patients with malignancies (46.4%) and 114 patients with primary sclerosing cholangitis (62.3%). Samples containing cells above the cutoff for polysomy (copy number gain of ≥2 probes) were classified as positive in FISH with the UroVysion and pancreatobiliary probes. Multivariable logistic regression was used to estimate associations between clinical and pathology findings and results from FISH. The combination of FISH probes 1q21, 7p12, 8q24, and 9p21 identified cancer cells with 93% sensitivity and 100% specificity in pancreatobiliary tissue samples and were therefore included in the pancreatobiliary probe set. In the validation cohort of

Toxicogenomic analysis reveals profibrogenic effects of trichloroethylene in autoimmune-mediated cholangitis in mice.

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Kopec, Anna K; Sullivan, Bradley P; Kassel, Karen M; Joshi, Nikita; Luyendyk, James P

2014-10-01

Epidemiological studies suggest that exposure to environmental chemicals increases the risk of developing autoimmune liver disease. However, the identity of specific chemical perpetrators and the mechanisms whereby environmental chemicals modify liver disease is unclear. Previous studies link exposure to trichloroethylene (TCE) with the development of autoimmune liver disease and exacerbation of autoimmunity in lupus-prone MRL mice. In this study, we utilized NOD.c3c4 mice, which spontaneously develop autoimmune cholangitis bearing resemblance to some features of primary biliary cirrhosis. Nine-week-old female NOD.c3c4 mice were given TCE (0.5 mg/ml) or its vehicle (1% Cremophor-EL) in drinking water for 4 weeks. TCE had little effect on clinical chemistry, biliary cyst formation, or hepatic CD3+ T-cell accumulation. Hepatic microarray profiling revealed a dramatic suppression of early growth response 1 (EGR1) mRNA in livers of TCE-treated mice, which was verified by qPCR and immunohistochemical staining. Consistent with a reported link between reduced EGR1 expression and liver fibrosis, TCE increased hepatic type I collagen (COL1A1) mRNA and protein levels in livers of NOD.c3c4 mice. In contrast, TCE did not increase COL1A1 expression in NOD.ShiLtJ mice, which do not develop autoimmune cholangitis. These results suggest that in the context of concurrent autoimmune liver disease with a genetic basis, modification of hepatic gene expression by TCE may increase profibrogenic signaling in the liver. Moreover, these studies suggest that NOD.c3c4 mice may be a novel model to study gene-environment interactions critical for the development of autoimmune liver disease. © The Author 2014. Published by Oxford University Press on behalf of the Society of Toxicology. All rights reserved. For permissions, please email: journals.permissions@oup.com.

Molecular and radiological diagnosis of sclerosing bone dysplasias

International Nuclear Information System (INIS)

Hul, Wim van; Vanhoenacker, Filip; Balemans, Wendy; Janssens, Katrien; Schepper, A.M. de

2001-01-01

Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights

Molecular and radiological diagnosis of sclerosing bone dysplasias

Energy Technology Data Exchange (ETDEWEB)

Hul, Wim van E-mail: vhul@uia.ac.be; Vanhoenacker, Filip; Balemans, Wendy; Janssens, Katrien; Schepper, A.M. de

2001-12-01

Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights.

Garre's chronic diffuse sclerosing osteomyelitis of the sacrum: a rare condition mimicking malignancy.

LENUS (Irish Health Repository)

Nasir, N

2012-02-03

Garre\\'s chronic diffuse sclerosing osteomyelitis (DSOM) is a rare disease that occurs most commonly in the mandible. We present a case of sacral DSOM that simulated an expanding destructive sacral tumour. Treatment was conducted on the basis of the available experience with the mandibular form of the disease, with partial symptomatic relief, but progressive sclerosis of the sacral lesion. To the best of our knowledge, this is the first case initially presenting in the sacrum. As an osteolytic expanding lesion simulating malignancy, it is important to recognize this entity in the sacrum.

Biliary fascioliasis – an uncommon cause of recurrent biliary colics: Report of a case and brief review [Biliäre Fasciolose – eine ungewöhnliche Ursache für wiederholte Gallenkoliken: Bericht eines Falles mit einer kurzen Übersicht

Directory of Open Access Journals (Sweden)

All, Gamal Nasr Ahmed Abdel

2012-05-01

Full Text Available [english]
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 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.[german]
In endemischen Gebieten ist der Parasitenbefall von Gallengängen eine häufige Ursache für Gallenkoliken. Aufgrund von Migration und Reisetätigkeiten kommen derartige Erkrankungen auch außerhalb endemischer Gebiete vor. Das Spektrum der Symptome bei Gallengangsfasciolose erstreckt sich von wiederholten Gallenkoliken bis zur akuten Cholangitis. Als Langzeitkomplikationen werden Gallensteine, sklerosierende Cholangitis und biliäre Zirrhose beschrieben. Wir berichten über eine Fasciolose als Ursache für Gallenkoliken bei einem jungen Mann, der über vier Jahre mehrfach wegen Gallenkoliken stationär behandelt wurde. Das Untersuchungsprofil brachte bisher jedes Mal keinen Erfolg, wenn er stationär wegen Bauchschmerzen aufgenommen wurde. Er hatte vorher niemals eine Cholangitis auf der Basis eines Befalls mit dem Leberegel. Nur durch endoskopisch retrograde

Surviving Sengstaken.

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Jayakumar, S; Odulaja, A; Patel, S; Davenport, M; Ade-Ajayi, N

2015-07-01

To report the outcomes of children who underwent Sengstaken-Blakemore tube (SBT) insertion for life-threatening haemetemesis. Single institution retrospective review (1997-2012) of children managed with SBT insertion. Patient demographics, diagnosis and outcomes were noted. Data are expressed as median (range). 19 children [10 male, age 1 (0.4-16) yr] were identified; 18 had gastro-oesophageal varices and 1 aorto-oesophageal fistula. Varices were secondary to: biliary atresia (n=8), portal vein thrombosis (n=5), alpha-1-anti-trypsin deficiency (n=1), cystic fibrosis (n=1), intrahepatic cholestasis (n=1), sclerosing cholangitis (n=1) and nodular hyperplasia with arterio-portal shunt (n=1). Three children deteriorated rapidly and did not survive to have post-SBT endoscopy. The child with an aortooesophageal fistula underwent aortic stent insertion and subsequently oesophageal replacement. Complications included gastric mucosal ulceration (n=3, 16%), pressure necrosis at lips and cheeks (n=6, 31%) and SBT dislodgment (n=1, 6%). Six (31%) children died. The remaining 13 have been followed up for 62 (2-165) months; five required liver transplantation, two underwent a mesocaval shunt procedure and 6 have completed endoscopic variceal obliteration and are under surveillance. SBT can be an effective, albeit temporary, life-saving manoeuvre in children with catastrophic haematemesis. Copyright © 2015 Elsevier Inc. All rights reserved.

Antibodies to SS-A/Ro-52kD and centromere in autoimmune liver disease: a clue to diagnosis and prognosis of primary biliary cirrhosis.

Science.gov (United States)

Granito, A; Muratori, P; Muratori, L; Pappas, G; Cassani, F; Worthington, J; Ferri, S; Quarneti, C; Cipriano, V; de Molo, C; Lenzi, M; Chapman, R W; Bianchi, F B

2007-09-15

Primary biliary cirrhosis (PBC) may be associated with various rheumatological disorders. To investigate the frequency and significance of 'rheumatological' antinuclear antibodies in the field of autoimmune chronic liver disease, with special regard to PBC. We studied 105 patients with PBC, 162 autoimmune liver disease controls (type 1 and 2 autoimmune hepatitis, primary sclerosing cholangitis), 30 systemic lupus erythematosus and 50 blood donors. Sera were tested for the presence of antibodies to extractable nuclear antigens (anti-ENA) by counterimmunoelectrophoresis, enzyme-linked and immunoblot (IB) assay, and for the presence of anti-centromere antibodies (ACA) by indirect immunofluorescence on HEp-2 cells and IB. The overall prevalence of IB-detected anti-ENA in PBC (30%) was higher than in type 1 autoimmune hepatitis (2.5%, P < 0.0001), type 2 autoimmune hepatitis (0%, P < 0.0001) and primary sclerosing cholangitis (11.5%, P = 0.006) and lower than in systemic lupus erythematosus (53%, P = 0.03). The most frequent anti-ENA reactivity in PBC was anti-SSA/Ro-52kD (28%). ACA were detected by IB in 21% PBC patients and never in the other subjects (P < 0.0001). Anti-SS-A/Ro/52kD positive PBC patients had at the time of diagnosis a more advanced histological stage (P = 0.01) and higher serum levels of bilirubin (P = 0.01) and IgM (P = 0.03) compared with negative ones. In the autoimmune liver disease setting, anti-SS-A/Ro-52kD and ACA have a high specificity for PBC and can thus be of diagnostic relevance in anti-mitochondrial antibodies negative cases. If confirmed in further studies with adequate follow-up, anti-SS-A/Ro-52kD antibodies might identify PBC patients with a more advanced and active disease.

Anti-Saccharomyces cerevisiae and perinuclear anti-neutrophil cytoplasmic antibodies in coeliac disease before and after gluten-free diet.

Science.gov (United States)

Granito, A; Zauli, D; Muratori, P; Muratori, L; Grassi, A; Bortolotti, R; Petrolini, N; Veronesi, L; Gionchetti, P; Bianchi, F B; Volta, U

2005-04-01

Anti-Saccharomyces cerevisiae and perinuclear anti-neutrophil cytoplasmic autoantibodies are markers of Crohn's disease and ulcerative colitis respectively. To determine the prevalence of anti-S. cerevisiae and perinuclear anti-neutrophil cytoplasmic autoantibodies in a large series of coeliac disease patients before and after gluten free diet, and to correlate anti-S. cerevisiae-positivity with intestinal mucosal damage. One hundred and five consecutive coeliac disease patients and 141 controls (22 ulcerative colitis, 24 Crohn's disease, 30 primary sclerosing cholangitis, 15 postenteritis syndrome, 50 blood donors) were tested for anti-S. cerevisiae by enzyme-linked immunosorbent assay and for perinuclear anti-neutrophil cytoplasmic autoantibodies by indirect immunofluorescence. In coeliac disease anti-S. cerevisiae (immunoglobulin G and/or immunoglobulin A) were slightly less frequent (59%) than in Crohn's disease (75%, P = 0.16) and significantly more frequent than in ulcerative colitis (27%), primary sclerosing cholangitis (30%), postenteritis syndrome (26%) and blood donors (4%) (P = 0.009, P = 0.0002, P = 0.025, P < 0.0001). No correlation was found between anti-S. cerevisiae and degree of mucosal damage. Perinuclear anti-neutrophil cytoplasmic autoantibodies were detected only in one coeliac. After gluten free diet the disappearance of anti-S. cerevisiae-immunoglobulin A (93%) was more frequent than that of immunoglobulin G (17%, P = 0.0001); perinuclear anti-neutrophil cytoplasmic autoantibodies disappeared in the only coeliac positive at diagnosis. More than half of untreated coeliacs are anti-S. cerevisiae-positive irrespective of the severity of mucosal damage. Differently from immunoglobulin A, anti-S. cerevisiae-immunoglobulin G persisted in more than 80% after gluten free diet. The high prevalence of anti-S. cerevisiae in coeliac disease suggests that they may be the effect of a non-specific immune response in course of chronic small bowel disease.

Osteomielitis crónica esclerosante difusa Chronic diffuse sclerosing osteomyelitis

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M. Paula Aparicio M

2008-12-01

Full Text Available La osteomielitis esclerosante difusa se considera como una osteomielitis crónica primaria consistente en un proceso inflamatorio, doloroso y prolongado en el tiempo. Afecta solo a la mandíbula y es generalmente unilateral, compromete hueso basal y alveolar, y se localiza al nivel de cuerpo, ángulo, rama e incluso cóndilo. La causa es aún controversial, ya que algunos le atribuyen un origen infeccioso, mientras otros lo consideran una condición no infecciosa, como producto de sobrecargas o asociado con síndrome SAPHO (sinovitis, acné, pustulosis, hiperostosis y osteítis, pero la literatura no es concluyente. Con respecto al tratamiento, al igual que su causa, no está totalmente esclarecido y se describen a lo largo del tiempo distintas alternativas, que van desde lo conservador a lo más radical. Se presenta el seguimiento y tratamiento durante 7 meses de una paciente afectada por osteomielitis esclerosante difusa con 18 años de evolución aproximadamente, que ha sido refractaria a las alternativas terapéuticas convencionales.Diffuse sclerosing osteomyelitis is considered a chronic primary osteomyelitis consisting in an inflammatory, painful and prolonged process. It only affects the mandible and it is generally unilateral. It involves the basal and alveolar bone and it is located at the level of body, angle, branch and even condyle. The cause is more controversial, since some attribute an infectious origin to it, whereas others consider it as a non-infectious condition resulting from the overloads or associated with SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis, but literature is not concluding. Treatment as well as its cause are not totally clear. Different alternatives are described that go from the conservative to the most radical position. The 7-month follow-up and treatment of a female patient suffering from diffuse sclerosing osteomyelitis with approximately18 years of evolution that has been refractory

Radiologic bone changes of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

International Nuclear Information System (INIS)

Maekelae, P.; Virtama, P.

1982-01-01

More than 50 cases of polycystic lipomembranous osteodysplasia (PLO) with sclerosing leukoencephalopathy (SL) have been described in Finland, Sweden, Japan, and in the USA. Radiographic bone changes, including symmetrical cystic lesions in the small bones of the extremities and trabecular loss in the distal ends of the long tubular bones, represent primary abnormalities in the diagnosis of the disease. Neuropsychiatric symptoms, frontal syndrome, and pyramidal signs make the patients dangerous to themselves. They are often involved in traffic accidents are prone to multiple spontaneous or almost spontaneous fractures. PLO usually starts with slight bone pain around the age of 20 years. Progress is very slow during the next ten years, but faster after the age of 40 years. The patients usually die before the age of 50 years having total dementia and epileptiform convulsions. (orig.)

Microbiological Assessment of Bile and Corresponding Antibiotic Treatment: A Strobe-Compliant Observational Study of 1401 Endoscopic Retrograde Cholangiographies.

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Rupp, Christian; Bode, Konrad; Weiss, Karl Heinz; Rudolph, Gerda; Bergemann, Janine; Kloeters-Plachky, Petra; Chahoud, Fadi; Stremmel, Wolfgang; Gotthardt, Daniel Nils; Sauer, Peter

2016-03-01

The aim of this study was to determine the antibiotic susceptibility profiles of bacteria in bile samples and to analyze the clinical relevance of the findings as only limited information about risk factors for elevated frequence of bacterial and fungal strains in routinely collected bile samples has been described so far.A prospective cohort study at a tertiary care center was conducted. Seven hundred forty-four patients underwent 1401 endoscopic retrograde cholangiographies (ERCs) as indicated by liver transplantation (427/1401), primary sclerosing cholangitis (222/1401), choledocholithiasis only (153/1401), obstruction due to malignancy (366/1401), or other conditions (233/1401). Bile samples for microbiological analysis were obtained in all patients.The 71.6% (823/1150) samples had a positive microbiological finding, and 57% (840/1491) of the bacterial isolates were gram-positive. The main species were Enterococcus spp (33%; 494/1491) and Escherichia coli (12%; 179/1491). Of the samples, 53.8% had enteric bacteria and 24.7% had Candida spp; both were associated with clinical and laboratory signs of cholangitis (C-reactive proteins 35.0 ± 50.1 vs 44.8 ± 57.6; 34.5 ± 51.2 vs 52.9 ± 59.7; P bile sampling was achieved in 56.3% (89/158) of the patients. In cases with a positive bile culture and available blood culture, blood cultures were positive in 29% of cases (36/124), and 94% (34/36) of blood cultures had microbial species identical to the bile cultures.Bactobilia and fungobilia can usually be detected by routine microbiological sampling, allowing optimized, strain-specific antibiotic treatment. Previous endoscopic intervention, clinical and laboratory signs of cholangitis, and age are independent risk factors. MR bacteria and fungi are an evolving problem in cholangitis, especially in immunocompromised patients.

Transjugular intrahepatic portosystemic shunt in a patient with cavernomatous portal vein occlusion

International Nuclear Information System (INIS)

Kawamata, Hiroshi; Kumazaki, Tatsuo; Kanazawa, Hidenori; Takahashi, Shuji; Tajima, Hiroyuki; Hayashi, Hiromitsu

2000-01-01

A 23-year-old woman with liver cirrhosis secondary to primary sclerosing cholangitis was referred to us for the treatment of recurrent bleeding from esophageal varices that had been refractory to endoscopic sclerotherapy. Her portal vein was occluded, associated with cavernous transformation. A transjugular intrahepatic portosystemic shunt (TIPS) was performed after a preprocedural three-dimensional computed tomographic angiography evaluation to determine feasibility. The portal vein system was recanalized and portal blood flow increased markedly after TIPS. Esophageal varices disappeared 3 weeks after TIPS. Re-bleeding and hepatic encephalopathy were absent for 3 years after the procedure. We conclude that with adequate preprocedural evaluation, TIPS can be performed safely even in patients with portal vein occlusion associated with cavernous transformation.

A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl

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Soo Min Park

2011-05-01

Full Text Available A sclerosing stromal tumor of the ovary is an extremely rare benign tumor; it usually is found during the second and third decades of life. Patients present with pelvic pain or a palpable abdominal mass. Hormonal effects such as masculinization are uncommon. Here, an 11-year old premenarchal girl presented with deepening of the voice. In addition, clitoromegaly and hirsutism with a male suprapubic hair pattern were observed. The laboratory findings showed that the testosterone level was elevated to 3.67 ng/mL, andostenedione to above 10 ng/mL, dehydroepiandrosterone-sulfate to 346 μg/dL and 17-hydroxy progesterone (17-OHP to 11.28 ng/mL. The chromosome evaluation revealed a 46,XX female karyotype. An adrenocorticotropic hormone stimulation test was performed. The 17-OHP to cortisol ratio in 30 minutes was 0.045, which suggested a heterozygote for the 21-hydroxylase deficiency. However, the CYP21A2 gene encoding steroid 21-hydroxylase showed normal. The pelvic ultrasound showed a heterogeneous mass consisting of predominantly solid tissue in the pelvic cavity. The pelvic magnetic resonance imaging revealed an 8.9× 6.2×6.6 cm mass of the left ovary. A left oophrectomy was performed and microscopic examination confirmed a sclerosing stromal tumor. Immunohistochemical studies showed that the tumor was positive for smooth muscle actin and vimentin, but negative for S-100 protein and cytokeratin. Following surgery, the hormone levels returned to the normal range and the hirsutism resolved.

CD8+ T cells undergo activation and programmed death-1 repression in the liver of aged Ae2a,b-/- mice favoring autoimmune cholangitis

NARCIS (Netherlands)

Concepcion, Axel R.; Salas, January T.; Sáez, Elena; Sarvide, Sarai; Ferrer, Alex; Portu, Ainhoa; Uriarte, Iker; Hervás-Stubbs, Sandra; Oude Elferink, Ronald P. J.; Prieto, Jesús; Medina, Juan F.

2015-01-01

Primary biliary cirrhosis (PBC) is a chronic cholestatic disease of unknown etiopathogenesis showing progressive autoimmune-mediated cholangitis. In PBC patients, the liver and lymphocytes exhibit diminished expression of AE2/SLC4A2, a Cl-/HCO3- anion exchanger involved in biliary bicarbonate

PATHOMORPHOLOGICAL FEATURES OF BONE LESIONS AND CORRELATION OF CLINICAL, LABORATORY AND MORPHOMETRIC CRITERIA IN PATIENTS WITH LATENT SCLEROSING HEMATOGENOUS OSTEOMYELITIS (GARRÉ

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V. V. Grigorovsky

2018-01-01

Full Text Available Introduction. Sclerosing hematogenous osteomyelitis of Garré (SHO holds a significant place among cases of latent hematogenous osteomyelitis. Pathomorphological studies of sclerosing hematogenous osteomyelitis are needed to improve differentiated diagnostics, to prognosticate morphology specifics of nidus and to choose the optimal treatment tactics.Purpose of the study — to identify statistical differences between manifestation patterns of various disease types and correlation between clinical, laboratorial and morphometric criteria of bone lesions in patients with sclerosing hematogenous osteomyelitis.Material and methods. The authors studied tissue fragments of affected bones of 25 patients with SHO which was diagnosed by clinical, laboratorial, visualizing and morphological methods. Gradation morphometric criteria were used to reflect condition of nidus tissues. The authors made the analysis of qualitative characters and correlation analysis of dependencies between clinical and laboratorial criteria on the one hand, and with morphometric criteria — on the other, and identified association factor. Results. Pathomorphological study of SHO nidus demonstrated variances of bone lesions in separate disease cases despite the overall similarity of morphological manifestation. About 2/3 of all cases correspond to fibrosing type whereby even small exudative inflammation sites are absent. In about 1/3 of all cases apart from fibrosis, osteosclerosis and remodeling, the osteomyelitis niduses contain microabscesses indicative of suppurative inflammation as well as the authors observed small sequestration. The maximum differences in patients with various types of SHO were identified in such parameters as share of stab microphages and erythrocyte sedimentation rate (ESR. In cases of long term morbidity the fibrosing disease type is prevailing, in cases of short term lesion (1–2 years — a fibrosing type with microabscesses formation.A series of

Abdominal manifestations of autoimmune disorders

International Nuclear Information System (INIS)

Triantopoulou, C.

2015-01-01

Full text: Immunoglobulin G4-related disease was recognized as a systemic disease since various extrapancreatic lesions were observed in patients with autoimmune pancreatitis (AIP). The real etiology and pathogenesis of IgG4-RD is still not clearly understood. Moreover the exact role of IgG4 or IgG4-positive plasma cells in this disease has not yet been elucidated. only some inconsistent biological features such as hypergammaglobulinemia or hypocomplementemia support the autoimmune nature of the disease process. various names have been ascribed to this clinicopathological entity including IgG4-related sclerosing disease, IgG4-related systemic sclerosing disease, IgG4-related disease, IgG4-related autoimmune disease, hyper-IgG4 disease and IgG4-related systemic disease. The extrapancreatic lesions of IgG4-RD also exhibit the same characteristic histologic features including dense lymphoplasmacytic infiltrate, massive storiform fibrosis, and obliterative phlebitis as seen in IgG4-related pancreatitis. Abdominal manifestations include the following organs/systems: Bile ducts: Sclerosing cholangitis; Gallbladder and liver: Acalculous sclerosis cholecytitis with diffuse wall thickening; hepatic inflammatory pseudotumorts; Kidneys: round or wedge-shaped renal cortical nodules, peripheral cortical; lesions, mass like lesions or renal pelvic involvement; Prostate, urethra, seminal vesicle, vas deferens, uterine cervix; Autoimmune prostatitis; Retroperitoneum: Retroperitoneal fibrosis. thin or mildly thick homogeneous soft tissue lesion surrounding the abdominal aorta and its branches but also bulky masses causing hydronephroureterosis; Mesentery: Sclerosing mesenteritis usually involving the root of the mesentery; Bowel: Inflammatory bowel diseases mimicking Crohn’s disease or ulcerative colitis. various types of sclerosing nodular lesions of the bowel wall; Stomach: Gastritis, gastric ulcers and focal masses mimicking submucosal tumor; omentum: Infiltration mimicking

Shear wave elastography diagnosis of the diffuse sclerosing variant of papillary thyroid carcinoma: A case report

OpenAIRE

Xue, Nianyu; Xu, Youfeng; Huang, Pintong; Zhang, Shengmin; Wang, Hongwei; Yu, Fei

2016-01-01

The present study aimed to report the shear wave elastography (SWE) findings in a patient with the diffuse sclerosing variant of papillary thyroid carcinoma (DSVPTC). Since patients with DSVPTC may present with typical clinicopathological features and initially appear to have Hashimoto's thyroiditis, a thorough clinical evaluation and an early diagnosis are important. A 20-year-old female patient presented with a 1-month history of a neck mass and sore throat. Conventional ultrasound and SWE ...

Clinical endoscopic management and outcome of post-endoscopic sphincterotomy bleeding.

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Wei-Chen Lin

Full Text Available Post-endoscopic sphincterotomy bleeding is a common complication of biliary sphincterotomy, and the incidence varies from 1% to 48%. It can be challenging to localize the bleeder or to administer various interventions through a side-viewing endoscope. This study aimed to evaluate the risk factors of post-endoscopic sphincterotomy bleeding and the outcome of endoscopic intervention therapies. We retrospectively reviewed the records of 513 patients who underwent biliary sphincterotomy in Mackay Memorial Hospital between 2011 and 2016. The blood biochemistry, comorbidities, indication for sphincterotomy, severity of bleeding, endoscopic features of bleeder, and type of endoscopic therapy were analyzed. Post-endoscopic sphincterotomy bleeding occurred in 65 (12.6% patients. Forty-five patients had immediate bleeding and 20 patients had delayed bleeding. The multivariate analysis of risk factors associated with post-endoscopic sphincterotomy bleeding were liver cirrhosis (P = 0.029, end-stage renal disease (P = 0.038, previous antiplatelet drug use (P<0.001, and duodenal ulcer (P = 0.023. The complications of pancreatitis and cholangitis were higher in the bleeding group, with statistical significance. Delayed bleeding occurred within 1 to 7 days (mean, 2.5 days, and 60% (12/20 of the patients received endoscopic evaluation. In the delayed bleeding group, the successful hemostasis rate was 71.4% (5/7, and 65% (13/20 of the patients had ceased bleeding without endoscopic hemostasis therapy. Comparison of different therapeutic modalities showed that cholangitis was higher in patients who received epinephrine spray (P = 0.042 and pancreatitis was higher in patients who received epinephrine injection and electrocoagulation (P = 0.041 and P = 0.039 respectively. Clinically, post-endoscopic sphincterotomy bleeding and further endoscopic hemostasis therapy increase the complication rate of pancreatitis and cholangitis. Realizing the effectiveness of each

Novel strategies and therapeutic options for the management of primary biliary cholangitis.

Science.gov (United States)

Khanna, Amardeep; Jones, David E

2017-10-01

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease. It has a varied course of progression ranging from being completely asymptomatic to aggressive disease leading to cirrhosis and resulting in liver transplantation. In addition, symptoms can be debilitating and can have a major impact on quality of life. For decades, there was only one anti-cholestatic agent available to target this disease and that was only effective in around half of patients, with little or no effect on symptoms. With increasing understanding of the pathogenic mechanisms of PBC and potential targets for drug treatment, pharmaceutical companies have shown a greater interest in this rare disease. A large number of novel therapeutic molecules have been developed and are currently being evaluated. In this review article all the novel molecules in use and in trials targeting cholestasis and symptoms in PBC are discussed.

Congenital double bile duct presenting as recurrent cholangitis in a child

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K.D. Chakravarty

2015-12-01

Full Text Available Double common bile duct (DCBD is a rare congenital anomaly. Most of these bile duct anomalies are associated with bile duct stones, anomalous pancreaticobiliary junction (APBJ, pancreatitis and bile duct or gastric cancers. Early detection and treatment is important to avoid long term complications. Surgical resection of the anomalous bile duct and reconstruction of the biliary enteric anastomosis is the treatment of choice. We report a rare case of DCBD anomaly in a girl, who presented with recurrent cholangitis. She had type Va DCBD anomaly. She underwent successful resection of the bile duct and reconstruction of the biliary enteric anastomosis. Preoperative imaging and diagnosis of the congenital biliary anomaly is very important to avoid intraoperative bile duct injury. Review of the literature shows very few cases of type Va DCBD, presenting with either bile duct stones or APBJ.

[Sclerosing epithelioid fibrosarcoma of the paravertebral column. Case report and literature review].

Science.gov (United States)

Puerta Roldán, Patricia; Rodríguez Rodríguez, Rodrigo; Bagué Rossell, Silvia; de Juan Delago, Manel; Molet Teixidó, Joan

2013-01-01

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of low-grade fibrosarcoma, with specific histological and immunohistochemical features and a poor prognosis. We report a case of SEF of the paravertebral column in a 49-year old male who presented a paraspinal mass with extension into the L4-L5 neural foramen and invasion of the L5 nerve root. Histology of the tumourectomy specimen and its immunohistochemical study led to the diagnosis of SEF. This case was particularly unusual due to its paravertebral column location and, despite its low grade, illustrates the malignant potential of SEF. Copyright © 2012 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

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...

Expression of hepatic transporters OATP-C and MRP2 in primary sclerosing cholangitis

NARCIS (Netherlands)

Oswald, M.; Kullak-Ublick, G. A.; Paumgartner, G.; Beuers, U.

2001-01-01

In chronic cholestatic liver diseases, biliary excretion of organic anions from blood into bile is impaired. The aim of this study was to identify the underlying mechanism. Expression of the basolateral organic anion transporting polypeptide OATP-C (SLC21A6) and the canalicular multidrug resistance

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

Czech Academy of Sciences Publication Activity Database

Bajer, L.; Kverka, Miloslav; Kostovčík, Martin; Macinga, P.; Dvořák, Jiří; Stehlíková, Zuzana; Březina, J.; Wohl, P.; Špičák, J.; Drastich, P.

2017-01-01

Roč. 23, č. 25 (2017), s. 4548-4558 ISSN 1007-9327 R&D Projects: GA MZd(CZ) NV15-28064A Institutional support: RVO:61388971 Keywords : Dysbiosis * Inflammatory bowel disease * Ulcerative colitis Subject RIV: EE - Microbiology, Virology OBOR OECD: Microbiology Impact factor: 3.365, year: 2016

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

Czech Academy of Sciences Publication Activity Database

Bajer, L.; Kverka, Miloslav; Kostovčík, M.; Macinga, P.; Dvořák, J.; Stehlíková, Z.; Březina, J.; Wohl, P.; Špičák, J.; Drastich, P.

2017-01-01

Roč. 23, č. 25 (2017), s. 4548-4558 ISSN 1007-9327 R&D Projects: GA MZd(CZ) NV15-28064A Institutional support: RVO:68378041 Keywords : dysbiosis * inflammatory bowel disease * ulcerative colitis Subject RIV: FP - Other Medical Disciplines OBOR OECD: Gastroenterology and hepatology Impact factor: 3.365, year: 2016

European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

Science.gov (United States)

Knobler, R; Moinzadeh, P; Hunzelmann, N; Kreuter, A; Cozzio, A; Mouthon, L; Cutolo, M; Rongioletti, F; Denton, C P; Rudnicka, L; Frasin, L A; Smith, V; Gabrielli, A; Aberer, E; Bagot, M; Bali, G; Bouaziz, J; Braae Olesen, A; Foeldvari, I; Frances, C; Jalili, A; Just, U; Kähäri, V; Kárpáti, S; Kofoed, K; Krasowska, D; Olszewska, M; Orteu, C; Panelius, J; Parodi, A; Petit, A; Quaglino, P; Ranki, A; Sanchez Schmidt, J M; Seneschal, J; Skrok, A; Sticherling, M; Sunderkötter, C; Taieb, A; Tanew, A; Wolf, P; Worm, M; Wutte, N J; Krieg, T

2017-09-01

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum. © 2017 European Academy of Dermatology and Venereology.

Surgical management of isolated mesenteric autoimmune disease: addressing the spectrum of IgG4-related disease and sclerosing mesenteritis.

Science.gov (United States)

Greenbaum, Alissa; Yadak, Nour; Perez, Steven; Rajput, Ashwani

2017-06-08

IgG 4 -related disease (IgG 4 -RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG 4 and tissue IgG 4 levels, specific histopathological findings, multiorgan involvement and adequate response to glucocorticoid treatment. The low incidence and the heterogeneous nature of the disease has made consensus on diagnostic criteria for IgG 4 -RD difficult. Whether sclerosing mesenteritis (SM) is considered a manifestation of IgG 4 -RD is strongly debated. We present the case of a patient with a history of rheumatoid arthritis who presented with a calcified abdominal mass. She was found to have an isolated, pedunculated mesenteric mass positive for IgG 4 and concurrently elevated serum IgG 4 levels. Clinical features did not classify her disease as either SM or IgG 4 -RD as currently described in consensus statements. Concurrent diagnoses of IgG 4 -RD, SM and other autoimmune disorders, as well as postoperative recommendations for resected isolated IgG 4 -positive masses, are discussed. © 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.

Prevalence of hepatobiliary dysfunction in a regional group of patients with chronic inflammatory bowel disease

DEFF Research Database (Denmark)

Wewer, V; Gluud, C; Schlichting, P

1991-01-01

A regional group of outpatients with chronic inflammatory bowel disease (ulcerative colitis, n = 396, and Crohn's disease, n = 125) was biochemically screened to estimate the prevalence of hepatobiliary dysfunction. Among the 396 patients with ulcerative colitis, 69 (17%; 95% confidence limits, 14...... primary sclerosing cholangitis, of whom two were primarily diagnosed; one patient had cholangiocarcinoma also primarily diagnosed; and two patients were found to have alcoholic hepatic damage. Among the 125 patients with Crohn's disease, 38 (30%; 95% confidence limits, 23-38%) had at least 1 abnormal...... the criteria for further evaluation as described above. One patient appeared to have epithelioid granuloma in the liver and one patient had alcoholic liver disease, whereas one patient refused further examination.(ABSTRACT TRUNCATED AT 250 WORDS)...

IDIOPATHIC SCLEROSING ENCAPSULATING PERITONITIS CAUSING ACUTE INTESTINAL OBSTRUCTION AND GANGRENE: A CASE REPORT

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Nava

2016-04-01

Full Text Available INTRODUCTION Sclerosing encapsulating peritonitis (SEP is a relatively rare cause of intestinal obstruction resulting from encasement of variable lengths of bowel by dense fibro-collagenous membrane. It is more common in young females, and shows tropical and sub-tropical distribution. The idiopathic cases of SEP, which lack any identifiable cause from clinical, radiological and histopathological findings, are also reported under the descriptive term “abdominal cocoon syndrome”. SEP presents with acute or sub-acute intestinal obstruction with or without a mass. In the era of laparoscopic surgery, inadvertent damage to the small bowel at insertion of the trocar and cannula can occur by being unaware of this condition resulting in unnecessary bowel resection. Persistent untreated SEP may advance to bowel gangrene or intestinal perforation, representing life threatening conditions. We report the clinical presentation of a 75-year-old female presenting with signs of intestinal obstruction whose imaging findings revealed abdominal cocoon with bowel gangrene leading to perforation and the same confirmed at surgery. Surgical excision of the fibrotic sac encasing the bowel, resection of gangrenous bowel segment and end ileostomy was performed. Histopathology of the excised membrane confirmed sclerosing encapsulating peritonitis. To our knowledge, only a few cases of abdominal cocoon with perforation have been reported in literature so far. Radiologists should be aware of this relatively rare cause of intestinal obstruction, its imaging findings and complications, as preoperative diagnosis will prevent delay and aid in treatment planning to the surgeon. Identification of soft tissue density membrane encasing congregated small bowel loops into a single area on computed-tomography gives diagnostic clue. Surgical excision of sac, release of bowel loops and adhesions with partial intestinal resection when necessary is the treatment.

Subacute sclerosing panencephalitis with bilateral inferior collicular hyperintensity on magnetic resonance imaging brain

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Maya Thomas

2012-01-01

Full Text Available Subacute sclerosing panencephalitis (SSPE is chronic encephalitis occurring after infection with measles virus. An 8-year-old boy presented with progressive behavioral changes, cognitive decline and myoclonic jerks, progressing to a bed bound state over 2 months. Magnetic resonance imaging (MRI brain showed T2-weighted hyperintensities in the subcortical areas of the left occipital lobe and brachium of the inferior colliculus on both sides. EEG showed bilateral, synchronous periodic discharges. Serum/cerebrospinal fluid measles IgG titer was significantly positive. The overall features were suggestive of SSPE. MRI finding of bilateral inferior colliculus changes on MRI without significant involvement of other commonly involved areas suggests an uncommon/rare imaging pattern of SSPE.

IgG4 related sclerosing mastitis: expanding the morphological spectrum of IgG4 related diseases.

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Chougule, Abhijit; Bal, Amanjit; Das, Ashim; Singh, Gurpreet

2015-01-01

IgG4 related disease (IgG4RD) is a recently recognised condition characterised by mass forming lesions associated with storiform fibrosis, obliterative phlebitis, lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and elevated serum IgG4 levels. Although rare, mammary involvement has been reported as IgG4 related sclerosing mastitis, the morphological counterpart of a growing family of IgG4 related diseases. A total of 17 cases belonging to mass forming benign inflammatory breast lesions such as plasma cell mastitis, granulomatous lobular mastitis, non-specific mastitis and inflammatory pseudotumour were investigated as a possible member of IgG4 related sclerosing mastitis. Clinical, radiological, histopathological and immunohistochemistry findings were noted in all cases. Cases diagnosed as inflammatory pseudotumour showed all the histopathological features of IgG4RD along with increased number of IgG4 positive plasma cells and IgG4/IgG ratio >40%. However, only a few IgG4 positive cells were seen in plasma cell mastitis, granulomatous lobular mastitis and non-specific mastitis cases. These cases also did not fulfill the morphological criteria for the diagnosis of IgG4 related diseases. IgG4RD should be excluded in plasma cell rich lesions diagnosed on core biopsies by IgG4 immunostaining. This can avoid unnecessary surgery as IgG4 related diseases respond to simple and effective steroid treatment.

Subacute Sclerosing Panencephalitis: Clinical and Demographic Characteristics

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Rafique, A.; Amjad, N.; Chand, P.; Ahmed, K.; Ibrahim, S.; Zaidi, S. S. Z.; Rana, M. S.

2014-01-01

Objective: To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). Study Design: Case series. Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. Methodology: A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. Results were expressed as percentages. Results: Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. Conclusion: SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions. (author)

Segmentectomy for giant pulmonary sclerosing haemangiomas with high serum KL-6 levels

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Kuroda, Hiroaki; Mun, Mingyon; Okumura, Sakae; Nakagawa, Ken

2012-01-01

We describe a 61-year old female patient with a giant pulmonary sclerosing haemangioma (PSH) and an extremely high preoperative serum KL-6 level. During an annual health screening, the patient showed a posterior mediastinal mass on chest radiography. Chest computed tomography and magnetic resonance imaging revealed a well-circumscribed 60 mm diameter nodule with a marked contrast enhancement in the left lower lobe. The preoperative serum KL-6 level was elevated to 8204 U/ml. We performed a four-port thoracoscopic basal segmentectomy and lymph node sampling for diagnosis and therapy. The postoperative diagnosis showed PSH. The serum KL-6 level decreased dramatically with tumour resection. To the best of our knowledge, this is the first report of a patient with PSH showing a high serum KL-6 level. PMID:22454483

Radiotherapy With or Without Surgery for Patients With Idiopathic Sclerosing Orbital Inflammation Refractory or Intolerant to Steroid Therapy

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Lee, Jong Hoon; Kim, Yeon-Sil; Yang, Suk Woo; Cho, Won-Kyung; Lee, Sang Nam; Lee, Kyung Ji; Ryu, Mi-Ryeong; Jang, Hong Seok

2012-01-01

Purpose: To evaluate the outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) treated with radiotherapy with or without surgery. Methods and Materials: We retrospectively reviewed 22 patients with histopathologically confirmed ISOI who had been refractory or intolerant to steroid therapy and treated with radiation with or without surgery. The radiation dose ranged from 20 to 40 Gy (median, 20 Gy) at 2 Gy per fraction. Presenting signs and treatment outcomes were assessed. Results: Proptosis was the most common sign at presentation, seen in 19 (86.3%) patients, followed by restriction of extraocular movements in 10 (45.4%) patients. Response to radiotherapy was complete in 15 (68.1%) patients, partial in 3 (13.6%) patients, and none in 4 (18.2%) patients. At the median follow-up of 34 months, 14 (63.6%) patients had progression-free state of symptoms and signs, with the progression-free duration ranging from 3 to 75 months (median, 41.5 months), whereas 8 (36.4%) patients had recurrent or persistent disease although they had received radiotherapy. Of the 14 progression-free patients, 6 underwent a bimodality treatment of debulking surgery of ocular disease and radiotherapy. They had had no recurrent disease. Cataract was the most common late complications, and 2 patients experienced a Grade 3 cataract. Conclusion: Our study suggests that for patients with ISOI who are refractory or intolerant to steroid therapy, 20 Gy of radiotherapy appears to be effective for the control of disease with acceptable complications, especially when it is combined with surgery.

Acute Cholangitis After Bilioenteric Anastomosis for Bile Duct Injuries.

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Ortiz-Brizuela, Edgar; Sifuentes-Osornio, José; Manzur-Sandoval, Daniel; Terán-Ellis, Santiago Mier Y; Ponce-de-León, Sergio; Torres-González, Pedro; Mercado, Miguel Ángel

2017-10-01

The study aims to describe the clinical features, microbiology, and associated factors of acute cholangitis (AC) after bilioenteric anastomosis (BEA) for biliary duct injury (BDI). Additionally, we assessed the performance of the Tokyo Guidelines 2013 (TG13) recommendations in these patients. We conducted a case-control study of 524 adults with a history of BEA for BDI from January 2000 to January 2014. A propensity score adjustment was performed for the analysis of the independent role of the main factors identified during the univariate logistic regression procedure. We identified 117 episodes of AC in 70 patients; 51.3% were definitive AC according to the TG13 diagnostic criteria, and 39.3% did not fulfill the imaging criteria of AC. A history of post-operative biliary complications (OR 2.55, 95% CI 1.38-4.70) and the bile duct confluence preservation (OR 0.46, 95% CI 0.24-0.87) were associated with AC. Eighty-nine percent of the microorganisms were Enterobacteriaceae; of them, 28% were extended spectrum β-lactamase (ESBL) producers. AC is a common complication after BEA and must be suspected even in the absence of imaging findings, particulary in patients with a history of post-operative biliary complications, and/or without bile duct confluence preserved. An empirical treatment for ESBL-producing Enterobacteriaceae may be appropriate in patients living in countries with a high rate of bacterial drug resistance.

Laparoscopic Management of Sclerosing Stromal Tumors of the Ovary Combined with Ectopic Pregnancy.

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Liu, Hua-Qian; Liu, Qiang; Sun, Xue-Bing; Chang, Wen-Min

2015-01-01

Like other stromal-derived gynecological tumors, a sclerosing stromal tumor of the ovary (SSTO) is a rare benign tumor that is difficult to distinguish from a malignant ovarian tumor in clinical practice. An SSTO is routinely treated with laparotomy. Here, we present two extremely rare cases of SSTO with contralateral and ipsilateral tubal pregnancies, in which laparoscopic surgery was performed to remove the tumors. After surgery, one patient (case 1) became pregnant twice within 29 months, and the other patient (case 2) did not become pregnant within 6 months postoperatively. These two cases suggest that laparoscopic management is not only useful in treating SSTO and complicating diseases, but it may also help to reduce unnecessary surgical injury to the ovary. © 2015 S. Karger AG, Basel.

Mesenteric ossification in CT indicates sclerosing peritonitis in chronic bacterial infection and pancreatitis; Mesenteriale Verknoecherungen als computertomographische Zeichen einer sklerosierenden Peritonitis bei chronischer Bauchfellentzuendung und Pankreatitis

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Kirchner, J.; Kirchner, E.M. [Abt. fuer Diagnostische und Interventionelle Radiologie, Klinikum Niederberg Velbert (Germany); Kickuth, R. [Klinik fuer Radiologie und Nuklearmedizin, Katholisches Marienhospital Herne, Universitaetsklinik der Ruhr Univ. Bochum (Germany); Stein, A. [Klinik fuer Strahlentherapie und Onkologie, Universitaetsklinikum Frankfurt/Main (Germany)

2004-07-01

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.) [German] Die sklerosierende Peritonitis wurde wiederholt als ernste Komplikation der Bauchfelldialyse beschrieben. Aber auch verschiedene andere Reizzustaende wie chronische bakterielle Peritonitis und Pankreatitis koennen eine sklerosierende Peritonitis nach sich ziehen. Hierbei zeigen sich neben einer Verdickung des Peritoneums und der Waende des Duenndarms auch computertomographisch nachweisbare Kalkeinlagerungen. Wir stellen zwei Patienten mit chronischer Peritonitis nach Sigmaperforation und Pankreatitis mit Verknoecherungen des Peritoneums vor. (orig.)

Colesevelam attenuates cholestatic liver and bile duct injury in Mdr2-/- mice by modulating composition, signalling and excretion of faecal bile acids.

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Fuchs, Claudia Daniela; Paumgartner, Gustav; Mlitz, Veronika; Kunczer, Victoria; Halilbasic, Emina; Leditznig, Nadja; Wahlström, Annika; Ståhlman, Marcus; Thüringer, Andrea; Kashofer, Karl; Stojakovic, Tatjana; Marschall, Hanns-Ulrich; Trauner, Michael

2018-04-10

Interruption of the enterohepatic circulation of bile acids (BAs) may protect against BA-mediated cholestatic liver and bile duct injury. BA sequestrants are established to treat cholestatic pruritus, but their impact on the underlying cholestasis is still unclear. We aimed to explore the therapeutic effects and mechanisms of the BA sequestrant colesevelam in a mouse model of sclerosing cholangitis. Mdr2 -/- mice received colesevelam for 8 weeks. Gene expression profiles of BA homeostasis, inflammation and fibrosis were explored in liver, intestine and colon. Hepatic and faecal BA profiles and gut microbiome were analysed. Glucagon-like peptide 1 (GLP-1) levels in portal blood were measured by ELISA. Furthermore, Mdr2 -/- mice as well as wild-type 3,5-diethoxy-carbonyl-1,4-dihydrocollidine-fed mice were treated with GLP-1-receptor agonist exendin-4 for 2 weeks prior to analysis. Colesevelam reduced serum liver enzymes, BAs and expression of proinflammatory and profibrogenic markers. Faecal BA profiling revealed increased levels of secondary BAs after resin treatment, while hepatic and biliary BA composition showed a shift towards more hydrophilic BAs. Colonic GLP-1 secretion, portal venous GLP-1 levels and intestinal messenger RNA expression of gut hormone Proglucagon were increased, while ileal Fgf15 expression was abolished by colesevelam. Exendin-4 treatment increased bile duct mass without promoting a reactive cholangiocyte phenotype in mouse models of sclerosing cholangitis. Microbiota analysis showed an increase of the phylum δ-Proteobacteria after colesevelam treatment and a shift within the phyla Firmicutes from Clostridiales to Lactobacillus . Colesevelam increases faecal BA excretion and enhances BA conversion towards secondary BAs, thereby stimulating secretion of GLP-1 from enteroendocrine L-cells and attenuates liver and bile duct injury in Mdr2 -/- mice. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article

Autoimmune paediatric liver disease.

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Mieli-Vergani, Giorgina; Vergani, Diego

2008-06-07

Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. AIH is divided into two subtypes 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 partial 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 type 1. 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 normalization of biochemical parameters, and decreased inflammatory activity on follow up liver biopsies. However, the cholangiopathy can progress. There may be evolution from AIH to ASC over the years, despite treatment. De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH, including elevated titres of serum antibodies, hypergammaglobulinaemia, and histological findings of interface hepatitis, bridging fibrosis, and collapse. Like classical AIH, it responds to treatment with prednisolone and azathioprine. De novo AIH post liver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection. Whether this condition is a

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 thymoma: case report Timoma esclerosante: relato de caso

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Gesine Gregorio Siqueira

2012-08-01

Full Text Available We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%, sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.

Benign bile duct stenosis: diagnosis and treatment

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Garcia-Medina, J.; Casal, M.; Vieito, X.

1997-01-01

The bening injuries of the biliary ducts are relatively little frequent. Exist two groups of injuries: to due to them to a series for responsible pathologies for itself of the such injuries training, and that basically are the sclerosant cholangitis, the chronic pancreatitis and the stenosis of the sfinter of Oddi, and related them to previous surgery. On both groups eitological, the interventional radiology occupies a place in the diagnosis as well as in the treatment, complementing or substituting to the surgery. Due to the greater frequency of the postchirurgical injuries, we have centered us basically in them. We make a review of the current state of the topic and a bibliographical tracking, emphasizing the most relevant projects. We show some clinical cases of our subject-specific experience. (Author) 42 refs

Colonoscopy surveillance for dysplasia and colorectal cancer in patients with inflammatory bowel disease

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Aalykke, Claus; Jensen, Michael Dam; Fallingborg, Jan

2015-01-01

The risk of colorectal cancer (CRC) and dysplasia in patients with inflammatory bowel disease (IBD) has been highly debated as risk estimates from different studies vary greatly. The present national Danish guideline on colonoscopy surveillance for dysplasia and colorectal cancer in patients......, in some subgroups of patients the risk is increased. These subgroups of patients, who should be offered colonoscopy surveillance, include patients with ulcerative colitis having extensive disease and a long disease duration (10-13 years); early age at onset (less than 19 years of age) of ulcerative...... colitis; and patients with ulcerative colitis as well as Crohn´s disease with a concomitant diagnosis of primary sclerosing cholangitis. A colonoscopy surveillance program is recommended in these subgroups with intervals ranging from every 3-6 months to every 5 years, using chromoendoscopy with targeted...

Heterogeneity of ductular reactions in adult rat and human liver revealed by novel expression of deleted in malignant brain tumor 1

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Bisgaard, H.C.; Holmskov, U.; Santoni-Rugiu, E.

2002-01-01

The regenerative capacity of mammalian adult liver reflects the ability of a number of cell populations within the hepatic lineage to take action. Limited information is available regarding factors and mechanisms that determine the specific lineage level at which liver cells contribute to liver......), were specifically associated with the emergence of ductular (oval) cell populations in injured liver. Subsequent cloning and characterization of the rat DMBT1 homologue revealed a highly inducible expression in ductular reactions composed of transit-amplifying ductular (oval) cells, but not in ductular...... reactions after ligation of the common bile duct. In human liver diseases, DMBT1 was expressed in ductular reactions after infection with hepatitis B and acetaminophen intoxication, but not in primary biliary cirrhosis, primary sclerosing cholangitis, and obstruction of the large bile duct. The expression...

Enrichment of measles virus-like RNA in the nucleocapsid fraction isolated from subacute sclerosing panencephalitis brains

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Bedows, E.; Payne, F.E.; Kohne, D.E.; Tourtellotte, W.W.

1982-01-01

A procedure has been developed which facilitates the detection of measles virus RNA sequences in human brains. The procedure involves isolating subviral components (nucleocapsids) from brain tissues prior to RNA purification, followed by hybridization of these RNAs to cDNA synthesized from measles virus 50 S RNA template. Using these techniques we were able to obtain an RNA fraction which was manyfold enriched in measles virus-specific RNA, relative to unfractionated subacute sclerosing panencephalitis (SSPE) brain RNAs. 70-100% of the measles virus-specific RNA present in these SSPE brain samples were recovered in this enriched fraction. (Auth.)

Enrichment of measles virus-like RNA in the nucleocapsid fraction isolated from subacute sclerosing panencephalitis brains

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Bedows, E; Payne, F E [Michigan Univ., Ann Arbor (USA). School of Public Health; Kohne, D E [Center for Neurologic Study, San Diego, CA, USA; Tourtellotte, W W [Neurology Service, V.A. Wadsworth Hospital Center, Los Angeles, CA, USA

1982-02-01

A procedure has been developed which facilitates the detection of measles virus RNA sequences in human brains. The procedure involves isolating subviral components (nucleocapsids) from brain tissues prior to RNA purification, followed by hybridization of these RNAs to cDNA synthesized from measles virus 50 S RNA template. Using these techniques we were able to obtain an RNA fraction which was manyfold enriched in measles virus-specific RNA, relative to unfractionated subacute sclerosing panencephalitis (SSPE) brain RNAs. 70-100% of the measles virus-specific RNA present in these SSPE brain samples were recovered in this enriched fraction.

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

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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

Subacute sclerosing panencephalitis: A clinical appraisal

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Sujit Abajirao Abajirao

2013-01-01

Full Text Available Introduction: Subacute sclerosing panencephalitis (SSPE is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken′s criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5% males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5% followed by seizures (23.5%. Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years. Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.

A randomized trial of obeticholic acid monotherapy in patients with primary biliary cholangitis.

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Kowdley, Kris V; Luketic, Velimir; Chapman, Roger; Hirschfield, Gideon M; Poupon, Raoul; Schramm, Christoph; Vincent, Catherine; Rust, Christian; Parés, Albert; Mason, Andrew; Marschall, Hanns-Ulrich; Shapiro, David; Adorini, Luciano; Sciacca, Cathi; Beecher-Jones, Tessa; Böhm, Olaf; Pencek, Richard; Jones, David

2018-05-01

Obeticholic acid (OCA), a potent farnesoid X receptor agonist, was studied as monotherapy in an international, randomized, double-blind, placebo-controlled phase 2 study in patients with primary biliary cholangitis who were then followed for up to 6 years. The goals of the study were to assess the benefit of OCA in the absence of ursodeoxycholic acid, which is relevant for patients who are intolerant of ursodeoxycholic acid and at higher risk of disease progression. Patients were randomized and dosed with placebo (n = 23), OCA 10 mg (n = 20), or OCA 50 mg (n = 16) given as monotherapy once daily for 3 months (1 randomized patient withdrew prior to dosing). The primary endpoint was the percent change in alkaline phosphatase from baseline to the end of the double-blind phase of the study. Secondary and exploratory endpoints included change from baseline to month 3/early termination in markers of cholestasis, hepatocellular injury, and farnesoid X receptor activation. Efficacy and safety continue to be monitored through an ongoing 6-year open-label extension (N = 28). Alkaline phosphatase was reduced in both OCA groups (median% [Q1, Q3], OCA 10 mg -53.9% [-62.5, -29.3], OCA 50 mg -37.2% [-54.8, -24.6]) compared to placebo (-0.8% [-6.4, 8.7]; P OCA improved many secondary and exploratory endpoints (including γ-glutamyl transpeptidase, alanine aminotransferase, conjugated bilirubin, and immunoglobulin M). Pruritus was the most common adverse event; 15% (OCA 10 mg) and 38% (OCA 50 mg) discontinued due to pruritus. OCA monotherapy significantly improved alkaline phosphatase and other biochemical markers predictive of improved long-term clinical outcomes. Pruritus increased dose-dependently with OCA treatment. Biochemical improvements were observed through 6 years of open-label extension treatment. (Hepatology 2018;67:1890-1902). © 2017 The Authors. Hepatology published by Wiley Periodicals, Inc. on behalf of American Association for the Study of Liver Diseases.

Molecular Mechanisms of Ursodeoxycholic Acid Toxicity & Side Effects: Ursodeoxycholic Acid Freezes Regeneration & Induces Hibernation Mode

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Kotb, Magd A.

2012-01-01

Ursodeoxycholic acid (UDCA) is a steroid bile acid approved for primary biliary cirrhosis (PBC). UDCA is reported to have “hepato-protective properties”. Yet, UDCA has “unanticipated” toxicity, pronounced by more than double number of deaths, and eligibility for liver transplantation compared to the control group in 28 mg/kg/day in primary sclerosing cholangitis, necessitating trial halt in North America. UDCA is associated with increase in hepatocellular carcinoma in PBC especially when it fails to achieve biochemical response (10 and 15 years incidence of 9% and 20% respectively). “Unanticipated” UDCA toxicity includes hepatitis, pruritus, cholangitis, ascites, vanishing bile duct syndrome, liver cell failure, death, severe watery diarrhea, pneumonia, dysuria, immune-suppression, mutagenic effects and withdrawal syndrome upon sudden halt. UDCA inhibits DNA repair, co-enzyme A, cyclic AMP, p53, phagocytosis, and inhibits induction of nitric oxide synthatase. It is genotoxic, exerts aneugenic activity, and arrests apoptosis even after cellular phosphatidylserine externalization. UDCA toxicity is related to its interference with drug detoxification, being hydrophilic and anti-apoptotic, has a long half-life, has transcriptional mutational abilities, down-regulates cellular functions, has a very narrow difference between the recommended (13 mg/kg/day) and toxic dose (28 mg/kg/day), and it typically transforms into lithocholic acid that induces DNA strand breakage, it is uniquely co-mutagenic, and promotes cell transformation. UDCA beyond PBC is unjustified. PMID:22942741

Molecular Mechanisms of Ursodeoxycholic Acid Toxicity & Side Effects: Ursodeoxycholic Acid Freezes Regeneration & Induces Hibernation Mode

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Magd A. Kotb

2012-07-01

Full Text Available Ursodeoxycholic acid (UDCA is a steroid bile acid approved for primary biliary cirrhosis (PBC. UDCA is reported to have “hepato-protective properties”. Yet, UDCA has “unanticipated” toxicity, pronounced by more than double number of deaths, and eligibility for liver transplantation compared to the control group in 28 mg/kg/day in primary sclerosing cholangitis, necessitating trial halt in North America. UDCA is associated with increase in hepatocellular carcinoma in PBC especially when it fails to achieve biochemical response (10 and 15 years incidence of 9% and 20% respectively. “Unanticipated” UDCA toxicity includes hepatitis, pruritus, cholangitis, ascites, vanishing bile duct syndrome, liver cell failure, death, severe watery diarrhea, pneumonia, dysuria, immune-suppression, mutagenic effects and withdrawal syndrome upon sudden halt. UDCA inhibits DNA repair, co-enzyme A, cyclic AMP, p53, phagocytosis, and inhibits induction of nitric oxide synthatase. It is genotoxic, exerts aneugenic activity, and arrests apoptosis even after cellular phosphatidylserine externalization. UDCA toxicity is related to its interference with drug detoxification, being hydrophilic and anti-apoptotic, has a long half-life, has transcriptional mutational abilities, down-regulates cellular functions, has a very narrow difference between the recommended (13 mg/kg/day and toxic dose (28 mg/kg/day, and it typically transforms into lithocholic acid that induces DNA strand breakage, it is uniquely co-mutagenic, and promotes cell transformation. UDCA beyond PBC is unjustified.

Prospective observational multicenter study to define a diagnostic algorithm for biliary candidiasis.

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Lenz, Philipp; Eckelskemper, Franziska; Erichsen, Thomas; Lankisch, Tim; Dechêne, Alexander; Lubritz, Gabriele; Lenze, Frank; Beyna, Torsten; Ullerich, Hansjörg; Schmedt, Andre; Domagk, Dirk

2014-09-14

To develop an algorithm to improve the diagnosis and treatment of patients with biliary candidiasis. We performed a prospective study of 127 patients who underwent endoscopic retrograde cholangiopancreatography, for various biliary disorders, at 3 tertiary referral centers in Germany from July 2011 through July 2012 (ClinicalTrials.gov: NCT01109550). Bile, buccal, and stool samples were collected. When indicated, endoscopic transpapillary bile duct biopsies were performed to clarify the etiology of bile duct strictures and to prove invasive fungal infections. Candida species were detected in 38 of the 127 bile samples (29.9%). By multivariate analysis patients' age and previous endoscopic sphincterotomy were independent risk factors for biliary candidiasis (P 7 d) (P = 0.089) tend to be at risk for biliary candidiasis. One patient was negative in mycological culture of bile fluid but invasive biliary candidiasis was diagnosed histologically. Of Candida subspecies detected, 36.7% were azole-resistant, such as C glabrata. Eight patients received anti-mycotic therapy, based on our algorithm. Of these, 3 had cancer with biliary tract involvement, 2 had secondary sclerosing cholangitis, 1 had retroperitoneal fibrosis, and 5 had septicemia. In all patients contamination was ruled out by smears of the endoscope channel. Gastroenterologists should be aware of frequent candida colonization in patients with cholangitis and biliary disorders. Our suggested algorithm facilitates the further clinical management.

Prospective observational multicenter study to define a diagnostic algorithm for biliary candidiasis

Science.gov (United States)

Lenz, Philipp; Eckelskemper, Franziska; Erichsen, Thomas; Lankisch, Tim; Dechêne, Alexander; Lubritz, Gabriele; Lenze, Frank; Beyna, Torsten; Ullerich, Hansjörg; Schmedt, Andre; Domagk, Dirk

2014-01-01

AIM: To develop an algorithm to improve the diagnosis and treatment of patients with biliary candidiasis. METHODS: We performed a prospective study of 127 patients who underwent endoscopic retrograde cholangiopancreatography, for various biliary disorders, at 3 tertiary referral centers in Germany from July 2011 through July 2012 (ClinicalTrials.gov: NCT01109550). Bile, buccal, and stool samples were collected. When indicated, endoscopic transpapillary bile duct biopsies were performed to clarify the etiology of bile duct strictures and to prove invasive fungal infections. RESULTS: Candida species were detected in 38 of the 127 bile samples (29.9%). By multivariate analysis patients’ age and previous endoscopic sphincterotomy were independent risk factors for biliary candidiasis (P 7 d) (P = 0.089) tend to be at risk for biliary candidiasis. One patient was negative in mycological culture of bile fluid but invasive biliary candidiasis was diagnosed histologically. Of Candida subspecies detected, 36.7% were azole-resistant, such as C glabrata. Eight patients received anti-mycotic therapy, based on our algorithm. Of these, 3 had cancer with biliary tract involvement, 2 had secondary sclerosing cholangitis, 1 had retroperitoneal fibrosis, and 5 had septicemia. In all patients contamination was ruled out by smears of the endoscope channel. CONCLUSION: Gastroenterologists should be aware of frequent candida colonization in patients with cholangitis and biliary disorders. Our suggested algorithm facilitates the further clinical management. PMID:25232260

[Autoimmune hepatitis: Immunological diagnosis].

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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.

Autoimmune Hepatitis: A Risk Factor for Cholangiocarcinoma

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Rajat Garg

2017-11-01

Full Text Available Cholangiocarcinoma (CCA is a very aggressive and lethal tumor, which arises from the epithelial cells of bile ducts. CCA comprises about 3% of all gastrointestinal malignancies and its incidence is on the rise in the recent years. Anatomically, it is classified into intrahepatic, perihilar, or extrahepatic (distal CCA. There are a number of risk factors associated with CCA including primary sclerosing cholangitis, fibropolycystic liver disease, parasitic infection, viral hepatitis, chronic liver disease, and genetic disorders like Lynch syndrome. Autoimmune hepatitis is also recently reported to have an association with development of CCA. We report an interesting case of perihilar CCA in the setting of autoimmune hepatitis along with a literature review. This case highlights the importance of early treatment and close clinical follow-up of patients with autoimmune hepatitis for development of CCA.

Novel developments in foam sclerotherapy: Focus on Varithena® (polidocanol endovenous microfoam) in the management of varicose veins.

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Star, Phoebe; Connor, David E; Parsi, Kurosh

2018-04-01

Scope Varithena® is a recently approved commercially available drug/delivery unit that produces foam using 1% polidocanol for the management of varicose veins. The purpose of this review is to examine the benefits of foam sclerotherapy, features of the ideal foam sclerosant and the strengths and limitations of Varithena® in the context of current foam sclerotherapy practices. Method Electronic databases including PubMed, Medline (Ovid) SP as well as trial registries and product information sheets were searched using the keywords, 'Varithena', 'Varisolve', 'polidocanol endovenous microfoam', 'polidocanol' and/or 'foam sclerotherapy/sclerosant'. Articles published prior to 20 September 2016 were identified. Results Foam sclerosants have effectively replaced liquid agents due to their physiochemical properties resulting in better clinical outcomes. Medical practitioners commonly prepare sclerosant foam at the bedside by agitating liquid sclerosant with a gas such as room air, using techniques as described by Tessari or the double syringe method. Such physician-compounded foams are highly operator dependent producing inconsistent foams of different gas/liquid compositions, bubble size, foam behaviour and varied safety profiles. Varithena® overcomes the variability and inconsistencies of physician-compounded foam. However, Varithena® has limited applications due to its fixed sclerosant type and concentration, cost and lack of worldwide availability. Clinical trials of Varithena® have demonstrated efficacy and safety outcomes equivalent or better than physician-compounded foam but only in comparison to placebo alone. Conclusion Varithena® is a promising step towards the creation of an ideal sclerosant foam. Further assessment in independent randomised controlled clinical trials is required to establish the advantages of Varithena® over and above the current best practice physician-compounded foam.

Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time.

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Cipolla, Calogero; Florena, Ada Maria; Ferrara, Gabriella; Di Gregorio, Riccardo; Unti, Elettra; Giannone, Antonino G; Lazzaro, Luigi A; Graceffa, Giuseppa; Pantuso, Gianni

2018-01-01

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

Radial scar/complex sclerosing lesion of the breast--value of ultrasound.

Science.gov (United States)

Grunwald, S; Heyer, H; Kühl, A; Schwesinger, G; Schimming, A; Köhler, G; Ohlinger, R

2007-04-01

Although benign, radial scar/complex sclerosing adenosis is a lesion which histopathologically resembles tubular carcinoma. On physical examination, it is difficult to distinguish radial scar from a malignant tumour. Mammography cannot differentiate radial scar from malignancy. This clinical study aims to delineate the role of preoperative ultrasonography with emphasis on the question whether ultrasonography could lower the number of false-positive readings and therefore the number of open biopsies required. In this examination, we present the clinical, mammographic, ultrasonographic, and histopathological features of 6 cases of radial scars. Although most authors describe radial scars as non-palpable, 2 of 6 lesions were indeed palpable. On mammograms, radial scars have a spiculated appearance, a feature observed in all of our cases. Numerous ultrasonographic characteristics are listed in the literature, but ultrasonography is not reported to have clear-cut advantages. Although this study did not elucidate any unique ultrasonographic features to characterise these lesions, the analysis of all ultrasonographic results made us recognise a set of "nearly specific ultrasonographic features" of radial scars. Current B-mode imaging does not appear to lead to the desirable reduction of the rate of unnecessary open biopsies.

Increasing incidences of inflammatory bowel disease and decreasing surgery rates in Copenhagen City and County, 2003-2005: a population-based study from the Danish Crohn colitis database

DEFF Research Database (Denmark)

Vind, Ida; Riis, L; Jess, T

2006-01-01

incidence rates and patient characteristics in Copenhagen County and City. METHODS: All patients diagnosed with IBD during 2003-2005 were followed prospectively. Demographic and clinical characteristics, such as disease extent, extraintestinal manifestations, smoking habits, medical treatment, surgical...... interventions, cancer, and death, were registered. RESULTS: Five-hundred sixty-two patients were diagnosed with IBD, resulting in mean annual incidences of 8.6/10(5) for CD, 13.4/10(5) for UC, and 1.1/10(5) for IC. Time from onset to diagnosis was 8.3 months in CD and 4.5 months in UC patients. A family history...... of IBD, smoking, and extraintestinal manifestations was significantly more common in CD than in UC patients. Only 0.6% of UC patients had primary sclerosing cholangitis. In CD, old age at diagnosis was related to pure colonic disease, whereas children significantly more often had proximal and extensive...

Colorectal Cancer Chemoprevention by Mesalazine and Its Derivatives

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Carmine Stolfi

2012-01-01

Full Text Available Patients with inflammatory bowel disease (IBD face an increased lifetime risk of developing colorectal cancer (CRC. Independent factors associated with increased risk include long disease duration, extensive colonic involvement, young age at onset of IBD, severity of inflammation, primary sclerosing cholangitis, backwash ileitis, and a family history of CRC, thus emphasising the role of intestinal inflammation as an underlying mechanism. This notion is also supported by the demonstration that the use of certain drugs used to attenuate the ongoing mucosal inflammation, such as mesalazine, seems to associate with a reduced incidence of colitis-associated CRC. In the last decade, work from many laboratories has contributed to delineate the mechanisms by which mesalazine alters CRC cell behaviour. In this paper, we review the available experimental data supporting the ability of mesalazine and its derivatives to interfere with intracellular signals involved in CRC cell growth.

Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time

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Calogero Cipolla

2018-01-01

Full Text Available Introduction. Sclerosing angiomatoid nodular transformation (SANT of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. Case Description. We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. Conclusions. SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

Immunoglobulin G4-related sclerosing disease manifesting as bilateral tonsillar hypertrophy on MRI images: A case report

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Park, Mee Hyun; Woo, Ji Young; Lee, Yul; Yoon, Dae Young; Hong, Hye Sook; Hong, Min Eui [Hallym University College of Medicine, Kangnam Sacred Heart Hospital, Seoul (Korea, Republic of)

2016-02-15

Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication.

Immunoglobulin G4-related sclerosing disease manifesting as bilateral tonsillar hypertrophy on MRI images: A case report

International Nuclear Information System (INIS)

Park, Mee Hyun; Woo, Ji Young; Lee, Yul; Yoon, Dae Young; Hong, Hye Sook; Hong, Min Eui

2016-01-01

Immunoglobulin G4-related sclerosing disease (IgG4-SD) is currently recognized as a distinct systemic disease involving various organs. We reported the imaging findings of a case of pathologically confirmed IgG4-SD involving bilateral palatine tonsils. CT and MRI showed diffuse enlargement of both palatine tonsils with homogeneous contrast enhancement. Focal contour bulging was noted in the right palatine tonsil. Lesions appeared as isointense on T1-weighted and slightly hyperintense on T2-weighted MRI images, as compared with muscle. The T2-weighted MRI image showed a striated pattern in both tonsils. Despite its rare occurrence, IgG4-SD should be included in the differential diagnoses of patients with symptomatic bilateral tonsillar hypertrophy that is non-responsive to medication

Fibrosis of the thyroid gland caused by an IgG4-related sclerosing disease: three years of follow-up.

Science.gov (United States)

Oriot, P; Amraoui, A; Rousseau, E; Malvaux, P; Dechambre, S; Delcourt, A

2014-12-01

Immunoglobulin G4-related sclerosing disease (IgG4-RSD) represents a recently identified inflammatory disorder in which infiltration of IgG4 plasma cells causes fibrosis in organs. While IgG4-RSD is well documented in the pancreas and other organs, it is poorly characterized in the thyroid gland. We report a case of a 48-year-old female with a fibrotic thyroid mass associated with a retroperitoneal fibrosis. Diagnosed early as Riedel disease, the high serum IgG4, immunohistopathology and decreased fibrosis with corticosteroid therapy, finally confirm for the first time, the origin of IgG4-RSD fibrosis of the thyroid.

Mesenchymal Stem Cells as New Therapeutic Agents for the Treatment of Primary Biliary Cholangitis

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Aleksandar Arsenijevic

2017-01-01

Full Text Available Primary biliary cholangitis (PBC is a chronic autoimmune cholestatic liver disease characterized by the progressive destruction of small- and medium-sized intrahepatic bile ducts with resultant cholestasis and progressive fibrosis. Ursodeoxycholic acid and obethicholic acid are the only agents approved by the US Food and Drug Administration (FDA for the treatment of PBC. However, for patients with advanced, end-stage PBC, liver transplantation is still the most effective treatment. Accordingly, the alternative approaches, such as mesenchymal stem cell (MSC transplantation, have been suggested as an effective alternative therapy for these patients. Due to their immunomodulatory characteristics, MSCs are considered as promising therapeutic agents for the therapy of autoimmune liver diseases, including PBC. In this review, we have summarized the therapeutic potential of MSCs for the treatment of these diseases, emphasizing molecular and cellular mechanisms responsible for MSC-based effects in an animal model of PBC and therapeutic potential observed in recently conducted clinical trials. We have also presented several outstanding problems including safety issues regarding unwanted differentiation of transplanted MSCs which limit their therapeutic use. Efficient and safe MSC-based therapy for PBC remains a challenging issue that requires continuous cooperation between clinicians, researchers, and patients.

Comparative analysis of cytokeratin 15, TDAG51, cytokeratin 20 and androgen receptor in sclerosing adnexal neoplasms and variants of basal cell carcinoma.

Science.gov (United States)

Evangelista, Mara Therese P; North, Jeffrey P

2015-11-01

Desmoplastic trichoepithelioma (DTE), morpheaform basal cell carcinoma (BCC) and microcystic adnexal carcinoma (MAC) are sclerosing adnexal neoplasms with overlapping histopathologic features. We compared cytokeratin 15, (CK15), T-cell death-associated gene 51 (TDAG51), cytokeratin 20 (CK20) and androgen receptor (AR) in differentiating these tumors and assessed their expression in BCC subtypes. Fifteen DTE, 15 infundibulocystic BCC, 18 micronodular BCC, 18 morpheaform BCC and 6 MAC were assessed for CK15, TDAG51, CK20 and AR expression. Quantitative CK15 staining was higher in DTE compared with BCC (p < 0.0001) and MAC (p = 0.02). Quantitative TDAG51 staining was higher in DTE than BCC (p < 0.0001). The CK20+AR- immunophenotype was 100% sensitive and specific in diagnosing DTE. The CK20-AR+ immunophenotype was 95.24% specific and 83.33% sensitive for BCC. The CK20-AR- immunophenotype was 83.33% sensitive and 90.91% specific for MAC. CK15, CK20 and AR were positive in 87, 53 and 67% of infundibulocystic BCC cases, respectively. Combination of CK20 and AR best differentiated these sclerosing adnexal neoplasms. Greater positivity for CK15 and TDAG51 generally favors benign lesions. Infundibulocystic BCC has higher CK20 and lower AR immunopositivity than other BCC variants and a high degree of CK15 and TDAG51 positivity. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Regional cerebral metabolic rate for glucose and cerebrospinal fluid monoamine metabolites in subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Yanai, Kazuhiko; Miyabayashi, Shigeaki; Iinuma, Kazuie; Tada, Keiya; Fukuda, Hiroshi; Ito, Masatoshi; Matsuzawa, Taiju.

1987-01-01

Regional cerebral metabolic rate for glucose (rCMRglu) and cerebrospinal fluid monoamine metabolites were measured in two cases of subacute sclerosing panencephalitis (SSPE) with different clinical courses. A marked decrease in rCMRglu was found in the cortical gray matter of a patient with rapidly developing SSPE (3.6 - 4.2 mg/100 g brain tissue/min). However, the rCMRglu was preserved in the caudate and lenticular nuclei of the patient (7.7 mg/100 g/min). The rCMRglu in a patient with slowly developing SSPE revealed patterns and values similar to those of the control. Cerebrospinal fluid monoamine metabolites ; homovanilic acid and 5-hydroxyindoleacetic acid, were decreased in both rapidly and slowly developing SSPE. These data indicated that rCMRglu correlated better with the neurological and psychological status and that dopaminergic and serotonergic abnormalities have been implicated in pathophysiology of SSPE. (author)

Use of ursodeoxycholic acid in patients with hypertransaminasemia

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Claudio Puoti

2014-09-01

Full Text Available Ursodeoxycholic acid (UDCA is a hydrophilic bile acid deriving from 7β epimerization of primary bile compound chenodeoxycholic acid. It may have antioxidant, immunomodulatory, antifibrotic and cytoprotective effects, therefore it has been extensively used in the treatment of cholestatic chronic liver diseases (primary biliary cirrhosis, primary sclerosing cholangitis, cholestasis of pregnancy, etc.. Due to the effectiveness of UDCA in decreasing serum liver enzyme levels also in non-cholestatic patients with chronic liver damage of various etiologies, this bile acid is now extensively used in clinical practice in combination with standard therapies, or as alternative treatment. In this paper, after a review of the biochemistry and physiology of UDCA, we have analyzed available data from the international literature on the efficacy and safety of UDCA in patients with hypertransaminasemia due to non-cholestatic chronic liver diseases, such as chronic viral hepatitis B and C and liver steatosis, the three main causes of aminotransferase elevation in western countries.

Andrographolide Ameliorates Beta-Naphthoflavone-Induced CYP1A Enzyme Activity and Lipid Peroxidation in Hamsters with Acute Opisthorchiasis.

Science.gov (United States)

Udomsuk, Latiporn; Chatuphonprasert, Waranya; Jarukamjorn, Kanokwan; Sithithaworn, Paiboon

2016-01-01

Opisthorchis viverrini (OV) infection generates oxidative stress/free radicals and is considered as a primary cause ofcholangiocarcinoma since it primarily triggers sclerosing cholangitis. In this study, the impacts of andrographolide on acute opisthorchaisis in β-naphthoflavone (BNF)-exposed hamsters were investigated. Ethoxyresorufin O-deethylase (EROD) and methoxyresorufin O-demethylase (MROD) activities and Thiobarbituric acid reaction substances (TBARS) assay of andrographolide in acute opisthorchiasis in the BNF-exposed hamsters were assessed. The results showed that andrographolide ameliorated the hepatic CYP1A1 and CYP1A2 activities by decreases of the specific enzymatic reactions of EROD and MROD, respectively, in the BNF-exposed hamsters. Moreover, andrographolide lowered the formation of malondialdehyde in the livers and brains of the hamsters. These observations revealed the promising chemo-protective and antioxidant activities of andrographolide via suppression of the specific EROD and MROD reactions and lipid peroxidation against acute opisthorchiasis in the BNF-exposed hamsters.

Endoscopic management of benign biliary strictures: Possibility or exercise in futility?

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Pierre H Deprez

2012-01-01

Full Text Available Benign biliary strictures for which endoscopic treatment is proposed are mostly related to liver transplantation or chronic pancreatitis (one third of cases each and, less frequently, to other causes (e. g., cholecystectomy, sphincterotomy. The question of futility of exercise may therefore be of importance before embarking in these techniques. Endoscopic treatment of iatrogenic (post-operative benign strictures may be considered as the gold standard since 90% of success is achieved with multiple stent placement. In strictures due to chronic pancreatitis, success rates are lower and surgery may be an appropriate alternative, although it may not be futile to propose an endoscopic try, especially when strictures are related to acute pancreatitis, pseudocyst obstruction or any reversible pancreatic cause of obstruction. In sclerosing cholangitis, endoscopic management is also focused on detection of malignancy. It should therefore not be considered as a futile exercise, but indications and aims of endotherapy should be discussed in a multidisciplinary team involving gastroenterologists, radiologists, and surgical specialists.

Current topics in autoimmune hepatitis.

Science.gov (United States)

Muratori, Luigi; Muratori, Paolo; Granito, Alessandro; Pappas, Giorgios; Cassani, Fabio; Lenzi, Marco

2010-11-01

Autoimmune hepatitis is a chronic liver disease of unknown aetiology characterized by interface hepatitis, hypergammaglobulinaemia and circulating autoantibodies. In the last decade a number of advancements have been made in the field of clinical and basic research: the simplified diagnostic criteria, the complete response defined as normalization of transaminase levels, the molecular identification of the antigenic targets of anti-liver cytosol antibody type 1 and anti-soluble liver antigen, the detection of anti-actin antibodies, the description of de novo autoimmune hepatitis after liver transplantation for non-autoimmune liver diseases, the characterization of autoimmune hepatitis with overlapping features of primary biliary cirrhosis or primary sclerosing cholangitis, the preliminary experience with novel treatment strategies based on cyclosporine, mycophenolate mofetil and budesonide, the role played by "impaired" regulatory T cells and the development of novel animal models of autoimmune hepatitis. Copyright © 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

IgG4 Cholangiopathy

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Yoh Zen

2012-01-01

Full Text Available IgG4 cholangiopathy can involve any level of the biliary tree which exhibits sclerosing cholangitis or pseudotumorous hilar lesions. Most cases are associated with autoimmune pancreatitis, an important diagnostic clue. Without autoimmune pancreatitis, however, the diagnosis of IgG4-cholangiopathy is challenging. Indeed such cases have been treated surgically. IgG4-cholangiopathy should be diagnosed based on serological examinations including serum IgG4 concentrations, radiological features, and histological evidence of IgG4+ plasma cell infiltration. Steroid therapy is very effective even at disease relapse. A Th2-dominant immune response or the activation of regulatory T cells seems to be involved in the underlying immune reaction. It is still unknown why IgG4 levels are specifically elevated in patients with this disease. IgG4 might be secondarily overexpressed by Th2 or regulatory cytokines given the lack of evidence that IgG4 is an autoantibody.

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

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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.

Risk for colorectal cancer in ulcerative colitis: changes, causes and management strategies.

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Lakatos, Peter-Laszlo; Lakatos, Laszlo

2008-07-07

The risk of colorectal cancer for any patient with ulcerative colitis is known to be elevated, and is estimated to be 2% after 10 years, 8% after 20 years and 18% after 30 years of disease. Risk factors for cancer include extent and duration of ulcerative colitis, primary sclerosing cholangitis, a family history of sporadic colorectal cancer, severity of histologic bowel inflammation, and in some studies, young age at onset of colitis. In this review, the authors discuss recent epidemiological trends and causes for the observed changes. Population-based studies published within the past 5 years suggest that this risk has decreased over time, despite the low frequency of colectomies. The crude annual incidence rate of colorectal cancer in ulcerative colitis ranges from approximately 0.06% to 0.16% with a relative risk of 1.0-2.75. The exact mechanism for this change is unknown; it may partly be explained by the more widespread use of maintenance therapy and surveillance colonoscopy.

Liver transplantation in the Nordic countries – An intention to treat and post-transplant analysis from The Nordic Liver Transplant Registry 1982–2013

Science.gov (United States)

Fosby, Bjarte; Melum, Espen; Bjøro, Kristian; Bennet, William; Rasmussen, Allan; Andersen, Ina Marie; Castedal, Maria; Olausson, Michael; Wibeck, Christina; Gotlieb, Mette; Gjertsen, Henrik; Toivonen, Leena; Foss, Stein; Makisalo, Heikki; Nordin, Arno; Sanengen, Truls; Bergquist, Annika; Larsson, Marie E.; Soderdahl, Gunnar; Nowak, Greg; Boberg, Kirsten Muri; Isoniemi, Helena; Keiding, Susanne; Foss, Aksel; Line, Pål-Dag; Friman, Styrbjörn; Schrumpf, Erik; Ericzon, Bo-Göran; Höckerstedt, Krister; Karlsen, Tom H.

2015-01-01

Abstract Aim and background. The Nordic Liver Transplant Registry (NLTR) accounts for all liver transplants performed in the Nordic countries since the start of the transplant program in 1982. Due to short waiting times, donor liver allocation has been made without considerations of the model of end-stage liver disease (MELD) score. We aimed to summarize key outcome measures and developments for the activity up to December 2013. Materials and methods. The registry is integrated with the operational waiting-list and liver allocation system of Scandiatransplant (www.scandiatransplant.org) and accounted at the end of 2013 for 6019 patients out of whom 5198 were transplanted. Data for recipient and donor characteristics and relevant end-points retransplantation and death are manually curated on an annual basis to allow for statistical analysis and the annual report. Results. Primary sclerosing cholangitis, acute hepatic failure, alcoholic liver disease, primary biliary cirrhosis and hepatocellular carcinoma are the five most frequent diagnoses (accounting for 15.3%, 10.8%, 10.6%, 9.3% and 9.0% of all transplants, respectively). Median waiting time for non-urgent liver transplantation during the last 10-year period was 39 days. Outcome has improved over time, and for patients transplanted during 2004–2013, overall one-, five- and 10-year survival rates were 91%, 80% and 71%, respectively. In an intention-to-treat analysis, corresponding numbers during the same time period were 87%, 75% and 66%, respectively. Conclusion. The liver transplant program in the Nordic countries provides comparable outcomes to programs with a MELD-based donor liver allocation system. Unique features comprise the diagnostic spectrum, waiting times and the availability of an integrated waiting list and transplant registry (NLTR). PMID:25959101

Coffee and autoimmunity: More than a mere hot beverage!

Science.gov (United States)

Sharif, Kassem; Watad, Abdulla; Bragazzi, Nicola Luigi; Adawi, Mohammad; Amital, Howard; Shoenfeld, Yehuda

2017-07-01

Coffee is one of the world's most consumed beverage. In the last decades, coffee consumption has attracted a huge body of research due to its impact on health. Recent scientific evidences showed that coffee intake could be associated with decreased mortality from cardiovascular and neurological diseases, diabetes type II, as well as from endometrial and liver cancer, among others. In this review, on the basis of available data in the literature, we aimed to investigate the association between coffee intake and its influence on the immune system and the insurgence of the most relevant autoimmune diseases. While some studies reported conflicting results, general trends have been identified. Coffee consumption seems to increase the risk of developing rheumatoid arthritis (RA) and type 1 diabetes mellitus (T1DM). By contrast, coffee consumption may exert a protective role against multiple sclerosis, primary sclerosing cholangitis, and ulcerative colitis. Concerning other autoimmune diseases such as systemic lupus erythematosus, psoriasis, primary biliary cholangitis and Crohn's disease, no significant association was found. In other studies, coffee consumption was shown to influence disease course and management options. Coffee intake led to a decrease in insulin sensitivity in T1DM, in methotrexate efficacy in RA, and in levothyroxine absorption in Hashimoto's disease. Further, coffee consumption was associated with cross reactivity with gliadin antibodies in celiac patients. Data on certain autoimmune diseases like systemic sclerosis, Sjögren's syndrome, and Behçet's disease, among others, are lacking in the existent literature. As such, further research is warranted. Copyright © 2017 Elsevier B.V. All rights reserved.

IgG4-related disease of the biliary tract and pancreas: clinical and experimental advances.

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Hubers, Lowiek M; Beuers, Ulrich

2017-07-01

IgG4-related disease (IgG4-RD) is an immune-mediated disease of unknown cause. It predominantly affects the biliary tract [IgG4-associated cholangitis (IAC)] and pancreas [autoimmune pancreatitis (AIP)] of mostly elderly men. Accurate diagnostic tests are lacking. Patients benefit from predniso(lo)ne treatment. However, disease relapse is often seen. This review will address pathophysiological aspects and advances in diagnostic and therapeutic strategies. The role of IgG1 and IgG4 in the pathophysiology of IgG4-RD was studied in mice which showed more intense organ damage of pancreas and salivary glands when IgG1 rather than IgG4 of patients with IgG4-RD was injected. Coadministration of IgG1+IgG4 led to dampening of IgG1-mediated injury supporting the view that IgG4 exerts immune-dampening effects. IgG4+ B-cell receptor clones identified by next-generation sequencing and the IgG4/IgG RNA ratio in human blood assessed by quantitative PCR were able to accurately distinguish IAC/AIP from primary sclerosing cholangitis or pancreatobiliary malignancies. Long-term treatment with low-dose prednisolone was safe and reduced the number of flare-ups in patients with AIP. Early diagnosis by a novel accurate and easy-to-use qPCR test may prevent life-threatening complications, unnecessary interventions and fatal course because of misdiagnosis. Prednisolone treatment remains the standard of care in patients with IgG4-RD.

Percutaneous transhepatic cholangiography: analysis of 120 cases

International Nuclear Information System (INIS)

Sohn, Hyung Sun; Shin, Kyung Sub; Kang, Hyo Seok

1981-01-01

Percutaneous transhepatic cholangiography (PTC) is of value in differential diagnosis of cholestatic diseases. Authors had performed PTC in 120 patients with flexible needle of 23 gauge at the Department of Radiology, Kang Nam St. Mary's Hospital and Myung Dong St. Mary's Hospital during the period from January, 1976 to April, 1980. PTC was accomplished successfully in 112 of 120 patients. Diagnoses included cholangiocarcinomas (27 cases), carcinomas of pancreas head (21 cases), ampullary carcinomas (4 cases), metastatic carcinomas (5 cases), bile duct stones (27 cases), sclerosing cholangitis (6 cases), hepatitis (6 cases), liver cirrhosis (6 cases), post operative adhesions (5 cases), chronic pancreatitis (3 cases), stomach carcinomas (3 cases), clonorchiasis (2 cases), blood clot (1 case), and remaining normal 4 cases. Some characteristic PTC findings were: (1) segmental annular narrowing and abrupt complete obstruction and polyploid filling defects of the bile duct in cholangiocarcinoma, (2) typical nipple or rat-tail appearance of the distal common bile duct in pancreatic head carcinoma, (3) single or multiple sharply and smoothly outlined filling defects within bile duct in all cases of stones, (4) complete obstruction with shallow concavity in ampullary carcinoma, (5) diffuse or segmental narrowing of the intrahepatic bile duct and common bile duct in sclerosing cholangitis, (6) multiple tiny polypoid filling defects of gall bladder, common hepatic duct, and common bile duct in clonorchiasis. (7) normal appearance in hepatitis. The overall diagnostic accuracy of PTC in 66 operated cases was 89.4%, and complications following the examination were significantly reduced by using a fine flexible needle (23 gauge). From the present study it is concluded as follows: 1. To evaluate obstructed or stenosed site accurately, aspiration of bile juice must be preceded by a 23 gauge needle connected to either 10 ml or 5 ml syringe. 2. To diagnose carcinoma of the

A comparative evaluation of early stent occlusion among biliary conventional versus wing stents.

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Khashab, Mouen A; Hutfless, Susan; Kim, Katherine; Lennon, Anne Marie; Canto, Marcia I; Jagannath, Sanjay B; Okolo, Patrick I; Shin, Eun Ji; Singh, Vikesh K

2012-06-01

Conventional plastic stents with a lumen typically have limited patency. The lumenless wing stent was engineered to overcome this problem. The objective of this study was to compare the incidence of early stent occlusion (symptomatic occlusion/cholangitis necessitating re-insertion within 90 days) for wing stents and conventional plastic stents. Patients with biliary pathology treated with plastic biliary stenting during the period 2003-2009 comprised the study cohort. Patients who had at least one biliary wing stent placed comprised the wing stent group, whereas patients who underwent only conventional stent plastic placement comprised the conventional stent group. Patients were stratified by indication: benign biliary strictures (group 1), malignant biliary strictures (group 2), or benign biliary non-stricture pathology (group 3). The association of stent type with the occurrence of primary outcome by indication was analyzed by use of multivariable logistic regression. Three-hundred and forty-six patients underwent 612 ERCP procedures with placement of plastic biliary stent(s). On multivariate analysis, early stent occlusion did not differ between the wing and conventional groups in groups 1, 2, and 3. Among patients who achieved primary outcome in group 2, significantly fewer patients in the wing group had cholangitis (6.7% vs. 39.1%, P = 0.03). Among patients who achieved primary outcome in group 3, significantly fewer patients in the wing group had cholangitis (10% vs. 50%, P = 0.03). Early stent occlusion was similar for wing stents and conventional plastic stents. Wing stents, however, were associated with a lower incidence of cholangitis in patients with malignant biliary obstruction and benign non-stricturing biliary pathology.

A possible case of Garre's sclerosing osteomyelitis from Medieval Tuscany (11th-12th centuries).

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Giuffra, Valentina; Vitiello, Angelica; Giusiani, Sara; Caramella, Davide; Fornaciari, Gino

2015-12-01

Archaeological excavations carried out at the castle of Monte di Croce near Florence brought to light a small cemetery complex belonging to the castle church, dated back to the 11th-12th centuries. An elite stone tomb contained the skeletal remains of a male aged 35-45 years with obvious pathology of the right tibia. The proximal metaphysis and the upper half of the diaphysis appear massively enlarged as a result of severe chronic periostitis. A transverse section illustrates complete obliteration of the medullary cavity by new spongy bone, with some large cavitations. The primary, but completely remodeled tibial shaft is still recognizable. This finding and the strong sclerotic reaction with some central cavitations rule out any form of bone tumor and indicate a chronic inflammatory disease. The morphological and radiological picture and the tibial localization suggest a diagnosis of chronic sclerosing osteomyelitis of Garré, a rare form of chronic osteomyelitis characterized by an intense periosteal reaction with little or no suppuration. Copyright © 2015. Published by Elsevier Inc.

Diffuse sclerosing variant of thyroid papillary carcinoma: Diagnostic challenges occur with Hashimoto's thyroiditis

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Chien-Chin Chen

2013-06-01

Full Text Available Diffuse sclerosing papillary thyroid carcinoma (DSPTC is a relatively rare variant of papillary thyroid carcinoma with distinct histological features, radiological characteristics, and biological aggressiveness. Compared with conventional papillary thyroid carcinoma, DSPTC is characterized by scattered microscopic tumor islands, diffuse fibrosis, calcification, and abundant lymphocytic aggregation. A preoperative diagnosis is challenging in the absence of nodules and scanty fine needle aspiration cytology samples. We describe a unique DSPTC patient, an 18-year-old woman who presented with a neck mass that grew slowly for 2 years. The palpable neck mass was nontender, well defined, firm, and unmovable. Laboratory studies showed normal thyroid function and positive autoimmune markers: antithyroglobulin antibody = 1:1600 and antimicrosomal antibody = 1:1600. A neck ultrasound showed diffusely prominent microcalcifications with one small vague nodule. Hashimoto's thyroiditis with an accompanying malignancy was suspected. Based on the result of intraoperative pathology reports, the patient was given a total thyroidectomy. Lymph node dissection and histological analysis revealed bilateral DSPTC in addition to lymphocytic thyroiditis in nonmalignant areas of the thyroid. Clinical and histological diagnostic challenges usually occur when DSPTC presents with a diffuse thyroid enlargement, dispersed microscopic tumor islands (frequently without mass formation, extensive fibrosis, and abundant lymphocytic infiltration mimicking thyroiditis.

Biomarkers in premalignant conditions of the gastrointestinal tract: Studies on Barrett’s esophagus and primary sclerosing cholangitis

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Timmer, M.R.

2016-01-01

In this thesis we have described our research on genetic abnormalities in (pre)malignant conditions of the gastrointestinal tract. The wide variation in biological behavior of cancerous and precancerous conditions may be largely explained by differences in genetic abnormalities. They are a source of

Central Retinal and Posterior Ciliary Artery Occlusion After Intralesional Injection of Sclerosant to Glabellar Subcutaneous Hemangioma

International Nuclear Information System (INIS)

Matsuo, Toshihiko; Fujiwara, Hiroyasu; Gobara, Hideo; Mimura, Hidefumi; Kanazawa, Susumu

2009-01-01

The aim of this study is to describe vision loss caused by central retinal artery and posterior ciliary artery occlusion as a consequence of sclerotherapy with a polidocanol injection to a glabellar hemangioma. An 18-year-old man underwent direct injection with a 23-gauge needle of 1 mL of a polidocanol-carbon dioxide emulsion into the glabellar subcutaneous hemangioma under ultrasound visualization of the needle tip by radiologists. He developed lid swelling the next day, and 3 days later at referral, the visual acuity in the left eye was no light perception. Funduscopy revealed central retinal artery occlusion and fluorescein angiography disclosed no perfusion at all in the left fundus, indicating concurrent posterior ciliary artery occlusion. The patient also showed mydriasis, blepharoptosis, and total external ophthalmoplegia on the left side. Magnetic resonance imaging demonstrated the swollen medial rectus muscle. In a month, blepharoptosis and ophthalmoplegia resolved but the visual acuity remained no light perception. Sclerosing therapy for facial hemangioma may develop a severe complication such as permanent visual loss.

Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature.

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Terziroli Beretta-Piccoli, Benedetta; Guillod, Caroline; Marsteller, Igor; Blum, Roland; Mazzucchelli, Luca; Mondino, Chiara; Invernizzi, Pietro; Gershwin, M Eric; Mainetti, Carlo

2017-08-01

Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease. It is often associated with extrahepatic autoimmune diseases. Skin disorders are sporadically reported in association with PBC. We report an unusual case of PBC associated with acquired reactive perforating dermatosis (ARPD) and present a review of the literature on skin disorders associated with PBC. Our patient presented to the dermatology department with generalized pruritus associated with nodular perforating skin lesions on the trunk, and cholestatic liver disease of unknown origin. After having established both diagnosis of ARPD and PBC, she was managed in an interdisciplinary manner, and both her skin and liver conditions improved gradually. Only one similar case is reported in the literature, in that case, the liver disease was not treated. By reviewing the literature, we found that lichen planus, vitiligo, and psoriasis are the most frequent skin disorders associated with PBC. However, there is only limited data about specific skin disorders associated with PBC. This case report of a patient with PBC associated with ARPD underlines the importance of interdisciplinary management of patients with rare liver diseases combined with rare skin disorders. The present review of the literature shows that probably, immune-mediated skin conditions are not more frequent in PBC patients than in the general population. However, the available data are scant; there is a need for high-quality data on skin conditions associated with PBC.

Hemagglutinin-specific neutralization of subacute sclerosing panencephalitis viruses.

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Miguel Ángel Muñoz-Alía

Full Text Available Subacute sclerosing panencephalitis (SSPE is a progressive, lethal complication of measles caused by particular mutants of measles virus (MeV that persist in the brain despite high levels of neutralizing antibodies. We addressed the hypothesis that antigenic drift is involved in the pathogenetic mechanism of SSPE by analyzing antigenic alterations in the MeV envelope hemagglutinin protein (MeV-H found in patients with SSPE in relation to major circulating MeV genotypes. To this aim, we obtained cDNA for the MeV-H gene from tissue taken at brain autopsy from 3 deceased persons with SSPE who had short (3-4 months, SMa79, average (3.5 years, SMa84, and long (18 years, SMa94 disease courses. Recombinant MeVs with a substituted MeV-H gene were generated by a reverse genetic system. Virus neutralization assays with a panel of anti-MeV-H murine monoclonal antibodies (mAbs or vaccine-immunized mouse anti-MeV-H polyclonal sera were performed to determine the antigenic relatedness. Functional and receptor-binding analysis of the SSPE MeV-H showed activity in a SLAM/nectin-4-dependent manner. Similar to our panel of wild-type viruses, our SSPE viruses showed an altered antigenic profile. Genotypes A, G3, and F (SSPE case SMa79 were the exception, with an intact antigenic structure. Genotypes D7 and F (SSPE SMa79 showed enhanced neutralization by mAbs targeting antigenic site IIa. Genotypes H1 and the recently reported D4.2 were the most antigenically altered genotypes. Epitope mapping of neutralizing mAbs BH015 and BH130 reveal a new antigenic site on MeV-H, which we designated Φ for its intermediate position between previously defined antigenic sites Ia and Ib. We conclude that SSPE-causing viruses show similar antigenic properties to currently circulating MeV genotypes. The absence of a direct correlation between antigenic changes and predisposition of a certain genotype to cause SSPE does not lend support to the proposed antigenic drift as a

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

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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.

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

Science.gov (United States)

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; Hadzic, Nedim

2017-11-03

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 (ASC), and de novo AIH after liver transplantation. Two types of paediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (anti-LKM-1/anti-LC-1; AIH-2).Pertinent issues addressing the diagnosis, treatment and long term follow up were formulated by a core group of ESPGHAN members. They have commissioned the first authors with execution of this project. Initially, they have performed a systematic literature search on MEDLINE, ResearchGate and Mendeley databases over the last 30 years and produced a document focusing on prospective and retrospective studies in children. The ESPGHAN core group and ESPGHAN Hepatology Committee members voted on each recommendation, using a formal voting technique.

The ability of anti-carbonic anhydrase II antibody to distinguish autoimmune cholangitis from primary biliary cirrhosis in Japanese patients.

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Akisawa, N; Nishimori, I; Miyaji, E; Iwasaki, S; Maeda, T; Shimizu, H; Sato, N; Onishi, S

1999-06-01

Serum antibody against carbonic anhydrase (CA) II has been described as a serological marker for distinguishing autoimmune cholangitis (AIC) from primary biliary cirrhosis (PBC). To validate this finding in a Japanese population, we evaluated sera from patients with PBC and AIC for antibody to human CA II. An enzyme-linked immunosorbent assay was employed to quantify serum antibody against CA II in patients with PBC (n = 40), AIC (n = 23), autoimmune hepatitis (n = 10), and extrahepatic obstructive jaundice (n = 10). Compared with the finding of a 4% prevalence of anti-CAII antibody in healthy subjects (n = 24), a significantly higher prevalence of anti-CA II antibody was detected in patients with PBC (35%) and AIC (30%) (P jaundice. No significant difference was observed between PBC and AIC patients. These results showed that AIC and PBC would be indistinguishable by anti-CA II antibody testing in Japanese patients. However, the finding of serum anti-CA II antibody in patients with PBC and AIC supports the disease concept of autoimmune exocrinopathy.

18F-FDG PET and MRS of the early stages of subacute sclerosing panencephalitis in a child with a normal initial MRI

International Nuclear Information System (INIS)

Seo, Yeong-Seon; Jung, Da-Eun; Kim, Ho-Sung

2010-01-01

In subacute sclerosing panencephalitis (SSPE), conventional MRI findings have been reported. However, in the early clinical stages, imaging studies can appear normal. Moreover, with no history of infant measles infection, the diagnosis of SSPE can only be arrived at after extensive investigation that must eliminate a number of neurodegenerative diseases. We report here on 18 F-fluorodeoxyglucose positron emission tomography ( 18 F-FDG PET) and magnetic resonance spectroscopy (MRS) findings in a 14-year-old girl with a normal initial MRI who had not contracted measles. Although 18 F-FDG PET and MRS are not specific or diagnostic for SSPE, these techniques can demonstrate substantial metabolic impairments when MRI findings show no obvious abnormalities, as is often the case in the early stages of this disease. (orig.)

Complications of Sclerotherapy with Sclerosing Foam in Lower Extremity Varicose Veins

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Hossein Hemmati

2015-01-01

Full Text Available Background: Using sclerotherapy with foam has caused a great change in treatment of varicose veins. Although, it is more than a century that it is being used, no exact and comprehensive knowledge of its complications has been published yet with regard to the existing facilities in Iran. Materials and Methods: In this cross-sectional study, Patients with varicose veins of lower extremity referred to cardiology clinic of Razi hospital, Rasht were included in the study after doppler sonography and in case of presence of varicose veins with more than 2 mm diameter in lower extremities without inadequacy of saphenofemoral and saphenopopliteal valve. They then underwent sclerosing with foam treatment. The total number of patients was 156 who were examined 2 weeks and 3 months after sclerotherapy in terms of complications such as pain, pigmentation, recurrence, phlebitis, deep vein thrombosis and skin necrosis. Results: Out of 156 patients, 47 were men and 109 women whose mean±SD age was 46.5±12.2 years. Two weeks after sclerotherapy, pain in 95 patients (65.1%, pigmentation in 79 patients (53.4%, recurrence in 5 patients (3.4%, necrosis in 8 patients (5.5% and no phlebitis was witnessed. Three months after sclerotherapy, pain in 10 patients (6.8%, pigmentation in 52 patients (35.1%, recurrence in 13 patients (8.8% and phlebitis in 13 patients (8.8% were seen; however, necrosis was not observed Conclusion: Sclerotherapy with foam is an effective, safe and inexpensive method for treatment of varicose veins of lower extremities. Thus, in case of a careful selection of patients and conduction in an equipped center, few complications will be seen.

Recurrent pyogenic cholangitis: clinico-pathologic correlation of focal attenuation differences on multi-phasic spiral CT

International Nuclear Information System (INIS)

Jeong, Jun Yong; Han, Joon Koo; Kim, Tae Kyoung; Kim, Seog Joon; Kim, Hyun Bum; Choi, Byung Ihn

2002-01-01

To determine the clinical and the pathologic significance of the focal attenuation differences (FAD) and bile duct wall enhancement occurring in recurrent pyogenic cholangitis (RPC) and seen at multiphasic spiral CT. Among the multiphasic (non-contrast, arterial and portal or delayed phase) spiral CT findings of 60 consecutive patients, two types of FAD were noted during the non-contrast phase. These were Type A (iso) and Type B (low attenuation), and their distribution pattern (lobar versus patchy, multifocal) and the and the presence or absence of bile duct wall enhancement were recorded. The radiologic findings were correlated with the clinical and pathologic findings. Two types of FAD were noted in 40 of the 60 patients. Active in flammation was present in 19 of the 27 with Type-A and in ten of the 15 in whom the presence of RPC was pathologically proven. Ten of the 13 with Type-B FAD were in a subclinical state, and nine of the ten in whom RPC was pathologically proven had chronic inflammation. Among 20 patients who did not have FAD, RPC was subclinical in 18 and dormant in nine of the eleven in whom its presence was pathologically proven (p<0.001). Clinico-pathologic correlation with bile duct wall enhancement and the distribution pattern of FAD showed no statistical significance. The inflammatory activity of RPC can be predicted by analysis of the FAD seen at multiphasic spiral CT

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...

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...

Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

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Seokhwi Kim

2016-07-01

Full Text Available IgG4-related disease (IgG4-RD may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.

Expression of defective measles virus genes in brain tissues of patients with subacute sclerosing panencephalitis

International Nuclear Information System (INIS)

Baczko, K.; Liebert, U.G.; Billeter, M.; Cattaneo, R.; Budka, H.; Ter Meulen, V.

1986-01-01

The persistence of measles virus in selected areas of the brains of four patients with subacute sclerosing panencephalitis (SSPE) was characterized by immunohistological and biochemical techniques. The five measles virus structural proteins were never simultaneously detectable in any of the bran sections. Nucleocapsid proteins and phosphoproteins were found in every diseased brain area, whereas hemagglutinin protein was detected in two cases, fusion protein was detected in three cases, and matrix protein was detected in only one case. Also, it could be shown that the amounts of measles virus RNA in the brains differed from patient to patient and in the different regions investigated. In all patients, plus-strand RNAs specific for these five viral genes could be detected. However, the amounts of fusion and hemagglutinin mRNAs were low compared with the amounts in lytically infected cells. The presence of particular measles virus RNAs in SSPE-infected brains did not always correlate with mRNA activity. In in vitro translations, the matrix protein was produced in only one case, and the hemagglutinin protein was produced in none. These results indicate that measles virus persistence in SSPE is correlated with different defects of several genes which probably prevent assembly of viral particles in SSPE-infected brain tissue

Radio-opaque ethylcellulose-ethanol is a safe and efficient sclerosing agent for venous malformations

International Nuclear Information System (INIS)

Dompmartin, Anne; Barrellier, Marie-Therese; Blaizot, Xavier; Chene, Yannick; Gaillard, Cathy; Theron, Jacques; Hammer, Frank; Labbe, Daniel; Leroyer, Robert; Chedru, Valerie; Ollivier, Catherine; Vikkula, Miikka; Boon, Laurence M.

2011-01-01

To evaluate the efficacy and safety of gelified ethanol, a newly developed sclerosing agent for slow-flow vascular malformations. Seventy-nine sclerotherapy procedures were performed on 44 patients with 37 venous malformations, 2 glomuvenous malformations, 2 lymphatic malformations, 2 lymphatico-venous malformations, and 1 Klippel-Trenaunay syndrome. The median injected volume was 1.00 mL/site of injection. Effects of sclerotherapy on pain, functional and cosmetic disturbance were statistically evaluated with a final result score. Local and systemic complications were recorded. The mean Visual Analogue Scores were 5.20 ± 2.81 before and 1.52 ± 1.25 after treatment (p < 0.001). Functional and aesthetic improvement was achieved in 31/35 patients (89%) and in 33/41 (80%), respectively. Minor local side effects included necrosis with or without issue of ethylcellulose, palpable residue, and hematoma. No systemic side-effects occurred. Per mL used, radio-opaque gelified ethanol is at least as effective as absolute ethanol. No systemic complication was observed, as only a low dose of ethanol was injected. Indications for sclerotherapy can be widened to areas with higher risk for local side effects (hands and periocular region), as ethanol is trapped in the lesion. Careful injection procedure is though necessary, because only a limited amount of ethylcellulose can be used per puncture. (orig.)

Trichloroethylene Exposure Reduces Liver Injury in a Mouse Model of Primary Biliary Cholangitis.

Science.gov (United States)

Ray, Jessica L; Kopec, Anna K; Joshi, Nikita; Cline-Fedewa, Holly; Lash, Lawrence H; Williams, Kurt J; Leung, Patrick S; Gershwin, M Eric; Luyendyk, James P

2017-04-01

Trichloroethylene (TCE) is a persistent environmental contaminant proposed to contribute to autoimmune disease. Experimental studies in lupus-prone MRL+/+ mice have suggested that TCE exposure can trigger autoimmune hepatitis. The vast majority of studies examining the connection between TCE and autoimmunity utilize this model, and the impact of TCE exposure in other established models of autoimmune liver disease is not known. We tested the hypothesis that TCE exposure exacerbates experimental hepatic autoimmunity in dominant negative transforming growth factor beta receptor type II (dnTGFBRII) mice, which develop serological and histological features resembling human primary biliary cholangitis. Female 8-week-old wild-type and dnTGFBRII mice were exposed to TCE (0.5 mg/ml) or vehicle (1% ethoxylated castor oil) in the drinking water for 12 or 22 weeks. Liver histopathology in 20- and 30-week-old wild-type mice was unremarkable irrespective of treatment. Mild portal inflammation was observed in vehicle-exposed 20-week-old dnTGFBRII mice and was not exacerbated by TCE exposure. Vehicle-exposed 30-week-old dnTGFBRII mice developed anti-mitochondrial antibodies, marked hepatic inflammation with necrosis, and hepatic accumulation of both B and T lymphocytes. To our surprise, TCE exposure dramatically reduced hepatic parenchymal inflammation and injury in 30-week-old dnTGFBRII mice, reflected by changes in hepatic proinflammatory gene expression, serum chemistry, and histopathology. Interestingly, TCE did not affect hepatic B cell accumulation or induction of the anti-inflammatory cytokine IL10. These data indicate that TCE exposure reduces autoimmune liver injury in female dnTGFBRII mice and suggests that the precise effect of environmental chemicals in autoimmunity depends on the experimental model. © The Author 2017. Published by Oxford University Press on behalf of the Society of Toxicology. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

{sup 18}F-FDG PET and MRS of the early stages of subacute sclerosing panencephalitis in a child with a normal initial MRI

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Seo, Yeong-Seon; Jung, Da-Eun [Ajou University School of Medicine, Department of Pediatrics, Suwon, Kyungki-do (Korea, Republic of); Kim, Ho-Sung [Ajou University School of Medicine, Department of Radiology, Suwon, Kyungki-do (Korea, Republic of)

2010-11-15

In subacute sclerosing panencephalitis (SSPE), conventional MRI findings have been reported. However, in the early clinical stages, imaging studies can appear normal. Moreover, with no history of infant measles infection, the diagnosis of SSPE can only be arrived at after extensive investigation that must eliminate a number of neurodegenerative diseases. We report here on {sup 18} F-fluorodeoxyglucose positron emission tomography ({sup 18}F-FDG PET) and magnetic resonance spectroscopy (MRS) findings in a 14-year-old girl with a normal initial MRI who had not contracted measles. Although {sup 18} F-FDG PET and MRS are not specific or diagnostic for SSPE, these techniques can demonstrate substantial metabolic impairments when MRI findings show no obvious abnormalities, as is often the case in the early stages of this disease. (orig.)

Sclerosing Epithelioid Fibrosarcoma of the Bone: A Case Report of High Resistance to Chemotherapy and a Survey of the Literature

Directory of Open Access Journals (Sweden)

Thomas G. P. Grunewald

2010-01-01

Full Text Available Sclerosing epithelioid fibrosarcoma (SEF is a rare soft tissue sarcoma mostly occurring in extraosseous sites. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. Intraosseous localization is an additional challenge with respect to the therapeutical approach. We report on a 16-year-old patient with SEF of the right proximal tibia. The patient underwent standardized neoadjuvant chemotherapy analogous to the EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprosthetic reconstruction. Histopathological analysis of the resected tumor showed >90% vital tumor cells suggesting no response to chemotherapy. Therefore, therapy was reassigned to the CWS 2002 High-Risk protocol for the treatment of soft tissue sarcoma. To date (22 months after diagnosis, there is no evidence of relapse or metastasis. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin, and Methotrexate, which should be considered in planning treatment for patients with SEF.

Coexisting Sclerosing Angiomatoid Nodular Transformation of the Spleen with Multiple Calcifying Fibrous Pseudotumors in a Patient

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Jen-Chieh Lee

2007-01-01

Full Text Available Primary tumor or tumor-like lesions of the spleen are rare. Among them, vascular lesions are the most common. Vascular tumor of the spleen is different from the usual hemangioma of soft tissue because the vascular structure of the spleen is unique. Sclerosing angiomatoid nodular transformation (SANT is a recently described vascular lesion of the spleen. Grossly, it is a multinodular, well-circumscribed tumor containing a hypervascular core. Microscopically, it comprises three types of vessels, and each type recapitulates the immunohistochemical characteristics of the normal vascular elements of the splenic red pulp, i.e. capillaries, sinusoids, and small veins, respectively. Because of the rarity of this entity, its actual pathogenesis is still unknown. In this study, we report a case of SANT occurring in a 43-year-old woman, in whom there were also multiple calcifying fibrous pseudotumors (CFPTs in the abdominal cavity. Both SANT and CFPT are thought to be variants of inflammatory pseudotumor. Coexistence of these two rare entities in a patient has never been reported, and this fact suggests that there might be a common mechanism contributing to the formation of these two types of lesions. [J Formos Med Assoc 2007;106(3:234-239

Immunohistochemical Examination of a Resected Advanced Hilar Cholangiocarcinoma Arising in a 29-Year-Old Male without Primary Sclerosing Cholangitis

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Taketoshi Suehiro

2010-05-01

Full Text Available A 29-year-old man with advanced hilar cholangiocarcinoma was successfully treated with an extended right lobectomy. The carbohydrate antigen 19-9 (CA19-9 level was elevated to 939 IU/l, and the pathological findings revealed moderately differentiated tubular adenocarcinoma which involved almost the entire thickness of the hepatic duct and the adjacent liver tissue (T3 and which was associated with lymph node metastasis (N1. It was a stage IIB (T3N1M0 tubular adenocarcinoma according to UICC pathological staging. Immunohistochemical examination revealed that Ki-67, cyclin D1, and MMP-7 were positive, and 14-3-3σ and p27 were negative. The pathological and immunohistochemical findings indicated high malignant potential indicating poor prognosis. We administrated the postoperative adjunct gemcitabine combined with S-1 chemotherapy. The patient is alive without recurrence and doing well two years after surgery. We also review other reports of cholangiocarcinoma patients aged less than 30 years.

Analysis of MxA, IL-4, and IRF-1 genes in Filipino patients with subacute sclerosing panencephalitis.

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Pipo-Deveza, J R; Kusuhara, K; Silao, C L T; Lukban, M B; Salonga, A M; Sanchez, B C; Kira, R; Takemoto, M; Torisu, H; Hara, T

2006-08-01

Subacute sclerosing panencephalitis (SSPE) is a chronic and debilitating disease of the central nervous system caused by a latent measles virus infection. Three candidate genes, MxA, IL-4, and IRF-1 genes were shown to be associated with SSPE in Japanese patients. These genes have been suggested to play a role in the establishment of persistent viral infection in the central nervous system. Sixty Filipino SSPE patients and 120 healthy control subjects were included in the study. Single nucleotide polymorphisms at promoter regions ( IL-4-590C/T and MXA-88G/T) were screened using PCR-RFLP method. Genotyping was done for GT repeat polymorphism within intron 7 of IRF-1. The TT genotype of MXA, as well as the CT genotype of IL-4, were seen a little more frequently among the SSPE patients as compared to the control subjects. The values though, did not reach statistical significance. IRF-1 analysis did not differ between the two groups. Our study failed to demonstrate a significant association between IL-4, MXA, or IRF-1, and SSPE in the Filipino population. Our results might be explained by a greater contribution of environmental factors such as the socio-economic and nutritional factors in the susceptibility of Filipinos to SSPE other than genetic factors.

Diffuse sclerosing variant of papillary carcinoma of the thyroid. Clinical importance, surgical treatment, and follow-up study

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Fujimoto, Y.; Obara, T.; Ito, Y.; Kodama, T.; Aiba, M.; Yamaguchi, K. (Tokyo Women' s Medical College (Japan))

1990-12-01

A diffuse sclerosing variant is not very rare among papillary carcinomas of the thyroid when the patients are female and younger than 30 years of age. The variant is characterized by diffuse involvement of one or both thyroid lobes, with dense sclerosis, patchy lymphocytic infiltration, and abundant psammoma bodies. Controversy still exists concerning its prognosis. We reviewed our experience with 14 patients treated between 1958 and 1988. All patients were young females, their age being from 10 to 28 years with a mean of 19.6. Hashimoto's thyroiditis had been suspected in nine patients before they came to our clinic. Nowadays the diagnosis of this cancer is possible when we have this entity in mind and detect abundant psammoma bodies either by ultrasonography or by soft-tissue roentgenography of the neck. Total thyroidectomy with modified neck dissection was carried out in eight patients, subtotal thyroidectomy with neck dissection in five, and lobectomy with neck dissection in one. All of them are alive and well without distant metastasis at a mean follow-up of 16 years. Because most of the patients with this variant of papillary carcinoma are young women and the prognosis is favorable, a complete resection without causing later recurrence, but also cosmetic and complication-free surgery, should be considered.

Massive Infestation of the biliary duct for Ascaris lumbricoides: case report

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Aristizabal, Humberto; Sanabria, Alvaro

2001-01-01

We present a case of a woman who arrived with acute cholangitis symptomatology, and was diagnosed preoperatively by ultrasound as biliary ascariasis. During surgery, we extracted 32 worms of Ascaris lumbricoides from the Gallbladder and the common bile duct. Clinical outcome was successful, with out retention of worms

Comparison of efficacy between sodium morrhuate and lauromacrogol as sclerosing agents in treatment of hepatic cyst

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QIN Zuyun

2015-07-01

Full Text Available Objective To compare the efficacy of sodium morrhuate versus lauromacrogol in the treatment of hepatic cyst. Methods Seventy-four patients with hepatic cyst who were admitted to our hospital from January 2009 to May 2013 were enrolled as subjects and divided into two groups. After the cystic fluid was drained by percutaneous liver biopsy, sodium morrhuate solution was injected into the cystic cavity for adhesion and sclerosis in 46 patients in group A, and lauromacrogol solution was injected in 28 patients in group B. The incidence rates of pain in patients during and after surgery were compared between the two groups. The follow-up comparison of hepatic cyst recurrence rates within one year after surgery was performed between the two groups. Between-group comparison was performed by χ2 test. Results Five patients (10.87% in group A and two patients (7.14% in group B had recurrence within one year after treatment. There was no significant difference in recurrence rate between the two groups (χ2=0.283, P＞0.05. The incidence of pain in group A was significantly higher than that in group B (χ2=5.258, P＜0.05. Conclusion With the same efficacy as sodium morrhuate in the treatment of hepatic cyst, lauromacrogol can be routinely used as a sclerosing agent due to its mild side effects.

Shear wave elastography diagnosis of the diffuse sclerosing variant of papillary thyroid carcinoma: A case report.

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Xue, Nianyu; Xu, Youfeng; Huang, Pintong; Zhang, Shengmin; Wang, Hongwei; Yu, Fei

2016-08-01

The present study aimed to report the shear wave elastography (SWE) findings in a patient with the diffuse sclerosing variant of papillary thyroid carcinoma (DSVPTC). Since patients with DSVPTC may present with typical clinicopathological features and initially appear to have Hashimoto's thyroiditis, a thorough clinical evaluation and an early diagnosis are important. A 20-year-old female patient presented with a 1-month history of a neck mass and sore throat. Conventional ultrasound and SWE were performed using an AIXPLORER system with 14-5 MHz linear transducer. The patient had undergone total thyroidectomy and bilateral neck lymph node dissection, and an intraoperative pathology consultation to confirm the malignancy of lymph node metastasis. Pathological diagnosis was DSVPTC in both lobes, with lymph node metastases in the bilateral neck. The clinical presentation and serological findings were all indicative of Hashimoto's thyroiditis. Thyroid ultrasonography revealed diffuse enlargement of the both lobes, heterogenous echogenicity without mass formation, diffuse scattered microcalcifications and poor vascularization. SWE revealed stiff values of the thyroid: The mean stiffness was 99.7 kpa, the minimum stiffness was 59.1 kpa and the maximum stiffness was 180.1 kpa. The maximum stiffness of the DSVPTC (180.1 kpa) was higher compared with the diagnostic criteria of malignant thyroid nodules (65 kPa). SWE may be considered as a novel and valuable method to diagnose DSVPC.

A new endoscopic therapeutic method for acute obstructive suppurative cholangitis post Roux-en-Y anastomosis: endoscopic retrograde cholangiography through jejunostomy

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Zhuo YANG

2012-01-01

Full Text Available  Objective  To probe the value of endoscopic retrograde cholangiography (ERC through jejunostomy in patients in whom ERC could not be performed via the mouth after Roux-en-Y anastomosis on the upper gastrointestinal tract. Methods  In two patients suffering from acute obstructive suppurative cholangitis after a radical operation for cholangiocarcinoma, ERC could not be performed through the mouth due to the presence of a long non-functional jejunal loop. A jejunostomy was first done in the afferent loop of the jejunum, and a gastroscope was then inserted via the jejunostomy and passed retrogradely, to find the stoma of the cholangiointestinal anastomosis. ERC was then successfully performed, and followed by endoscopic retrograde biliary drainage (ERBD. Results  The operation was successful. It was found that cholangio-jejunostomy stoma was narrow, and a large amount of purulent mucus was present in the enlarged intrahepatic duct. ERC was done to enlarge the stoma, and a stent was placed into the main branch of the intrahepatic duct. Two patients achieved surgical success and smooth recovery after the operation. Conclusion  ERC through a jejunostomy in the patients who had Roux-en-Y cholangiojejunostomy following radical resection for cholangiocarcinoma, is a safe and effective surgical procedure.

Gut microbial profile is altered in primary biliary cholangitis and partially restored after UDCA therapy.

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Tang, Ruqi; Wei, Yiran; Li, Yanmei; Chen, Weihua; Chen, Haoyan; Wang, Qixia; Yang, Fan; Miao, Qi; Xiao, Xiao; Zhang, Haiyan; Lian, Min; Jiang, Xiang; Zhang, Jun; Cao, Qin; Fan, Zhuping; Wu, Maoying; Qiu, Dekai; Fang, Jing-Yuan; Ansari, Aftab; Gershwin, M Eric; Ma, Xiong

2018-03-01

A close relationship between gut microbiota and some chronic liver disorders has recently been described. Herein, we systematically performed a comparative analysis of the gut microbiome in primary biliary cholangitis (PBC) and healthy controls. We first conducted a cross-sectional study of 60 ursodeoxycholic acid (UDCA) treatment-naïve patients with PBC and 80 matched healthy controls. Second, an independent cohort composed of 19 treatment-naïve patients and 34 controls was used to validate the results. Finally, a prospective study was performed in a subgroup of 37 patients with PBC who underwent analysis before and after 6 months of UDCA treatment. Faecal samples were collected, and microbiomes were analysed by 16S ribosomal RNA gene sequencing. A significant reduction of within-individual microbial diversity was noted in PBC (p=0.03). A signature defined by decreased abundance of four genera and increased abundance of eight genera strongly correlated with PBC (area under curve=0.86, 0.84 in exploration and validation data, respectively). Notably, the abundance of six PBC-associated genera was reversed after 6 months of UDCA treatment. In particular, Faecalibacterium , enriched in controls, was further decreased in gp210-positive than gp210-negative patients (p=0.002). Of interest was the finding that the increased capacity for the inferred pathway, bacterial invasion of epithelial cells in PBC, highly correlated with the abundance of bacteria belonging to Enterobacteriaceae . This study presents a comprehensive landscape of gut microbiota in PBC. Dysbiosis was found in the gut microbiome in PBC and partially relieved by UDCA. Our study suggests that gut microbiota is a potential therapeutic target and diagnostic biomarker for PBC. 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/.

Combined MEG-EEG source localisation in patients with sub-acute sclerosing pan-encephalitis.

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Velmurugan, J; Sinha, Sanjib; Nagappa, Madhu; Mariyappa, N; Bindu, P S; Ravi, G S; Hazra, Nandita; Thennarasu, K; Ravi, V; Taly, A B; Satishchandra, P

2016-08-01

To study the genesis and propagation patterns of periodic complexes (PCs) associated with myoclonic jerks in sub-acute sclerosing pan-encephalitis (SSPE) using magnetoencephalography (MEG) and electroencephalography (EEG). Simultaneous recording of MEG (306 channels) and EEG (64 channels) in five patients of SSPE (M:F = 3:2; age 10.8 ± 3.2 years; symptom-duration 6.2 ± 10 months) was carried out using Elekta Neuromag(®) TRIUX™ system. Qualitative analysis of 80-160 PCs per patient was performed. Ten isomorphic classical PCs with significant field topography per patient were analysed at the 'onset' and at 'earliest significant peak' of the burst using discrete and distributed source imaging methods. MEG background was asymmetrical in 2 and slow in 3 patients. Complexes were periodic (3) or quasi-periodic (2), occurring every 4-16 s and varied in morphology among patients. Mean source localization at onset of bursts using discrete and distributed source imaging in magnetic source imaging (MSI) was in thalami and or insula (50 and 50 %, respectively) and in electric source imaging (ESI) was also in thalami and or insula (38 and 46 %, respectively). Mean source localization at the earliest rising phase of peak in MSI was in peri-central gyrus (49 and 42 %) and in ESI it was in frontal cortex (52 and 56 %). Further analysis revealed that PCs were generated in thalami and or insula and thereafter propagated to anterolateral surface of the cortices (viz. sensori-motor cortex and frontal cortex) to same side as that of the onset. This novel MEG-EEG based case series of PCs provides newer insights for understanding the plausible generators of myoclonus in SSPE and patterns of their propagation.

Subacute sclerosing panencephalitis in papua new guinean children: the cost of continuing inadequate measles vaccine coverage.

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Laurens Manning

2011-01-01

Full Text Available subacute sclerosing panencephalitis (SSPE is a late, rare and usually fatal complication of measles infection. Although a very high incidence of SSPE in Papua New Guinea (PNG was first recognized 20 years ago, estimated measles vaccine coverage has remained at ≤ 70% since and a large measles epidemic occurred in 2002. We report a series of 22 SSPE cases presenting between November 2007 and July 2009 in Madang Province, PNG, including localized clusters with the highest ever reported annual incidence.as part of a prospective observational study of severe childhood illness at Modilon Hospital, the provincial referral center, children presenting with evidence of meningo-encephalitis were assessed in detail including lumbar puncture in most cases. A diagnosis of SSPE was based on clinical features and presence of measles-specific IgG in cerebrospinal fluid and/or plasma. The estimated annual SSPE incidence in Madang province was 54/million population aged 100/million/year. The distribution of year of birth of the 22 children with SSPE closely matched the reported annual measles incidence in PNG, including a peak in 2002.SSPE follows measles infections in very young PNG children. Because PNG children have known low seroconversion rates to the first measles vaccine given at 6 months of age, efforts such as supplementary measles immunisation programs should continue in order to reduce the pool of non-immune people surrounding the youngest and most vulnerable members of PNG communities.

Hilar cholangiocarcinoma: diagnosis, treatment options, and management

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Soares, Kevin C.; Kamel, Ihab; Cosgrove, David P.; Herman, Joseph M.

2014-01-01

Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. Of the 3,000 cases seen annually in the United States, less than one half of these tumors are resectable. A variety of risk factors have been associated with HC, most notably primary sclerosing cholangitis (PSC), biliary stone disease and parasitic liver disease. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. Computed topography (CT), magnetic resonance imaging (MRI), and ultrasound (US) are used to characterize biliary lesions. Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) assess local ductal extent of the tumor while allowing for therapeutic biliary drainage. MRCP has demonstrated similar efficacies to PTC and ERCP in identifying anatomic extension of tumors with less complications. Treatment consists of surgery, radiation, chemotherapy and photodynamic therapy. Biliary drainage of the future liver remnant should be performed to decrease bilirubin levels thereby facilitating future liver hypertrophy. Standard therapy consists of surgical margin-negative (R0) resection with extrahepatic bile duct resection, hepatectomy and en bloc lymphadenectomy. Local resection should not be undertaken. Lymph node invasion, tumor grade and negative margins are important prognostic indicators. In instances where curative resection is not possible, liver transplantation has demonstrated acceptable outcomes in highly selected patients. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40% however, recurrence can be as high as 50-70% even after R0 resection. Due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended for this complex disease. PMID:24696835

Cholangitis and multiple liver abscesses after percutaneous ethanol injection (PEI for recurrent hepatocellular carcinoma (HCC Colangitis y abscesos hepáticos múltiples tras la inyección percutánea de etanol (IPE en el tratamiento del carcinoma hepatocelular recurrente

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Fernando Macias-García

2013-02-01

Full Text Available Percutaneous ablation procedures are minimally invasive treatments for unresectable early stage hepatocellular carcinoma (HCC. These techniques are usually safe, but rare and even fatal complications have been described. We present a fatal result after percutaneous ethanol injection (PEI for the treatment of a recurrent HCC in a non-cirrhotic liver, with subsequent development of diffuse cholangitis and multiple liver abscesses. Although percutaneous drainage and intensive antibiotic treatment were employed, the patient finally died. We discuss about the etiology and the physiopathology of this rare complication in which the therapeutic options are limited and usually unsuccessful.

Laparoscopic Treatment of Sclerosing Stromal Tumor of the Ovary in a Woman With Gorlin-Goltz Syndrome: A Case Report and Review of the Literature.

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Grechi, Gianluca; Clemente, Nicolò; Tozzi, Alessandra; Ciavattini, Andrea

2015-01-01

Gorlin-Goltz syndrome is a rare hereditary multisystemic disease. Multiple basal cell carcinomas, odontogenic keratocysts, and skeletal abnormalities are the main clinical manifestations of the syndrome, but several organs can be involved. Moreover, this condition is associated with the development of various benign and malignant tumors, even in the genital tract. This report describes a rare association between Gorlin-Goltz syndrome and the sclerosing stromal tumor of the ovary. Because the ultrasound and magnetic resonance imaging patterns of this tumor can be similar to those of a malignant neoplasm, prompt surgical intervention and histological confirmation of diagnosis is mandatory; however, this is a benign lesion and thus can be approached with a laparoscopic fertility-sparing surgery. Gynecologists should be aware of this possible association to provide appropriate counseling for these women, and to take a fertility-sparing laparoscopic approach whenever possible. Copyright © 2015 AAGL. Published by Elsevier Inc. All rights reserved.

[Liver involvement in coeliac disease].

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Riestra, S; Fernández, E; Rodrigo, L

1999-12-01

Coeliac disease is a gluten-sensitive enteropathy in which, genetic, immunologic and environmental factors are implied. Several extradigestive diseases have been described in association with coeliac disease, which share most of the times an immunologic mechanism. The liver is damaged in coeliac disease, and it has been considered by some authors as an extraintestinal manifestation of the disease. In the present revision we discuss the different hepatic diseases related with the coeliac disease, as well as the best approach to diagnosis and therapy of choice. At diagnosis, it is very frequent to find an asymptomatic hipertransaminasemia, which frequently disappears after gluten suppression; the morphological substratum found in this alteration is a non-specific reactive hepatitis in the majority of cases. Coeliac disease is a demonstrated cause of cryptogenic hipertransaminasemia. In a small percentage of patient with coeliac disease an association has been found with other immunological liver diseases, such as primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune hepatitis. Few studies exist that include a large number of patient, and the results on occasions are discordant. Nevertheless, the strongest association is with autoimmune hepatitis and with primary biliary cirrhosis. Several communications of isolated cases of rare hepatic diseases, which probably, only reflect a fortuitous association, have been cited in the literature.

Etiologies of chronic liver disease in children

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Farahmand F

2001-11-01

Full Text Available Chronic Liver diseases in children is the result of many different diseases including: metabolic, genetic, infectious, toxic and idiopathic causes. This was a case series study on 133 infants and children with age range 6 month to 12 years old, who presented clinically with manifestation of chronic liver disease and were admitted to Children Hospital Medical Center from year 1999 to 2000. In this study, 32 (24.5 percent patients had autoimmune chronic hepatitis, 15 (11.3 percent Glycogen storage diseases, 12 (9 percent extrahepatic biliary atresia, 11 (8.2 percent willson disease, 10 (7.5 percent cryptogenic cirrhosis, 6 (4.5 percent chronic hepatitis C, 5 (3.8 percen chronic hepatitic B, 5 (3.8 percent galactosemia 3 (2.25 percent congenital hepatic fibrosis, 3 (3.8 percent histiocytosis X, 3 (2.25 percent sclerosing cholangitis, 2 (1.5 percent byler’s disease 2 (1.5 percent primary tuberculosis, 1 (0.75 percent choledocalcyst, 1 (0.75 percent Alagyle syndrome. According to our data, chronic liver disease should be considered in infants and children. In our study, the most common causes are found to be: metabolic and genetic diseases (37.5 percent, chronic autoimmune hepatitis (24 percent and biliary disorders (14 percent, that encompass 86 percent of the patients.

Current approach to the diagnosis of IgG4-related disease - Combination of comprehensive diagnostic and organ-specific criteria.

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Umehara, Hisanori; Okazaki, Kazuichi; Nakamura, Takuji; Satoh-Nakamura, Tomomi; Nakajima, Akio; Kawano, Mitsuhiro; Mimori, Tsuneyo; Chiba, Tsutomu

2017-05-01

IgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criterion has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan, published comprehensive diagnostic criteria for IgG4-RD. Several problems with these criteria have arisen in clinical practice, however, including the difficulty obtaining biopsy samples from some patients, and the sensitivity and the specificity of techniques used to measure serum IgG4 concentrations. Although serum IgG4 concentration is an important clinical marker for IgG4-RD, its diagnostic utility in differentiating IgG4-RD from other diseases, called IgG4-RD mimickers, remains unclear. This review describes the current optimal approach for the diagnosis of IgG4-RD, based on both comprehensive and organ-specific diagnostic criteria, in patients with diseases such as IgG4-related pancreatitis (AIP), sclerosing cholangitis, and renal, lung and orbital diseases.

Macrophage Depletion Attenuates Extracellular Matrix Deposition and Ductular Reaction in a Mouse Model of Chronic Cholangiopathies

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Syn, Wing-Kin; Lagaisse, Kimberly; van Hul, Noemi; Heindryckx, Femke; Sowa, Jan-Peter; Peeters, Liesbeth; Van Vlierberghe, Hans; Leclercq, Isabelle A.; Canbay, Ali

2016-01-01

Chronic cholangiopathies, such as primary and secondary sclerosing cholangitis, are progressive disease entities, associated with periportal accumulation of inflammatory cells, encompassing monocytes and macrophages, peribiliary extracellular matrix (ECM) deposition and ductular reaction (DR). This study aimed to elucidate the relevance of macrophages in the progression of chronic cholangiopathies through macrophage depletion in a 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) mouse model. One group of mice received a single i.p. injection of Clodronate encapsulated liposomes (CLOLipo) at day 7 of a 14 day DDC treatment, while control animals were co-treated with PBSLipo instead. Mice were sacrificed after 7 or respectively 14 days of treatment for immunohistochemical assessment of macrophage recruitment (F4/80), ECM deposition (Sirius Red, Laminin) and DR (CK19). Macrophage depletion during a 14 day DDC treatment resulted in a significant inhibition of ECM deposition. Porto-lobular migration patterns of laminin-rich ECM and ductular structures were significantly attenuated and a progression of DR was effectively inhibited by macrophage depletion. CLOLipo co-treatment resulted in a confined DR to portal regions without amorphous cell clusters. This study suggests that therapeutic options selectively directed towards macrophages might represent a feasible treatment for chronic cholestatic liver diseases. PMID:27618307

Autoimmune liver disease 2007.

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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.

Successful Sequential Liver and Hematopoietic Stem Cell Transplantation in a Child With CD40 Ligand Deficiency and Cryptosporidium-Induced Liver Cirrhosis.

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Quarello, Paola; Tandoi, Francesco; Carraro, Francesca; Vassallo, Elena; Pinon, Michele; Romagnoli, Renato; David, Ezio; Dell Olio, Dominic; Salizzoni, Mauro; Fagioli, Franca; Calvo, Pier Luigi

2018-05-01

Hematopoietic stem cell transplantation (HSCT) is curative in patients with primary immunodeficiencies. However, pre-HSCT conditioning entails unacceptably high risks if the liver is compromised. The presence of a recurrent opportunistic infection affecting the biliary tree and determining liver cirrhosis with portal hypertension posed particular decisional difficulties in a 7-year-old child with X-linked CD40-ligand deficiency. We aim at adding to the scanty experience available on such rare cases, as successful management with sequential liver transplantation (LT) and HSCT has been reported in detail only in 1 young adult to date. A closely sequential strategy, with a surgical complication-free LT, followed by reduced-intensity conditioning, allowed HSCT to be performed only one month after LT, preventing Cryptosporidium parvum recolonization of the liver graft. Combined sequential LT and HSCT resolved the cirrhotic evolution and corrected the immunodeficiency so that the infection responsible for the progressive sclerosing cholangitis did not recur. Hopefully, this report of the successful resolution of a potentially fatal combination of immunodeficiency and chronic opportunistic infection with end-stage organ damage in a child will encourage others to adapt a sequential transplant approach to this highly complex pathology. However, caution is to be exercised to carefully balance the risks intrinsic to transplant surgery and immunosuppression in primary immunodeficiencies.

Biliary-duodenal anastomosis using magnetic compression following massive resection of small intestine due to strangulated ileus after living donor liver transplantation: a case report.

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Saito, Ryusuke; Tahara, Hiroyuki; Shimizu, Seiichi; Ohira, Masahiro; Ide, Kentaro; Ishiyama, Kohei; Kobayashi, Tsuyoshi; Ohdan, Hideki

2017-12-01

Despite the improvements of surgical techniques and postoperative management of patients with liver transplantation, biliary complications are one of the most common and important adverse events. We present a first case of choledochoduodenostomy using magnetic compression following a massive resection of the small intestine due to strangulated ileus after living donor liver transplantation. The 54-year-old female patient had end-stage liver disease, secondary to liver cirrhosis, due to primary sclerosing cholangitis with ulcerative colitis. Five years earlier, she had received living donor liver transplantation using a left lobe graft, with resection of the extrahepatic bile duct and Roux-en-Y anastomosis. The patient experienced sudden onset of intense abdominal pain. An emergency surgery was performed, and the diagnosis was confirmed as strangulated ileus due to twisting of the mesentery. Resection of the massive small intestine, including choledochojejunostomy, was performed. Only 70 cm of the small intestine remained. She was transferred to our hospital with an external drainage tube from the biliary cavity and jejunostomy. We initiated total parenteral nutrition, and percutaneous transhepatic biliary drainage was established to treat the cholangitis. Computed tomography revealed that the biliary duct was close to the duodenum; hence, we planned magnetic compression anastomosis of the biliary duct and the duodenum. The daughter magnet was placed in the biliary drainage tube, and the parent magnet was positioned in the bulbus duodeni using a fiberscope. Anastomosis between the left hepatic duct and the duodenum was accomplished after 25 days, and the biliary drainage stent was placed over the anastomosis to prevent re-stenosis. Contributions to the successful withdrawal of parenteral nutrition were closure of the ileostomy in the adaptive period, preservation of the ileocecal valve, internal drainage of bile, and side-to-side anastomosis

Measles Virus: Identification in the M Protein Primary Sequence of a Potential Molecular Marker for Subacute Sclerosing Panencephalitis

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Hasan Kweder

2015-01-01

Full Text Available Subacute Sclerosing Panencephalitis (SSPE, a rare lethal disease of children and young adults due to persistence of measles virus (MeV in the brain, is caused by wild type (wt MeV. Why MeV vaccine strains never cause SSPE is completely unknown. Hypothesizing that this phenotypic difference could potentially be represented by a molecular marker, we compared glycoprotein and matrix (M genes from SSPE cases with those from the Moraten vaccine strain, searching for differential structural motifs. We observed that all known SSPE viruses have residues P64, E89, and A209 (PEA in their M proteins whereas the equivalent residues for vaccine strains are either S64, K89, and T209 (SKT as in Moraten or PKT. Through the construction of MeV recombinants, we have obtained evidence that the wt MeV-M protein PEA motif, in particular A209, is linked to increased viral spread. Importantly, for the 10 wt genotypes (of 23 that have had their M proteins sequenced, 9 have the PEA motif, the exception being B3, which has PET. Interestingly, cases of SSPE caused by genotype B3 have yet to be reported. In conclusion, our results strongly suggest that the PEA motif is a molecular marker for wt MeV at risk to cause SSPE.

Clinical presentation and outcome prediction of clinical, serological, and histopathological classification schemes in ANCA-associated vasculitis with renal involvement.

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Córdova-Sánchez, Bertha M; Mejía-Vilet, Juan M; Morales-Buenrostro, Luis E; Loyola-Rodríguez, Georgina; Uribe-Uribe, Norma O; Correa-Rotter, Ricardo

2016-07-01

Several classification schemes have been developed for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), with actual debate focusing on their clinical and prognostic performance. Sixty-two patients with renal biopsy-proven AAV from a single center in Mexico City diagnosed between 2004 and 2013 were analyzed and classified under clinical (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], renal limited vasculitis [RLV]), serological (proteinase 3 anti-neutrophil cytoplasmic antibodies [PR3-ANCA], myeloperoxidase anti-neutrophil cytoplasmic antibodies [MPO-ANCA], ANCA negative), and histopathological (focal, crescenteric, mixed-type, sclerosing) categories. Clinical presentation parameters were compared at baseline between classification groups, and the predictive value of different classification categories for disease and renal remission, relapse, renal, and patient survival was analyzed. Serological classification predicted relapse rate (PR3-ANCA hazard ratio for relapse 2.93, 1.20-7.17, p = 0.019). There were no differences in disease or renal remission, renal, or patient survival between clinical and serological categories. Histopathological classification predicted response to therapy, with a poorer renal remission rate for sclerosing group and those with less than 25 % normal glomeruli; in addition, it adequately delimited 24-month glomerular filtration rate (eGFR) evolution, but it did not predict renal nor patient survival. On multivariate models, renal replacement therapy (RRT) requirement (HR 8.07, CI 1.75-37.4, p = 0.008) and proteinuria (HR 1.49, CI 1.03-2.14, p = 0.034) at presentation predicted renal survival, while age (HR 1.10, CI 1.01-1.21, p = 0.041) and infective events during the induction phase (HR 4.72, 1.01-22.1, p = 0.049) negatively influenced patient survival. At present, ANCA-based serological classification may predict AAV relapses, but neither clinical nor serological

[Study of androgen receptor and phosphoglycerate kinase gene polymorphism in major cellular components of the so-called pulmonary sclerosing hemangioma].

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Qi, Feng-jie; Zhang, Xiu-wei; Zhang, Yong-xing; Dai, Shun-dong; Wang, En-hua

2006-05-01

To study the clonality of polygonal cells and surface cuboidal cells in the so-called pulmonary sclerosing hemangioma (PSH). 17 female surgically resected PSH were found. The polygonal cells and surface cuboidal cells of the 17 PSH cases were microdissected from routine hematoxylin and eosin-stained sections. Genomic DNA was extracted, pretreated through incubation with methylation-sensitive restrictive endonuclease HhaI or HpaII, and amplified by nested polymerase chain reaction for X chromosome-linked androgen receptor (AR) and phosphoglycerate kinase (PGK) genes. The length polymorphism of AR gene was demonstrated by denaturing polyacrylamide gel electrophoresis and silver staining. The PGK gene products were treated with Bst XI and resolved on agarose gel. Amongst the 17 female cases of PSH, 15 samples were successfully amplified for AR and PGK genes. The rates of polymorphism were 53% (8/15) and 27% (4/15) for AR and PGK genes respectively. Polygonal cells and surface cuboidal cells of 10 cases which were suitable for clonality study, showed the same loss of alleles (clonality ratio = 0) or unbalanced methylation pattern (clonality ratio < 0.25). The polygonal cells and surface cuboidal cells in PSH demonstrate patterns of monoclonal proliferation, indicating that both represent true neoplastic cells.

Pulmonary sclerosing hemangioma in a 21-year-old male with metastatic hereditary non-polyposis colorectal cancer: Report of a case

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Angele Martin K

2011-06-01

Full Text Available Abstract Background Pulmonary sclerosing hemangioma (SH is a rare tumor of the lung predominantly affecting Asian women in their fifth decade of life. SH is thought to evolve from primitive respiratory epithelium and mostly shows benign biological behavior; however, cases of lymph node metastases, local recurrence and multiple lesions have been described. Case Presentation We report the case of a 21-year-old Caucasian male with a history of locally advanced and metastatic rectal carcinoma (UICC IV; pT4, pN1, M1(hep that was eventually identified as having hereditary non-polyposis colorectal cancer (HNPCC, Lynch syndrome. After neoadjuvant chemotherapy followed by low anterior resection, adjuvant chemotherapy and metachronous partial hepatectomy, he was admitted for treatment of newly diagnosed bilateral pulmonary metastases. Thoracic computed tomography showed a homogenous, sharply marked nodule in the left lower lobe. We decided in favor of atypical resection followed by systematic lymphadenectomy. Histopathological analysis revealed the diagnosis of SH. Conclusions Cases have been published with familial adenomatous polyposis (FAP and simultaneous SH. FAP, Gardner syndrome and Li-Fraumeni syndrome, however, had been ruled out in the present case. To the best of our knowledge, this is the first report describing SH associated with Lynch syndrome.

Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience

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S K Rampelli

2016-01-01

Full Text Available There is limited data on the etiology, clinical and histopathological spectrum and outcomes of crescentic glomerulonephritis (CrGN in adult Indian population. This prospective study was done to evaluate the etiology, clinicohistological patterns and predictors of outcome of CrGN in South Indian population. All the patients received standard protocol based immunosuppression in addition to supportive care. Immune-complex glomerulonephritis (ICGN was the most common etiology (n = 31; 77.5% followed by pauci-immune glomerulonephritis (PauciGN; n = 8; 20% and anti-glomerular basement membrane disease (n = 1; 2.5%. The most common etiology of ICGN was IgA nephropathy (n = 11; 27.5% followed by lupus nephritis (n = 7; 17.5% and post-infectious glomerulonephritis (PIGN (n = 7; 17.5%. The patients with PauciGN were significantly older compared to those with ICGN (44.5 ± 15 years vs. 31.8 ± 11 years; P = 0.01. The patients with PauciGN presented with significantly higher serum creatinine (9.7 ± 4.4 vs. 6.6 ± 3.3 mg/dl; P = 0.03. The histopathologic parameters of ICGN and PauciGN were comparable except for a higher proportion of sclerosed glomeruli in ICGN. At the end of 3 months follow-up, only two patients went into complete remission (5.4%. Majority of the patients had end-stage renal failure (48.6% and were dialysis dependent and seven patients (18.9% expired. There was no signifi difference in the renal survival (10.9 ± 1.9 vs. 9.6 ± 3.3 months or patient survival (17.5 ± 2.1 vs. 17.3 ± 4.3 months. The parameters associated with adverse outcomes at 3 months were hypertension (odds ratio [OR]: 0.58; confidence interval [CI]: 0.36–0.94, need for renal replacement therapy (OR: 0.19; CI: 0.04–0.9, serum creatinine at admission (P = 0.019, estimated glomerular filtration rate (P = 0.022 and percentage of fibrocellular crescents (P = 0.022.

Clinical Feasibility and Usefulness of CT Fluoroscopy-Guided Percutaneous Transhepatic Biliary Drainage in Emergency Patients with Acute Obstructive Cholangitis

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Kim, Ji Hyung [Sam Anyang Hospital, Anyang (Korea, Republic of)

2009-04-15

To evaluate the feasibility of CT fluoroscopy (CTF)-guided percutaneous transhepatic biliary drainage (PTBD) in emergency patients with acute obstructive cholangitis. The study included 28 patients admitted to the emergency center due to obstructive jaundice and found to require urgent biliary drainage, as well as judged to have a suitable peripheral bile duct for a CTF-guided puncture (at least 4 mm in width). Prior to the CTF-guided puncture, a CT scan was performed to evaluate bile duct dilatation and the underlying causes of biliary obstruction. If the patient was judged to be a suitable candidate, a CTF-guided PTBD was performed in the same CT unit without additional fluoroscopic guidance. Technical feasibility of the procedure was investigated with the evaluation of overall success rate and causes of failure. A hepatic puncture was attempted at the left lobe in 23 patients and right lobe in five patients. The procedure was successful in 24 of 28 patients (86%) Successful biliary puncture was achieved on the first attempt in 16 patients, the second attempt in five patients, and the third attempt in three patients. The causes of failure included guide wire twisting in one patient, biliary puncture failure in two patients, and poor visualization of the guide wire in one patient. There were no significant procedure-related complication. The CTF-guided PTBD is technically feasible and highly successful in patients judged to have a suitable indication. Moreover, although the procedure is unfamiliar and inconvenient to interventionalists, it has economical advantages in that it saves time and manpower. We believe this method can be used in the emergency patients requiring urgent biliary drainage as an alternative for the fluoroscopy-guided PTBD.

IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease

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Divatia, Mukul; Kim, Sun A

2012-01-01

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies. PMID:22187229

Isolated Liver Hilar Infiltration by IgG4 Inflammation Mimicking Cholangiocarcinoma

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Laurent Bochatay

2016-10-01

Full Text Available IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria. The patient presented with progressive cholestasis identified by MR cholangiography as an isolated hilar mass responsible for dilatation of the left and right intrahepatic bile duct suspicious for a Klatskin tumor. The IgG4 blood level was normal as was biliary cytology. The patient underwent right portal embolization followed by right extended hepatectomy. Pathologic examination found no tumor but intense fibrosclerotic infiltration with a marked inflammatory infiltrate characterized by IgG4-positive plasmocytes. Despite immunosuppressive treatment, cholestasis was never controlled and successive biopsies of the remaining liver showed progressive cholestasis, liver infiltrate and no bile duct regeneration. The patient finally presented an upper gastrointestinal hemorrhage leading to death 4 months after hepatectomy and appropriate immunosuppressive therapy.

Epidemiology of Inflammatory Bowel Diseases in Iran and Asia; A Mini Review

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Ali Reza Safarpour

2013-06-01

Full Text Available The prevalence of inflammatory bowel diseases (IBDs is set to stabilize in Western Europe and North America, as opposed to its increasing trend in developing countries in Asia. The epidemiology of IBDs in areas where the incidence and prevalence are relatively low provides an opportunity for researchers to determine the unknown aspects of them. In this review article, the PubMed and MEDLINE databases were searched from 1970 to 2012 and the epidemiological aspects assessed in Iranian articles were compared with identical subjects in other Asian countries. During this period, there were 21 documented articles on IBD epidemiology in Iran and 52 in Asia. According to the present review, CTLA-gene polymorphism and male/female ratio in ulcerative colitis (UC, incidence of extra-intestinal manifestations, extent of intestinal involvement, and family history in both UC and Crohn’s disease (CD seemed to be different between Asia and Iran. In contrast, the incidence of primary sclerosing cholangitis in IBD patients and association between NO2/CARD15 mutation and CD as C3435-T allele and UC were nearly the same. The rate of IBD has increased significantly in Iran, as has that of other Asian countries during the last decade. A thorough, well-designed, population-based, multi-regional epidemiologic study seems mandatory due to the substantial demographic and characteristic variability in IBD patients in our region.

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

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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.

Biliary Innate Immunity: Function and Modulation

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Kenichi Harada

2010-01-01

Full Text Available Biliary innate immunity is involved in the pathogenesis of cholangiopathies in patients with primary biliary cirrhosis (PBC and biliary atresia. Biliary epithelial cells possess an innate immune system consisting of the Toll-like receptor (TLR family and recognize pathogen-associated molecular patterns (PAMPs. Tolerance to bacterial PAMPs such as lipopolysaccharides is also important to maintain homeostasis in the biliary tree, but tolerance to double-stranded RNA (dsRNA is not found. In PBC, CD4-positive Th17 cells characterized by the secretion of IL-17 are implicated in the chronic inflammation of bile ducts and the presence of Th17 cells around bile ducts is causally associated with the biliary innate immune responses to PAMPs. Moreover, a negative regulator of intracellular TLR signaling, peroxisome proliferator-activated receptor-γ (PPARγ, is involved in the pathogenesis of cholangitis. Immunosuppression using PPARγ ligands may help to attenuate the bile duct damage in PBC patients. In biliary atresia characterized by a progressive, inflammatory, and sclerosing cholangiopathy, dsRNA viruses are speculated to be an etiological agent and to directly induce enhanced biliary apoptosis via the expression of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL. Moreover, the epithelial-mesenchymal transition (EMT of biliary epithelial cells is also evoked by the biliary innate immune response to dsRNA.

Cholangiocarcinoma with respect to IgG4 Reaction

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Kenichi Harada

2014-01-01

Full Text Available IgG4 reactions marked by infiltration of IgG4-positive plasma cells in affected organs occur in cancer patients and in patients with IgG4-related diseases. Extrahepatic cholangiocarcinomas including gall bladder cancer are often accompanied by significant IgG4 reactions; these reactions show a negative correlation with CD8-positive cytotoxic T cells, suggesting that the evasion of immune surveillance is associated with cytotoxic T cells. The regulatory cytokine IL-10 may induce IgG4-positive plasma cell differentiation or promote B cell switching to IgG4 in the presence of IL-4. Cholangiocarcinoma cells may function as nonprofessional antigen presenting cells that indirectly induce IgG4 reactions via the IL-10-producing cells and/or these may act as Foxp3-positive and IL-10-producing cells that directly induce IgG4 reactions. Moreover, IgG4-related disease is a high-risk factor for cancer development; IgG4-related sclerosing cholangitis (IgG4-SC cases associated with cholangiocarcinoma or its precursor lesion biliary intraepithelial neoplasia (BilIN have been reported. IgG4-positive cell infiltration is an important finding of IgG4-SC but is not a histological hallmark of IgG4-SC. For the diagnosis of IgG4-SC, its differentiation from cholangiocarcinoma remains important.

The CXC Chemokine Receptor 3 Inhibits Autoimmune Cholangitis via CD8+ T Cells but Promotes Colitis via CD4+ T Cells

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Qing-Zhi Liu

2018-05-01

Full Text Available CXC chemokine receptor 3 (CXCR3, a receptor for the C-X-C motif chemokines (CXCL CXCL9, CXCL10, and CXCL11, which not only plays a role in chemotaxis but also regulates differentiation and development of memory and effector T cell populations. Herein, we explored the function of CXCR3 in the modulation of different organ-specific autoimmune diseases in interleukin (IL-2 receptor deficiency (CD25−/− mice, a murine model for both cholangitis and colitis. We observed higher levels of CXCL9 and CXCL10 in the liver and colon and higher expression of CXCR3 on T cells of the CD25−/− mice compared with control animals. Deletion of CXCR3 resulted in enhanced liver inflammation but alleviated colitis. These changes in liver and colon pathology after CXCR3 deletion were associated with increased numbers of hepatic CD4+ and CD8+ T cells, in particular effector memory CD8+ T cells, as well as decreased T cells in mesenteric lymph nodes and colon lamina propria. In addition, increased interferon-γ response and decreased IL-17A response was observed in both liver and colon after CXCR3 deletion. CXCR3 modulated the functions of T cells involved in different autoimmune diseases, whereas the consequence of such modulation was organ-specific regarding to their effects on disease severity. Our findings emphasize the importance of extra caution in immunotherapy for organ-specific autoimmune diseases, as therapeutic interventions aiming at a target such as CXCR3 for certain disease could result in adverse effects in an unrelated organ.

Evidence for the association between IgG-antimitochondrial antibody and biochemical response to ursodeoxycholic acid treatment in primary biliary cholangitis.

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Tang, Libo; Zhong, Ruihua; He, Xuanqiu; Wang, Weibin; Liu, Jinhong; Zhu, Youfu; Li, Yongyin; Hou, Jinlin

2017-03-01

Antimitochondrial antibody (AMA) is considered the serological hallmark of primary biliary cholangitis (PBC), while data regarding the profile of AMA during ursodeoxycholic acid (UDCA) treatment are scarce. Here, we assessed the influence of UDCA treatment on titers of AMA and factors relevant to its production. Serum IgA-AMA, IgM-AMA, IgG-AMA, B cell-activating factor of the tumor necrosis factor family (BAFF), and the frequency of circulating plasmablasts were detected in PBC patients, including those who received UDCA therapy for 24 weeks, healthy controls, chronic hepatitis B patients, and autoimmune hepatitis patients. Consecutive liver sections from controls and PBC patients were stained by immunohistochemistry for detection of intrahepatic CD38 + , IgA + , IgM + , and IgG + cells. Significant decrease in titers of IgG-AMA was found only confined to PBC patients with biochemical response to UDCA treatment (P = 0.005), and similar pattern was also observed at week 24 in quantifying circulating plasmablasts (P = 0.025) and serum BAFF (P = 0.013). Notably, positive correlation between serum BAFF levels and titers of IgG-AMA, and the frequency of circulating plasmablasts were observed in PBC patients (r = 0.464, P = 0.034 and r = 0.700, P < 0.001, respectively). Additionally, in situ staining revealed significant accumulation of CD38 + and IgG + cells within the portal tracts of PBC liver. Decreased titers of serum IgG-AMA are associated with biochemical response to UDCA treatment, implicating the potentiality of this hallmark in therapeutic response evaluation and the beneficial effect of UDCA on humoral immunity in PBC patients. © 2016 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

Acute Hemorrhagic Encephalitis Responding to Combined Decompressive Craniectomy, Intravenous Immunoglobulin, and Corticosteroid Therapies: Association with Novel RANBP2 Variant

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Abdulla Alawadhi

2018-03-01

Full Text Available BackgroundAcute hemorrhagic encephalomyelitis (AHEM is considered as a rare form of acute disseminated encephalomyelitis characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome.ObjectiveTo report the association with Ran Binding Protein (RANBP2 gene variant and the response to decompressive craniectomy and high-dose intravenous methylprednisolone (IVMP in life-threatening AHEM.DesignSingle case study.Case reportA 6-year-old girl known to have sickle cell disease (SCD presented an acquired demyelinating syndrome (ADS with diplopia due to sudden unilateral fourth nerve palsy. She received five pulses of IVMP (30 mg/kg/day. Two weeks after steroid weaning, she developed right hemiplegia and coma. Brain magnetic resonance imaging showed a left frontal necrotico-hemorrhagic lesion and new multifocal areas of demyelination. She underwent decompressive craniotomy and evacuation of an ongoing left frontoparietal hemorrhage. Comprehensive investigations ruled out vascular and infectious process. The neurological deterioration stopped concomitantly with combined neurosurgical drainage of the hematoma, decompressive craniotomy, IVMP, and intravenous immunoglobulins (IVIG. She developed during the following months Crohn disease and sclerosing cholangitis. After 2-year follow-up, there was no new neurological manifestation. The patient still suffered right hemiplegia and aphasia, but was able to walk. Cognitive/behavioral abilities significantly recovered. A heterozygous novel rare missense variant (c.4993A>G, p.Lys1665Glu was identified in RANBP2, a gene associated with acute necrotizing encephalopathy. RANBP2 is a protein playing an important role in the energy homeostasis of neuronal cells.ConclusionIn any ADS occurring in the context of SCD and/or autoimmune condition, we recommend to slowly wean steroids and to closely monitor the patient after weaning to quickly treat any recurrence of neurological symptom

Balloon-occluded Retrograde Transvenous Obliteration (BRTO): Technique and Intraprocedural Imaging

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Sabri, Saher S.; Saad, Wael E. A.

2011-01-01

Balloon-occluded retrograde transvenous obliteration (BRTO) is an endovascular technique used as a therapeutic adjunct or alternative to transjugular intrahepatic shunts (TIPS) in the management of gastric varices. Occlusion balloons are strategically placed to modulate flow within the gastrorenal or gastrocaval shunt to allow stagnation of the sclerosant material within the gastric varix. The approach and complexity of the procedure depends on the anatomic classification of inflow and outflow veins of the varix. Ethanolamine oleate has been described as the main sclerosant used in this procedure. Recently, foam sclerosants have gained popularity as alternative embolization agents, which provide the advantage of better variceal wall contact and potentially less dose of sclerosant. PMID:22942548

Pancreatitis autoinmune: pseudotumor inflamatorio, afectación multifocal, hipertensión portal y evolución a largo plazo Autoimmune pancreatitis: inflammatory pseudotumor, multifocal fibrosclerosis, portal hypertension, and long-term outcome

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J. L. Beristain

2008-10-01

-year-old female presented with obstructive jaundice and abdominal tenderness, as well as a mass at the pancreatic head on a CT scan, suggestive of pancreatic neoplasia. Surgery showed an increase of the whole pancreas, malignancy was intraoperatively ruled out, and a cholecystectomy and choledochoduodenostomy were carried out. The diagnosis was chronic pancreatitis. Over the following years different autoimmune complications developed, including asthma, salivary gland swelling, and sclerosing cholangitis, as well as recurrent episodes of jaundice, and exocrine and endocrine pancreatic failure. The development of these complications combined with the demonstration of high serum levels of IgG4 and carbonic anhydrase II led to a re-evaluation of the initial histology of the pancreas, leading to a final diagnosis of autoimmune pancreatitis: IgG4+ lymphoplasmacytic infiltrates, fibrosis, and obliterative phlebitis. New complications developed during the last few years: retroperitoneal fibrosis with portal hypertension, esophageal varices, and splenomegaly.

Clinical features and prognosis of patients with primary biliary cholangitis complicated by hepatitis virus infection

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ZHAO Dantong

2017-08-01

Full Text Available ObjectiveTo investigate the clinical features and prognosis of patients with primary biliary cholangitis(PBC complicated by hepatitis virus infection. MethodsA total of 16 patients who were admitted to Beijing YouAn Hospital from October 2004 to October 2012 and diagnosed with PBC complicated by hepatitis virus infection were enrolled, among whom 7 had chronic hepatitis B virus infection, 3 had hepatitis C, 4 had hepatitis E, 1 had hepatitis B and hepatitis C, and 1 had hepatitis A. A total of 76 hospitalized patients with PBC alone were enrolled as controls. The two groups were compared in terms of clinical features, laboratory markers, and autoantibodies, and follow-up visits were performed to investigate prognostic features. The independent samples t-test was used for comparison of normally distributed continuous data, and the Mann-Whitney U rank sum test was used for comparison of non-normally distributed continuous data; the chi-square test or Fisher′s exact test was used for comparison of categorical data. The Kaplan-Meier method was used to calculate survival rates and the log-rank test was used to compare survival rates between groups. ResultsCompared with the control group, the PBC-hepatitis virus infection group had significantly lower proportion of female patients (χ2=12.22, P=0.002, alkaline phosphatase (U=225.00, P＜0.001, CHO (U=363.50, P=0.036, and IgG level (t=2.79, P=0.007, and no patients in the PBC-hepatitis virus infection group experienced abdominal wall varices, upper gastrointestinal bleeding, or hepatic encephalopathy. The PBC-hepatitis virus infection group had various autoantibodies including anti-nuclear antibody, smooth muscle antibody, anti-parietal cell antibody (APCA, anti-liver specific protein antibody, and anti-myocardial antibody, as well as a significantly higher APCA positive rate than the control group (25% vs 3.9%, χ2=5.608, P=0.016. The median follow-up time was 49.5 months (2-312 months. The PBC

Effects of Liver Fibrosis Progression on Tissue Relaxation Times in Different Mouse Models Assessed by Ultrahigh Field Magnetic Resonance Imaging

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Andreas Müller

2017-01-01

Full Text Available Recently, clinical studies demonstrated that magnetic resonance relaxometry with determination of relaxation times T1 and T2⁎ may aid in staging and management of liver fibrosis in patients suffering from viral hepatitis and steatohepatitis. In the present study we investigated T1 and T2⁎ in different models of liver fibrosis to compare alternate pathophysiologies in their effects on relaxation times and to further develop noninvasive quantification methods of liver fibrosis. MRI was performed with a fast spin echo sequence for measurement of T1 and a multigradient echo sequence for determination of T2⁎. Toxic liver fibrosis was induced by injections of carbon tetrachloride (1.4 mL CCl4 per kg bodyweight and week, for 3 or 6 weeks in BALB/cJ mice. Chronic sclerosing cholangitis was mimicked using the ATP-binding cassette transporter B4 knockout (Abcb4 -/- mouse model. Untreated BALB/cJ mice served as controls. To assess hepatic fibrosis, we ascertained collagen contents and fibrosis scores after Sirius red staining. T1 and T2⁎ correlate differently to disease severity and etiology of liver fibrosis. T2⁎ shows significant decrease correlating with fibrosis in CCl4 treated animals, while demonstrating significant increase with disease severity in Abcb4 -/- mice. Measurements of T1 and T2⁎ may therefore facilitate discrimination between different stages and causes of liver fibrosis.

Short-term side effects and patient-reported outcomes of bleomycin sclerotherapy in vascular malformations.

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Mack, Joana M; Richter, Gresham T; Becton, David; Salem, Omar; Hill, Sarah E M; Crary, Shelley E

2018-06-01

Vascular malformations (VM) are congenital lesions that can be debilitating and cause significant aesthetic and functional limitations. The chemotherapeutic agent bleomycin has been utilized as a sclerosant, directly injected percutaneously into the VM. Unfortunately, little is known about the benefits and short-term side effects of bleomycin with intralesional injections. An IRB approved, retrospective chart review was performed on patients with VM who had been treated with intralesional bleomycin. Data included type of VM, number of treatments, total bleomycin dose per m², and adverse effects. A questionnaire was administered to available patients to assess subjective outcomes and side effects. Forty-six patients were treated with 141 procedures of bleomycin sclerotherapy for VM. Patient ages ranged from 1 to 20 years (median age 10 years). The median cumulative bleomycin dose was 16.3 units/m²/person (range of 1.7-97.0 units/m²/person). Sixty-three percent of patients were reached for a questionnaire to assess short-term side effects. Ninety percent of patients surveyed were satisfied to very satisfied with the results from the procedure. About 24% of patients experienced transient nausea, vomiting and/or local hyperpigmentation. Bleomycin sclerotherapy can be an effective treatment of VM with repeat exposure with minor risk of short-term side effects, however, long-term risks are of great concern. Further studies are required to assess systemic absorption and long-term risks. © 2018 Wiley Periodicals, Inc.

Ustekinumab for patients with primary biliary cholangitis who have an inadequate response to ursodeoxycholic acid: A proof-of-concept study.

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Hirschfield, Gideon M; Gershwin, M Eric; Strauss, Richard; Mayo, Marlyn J; Levy, Cynthia; Zou, Bin; Johanns, Jewel; Nnane, Ivo P; Dasgupta, Bidisha; Li, Katherine; Selmi, Carlo; Marschall, Hanns-Ulrich; Jones, David; Lindor, Keith

2016-07-01

The interleukin (IL)-12 signaling cascade has been associated with primary biliary cholangitis (PBC). This multicenter, open-label, proof-of-concept study evaluated the anti-IL12/23 monoclonal antibody, ustekinumab (90 mg subcutaneous at weeks 0 and 4, then every 8 weeks through week 20), in adults with PBC and an inadequate response to ursodeoxycholic acid therapy (i.e., alkaline phosphatase [ALP] >1.67× upper limit of normal [ULN] after ≥6 months). ALP response was defined as a >40% decrease from baseline and ALP remission as ALP normalization (if baseline ALP 1.67×-2.8× ULN) or 2.8× ULN). Changes in Enhanced Liver Fibrosis (ELF) scores and serum bile acids were also assessed. At baseline, patients had median disease duration of 3.2 years, median ELF score of 9.8, and highly elevated total bile acid concentration (median, 43.3 μmol/L); 13 of 20 (65%) patients had baseline ALP >3× ULN. Although steady-state serum ustekinumab concentrations were reached by week 12, no patient achieved ALP response or remission. Median percent ALP reduction from baseline to week 28 was 12.1%. ELF score decreased slightly from baseline to week 28 (median reduction: 0.173), and total serum bile acid concentrations decreased from baseline to week 28 (median reduction: 8.8 μmol/L). No serious infections or discontinuations resulting from adverse events were reported through week 28. One patient had a serious upper gastrointestinal hemorrhage considered unrelated to test agent by the investigator. Open-label ustekinumab therapy, though associated with a modest decrease in ALP after 28 weeks of therapy, did not otherwise appreciably change ALP and overt proof-of-concept was not established as per prespecified primary endpoint of proposed efficacy. No new ustekinumab safety signals were observed. (Hepatology 2016;64:189-199). © 2015 by the American Association for the Study of Liver Diseases.

Clinico-morphological comparisons in ultrasound diagnosis of carcinoma of the thyroid in children exposed to radiation

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Cherstvoj, E D [Minsk Medical Inst., Minsk (Belarus); Demidchik, E P [Republican Centre of Hygiene and Epidemiology, Minsk (Belarus); Drozd, V M [Scientific Research Inst. for Radiation Medicine, Belarus Ministry of Health, Minsk (Belarus)

1997-09-01

The purpose of the study was to compare morphologic and ultrasonic pictures of the thyroid. The study included 42 children with thyroid cancer exposed to ionizing radiation after the Chernobyl accident. All children were operated on during 1990-1994 yy. This study has shown that diffuse sclerosing variant of papillary carcinoma (PC) occurred more frequently in patients with higher absorbed doses to the thyroid. This variant of PC was rarely found at pT1 stage, besides, diffuse sclerosing PC was more frequently accompanied by bilateral and median metastases (``pure`` PC and follicular variant of PC). Biometry of thyroid carcinoma showed a wider spread of neoplastic process in diffuse sclerosing PC than in pure papillary carcinoma. The patients with diffuse sclerosing PC had the second operation more frequently than those with follicular PC. 14 refs, 5 tabs.

Upper motor and extra-motor neuron involvement in recent-onset motor neuron disease

NARCIS (Netherlands)

van der Graaff, M.M.

2012-01-01

Amyotrofische laterale sclerose (ALS), progressieve spinale spieratrofie en primaire laterale sclerose zijn ziekten in de motorische voorhoorncel. De aandoeningen worden vooral op klinische gronden van elkaar onderscheiden. Maaike van der Graaff bekeek of motorische en niet-motorische neuronen in de

Patients with primary biliary cholangitis and fatigue present with depressive symptoms and selected cognitive deficits, but with normal attention performance and brain structure.

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Roman Zenouzi

Full Text Available In primary biliary cholangitis (PBC fatigue is a major clinical challenge of unknown etiology. By demonstrating that fatigue in PBC is associated with an impaired cognitive performance, previous studies have pointed out the possibility of brain abnormalities underlying fatigue in PBC. Whether structural brain changes are present in PBC patients with fatigue, however, is unclear. To evaluate the role of structural brain abnormalities in PBC patients severely affected from fatigue we, therefore, performed a case-control cerebral magnetic resonance imaging (cMRI study and correlated changes of white and grey brain matter with the cognitive and attention performance.20 female patients with PBC and 20 female age-matched controls were examined in this study. The assessment of fatigue, psychological symptoms, cognitive and attention performance included clinical questionnaires, established cognition tests and a computerized test battery of attention performance. T1-weighted cMRI and diffusion tensor imaging (DTI scans were acquired with a 3 Tesla scanner. Structural brain alterations were investigated with voxel-based morphometry (VBM and DTI analyses. Results were correlated to the cognitive and attention performance.Compared to healthy controls, PBC patients had significantly higher levels of fatigue and associated psychological symptoms. Except for an impairment of verbal fluency, no cognitive or attention deficits were found in the PBC cohort. The VBM and DTI analyses revealed neither major structural brain abnormalities in the PBC cohort nor correlations with the cognitive and attention performance.Despite the high burden of fatigue and selected cognitive deficits, the attention performance of PBC patients appears to be comparable to healthy people. As structural brain alterations do not seem to be present in PBC patients with fatigue, fatigue in PBC must be regarded as purely functional. Future studies should evaluate, whether functional brain

Patients with primary biliary cholangitis and fatigue present with depressive symptoms and selected cognitive deficits, but with normal attention performance and brain structure.

Science.gov (United States)

Zenouzi, Roman; von der Gablentz, Janina; Heldmann, Marcus; Göttlich, Martin; Weiler-Normann, Christina; Sebode, Marcial; Ehlken, Hanno; Hartl, Johannes; Fellbrich, Anja; Siemonsen, Susanne; Schramm, Christoph; Münte, Thomas F; Lohse, Ansgar W

2018-01-01

In primary biliary cholangitis (PBC) fatigue is a major clinical challenge of unknown etiology. By demonstrating that fatigue in PBC is associated with an impaired cognitive performance, previous studies have pointed out the possibility of brain abnormalities underlying fatigue in PBC. Whether structural brain changes are present in PBC patients with fatigue, however, is unclear. To evaluate the role of structural brain abnormalities in PBC patients severely affected from fatigue we, therefore, performed a case-control cerebral magnetic resonance imaging (cMRI) study and correlated changes of white and grey brain matter with the cognitive and attention performance. 20 female patients with PBC and 20 female age-matched controls were examined in this study. The assessment of fatigue, psychological symptoms, cognitive and attention performance included clinical questionnaires, established cognition tests and a computerized test battery of attention performance. T1-weighted cMRI and diffusion tensor imaging (DTI) scans were acquired with a 3 Tesla scanner. Structural brain alterations were investigated with voxel-based morphometry (VBM) and DTI analyses. Results were correlated to the cognitive and attention performance. Compared to healthy controls, PBC patients had significantly higher levels of fatigue and associated psychological symptoms. Except for an impairment of verbal fluency, no cognitive or attention deficits were found in the PBC cohort. The VBM and DTI analyses revealed neither major structural brain abnormalities in the PBC cohort nor correlations with the cognitive and attention performance. Despite the high burden of fatigue and selected cognitive deficits, the attention performance of PBC patients appears to be comparable to healthy people. As structural brain alterations do not seem to be present in PBC patients with fatigue, fatigue in PBC must be regarded as purely functional. Future studies should evaluate, whether functional brain changes

Pathomorphological characteristic of IgG4-related diseases

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O. O. Dyadyk

2016-08-01

Full Text Available IgG4-related diseases are a relatively new group of diseases of unknown etiology which are characterized by the development of fibrosis of organs with the presence of big amounts of IgG4-positive plasma-cells in the area of the lesions and increased levels of IgG4 in serum. The organs that may be affected are pancreas, salivary gland, and others, clinical cases of kidney damage are described as well. Renal involvement in IgG4-related diseases most often occurs on the type of tubulointerstitial nephritis, with the further development of acute or chronic kidney injury. The clinic may be represented by the pseudotumor of kidney, renal tissue heterogeneity on the results of CT-studies; acute or chronic renal disease; combination with other organ damage (autoimmune pancreatitis, sclerosing cholangitis, sclerosing lymphoplasmacytic cholecystitis, colitis, sialadenitis, retroperitoneal fibrosis, etc.. Laboratory findings include an increased level of IgG4 in the blood serum, hypocomplementemia, eosinophilia. Histologically, there is interstitial inflammation with many plasma cells, interstitial fibrosis, tubular atrophy, thickening of the tubular basement membrane, some cases are a type of membranous glomerulonephritis. The aim of the study is to identify the patients with IgG4-related diseases with renal impairment and widening the pathological database of such patients with renal impairment to determine the classification criteria of this pathological condition. Materials and methods will include the deceased kidney screening, screening of patients with autoimmune and allergic diseases, nephrological patients screening with the lifetime biopsy (in some cases – repeat biopsy with chronic or acute kidney impairment. There will be clinical and pathological comparison in kidney damage and other diseases with the development of criteria for the classification of lesions in the presence of IgG4-positive substrates and further development of practical

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.

Dynamic mechanistic modeling of the multienzymatic one-pot reduction of dehydrocholic acid to 12-keto ursodeoxycholic acid with competing substrates and cofactors.

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Sun, Boqiao; Hartl, Florian; Castiglione, Kathrin; Weuster-Botz, Dirk

2015-01-01

Ursodeoxycholic acid (UDCA) is a bile acid which is used as pharmaceutical for the treatment of several diseases, such as cholesterol gallstones, primary sclerosing cholangitis or primary biliary cirrhosis. A potential chemoenzymatic synthesis route of UDCA comprises the two-step reduction of dehydrocholic acid to 12-keto-ursodeoxycholic acid (12-keto-UDCA), which can be conducted in a multienzymatic one-pot process using 3α-hydroxysteroid dehydrogenase (3α-HSDH), 7β-hydroxysteroid dehydrogenase (7β-HSDH), and glucose dehydrogenase (GDH) with glucose as cosubstrate for the regeneration of cofactor. Here, we present a dynamic mechanistic model of this one-pot reduction which involves three enzymes, four different bile acids, and two different cofactors, each with different oxidation states. In addition, every enzyme faces two competing substrates, whereas each bile acid and cofactor is formed or converted by two different enzymes. First, the kinetic mechanisms of both HSDH were identified to follow an ordered bi-bi mechanism with EBQ-type uncompetitive substrate inhibition. Rate equations were then derived for this mechanism and for mechanisms describing competing substrates. After the estimation of the model parameters of each enzyme independently by progress curve analyses, the full process model of a simple batch-process was established by coupling rate equations and mass balances. Validation experiments of the one-pot multienzymatic batch process revealed high prediction accuracy of the process model and a model analysis offered important insight to the identification of optimum reaction conditions. © 2015 American Institute of Chemical Engineers.

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

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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.

PML nuclear body component Sp140 is a novel autoantigen in primary biliary cirrhosis.

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Granito, Alessandro; Yang, Wei-Hong; Muratori, Luigi; Lim, Mark J; Nakajima, Ayako; Ferri, Silvia; Pappas, Georgios; Quarneti, Chiara; Bianchi, Francesco B; Bloch, Donald B; Muratori, Paolo

2010-01-01

Some patients with primary biliary cirrhosis (PBC) have antinuclear antibodies (ANAs). These ANAs include the "multiple nuclear dots" (MND) staining pattern, targeting promyelocytic leukemia protein (PML) nuclear body (NB) components, such as "speckled 100-kD" protein (Sp100) and PML. A new PML NB protein, designated as Sp140, was identified using serum from a PBC patient. The aim of this study was to analyze the immune response against Sp140 protein in PBC patients. We studied 135 PBC patients and 157 pathological controls with type 1 autoimmune hepatitis, primary sclerosing cholangitis, and systemic lupus erythematosus. We used indirect immunofluorescence and a neuroblastoma cell line expressing Sp140 for detecting anti-Sp140 antibodies, and a commercially available immunoblot for detecting anti-Sp100 and anti-PML antibodies. Anti-Sp140 antibodies were present in 20 (15%) PBC patients but not in control samples, with a higher frequency in antimitochondrial antibody (AMA)-negative cases (53 vs. 9%, P<0.0001). Anti-Sp140 antibodies were found together with anti-Sp100 antibodies in all but one case (19 of 20, 90%) and with anti-PML antibodies in 12 (60%) cases. Anti-Sp140 positivity was not associated with a specific clinical feature of PBC. Our study identifies Sp140 as a new, highly specific autoantigen in PBC for the first time. The very frequent coexistence of anti-Sp140, anti-Sp100 and anti-PML antibodies suggests that the NB is a multiantigenic complex in PBC and enhances the diagnostic significance of these reactivities, which are particularly useful in AMA-negative cases.

Rheumatic Disease Autoantibodies in Autoimmune Liver Diseases.

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Utiyama, Shirley R R; Zenatti, Katiane B; Nóbrega, Heloisa A J; Soares, Juliana Z C; Skare, Thelma L; Matsubara, Caroline; Muzzilo, Dominique A; Nisihara, Renato M

2016-08-01

Autoimmune liver diseases (ALDs) are known to be associated with systemic autoimmune rheumatic diseases (SARDs) and their autoantibodies. We aimed to study the prevalence of SARDs and related autoantibodies, as well as their prognostic implications in a group of patients with ALDs. This was a cross-sectional study. Sixty patients with ALDs (38.3% with autoimmune hepatitis; 11.7% with primary biliary cirrhosis; 25% with primary sclerosing cholangitis and 25% with overlap syndrome) were studied for the presence of SARDs and their autoantibodies. There was autoimmune rheumatic disease in 20% of the studied sample. Systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) were the commonest (11.6% and 5%, respectively). Antinuclear antibodies (ANAs) were present in 35% of the patients, followed by anti-Ro (20.0%); anti-nucleosome (18.3%); rheumatoid factor (10%) anti-CCP (8.3%); anti-RNP (8.3%); anti-ds-DNA (6.6%); anti-La (3.3%); anti-Sm (3.3%), anti-ribosomal P (3.3%). Anti-Ro (p = 0.0004), anti-La (p = 0.03), anti-RNP (p = 0.04) and anti-Sm (p = 0.03) were commonly found in patients with SARD, but not anti-DNA, anti-nucleosome and anti-ribosomal P. No differences were found in liver function tests regarding to the presence of autoantibodies. There was a high prevalence of SARD and their autoantibodies in ALD patients. Anti-Ro, anti-La, anti-RNP and anti-Sm positivity points to an association with systemic autoimmune rheumatic diseases. The presence of autoantibodies was not related to liver function tests.

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.

Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands.

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van Gerven, Nicole M F; Verwer, Bart J; Witte, Birgit I; van Erpecum, Karel J; van Buuren, Henk R; Maijers, Ingrid; Visscher, Arjan P; Verschuren, Edwin C; van Hoek, Bart; Coenraad, Minneke J; Beuers, Ulrich H W; de Man, Robert A; Drenth, Joost P H; den Ouden, Jannie W; Verdonk, Robert C; Koek, Ger H; Brouwer, Johannes T; Guichelaar, Maureen M J; Vrolijk, Jan Maarten; Mulder, Chris J J; van Nieuwkerk, Carin M J; Bouma, Gerd

2014-10-01

Epidemiological data on autoimmune hepatitis (AIH) are scarce. In this study, we determined the clinical and epidemiological characteristics of AIH patients in the Netherlands (16.7 million inhabitants). Clinical characteristics were collected from 1313 AIH patients (78% females) from 31 centers, including all eight academic centers in the Netherlands. Additional data on ethnicity, family history and symptoms were obtained by the use of a questionnaire. The prevalence of AIH was 18.3 (95% confidential interval [CI]: 17.3-19.4) per 100,000 with an annual incidence of 1.1 (95% CI: 0.5-2) in adults. An incidence peak was found in middle-aged women. At diagnosis, 56% of patients had fibrosis and 12% cirrhosis in liver biopsy. Overall, 1% of patients developed HCC and 3% of patients underwent liver transplantation. Overlap with primary biliary cirrhosis and primary sclerosing cholangitis was found in 9% and 6%, respectively. The clinical course did not differ between Caucasian and non-Caucasian patients. Other autoimmune diseases were found in 26% of patients. Half of the patients reported persistent AIH-related symptoms despite treatment with a median treatment period of 8 years (range 1-44 years). Familial occurrence was reported in three cases. This is the largest epidemiological study of AIH in a geographically defined region and demonstrates that the prevalence of AIH in the Netherlands is uncommon. Although familial occurrence of AIH is extremely rare, our twin data may point towards a genetic predisposition. The high percentage of patients with cirrhosis or fibrosis at diagnosis urges the need of more awareness for AIH.

[Choledocholithiasis in Edgardo Rebagliati Martins Hospital. Lima-Peru. 2010-2011. Incidence, risk factors, diagnostic and therapeutic aspects].

Science.gov (United States)

Llatas Pérez, Juan; Hurtado Roca, Yamilee; Frisancho Velarde, Oscar

2011-01-01

to determine the incidence, frequency of risk factors, diagnostic aspects (clinics, biochemical, and images) and therapeutic aspects of the choledocholithiasis. Descriptive and prospective analysis of 51 patients who signed informed consent for study of choledocholithiasis by ERCP (endoscopic retrograde cholangiopancreatography). Of the 51 patients, 36 (70.6%) confirmed choledocholithiasis by ERCP. Its incidence was 10.4%. The mean age was 63.75 years, BMI 25.59 kg/m2 and 55.6% were women. Abdominal pain was present in 94.4% of patients. Total bilirubin in the group with choledocholithiasis was 5.8 mg / dl at baseline and 4.2 mg / dl in hospitalization. 11.1% debuted with pancreatitis and 30.6% with cholangitis. In 68.7% of patients with cholangitis was confirmed choledocholithiasis by ERCP. The specificity of ultrasound was 0.80 (95% CI 0.6- 1). The specificity of the nuclear magnetic resonance was 0.25 (95% CI 0.17-0.67). Of the 51 patients with ERCP, 4 (7.80%) do pancreatitis, 1(1.96%) cholangitis and 2 (3.92%) bleeding Choledocholithiasis occurs in older people, female and overweight with an incidence of 10.4%. Abdominal pain is the most common symptom. A total bilirubin more tan 4mg/dl occurs in patients with choledocholithiasis. The complications of choledocholithiasis (pancreatitis and cholangitis) were more frequent than reported in the literature. Both the specificity of cholangitis for choledocholithiasis, the specificity of ultrasound and the specificity of the nuclear magnetic resonance were lower than reported in the literature. ERCP complications were slightly higher than that reported in the literature.

Cholecystectomy or gallbladder in situ after endoscopic sphincterotomy and bile duct stone removal in Chinese patients.

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Lau, James Y W; Leow, Chon-Kar; Fung, Terence M K; Suen, Bing-Yee; Yu, Ly-Mee; Lai, Paul B S; Lam, Yuk-Hoi; Ng, Enders K W; Lau, Wan Yee; Chung, Sydney S C; Sung, Joseph J Y

2006-01-01

In patients with stones in their bile ducts and gallbladders, cholecystectomy is generally recommended after endoscopic sphincterotomy and clearance of bile duct stones. However, only approximately 10% of patients with gallbladders left in situ will return with further biliary complications. Expectant management is alternately advocated. In this study, we compared the treatment strategies of laparoscopic cholecystectomy and gallbladders left in situ. We randomized patients (>60 years of age) after endoscopic sphincterotomy and clearance of their bile duct stones to receive early laparoscopic cholecystectomy or expectant management. The primary outcome was further biliary complications. Other outcome measures included adverse events after cholecystectomy and late deaths from all causes. One hundred seventy-eight patients entered into the trial (89 in each group); 82 of 89 patients who were randomized to receive laparoscopic cholecystectomy underwent the procedure. Conversion to open surgery was needed in 16 of 82 patients (20%). Postoperative complications occurred in 8 patients (9%). Analysis was by intention to treat. With a median follow-up of approximately 5 years, 6 patients (7%) in the cholecystectomy group returned with further biliary events (cholangitis, n = 5; biliary pain, n = 1). Among those with gallbladders in situ, 21 (24%) returned with further biliary events (cholangitis, n = 13; acute cholecystitis, n = 5; biliary pain, n = 2; and jaundice, n = 1; log rank, P = .001). Late deaths were similar between groups (cholecystectomy, n = 19; gallbladder in situ, n = 11; P = .12). In the Chinese, cholecystectomy after endoscopic treatment of bile duct stones reduces recurrent biliary events and should be recommended.

Percutaneous sclerotherapy of pediatric lymphatic malformations: experience and outcomes according to the agent used.

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Gallego Herrero, C; Navarro Cutillas, V

Analyze statistically the success, number of sessions required and complete duration of treatment of agents used in pediatric percutaneous sclerotherapy of lymphatic malformations, to determine the most suitable. Retrospective study based on outcomes from percutaneous sclerotherapy performed on lymphatic malformations of 56 patients conducted by pediatric interventional radiologist for 14 years. As first approach, the procedure consists of ultrasound-guided introduction of sclerosing agent. Sessions were repeated until clinical resolution. Success, number of sessions and the duration of treatment were recorded and statistical treatment of the data was performed to obtain further conclusions. Lost patients in follow up and other minority agents used were excluded from the data. Eventually, 52 patients treated with OK432 (n=29), Ethibloc (n=5) and combination therapy (n=18) were included. The average number of sessions and duration in months of treatment was respectively 2.38 and 8.6 for OK432, 1.4 and 5.6 for Ethibloc, and 1.83 and 2.30 for dual therapy. The results were statistically significant for the difference in duration between OK432 and dual therapy. Also, 60-80% of patients reached proper results related to success, but the difference was no significant among the agents. Other demographic and anatomical variables were analyzed, not showing any difference, which supports the homogeneity of the sample. Despite of no significant difference in success and number of sessions among agents, longer duration of treatment with OK432 than dual therapy could mean greater health costs and probably greater disturb for patient and family. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

Biodegradable biliary stent implantation in the treatment of benign bilioplastic-refractory biliary strictures: preliminary experience.

Science.gov (United States)

Mauri, Giovanni; Michelozzi, Caterina; Melchiorre, Fabio; Poretti, Dario; Tramarin, Marco; Pedicini, Vittorio; Solbiati, Luigi; Cornalba, Gianpaolo; Sconfienza, Luca Maria

2013-12-01

To evaluate feasibility, safety, and outcome of patients treated with biodegradable biliary stents for benign biliary stenosis refractory to other treatments. Between March 2011 and September 2012, ten patients (seven men, three women; age 59 ± 7 years) with recurrent cholangitis due to postsurgical biliary stricture, previous multiple unsuccessful (two to five) bilioplasties, and unsuitability for surgical/endoscopic repair underwent percutaneous implantation of a biodegradable biliary stent. Patients were followed-up clinically and with ultrasound at 1, 3 and 6 months, and then at 6-month intervals. Stent implantation was always feasible. No immediate major or minor complications occurred. In all patients, 48-h cholangiographic control demonstrated optimal stent positioning and stenosis resolution. In a median follow-up time of 16.5 months (25th-75th percentiles = 11-20.25 months) no further invasive treatment was needed. Three patients experienced transient episodes of cholangitis. Neither re-stenosis nor dilatation of the biliary tree was documented during follow-up. No stent was visible at the 6-month follow-up. Percutaneous placement of biodegradable biliary stents represents a new option in treating benign biliary stenoses refractory to treatment with bilioplasty. This technique seems to be feasible, effective and free from major complications. Further investigations are warranted to confirm our preliminary results.

Structural defect linked to nonrandom mutations in the matrix gene of Biden strain subacute sclerosing panencephalitis virus defined by cDNA cloning and expression of chimeric genes

International Nuclear Information System (INIS)

Ayata, M.; Hirano, A.; Wong, T.C.

1989-01-01

Biken strain, a nonproductive measles viruslike agent isolated from a subacute sclerosing panencephalitis (SSPE) patient, contains a posttranscriptional defect affecting matrix (M) protein. A putative M protein was translated in vitro with RNA from Biken strain-infected cells. A similar protein was detected in vivo by an antiserum against a peptide synthesized from the cloned M gene of Edmonston strain measles virus. By using a novel method, full-length cDNAs of the Biken M gene were selectively cloned. The cloned Biken M gene contained an open reading frame which encoded 8 extra carboxy-terminal amino acid residues and 20 amino acid substitutions predicted to affect both the hydrophobicity and secondary structure of the gene product. The cloned gene was expressed in vitro and in vivo into a 37,500 M r protein electrophoretically and antigenically distinct from the M protein of Edmonston strain but identical to the M protein in Biken strain-infected cells. Chimeric M proteins synthesized in vitro and in vivo showed that the mutations in the carboxy-proximal region altered the local antigenicity and those in the amino region affected the overall protein conformation. The protein expressed from the Biken M gene was unstable in vivo. Instability was attributed to multiple mutations. These results offer insights into the basis of the defect in Biken strain and pose intriguing questions about the evolutionary origins of SSPE viruses in general

Standard guidelines for care: Sclerotherapy in dermatology

Directory of Open Access Journals (Sweden)

Niti Khunger

2011-01-01

Full Text Available Definition: Sclerotherapy is defined as the targeted elimination of small vessels, varicose veins and vascular anomalies by the injection of a sclerosant. The aim of sclerotherapy is to damage the vessel wall and transform it into a fibrous cord that cannot be recanalized. It is a simple, cost-effective, efficacious and esthetically acceptable modality for both therapeutic and esthetic purposes. Indications: Therapeutic indications include varicose veins and vascular malformations. Esthetic indications include telangiectasias and reticular veins. In the management of varicose veins, it may need to be combined with other surgical methods of treatment, such as ligation of the saphenofemoral junction, stab ligation of perforators and stripping. A surgical opinion may be necessary. Methodology: A thorough knowledge of the anatomy and physiology of the venous system of the legs, basic principles of venous insufficiency, methods of diagnosis and, in addition, uses, mechanisms of action and complications of sclerosing agents and proper compression techniques are important pre-requisites to successful sclerotherapy. Although various sclerosing agents are available, polidoconal and sodium tetradecyl sulfate are most commonly used. More recently, these sclerosants have been used in microfoam form for increased efficacy. The basic principle of a successful sclerotherapy technique is the use of an optimal volume and concentration of the sclerosant according to the size of the vessel. The sclerosant is injected carefully into the vessel and compression is applied. Contraindications: Contraindications include superficial and deep venous thrombosis, sapheno-femoral junction incompetence, pregnancy, myocardial decompensation, migraine, hypercoagulable state, serious systemic illness, dependency edema, immobility, arterial disease, diabetes mellitus and allergic reactions to sclerosants. Complications: While sclerotherapy is usually a safe procedure

Risk factors and classifications of hilar cholangiocarcinoma.

Science.gov (United States)

Suarez-Munoz, Miguel Angel; Fernandez-Aguilar, Jose Luis; Sanchez-Perez, Belinda; Perez-Daga, Jose Antonio; Garcia-Albiach, Beatriz; Pulido-Roa, Ysabel; Marin-Camero, Naiara; Santoyo-Santoyo, Julio

2013-07-15

Cholangiocarcinoma is the second most common primary malignant tumor of the liver. Perihilar cholangiocarcinoma or Klatskin tumor represents more than 50% of all biliary tract cholangiocarcinomas. A wide range of risk factors have been identified among patients with Perihilar cholangiocarcinoma including advanced age, male gender, primary sclerosing cholangitis, choledochal cysts, cholelithiasis, cholecystitis, parasitic infection (Opisthorchis viverrini and Clonorchis sinensis), inflammatory bowel disease, alcoholic cirrhosis, nonalcoholic cirrhosis, chronic pancreatitis and metabolic syndrome. Various classifications have been used to describe the pathologic and radiologic appearance of cholangiocarcinoma. The three systems most commonly used to evaluate Perihilar cholangiocarcinoma are the Bismuth-Corlette (BC) system, the Memorial Sloan-Kettering Cancer Center and the TNM classification. The BC classification provides preoperative assessment of local spread. The Memorial Sloan-Kettering cancer center proposes a staging system according to three factors related to local tumor extent: the location and extent of bile duct involvement, the presence or absence of portal venous invasion, and the presence or absence of hepatic lobar atrophy. The TNM classification, besides the usual descriptors, tumor, node and metastases, provides additional information concerning the possibility for the residual tumor (R) and the histological grade (G). Recently, in 2011, a new consensus classification for the Perihilar cholangiocarcinoma had been published. The consensus was organised by the European Hepato-Pancreato-Biliary Association which identified the need for a new staging system for this type of tumors. The classification includes information concerning biliary or vascular (portal or arterial) involvement, lymph node status or metastases, but also other essential aspects related to the surgical risk, such as remnant hepatic volume or the possibility of underlying disease.

Clinical outcomes and toxicity of proton beam therapy for advanced cholangiocarcinoma

International Nuclear Information System (INIS)

Makita, Chiyoko; Kikuchi, Yasuhiro; Hareyama, Masato; Murakami, Masao; Fuwa, Nobukazu; Hata, Masaharu; Inoue, Tomio; Nakamura, Tatsuya; Takada, Akinori; Takayama, Kanako; Suzuki, Motohisa; Ishikawa, Yojiro; Azami, Yusuke; Kato, Takahiro; Tsukiyama, Iwao

2014-01-01

We examined the efficacy and toxicity of proton beam therapy (PBT) for treating advanced cholangiocarcinoma. The clinical data and outcomes of 28 cholangiocarcinoma patients treated with PBT between January 2009 and August 2011 were retrospectively examined. The Kaplan–Meier method was used to estimate overall survival (OS), progression-free survival (PFS), and local control (LC) rates, and the log-rank test to analyze the effects of different clinical and treatment variables on survival. Acute and late toxicities were assessed using the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.0. The median age of the 17 male and 11 female patients was 71 years (range, 41 to 84 years; intrahepatic/peripheral cholangiocarcinoma, n = 6; hilar cholangiocarcinoma/Klatskin tumor, n = 6; distal extrahepatic cholangiocarcinoma, n = 3; gallbladder cancer, n = 3; local or lymph node recurrence, n = 10; size, 20–175 mm; median 52 mm). The median radiation dose was 68.2 Gy (relative biological effectiveness [RBE]) (range, 50.6 to 80 Gy (RBE)), with delivery of fractions of 2.0 to 3.2 Gy (RBE) daily. The median follow-up duration was 12 months (range, 3 to 29 months). Fifteen patients underwent chemotherapy and 8 patients, palliative biliary stent placement prior to PBT. OS, PFS, and LC rates at 1 year were 49.0%, 29.5%, and 67.7%, respectively. LC was achieved in 6 patients, and was better in patients administered a biologically equivalent dose of 10 (BED10) > 70 Gy compared to those administered < 70 Gy (83.1% vs. 22.2%, respectively, at 1 year). The variables of tumor size and performance status were associated with survival. Late gastrointestinal toxicities grade 2 or greater were observed in 7 patients <12 months after PBT. Cholangitis was observed in 11 patients and 3 patients required stent replacement. Relatively high LC rates after PBT for advanced cholangiocarcinoma can be achieved by delivery of a BED10 > 70 Gy. Gastrointestinal

Endoscopic stenting for common bile duct stenoses in chronic pancreatitis: results and impact on long-term outcome.

Science.gov (United States)

Eickhoff, A; Jakobs, R; Leonhardt, A; Eickhoff, J C; Riemann, J F

2001-10-01

The overall incidence of common bile duct strictures due to chronic pancreatitis is reported to be approximately 10-30%. It remains a challenging problem for gastroenterologists and surgeons. The exact role of endoscopic stenting has not yet been clearly defined. Thirty-nine patients with chronic pancreatitis and symptomatic common bile duct stenoses underwent endoscopic stenting and were studied retrospectively. We were particularly interested in how many patients would achieve resolution of the stricture and tolerate removal of the stents in the long term. Indications for endoscopic stenting were symptomatic cholestasis, jaundice or cholangitis. The initial serum bilirubin was 8.3 mg/dl and the diameter of the common bile duct was 14.2 mm before stenting. Within 3-7 days of stenting, all patients presented improvement of jaundice and cholestasis. After a median stenting time of 9 months (range 1-144 months), 46% of the patients demonstrated regression of the stricture and clinical improvement, 26% required further stenting, and 28% were referred to surgery. Five patients received a self-expandable metal Wallstent. Thirty-one per cent demonstrated complete clinical recovery of the stricture as well as 10.2% a complete, radiologically verified stricture regression in a median follow-up of 58 months. There seems to be a therapeutic benefit for short-term endoscopic treatment but medium-term and long-term outcome remains questionable. Endoscopic stenting should be applied as an initial therapy before surgery, but it can be the definitive approach for older and morbid patients or cases with complete stricture regression after stent removal. Overall, it should not be considered as a routine procedure for symptomatic cases.

Effects of Paclitaxel-conjugated N-Succinyl-Hydroxyethyl Chitosan Film for Proliferative Cholangitis in Rabbit Biliary Stricture Model.

Science.gov (United States)

Wang, Tao; Zou, Hao; Liu, Yun-Xia; Zhang, Xiao-Wen

2018-03-20

Paclitaxel (PTX) could inhibit the growth of fibroblasts, which occurs in proliferative cholangitis and leads to biliary stricture. However, its use has been limited due to poor bioavailability and local administration for short time. This study designed and synthesized a new PTX-conjugated chitosan film (N-succinyl-hydroxyethyl chitosan containing PTX [PTX-SHEC]) and evaluated its safety and efficiency using in vivo and in vitro experiments. The SHEC conjugated with PTX was confirmed by nuclear magnetic resonance (NMR) and Fourier-transform infrared spectroscopy (FT-IR) measurements. Drug releases in vitro and in vivo were determined using high-performance liquid chromatography. Cell viability in vitro was measured using 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide. Rabbit biliary stricture model was constructed. All rabbits randomly divided into five groups (n = 8 in each group): the sham-operated rabbits were used as control (Group A), Groups B received laparotomies and suture, Group C received laparotomies and covered SHEC suture without the PTX coating, Group D received laparotomies and covered PTX-SHEC suture, and Group E received laparotomies and 1000 μmol/L PTX administration. Liver function tests and residual dosage of PTX from each group were measured by enzyme-linked immunosorbent assay. Histological data and α-smooth muscle actin (SMA) immunohistochemical staining of common bile duct were examined. NMR and FT-IR indicated that PTX was successfully introduced, based on the appearance of signals at 7.41-7.99 ppm, 1.50 ppm, and 1.03 ppm, due to the presence of aromatic protons, methylene protons, and methyl protons of PTX, respectively. No bile leak was observed. The PTX-conjugated film could slowly release PTX for 4 weeks (8.89 ± 0.03 μg at day 30). The in vitro cell viability test revealed significantly different levels of toxicity between films with and without PTX (111.7 ± 4.0% vs. 68.1 ± 6.0%, P films (67.7 ± 5.4%, 67.2 ± 3

Galdevejsatresi. Hepatoportoenteroanastomose og/eller levertransplantation

DEFF Research Database (Denmark)

Kvist, N E; Thorup, Jørgen Mogens

1993-01-01

Long-term survival of biliary atresia patients after Kasai hepatoportoenterostomy is being increasingly reported. Favorable prognostic factors are: 1) Young age at operation (max. 60 days). 2) Few incidents of postoperative cholangitis. Jaundice-free long-term survival is achieved in one-fourth t......Long-term survival of biliary atresia patients after Kasai hepatoportoenterostomy is being increasingly reported. Favorable prognostic factors are: 1) Young age at operation (max. 60 days). 2) Few incidents of postoperative cholangitis. Jaundice-free long-term survival is achieved in one...

Melioidosis in acute cholangitis of diabetic patient: a forgotten diagnosis

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Mohamad N

2012-08-01

Full Text Available Nasir Mohamad,1 Suresh Ponnusamy,2 Sunita Devi,3 Rishya Manikam,4 Ilya Irinaz Idrus,1 Nor Hidayah Abu Bakar51Department of Emergency Medicine, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Malaysia; 2AIMST University, Bedong, Malaysia; 3Hospital Sultan Abdul Halim, Sungai Petani, Malaysia; 4University Malaya Medical Centre, Kuala Lumpur, Malaysia; 5Department of Pathology, Hospital Raja Perempuan Zainab II, Kota Bharu, MalaysiaAbstract: Melioidosis presents with a wide range of clinical presentations, which include severe community-acquired pneumonia, septicemia, central nervous system infection, and less severe soft tissue infection. Hence, its diagnosis depends heavily on the clinical microbiology laboratory for culture. In this case report, we describe an atypical presentation of melioidosis in a 52-year-old man who had fever, right upper-abdominal pain, and jaundice for 15 days. Melioidosis caused by Burkholderia pseudomallei was subsequently diagnosed from blood culture. As a primary care physician, high suspicion index is of great importance. High suspicion index of melioidosis in a high-risk group patient, such as the patient with diabetes mellitus and diabetic foot, is crucial in view of atypical presentations of pseudomonas sepsis. A correct combination of antibiotic administration in the early phase of therapy will determine its successful outcome.Keywords: Burkholderia pseudomallei, atypical, high suspicion, primary care

Adenomas of the common bile duct in familial adenomatous polyposis

Science.gov (United States)

Yan, Mao-Lin; Pan, Jun-Yong; Bai, Yan-Nan; Lai, Zhi-De; Chen, Zhong; Wang, Yao-Dong

2015-01-01

Familial adenomatous polyposis (FAP) or Gardner’s syndrome is often accompanied by adenomas of the stomach and duodenum. We experienced a case of adenomas of the common bile duct in a 40-year-old woman with FAP presenting with acute cholangitis. Only 8 cases of adenomas or adenocarcinoma of the common bile duct have been reported in the literature in patients with FAP or Gardner’s syndrome. Those patients presented with acute cholangitis or pancreatitis. Local excision or Whipple procedure may be the reasonable surgical option. PMID:25780319

Amplification and sequence analysis of partial bacterial 16S ribosomal RNA gene in gallbladder bile from patients with primary biliary cirrhosis.

Science.gov (United States)

Hiramatsu, K; Harada, K; Tsuneyama, K; Sasaki, M; Fujita, S; Hashimoto, T; Kaneko, S; Kobayashi, K; Nakanuma, Y

2000-07-01

The etiopathogenesis of bile duct lesion in primary biliary cirrhosis is unknown, though the participation of bacteria and/or their components and products is suspected. In this study, we tried to detect and identify bacteria in the bile of patients with primary biliary cirrhosis by polymerase chain reaction using universal bacterial primers of the 16S ribosomal RNA gene. Gallbladder bile samples from 15 patients with primary biliary cirrhosis, 5 with primary sclerosing cholangitis, 5 with hepatitis C virus-related liver cirrhosis, 11 with cholecystolithiasis, and from 12 normal adult gallbladders were used. In addition to the culture study, partial bacterial 16S ribosomal RNA gene was amplified by polymerase chain reaction (PCR) taking advantage of universal primers that can amplify the gene of almost all bacterial species, and the amplicons were cloned and sequenced. Sequence homology with specific bacterial species was analyzed by database research. Bacterial contamination at every step of the bile sampling, DNA extraction and PCR study was avoided. Furthermore, to confirm whether bacterial DNA is detectable in liver explants, the same analysis was performed using 10 liver explants of patients with primary biliary cirrhosis. In primary biliary cirrhosis, 75% (p<0.0001) of 100 clones were identified as so-called gram-positive cocci while these cocci were positive in only 5% in cholecystolithiasis (p<0.0001). In cholecystolithiasis gram-negative rods were predominant instead. One bacterial species detected in a normal adult was not related to those detected in primary biliary cirrhosis and cholecystolithiasis patients. No bacterial DNA was detected by PCR amplification in 10 liver explants of patients with primary biliary cirrhosis. The present results raise several possible roles of gram-positive bacteria in bile in the etiopathogenesis of primary biliary cirrhosis. However, these results could also reflect an epiphenomenon due to decreased bile flow in the

Visualization of the thymus by substance P receptor scintigraphy in man

Energy Technology Data Exchange (ETDEWEB)

Hagen, P.M. van [Department of Immunology, Erasmus University, Rotterdam (Netherlands)]|[Department of Internal Medicine III, Erasmus University, Rotterdam (Netherlands); Breeman, W.A.P. [Department of Nuclear Medicine, Erasmus University, Rotterdam (Netherlands); Reubi, J.C. [Department of Pathology, University of Berne (Switzerland); Postema, P.T.E. [Department of Internal Medicine III, Erasmus University, Rotterdam (Netherlands); Anker-Lugtenburg, P.J. van den [Department of Hematology, Erasmus University, Rotterdam (Netherlands); Kwekkeboom, D.J. [Department of Nuclear Medicine, Erasmus University, Rotterdam (Netherlands); Laissue, J. [Department of Pathology, University of Berne (Switzerland); Waser, B. [Department of Pathology, University of Berne (Switzerland); Lamberts, S.W.J. [Department of Internal Medicine III, Erasmus University, Rotterdam (Netherlands); Visser, T.J. [Department of Internal Medicine III, Erasmus University, Rotterdam (Netherlands); Krenning, E.P. [Department of Internal Medicine III, Erasmus University, Rotterdam (Netherlands)]|[Department of Nuclear Medicine, Erasmus University, Rotterdam (Netherlands)

1996-11-01

In this study we present the results concerning the metabolism of the substance P analogue [{sup 111}In-DTPA-Arg{sup 1}]-substance P in man, as well as the visualization of the thymus in patients with immune-mediated diseases. Twelve selected patients were investigated, comprising five with inflammatory bowel disease, one with ophthalmic Graves` disease, one with sclerosing cholangitis, one with Sjoegren`s syndrome, one with rheumatoid arthritis, one with systemic lupus erythematosus and two with myasthenia gravis. After intravenous administration of 150-250 MBq (2.5-5.0 {mu}g) [{sup 111}In-DTPA-Arg{sup 1}]-substance P, radioactivity was measured in blood, urine and faeces for 48 h. Planar and single-photon emission tomographic images were obtained 4 and 24 h after injection. After administration of [{sup 111}In-DTPA-Arg{sup 1}]-substance P, a transient flush was observed in all patients. Degradation of [{sup 111}In-DTPA-Arg{sup 1}]-substance P started in the first minutes after administration, resulting in a half-life of 10 min for the total plasma radioactivity, and of 4 min for the intact radiopharmaceutical. Urinary excretion accounted for >95% of the radioactivity within 24 h post injection, and up to 0.05% was found in the faeces up to 60 h. In all patients uptake of radioactivity was found in the areolae mammae (in women), liver, spleen, kidneys and urinary bladder. In eight patients a high uptake of [{sup 111}In-DTPA-Arg{sup 1}]-substance P was observed in the thymus. We conclude that, despite its short half-life, [{sup 111}In-DTPA-Arg{sup 1}]-substance P can be used to visualize the thymus. This may contribute to the investigation of the role of thymus in immune-mediated diseases. In addition, inflammatory sites in various diseases could be visualized. (orig.). With 6 figs., 1 tab.

CD8+ T-Cell Deficiency, Epstein-Barr Virus Infection, Vitamin D Deficiency, and Steps to Autoimmunity: A Unifying Hypothesis

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Michael P. Pender

2012-01-01

Full Text Available CD8+ T-cell deficiency is a feature of many chronic autoimmune diseases, including multiple sclerosis, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, systemic sclerosis, dermatomyositis, primary biliary cirrhosis, primary sclerosing cholangitis, ulcerative colitis, Crohn's disease, psoriasis, vitiligo, bullous pemphigoid, alopecia areata, idiopathic dilated cardiomyopathy, type 1 diabetes mellitus, Graves' disease, Hashimoto's thyroiditis, myasthenia gravis, IgA nephropathy, membranous nephropathy, and pernicious anaemia. It also occurs in healthy blood relatives of patients with autoimmune diseases, suggesting it is genetically determined. Here it is proposed that this CD8+ T-cell deficiency underlies the development of chronic autoimmune diseases by impairing CD8+ T-cell control of Epstein-Barr virus (EBV infection, with the result that EBV-infected autoreactive B cells accumulate in the target organ where they produce pathogenic autoantibodies and provide costimulatory survival signals to autoreactive T cells which would otherwise die in the target organ by activation-induced apoptosis. Autoimmunity is postulated to evolve in the following steps: (1 CD8+ T-cell deficiency, (2 primary EBV infection, (3 decreased CD8+ T-cell control of EBV, (4 increased EBV load and increased anti-EBV antibodies, (5 EBV infection in the target organ, (6 clonal expansion of EBV-infected autoreactive B cells in the target organ, (7 infiltration of autoreactive T cells into the target organ, and (8 development of ectopic lymphoid follicles in the target organ. It is also proposed that deprivation of sunlight and vitamin D at higher latitudes facilitates the development of autoimmune diseases by aggravating the CD8+ T-cell deficiency and thereby further impairing control of EBV. The hypothesis makes predictions which can be tested, including the prevention and successful treatment of chronic autoimmune diseases by controlling EBV infection.

Autoimmune pancreatitis

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Davorin Dajčman

2007-05-01

Full Text Available Background: Autoimmune pancreatitis is a recently described type of pancreatitis of presumed autoimmune etiology. Autoimmune pancreatitis is often misdiagnosed as pancreatic cancer difficult, since their clinical presentations are often similar. The concept of autoimmune pancreatitis was first published in 1961. Since then, autoimmune pancreatitis has often been treated not as an independent clinical entity but rather as a manifestation of systemic disease. The overall prevalence and incidence of the disease have yet to be determined, but three series have reported the prevalence as between 5 and 6 % of all patients with chronic pancreatitis. Patient vary widely in age, but most are older than 50 years. Patients with autoimmune pancreatitis usually complain of the painless jaundice, mild abdominal pain and weight loss. There is no laboratory hallmark of the disease, even if cholestatic profiles of liver dysfunction with only mild elevation of amylase and lipase levels have been reported.Conclusions: Proposed diagnostic criteria contains: (1 radiologic imaging, diffuse enlargement of the pancreas and diffusely irregular narrowing of the main pancreatic duct, (2 laboratory data, elevated levels of serum ã-globulin and/or IgG, specially IgG4, or the presence of autoantibodies and (3 histopathologic examination, fibrotic change with dense lymphoplasmacytic infiltration in the pancreas. For correct diagnosis of autoimmune pancreatitis, criterion 1 must be present with criterion 2 and/or 3. Autoimmune pancreatitis is frequently associated with rheumatoid arthritis, Sjogren’s syndrome, inflammatory bowel disease, tubulointersticial nephritis, primary sclerosing cholangitis and idiopathic retroperitoneal fibrosis. Pancreatic biopsy using an endoscopic ultrasound-guided fine needle aspiration biopsy is the most important diagnostic method today. Treatment with corticosteroids leads to the and resolution of pancreatic inflamation, obstruction and

Genome-wide association study identifies variants associated with autoimmune hepatitis type 1.

Science.gov (United States)

de Boer, Ynto S; van Gerven, Nicole M F; Zwiers, Antonie; Verwer, Bart J; van Hoek, Bart; van Erpecum, Karel J; Beuers, Ulrich; van Buuren, Henk R; Drenth, Joost P H; den Ouden, Jannie W; Verdonk, Robert C; Koek, Ger H; Brouwer, Johannes T; Guichelaar, Maureen M J; Vrolijk, Jan M; Kraal, Georg; Mulder, Chris J J; van Nieuwkerk, Carin M J; Fischer, Janett; Berg, Thomas; Stickel, Felix; Sarrazin, Christoph; Schramm, Christoph; Lohse, Ansgar W; Weiler-Normann, Christina; Lerch, Markus M; Nauck, Matthias; Völzke, Henry; Homuth, Georg; Bloemena, Elisabeth; Verspaget, Hein W; Kumar, Vinod; Zhernakova, Alexandra; Wijmenga, Cisca; Franke, Lude; Bouma, Gerd

2014-08-01

Autoimmune hepatitis (AIH) is an uncommon autoimmune liver disease of unknown etiology. We used a genome-wide approach to identify genetic variants that predispose individuals to AIH. We performed a genome-wide association study of 649 adults in The Netherlands with AIH type 1 and 13,436 controls. Initial associations were further analyzed in an independent replication panel comprising 451 patients with AIH type 1 in Germany and 4103 controls. We also performed an association analysis in the discovery cohort using imputed genotypes of the major histocompatibility complex region. We associated AIH with a variant in the major histocompatibility complex region at rs2187668 (P = 1.5 × 10(-78)). Analysis of this variant in the discovery cohort identified HLA-DRB1*0301 (P = 5.3 × 10(-49)) as a primary susceptibility genotype and HLA-DRB1*0401 (P = 2.8 × 10(-18)) as a secondary susceptibility genotype. We also associated AIH with variants of SH2B3 (rs3184504, 12q24; P = 7.7 × 10(-8)) and CARD10 (rs6000782, 22q13.1; P = 3.0 × 10(-6)). In addition, strong inflation of association signal was found with single-nucleotide polymorphisms associated with other immune-mediated diseases, including primary sclerosing cholangitis and primary biliary cirrhosis, but not with single-nucleotide polymorphisms associated with other genetic traits. In a genome-wide association study, we associated AIH type 1 with variants in the major histocompatibility complex region, and identified variants of SH2B3and CARD10 as likely risk factors. These findings support a complex genetic basis for AIH pathogenesis and indicate that part of the genetic susceptibility overlaps with that for other immune-mediated liver diseases. Copyright © 2014 AGA Institute. Published by Elsevier Inc. All rights reserved.

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

Science.gov (United States)

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.

Visualization of the thymus by substance P receptor scintigraphy in man

International Nuclear Information System (INIS)

Hagen, P.M. van; Breeman, W.A.P.; Reubi, J.C.; Postema, P.T.E.; Anker-Lugtenburg, P.J. van den; Kwekkeboom, D.J.; Laissue, J.; Waser, B.; Lamberts, S.W.J.; Visser, T.J.; Krenning, E.P.

1996-01-01

In this study we present the results concerning the metabolism of the substance P analogue [ 111 In-DTPA-Arg 1 ]-substance P in man, as well as the visualization of the thymus in patients with immune-mediated diseases. Twelve selected patients were investigated, comprising five with inflammatory bowel disease, one with ophthalmic Graves' disease, one with sclerosing cholangitis, one with Sjoegren's syndrome, one with rheumatoid arthritis, one with systemic lupus erythematosus and two with myasthenia gravis. After intravenous administration of 150-250 MBq (2.5-5.0 μg) [ 111 In-DTPA-Arg 1 ]-substance P, radioactivity was measured in blood, urine and faeces for 48 h. Planar and single-photon emission tomographic images were obtained 4 and 24 h after injection. After administration of [ 111 In-DTPA-Arg 1 ]-substance P, a transient flush was observed in all patients. Degradation of [ 111 In-DTPA-Arg 1 ]-substance P started in the first minutes after administration, resulting in a half-life of 10 min for the total plasma radioactivity, and of 4 min for the intact radiopharmaceutical. Urinary excretion accounted for >95% of the radioactivity within 24 h post injection, and up to 0.05% was found in the faeces up to 60 h. In all patients uptake of radioactivity was found in the areolae mammae (in women), liver, spleen, kidneys and urinary bladder. In eight patients a high uptake of [ 111 In-DTPA-Arg 1 ]-substance P was observed in the thymus. We conclude that, despite its short half-life, [ 111 In-DTPA-Arg 1 ]-substance P can be used to visualize the thymus. This may contribute to the investigation of the role of thymus in immune-mediated diseases. In addition, inflammatory sites in various diseases could be visualized. (orig.). With 6 figs., 1 tab

Altered Markers of Brain Development in Crohn's Disease with Extraintestinal Manifestations - A Pilot Study.

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Anne K Thomann

Full Text Available Alterations of brain morphology in Crohn's disease have been reported, but data is scarce and heterogenous and the possible impact of disease predisposition on brain development is unknown. Assuming a systemic course of the disease, brain involvement seems more probable in presence of extraintestinal manifestations, but this question has not yet been addressed. The present study examined the relationship between Crohn's disease and brain structure and focused on the connection with extraintestinal manifestations and markers of brain development.In a pilot study, brains of 15 patients with Crohn's disease (of which 9 had a history of extraintestinal manifestations, i.e. arthritis, erythema nodosum and primary sclerosing cholangitis were compared to matched healthy controls using high resolution magnetic resonance imaging. Patients and controls were tested for depression, fatigue and global cognitive function. Cortical thickness, surface area and folding were determined via cortical surface modeling.The overall group comparison (i.e. all patients vs. controls yielded no significant results. In the patient subgroup with extraintestinal manifestations, changes in cortical area and folding, but not thickness, were identified: Patients showed elevated cortical surface area in the left middle frontal lobe (p<0.05 and hypergyrification in the left lingual gyrus (p<0.001 compared to healthy controls. Hypogyrification of the right insular cortex (p<0.05 and hypergyrification of the right anterior cingulate cortex (p<0.001 were detected in the subgroup comparison of patients with against without extraintestinal manifestations. P-values are corrected for multiple comparisons.Our findings lend further support to the hypothesis that Crohn's disease is associated with aberrant brain structure and preliminary support for the hypothesis that these changes are associated with a systemic course of the disease as indicated by extraintestinal manifestations. Changes

Subacute Sclerosing Panencephalitis

Science.gov (United States)

... high in developing countries such as India and Eastern Europe. There is a higher incidence among males ... high in developing countries such as India and Eastern Europe. There is a higher incidence among males ...

The clinical efficacy observation of fluoroscopy-guided foam sclerotherapy with lauromacrogol for varicose veins of the lower extremities

International Nuclear Information System (INIS)

Zhu Yongqiang; Xu Jingxuan; Chen Junying; Wu Yan; Zhang Chuanhong; Li Liang

2012-01-01

Objective: To assess the clinical efficacy of foam sclerotherapy with lauromacrogol for varicose veins of the lower extremities. Methods: During the period from February to July 2011, we retrospectively analyzed 20 patients with varicose veins of the lower extremities, who were treated with lauromacrogol foam sclerosing agent injected directly at varicose veins, and in 5 extremities injected at the great saphenous vein (GSV) through a catheter at the same time. Elastic bandages were applied at the site of the injected varicosities after the therapy. The clogging of the varicose veins, the improvement of the clinical symptoms and the adverse reaction were observed. Results: Lauromacrogol foam sclerosing agent was successfully injected under the guidance of fluoroscopy in 20 patients with 28 affected extremities. The average volume of foam sclerosing agent in each affected extremity was 7.8 ml. All patients presented self limiting minor complications, including swelling and pain, which was obviously alleviated by the externally applied Mucopolysaccharide Polysulfate Cream. Two patients presented cough that was relieved after inhaling oxygen. Most of treated varicosities demonstrated pigmentation after the first week, which gradually disappeared after the four-month use of vitamin E capsule. A second foam sclerotherapy was carried out for 3 affected extremities of 3 patients two months after the first therapy. Two cases of leg venous ulcer were healed within a few weeks. All patients could walk immediately with no deep vein thrombosis, pulmonary embolism, anaphylaxis, or transient visual disturbance. Obvious abnormal varicose veins as well as the soreness and fatigue of the lower extremities disappeared in all patients at the 10th-month follow-up. The grading of the disease was significantly improved by the treatment (Z=5.103, P<0.01). Conclusions: The efficacy of lauromacrogol foam sclerosing agent in the treatment of varicose veins of the lower extremities is

A Young Man with Non-alcoholic Steatohepatitis and Serum Anti-mitochondrial Antibody Positivity: A Case Report.

Science.gov (United States)

Seike, Takuya; Komura, Takuya; Shimizu, Yoshiaki; Omura, Hitoshi; Kumai, Tatsuo; Kagaya, Takashi; Ohta, Hajime; Kawashima, Atsuhiro; Harada, Kenichi; Kaneko, Shuichi; Unoura, Masashi

2018-06-06

A 37-year-old obese man who was a social drinker was admitted to our hospital to undergo a detailed examination for liver injury with anti-mitochondrial antibody positivity. Abdominal ultrasonography revealed moderate fatty liver. A histological analysis showed steatosis of approximately 30% of the hepatocytes, focal necrosis, a few ballooning hepatocytes and lobular inflammation suggestive of steatohepatitis, epithelioid granuloma and irregularity of the sequence of the bile duct epithelium accompanied by lymphocyte infiltration suggestive of chronic cholangitis. He was diagnosed with non-alcoholic steatohepatitis complicated with primary biliary cholangitis. His liver injury was improved by weight loss and high-dose ursodeoxycholic acid treatment.

An Unusual Cause of Biliary Obstruction

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Sern Wei Yeoh

2012-01-01

Full Text Available Portal biliary ductopathy (PBD is a condition in which biliary and pancreatic ducts are extrinsically compressed by collateral branches of the portal venous system, which in turn have become dilated and varicosed due to portal hypertension. While the majority of patients with PBD are asymptomatic, a minority can present with symptoms of biliary obstruction and cholangitis with the potential of developing secondary chronic liver disease. This paper reports the case of a 29 year old male presenting with acute cholangitis, in whom PBD was diagnosed radiologically. A brief review of current literature regarding the diagnosis and management of this condition will also be presented.

Telesonography Adaptation and Use to Improve the Standard of Patient Care Within a Dominican Community

Science.gov (United States)

2009-05-06

Ascites; Blunt Abdominal Trauma; Cholelithiasis; Cholecystitis; Cholangitis; Pancreatitis; Hydronephrosis; Abdominal Aortic Aneurysm; Hepatitis; Portal Hypertension; Urolithiasis; Abnormal Uterine Bleeding; Ovarian Mass; Ovarian Torsion

Efficacy of short-term versus long-term chest tube drainage following talc slurry pleurodesis in patients with malignant pleural effusions: a randomised trial.

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Goodman, Anna; Davies, Christopher W H

2006-10-01

Talc pleurodesis is commonly used in the palliative treatment of malignant pleural effusions but the shortest and most effective regime has not been determined. In particular, it is not clear when the intercostal drain should be removed following the insertion of sclerosant. We conducted a single-centre, randomised, open trial of drain removal at 24 h versus 72 h following talc slurry pleurodesis. The primary outcome measure was success of pleurodesis (no recurrence of effusion on chest radiograph at 1-month follow-up) and secondary outcome measures included length of hospital stay and mortality. We found no difference between recurrence of pleural effusion in those randomised to drain removal at 24 h and those randomised to drain removal at 72 h (p>0.5). However, length of stay was significantly reduced when the chest drain was removed at 24 h (4 days versus 8 days; p<0.01). Mortality did not differ between the two groups. We conclude that this shorter pleurodesis regime is safe and effective.

Evaluation of classical and novel autoantibodies for the diagnosis of Primary Biliary Cholangitis-Autoimmune Hepatitis Overlap Syndrome (PBC-AIH OS).

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Nguyen, Henry H; Shaheen, Abdel Aziz; Baeza, Natalia; Lytvyak, Ellina; Urbanski, Stefan J; Mason, Andrew L; Norman, Gary L; Fritzler, Marvin J; Swain, Mark G

2018-01-01

Up to 20% of Primary Biliary Cholangitis (PBC) patients are estimated to have features that overlap with Autoimmune Hepatitis (AIH). Patients with PBC-AIH overlap syndrome (PBC-AIH OS) have been reported to exhibit suboptimal responses to ursodeoxycholic acid therapy, and are more likely to progress to cirrhosis. Anti-double stranded DNA (anti-dsDNA) and anti-p53 have been previously suggested to be potential autoantibodies for identifying patients with PBC-AIH OS. In our well defined PBC patient cohorts, a comprehensive assessment of various classical and novel autoantibodies was evaluated for their utility in identifying PBC-AIH OS patients. PBC-AIH OS was classified according to the Paris criteria and PBC as per the European Association for the Study of the Liver guidelines. Biobanked serum samples from 197 patients at the University of Calgary Liver Unit and the University of Alberta were analyzed for classical and novel autoantibodies. Anti-dsDNA was measured by the Crithidia luciliae immunofluorescence (CLIFT) assay (1:20 dilution) and chemiluminescence (CIA: QUANTA Flash®, Inova Diagnostics, San Diego). Anti-p53, anti-Ro52/TRIM21, anti-YB 1, anti-GW182, anti-Ge-1, and anti-Ago 2 were measured by either an addressable laser bead immunoassay (ALBIA) or line immunoassay (LIA). Autoantibodies against MIT3, gp210, sp100, LKM1, SLA, and the novel autoantibodies Hexokinase-1 (HK-1), and Kelch like protein 12 (KLHL-12) were measured using QUANTA Lite® ELISA assays. We applied non-parametric methods to compare the biomarkers frequencies between study groups. We used multivariate adjusted models and AUROC to compare the diagnostic accuracy of the different autoantibodies alone or in combination with serum biochemistry. 16 out of 197 PBC patients (8.1%) were classified as PBC-AIH OS. Compared to PBC patients, PBC-AIH OS patients were similar in age (median: 59 vs. 63, P = 0.21) and female predominance (94% vs. 89%, P = 1.00). Anti-dsDNA-by CLIFT (37.5% in PBC-AIH OS

Residual intrahepatic stones after percutaneous biliary extraction : longterm follow up of complications

International Nuclear Information System (INIS)

Yoo, Seung Min; Shim, Hyung Jin; Lee, Hwa Yeon; Lim, Sang Jun; Park, Hyo Jin; Kim, Yang Soo; Choi, Young Hee; Kwak, Byung Kuck; Park, Ji Young

1997-01-01

To evaluate and compare the radiologic and clinical follow-up of complications between a group in whom stone removal after percutaneous biliary extraction had been complete, and a group in whom this had been incomplete. Twenty-two patients in whom stone removal had been incomplete, and 20 from whom stones had been completely removed were evaluated with particular attention to complications such as cholangitis, liver abscess, biliary sepsis, and pain. Cholangitis was diagnosed on the basis of typical clinical symptoms such as pain, high fever, jaundice and leukocytosis. Pain without other cholangitic symptoms was excluded. Liver abscess was diagnosed by percutaneous aspiration of pus, and biliary sepsis by bacterial growth on blood culture, or laboratory findings such as increased fibrinogen products, decreased fibrinogen, and increased prothrombin time with cholangitic symptoms. 'Complete removal' means no residual stones on follow-up sonogram and cholangiogram performed within three to seven days after pecutaneous biliary extraction. Mean follow-up period was 26.5 months in the incomplete removal group and 34.2 months in the complete removal group. In twelve of 22 patients (54.5%) in the incomplete removal group, complications occurred, as follows:cholangitis, ten cases (45.5%);liver absces, one (4.5%);biliary sepsis, one (4.5%);and pain, seven(31.8%). In contrast, only two of twenty patients (10%) in the complete removal group suffered complications, all of which involved the recurrence of stones in the common duct, and cholangitis. Complete removal of intrahepatic stones significantly helps to reduce the incidence of possible complications. Even in the case of an impacted stone, aggressive interventional procedures, aimed at complete removal, should be considered. If nonsurgical procedures fail, early partial hepatectomy should be considered, particulary for the stones localized in the left intrahepatic duct

Development of a duodenal gallstone ileus with gastric outlet obstruction (Bouveret syndrome four months after successful treatment of symptomatic gallstone disease with cholecystitis and cholangitis: a case report

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Winnekendonk Guido

2010-11-01

Full Text Available Abstract Introduction Cases of gallstone ileus account for 1% to 4% of all instances of mechanical bowel obstruction. The majority of obstructing gallstones are located in the terminal ileum. Less than 10% of impacted gallstones are located in the duodenum. A gastric outlet obstruction secondary to a gallstone ileus is known as Bouveret syndrome. Gallstones usually enter the bowel through a biliary enteral fistula. Little is known about the formation of such fistulae in the course of gallstone disease. Case presentation We report the case of a 72-year-old Caucasian woman born in Germany with a gastric outlet obstruction due to a gallstone ileus (Bouveret syndrome, with a large gallstone impacted in the third part of the duodenum. Diagnostic investigations of our patient included plain abdominal films, gastroscopy and abdominal computed tomography, which showed a biliary enteric fistula between the gallbladder and the duodenal bulb. Our patient was successfully treated by laparotomy, duodenotomy, extraction of the stone, cholecystectomy, and resection of the fistula in a one-stage surgical approach. Histopathological examination showed chronic and acute cholecystitis, with perforated ulceration of the duodenal wall and acute purulent inflammation of the surrounding fatty tissue. Four months prior to developing a gallstone ileus our patient had been hospitalized for cholecystitis, a large gallstone in the gallbladder, cholangitis and a small obstructing gallstone in the common biliary duct. She had been treated with endoscopic retrograde cholangiopancreatography, endoscopic biliary sphincterotomy, balloon extraction of the common biliary duct gallstone, and intravenous antibiotics. At the time of her first presentation, abdominal ultrasound and endoscopic examination (including esophagogastroduodenoscopy and endoscopic retrograde cholangiopancreatography had not shown any evidence of a biliary enteral fistula. In the four months preceding the

Subacute sclerosing panencephalitis: clinical aspects and prognosis. The Brazilian Registry Panencefalite esclerosante subaguda, aspectos clínicos e prognóstico: Registro Brasileiro

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MAGDA LAHORGUE NUNES

1999-06-01

Full Text Available Subacute sclerosing panencephalitis (SSPE is an inflammatory neurodegenerative disease related to the persistence of measles virus. Although its frequency is declining because of measles eradication, we still have some cases being diagnosed. With the aim to describe epidemiological aspects of SSPE in Brazil, we sent a protocol to Child Neurologists around the country, 48 patients were registered, 27 (56 % were from the southeast region, 34 (71% were male and 35 (73% white, 27 (56% had measles, 9 (19% had measles and were also immunized, 7 (14% received only immunization, 1 patient had a probable neonatal form. Mean time between first symptoms and diagnosis was 12 months (22 started with myoclonus or tonic-clonic seizures, 7 (14% with behavioral disturbances; 36 patients (75% had EEG with pseudoperiodic complexes. Follow up performed in 28 (58 % patients showed: 12 died, 2 had complete remission and the others had variable neurological disability Our data shows endemic regions in the country, a high incidence of post-immunization SSPE and a delay between first symptom and diagnosis.A panencefalite esclerosante subaguda é doença neurodegenerativa inflamatória relacionada à persistência do vírus do sarampo no organismo. Sua incidência vem diminuindo significativamente com a erradicação do sarampo, mas eventualmente alguns casos ainda têm sido diagnosticados. Com o objetivo de descrever aspectos epidemiológicos da panencefalite no Brasil contactamos Neurologistas Infantis de todo país. Foram registrados 48 pacientes, 27% da região sudeste, 34 (70% do sexo masculino, 35 (73% brancos, 9 (19% apresentaram sarampo e receberam imunização, 7 (14% somente imunizados, um paciente apresentou provável forma neonatal. Intervalo médio entre primeiro sintoma e diagnóstico de 12 meses, 22 pacientes (45% iniciaram o quadro com mioclonus ou convulsões tônico-clônicas, 7 (14% com distúrbios comportamentais; 36 (75% apresentaram EEG com

Operable malignant jaundice: To stent or not to stent before the operation?

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Rerknimitr, Rungsun; Kullavanijaya, Pinit

2010-01-16

Traditionally, pre-operative biliary drainage (PBD) was believed to improve multi-organ dysfunction, and for this reason, was practiced worldwide. Over the last decade, this concept was challenged by many reports, including meta-analyses that showed no difference in morbidity and mortality between surgery with, and surgery without PBD, in operable malignant jaundice. The main disadvantages of PBD are seen to be the additional cost of the procedure itself, and the need for longer hospitalization. In addition, many studies showed the significance of specific complications resulting from PBD, such as recurrent jaundice, cholangitis, pancreatitis, cutaneous fistula, and bleeding. However, the results of these studies remain inconclusive as to date there has been no perfect study that equally randomized comparable patients according to the level of obstruction and technique used for PBD. Generally, endoscopic stent insertion (ES) is preferred for common duct obstruction, whereas endoscopic nasobiliary drainage and percutaneous biliary drainage is reserved for hilar obstruction, since ES in hilar block confers a high rate of cholangitis. Although, there is no guideline which either supports or refutes this approach, certain subgroups of patients, including those with symptomatic jaundice, cholangitis, impending renal failure, hilar block requiring preoperative portal vein embolization, and those who need pre-operative neoadjuvant therapy, are suitable candidates for PBD.

[Choledochal cyst during pregnancy. Report of 3 cases and a literature review].

Science.gov (United States)

Martínez-Ordaz, José Luis; Morales-Camacho, Magdely Yazmin; Centellas-Hinojosa, Sócrates; Román-Ramírez, Eduardo; Romero-Hernández, Teodoro; de la Fuente-Lira, Mauricio

2016-01-01

Choledochal cysts are rare. They usually present during childhood in women, but it can also be seen during pregnancy. Clinical signs and symptoms are obscured during this time, thus it can complicate the diagnosis and represent a life threatening complication for both the mother and the child. To communicate the case of 3 pregnant patients with choledochal cyst. Three pregnant women in which choledochal cyst were diagnosed. Two developed signs of cholangitis. The first one underwent a hepatic-jejunostomy, but had an abortion and died on postoperative day 10. The second one had a preterm caesarean operation due to foetal distress and underwent a hepatic-jejunostomy 4 weeks later; during her recovery she had a gastric perforation and died of septic complications. The third one did not develop cholangitis or jaundice. She had an uneventful pregnancy and had a hepatic-jejunostomy 4 weeks later with good results. Management of choledochal cysts during pregnancy is related to the presence of cholangitis. When they do not respond to medical treatment, decompression of the biliary tree is indicated. Definitive treatment should be performed after resolution of the pregnancy. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

A case illustrating successful eradication of recurrent, aggressive basal cell carcinoma located in a scar with vismodegib.

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Lucero, Olivia M; Fitzmaurice, Sarah; Thompson, Curtis; Leitenberge, Justin

2018-02-15

Vismodegib is a small molecule inhibitor of the Hedgehog signaling pathway that has shown efficacy in the control of locally advanced or metastatic basal cell carcinoma, although proof of its effectiveness in the elimination of aggressive tumors is lacking. We report a case and provide complete histological evidence of a 69-year-old gentleman who presented with a recurrent, infiltrative, and sclerosing (morpheiform) basal cell carcinoma on his left upper lip that was entirely eradicated with a three-month course of vismodegib 150 mg daily. Complete histologic clearance of a tumor in a recurrent, infiltrative, and sclerosing basal cell carcinoma with vismodegib is uncommon.

Percutaneous Endoscopic Management for Oriental Cholangiohepatitis: A Case Report and a Brief Review of the Literature

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Khalil Aloreidi

2017-01-01

Full Text Available Oriental cholangiohepatitis (OCH is a disease characterized by intrabiliary pigment stone formation, resulting in recurrent bouts of cholangitis. OCH is found mostly in Southeast Asia but it is occasionally recognized in Western societies. OCH etiology is largely unknown. We report our experience with a patient who presented with acute cholecystitis. Following laparoscopic cholecystectomy, she developed acute cholangitis due to multiple biliary tree stones. She underwent ERCP to clear the stones from common bile duct. For the intrahepatic stones, she underwent novel hybrid percutaneous endoscopic technique. The procedure resulted in complete clearance of biliary tree stones and resolution of her symptoms. The aim of this case is to increase awareness of this disease when patients from endemic areas present with biliary stones.

ATRESIA BILIER

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Julinar Julinar

2009-09-01

, that were a highly suggestive of fetal form of biliary atresia. A boy 58 days infant with fetal biliary atresia. He developed jaundice in 3 weeks of life, pale stool, dark urine, and liver was palpable. Kasai operation was not effective because he had cholangitis complicated and developed liver cirrhosis at five month of age. Fetal EHBA (Extra Hepatic Biliary Atresia with worse outcome after Kasai procedure becauseLAPORAN KASUS189of cholangitis complicated. Early diagnosis EHBA is very important for succesfull of Kasai procedure.Key words: biliary atresia, kasai, Cholangitis, liver cirrhosis

Abdominal ultrasonogram of autoimmune pancreatitis: Five cases of pancreatic lesions accompanied by Sjögren syndrome.

Science.gov (United States)

Yoshizaki, Hideo; Takeuchi, Kazuo; Okuda, Chikao; Honjyo, Hajime; Yamamoto, Takatugu; Kora, Tetuo; Takamori, Yoriyuki

2002-09-01

The concept of autoimmune pancreatitis has recently been established, and ultrasonographic findings we obtained from five cases consistent with autoimmune pancreatitis are reported here. Case 1, a 77-year-old man, was admitted complaining of loss of body weight. Serum hepatobiliary enzymes and γ-globulin levels were elevated, and antinuclear antibody was positive, Abdominal ultrasonography showed dilatation of the intrahepatic bile duct, wall thickening of the common bile duct and hypoechoic swelling of the pancreatic head and body. ERCP revealed multiple stenosis of the intra-and extra-hepatic bile ducts, and diffuse irregular narrowing of the main pancreatic duct. The patient complained of thirst, and the minor salivary gland was examined histologically. Our diagnosis was Sjögren syndrome accompanied by sclerosing cholangitis and a pancreatic lesion. Obstructive jaundice also developed, and PTCD was therefore performed. Both the pancreatic swelling and multiple stenosis of the bile duct improved after steroids were administered. Case 2, a 71-year-old man, was admitted with jaundice. Abdominal ultrasonography showed hypoechoic swelling of the pancreas. ERCP showed stenosis of the common bile duct in the pancreatic head region and diffuse irregular narrowing of the main pancreatic duct. Histological examination of the minor salivary gland suggested Sjögren syndrome. Steroids were therefore administered because the presence of both hyper-γ-globulinemia and positive antinuclear antibody suggested involvement of the autoimmune mechanism. Steroid therapy improved the jaundice as well as the findings from the cholangiograms and pancreatograms. We also encountered three similar cases, all consistent with the concept of autoimmune pancreatitis. The ultrasonographic findings of the pancreatic lesion (1) showed them as homogeneous and markedly hypoechoic areas and, (2) visualized the main pancreatic duct in the lesion, which facilitated a differential diagnosis of the

Risk factors for proximal migration of biliary tube stents.

Science.gov (United States)

Kawaguchi, Yoshiaki; Ogawa, Masami; Kawashima, Yohei; Mizukami, Hajime; Maruno, Atsuko; Ito, Hiroyuki; Mine, Tetsuya

2014-02-07

To analyze the risk factors for biliary stent migration in patients with benign and malignant strictures. Endoscopic stent placement was performed in 396 patients with bile duct stenosis, at our institution, between June 2003 and March 2009. The indications for bile duct stent implantation included common bile duct stone in 190 patients, malignant lesions in 112, chronic pancreatitis in 62, autoimmune pancreatitis in 14, trauma in eight, surgical complications in six, and primary sclerosing cholangitis (PSC) in four. We retrospectively examined the frequency of stent migration, and analyzed the patient factors (disease, whether endoscopic sphincterotomy was performed, location of bile duct stenosis and diameter of the bile duct) and stent characteristics (duration of stent placement, stent type, diameter and length). Moreover, we investigated retrieval methods for migrated stents and their associated success rates. The frequency of tube stent migration in the total patient population was 3.5%. The cases in which tube stent migration occurred included those with common bile duct stones (3/190; 1.6%), malignant lesions (2/112; 1.8%), chronic pancreatitis (4/62; 6.5%), autoimmune pancreatitis (2/14; 14.3%), trauma (1/8; 12.5%), surgical complications (2/6; 33.3%), and PSC (0/4; 0%). The potential risk factors for migration included bile duct stenosis secondary to benign disease such as chronic pancreatitis and autoimmune pancreatitis (P = 0.030); stenosis of the lower bile duct (P = 0.031); bile duct diameter > 10 mm (P = 0.023); duration of stent placement > 1 mo (P = 0.007); use of straight-type stents (P stents (P stents was successful in all cases. The grasping technique, using a basket or snare, was effective for pig-tailed or thin and straight stents, whereas the guidewire cannulation technique was effective for thick and straight stents. Migration of tube stents within the bile duct is rare but possible, and it is important to determine the risk factors

Serum Reactivity Against Bacterial Pyruvate Dehydrogenase: Increasing the Specificity of Anti-Mitochondrial Antibodies for the Diagnosis of Primary Biliary Cirrhosis

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Hiroshi Miyakawa

2006-01-01

Full Text Available Antimitochondrial antibodies (AMA are the serum hallmark of primary biliary cirrhosis (PBC. However, AMA-positivity can be found in non-PBC sera when lower dilutions are used, thus raising issues about the specificity and sensitivity of the test. AMA reacts primarily with the lipoylated domains of pyruvate dehydrogenase-E2 (PDC-E2 which is highly conserved across species, including bacteria. We studied 77 serum samples, including 24 from patients with anti-PDC-E2-positive PBC and 53 controls (16 with autoimmune hepatitis (AIH, 10 with primary sclerosing cholangitis (PSC, and 27 healthy individuals for their reactivities at serial dilutions (1:10, 1:20 and 1:40 against Escherichia coli DH5 alpha lysate overexpressing human PDC-E2 using immunoblotting (IB. A murine anti-human PDC-E2 monoclonal antibody (mAB was used as control. We further studied positive sera using adsorption with a synthetic E. coli peptide sharing similarity with human PDC-E2. Finally, we verified whether a unique buffer for E. coli preparation could reduce non-specific serum reactivity. Results demonstrated that 100% of anti-PDC-E2-positive PBC and up to 38% of control sera at 1:10 dilution recognized E. coli PDC-E2 at IB while dilution tests indicated that the overall potency of PBC reactivity was 100-fold higher compared to controls. In fact, a subgroup (20-38% of non-PBC sera were positive at low titers but lost the reactivity when absorbed with the synthetic E. coli peptide. Finally, our unique buffer reduced the reactivity of non-PBC sera as measured by ELISA. In conclusion, we demonstrated that weak cross-reactivity with E. coli PDC-E2 occurs in non-PBC sera at lower dilutions and that such reactivity is not due to AMA-positivity. The use of a specific buffer might avoid the risk of false positive AMA determinations when E. coli-expressed recombinant antigens are used.

Thiopurine Therapy Reduces the Incidence of Colorectal Neoplasia in Patients with Ulcerative Colitis. Data from the ENEIDA Registry.

Science.gov (United States)

Gordillo, Jordi; Cabré, Eduard; Garcia-Planella, Esther; Ricart, Elena; Ber-Nieto, Yolanda; Márquez, Lucía; Rodríguez-Moranta, Francisco; Ponferrada, Ángel; Vera, Isabel; Gisbert, Javier P; Barrio, Jesús; Esteve, Maria; Merino, Olga; Muñoz, Fernando; Domènech, Eugeni

2015-12-01

Patients with ulcerative colitis (UC) are at increased risk of developing colorectal cancer (CRC), but recent studies suggest a lower risk than previously reported. The aim was to evaluate the incidence of dysplasia, CRC and related risk factors in UC patients from a Spanish nationwide database. All UC patients were identified and retrospectively reviewed. Clinical-epidemiological data and the finding of dysplasia and/or CRC were collected. A total of 831 UC patients were included. Twenty-six cases of CRC in 26 patients and 29 cases of high-grade dysplasia (HGD) in 24 patients were found, accounting for 55 diagnoses of advanced neoplasia (AN = CRC and/or HGD) in 45 patients (33% of them within the first 8 years after UC diagnosis). The cumulative risk of AN was 2, 5.3 and 14.7% at 10, 20 and 30 years, respectively. Concomitant primary sclerosing cholangitis (odds ratio [OR] 10.90; 95% confidence interval [CI] 3.75-31.76, p < 0.001), extensive UC (OR 2.10, 95% CI 1.01-4.38, p = 0.048), UC diagnosis at an older age (OR 2.23, 95% CI 1.03-4.83, p = 0.043) and appendectomy prior to UC diagnosis (OR 2.66, 95% CI 1.06-6.71, p = 0.038) were independent risk factors for AN. Use of thiopurines (OR 0.21, 95% CI 0.06-0.74, p = 0.015) and being in a surveillance colonoscopy programme (OR 0.33; 95% CI 0.16-0.67; p = 0.002) were independent protective factors for AN. The risk of AN among UC patients is lower than previously reported but steadily increases from the time of UC diagnosis. The widespread use of thiopurines may have influenced this reduced incidence of UC-related neoplasias. 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.

Management of Bleeding Duodenal Varices with Combined TIPS Decompression and Trans-TIPS Transvenous Obliteration Utilizing 3% Sodium Tetradecyl Sulfate Foam Sclerosis.

Science.gov (United States)

Saad, Wael E; Lippert, Allison; Schwaner, Sandra; Al-Osaimi, Abdullah; Sabri, Saher; Saad, Nael

2014-01-01

Endoscopic experience in the management of duodenal varices (DVs) is limited and challenging given the anatomic constraints and limited experience. The endovascular management of DVs is not yet established and the controversy of whether to manage them by decompression with a transjugular intrahepatic portosystemic shunt (TIPS) or by transvenous obliteration is unresolved. In the literature, the 6-12 month rebleeding rate of DVs after TIPS is 21-37% and after transvenous obliteration is 13%. The purpose of the study is to evaluate the clinical outcome of combined TIPS decompression and transvenous obliteration/sclerosis. This is a retrospective study (case series) of two institutions, evaluating patients who underwent TIPS and/or transvenous obliteration/sclerosis for bleeding DVs (from January 2009 to June 2013). TIPS was performed according to a standard procedure using covered stents. Transvenous obliteration (variceal sclerosis) from the systemic and/or portal venous circulation was performed utilizing 3% sodium tetradecyl sulfate foam. Transvenous obliteration was commonly augmented with coils and/or vascular plugs. Technical (technical success of establishing TIPS and completely obliterating the DVs) and clinical outcomes (rebleeding rate and survival) were evaluated. Five patients with liver cirrhosis presenting with bleeding DVs were included in the study with all eventually (and coincidentally) receiving TIPS and transvenous obliteration. Two of the five patients underwent concomitant TIPS and transvenous obliteration in the same procedural setting. However, three patients underwent transvenous obliteration due to bleeding despite a patent TIPS that had been previously placed. The average time from TIPS placement to transvenous obliteration was 125 days (range: 3-324 days). After having both procedures, there was no rebleeding in the patients during a mean follow-up period of 22 months (6-50 months). Coils and/or metallic vascular plugs were used to augment

Management of stomal varices with transvenous obliteration utilizing sodium tetradecyl sulfate foam sclerosis.

Science.gov (United States)

Saad, Wael E A; Schwaner, Sandra; Lippert, Allison; Sabri, Saher S; Al-Osaimi, Abdullah; Matsumoto, Alan H; Angle, John F; Caldwell, Stephen

2014-12-01

The management of parastomal varices is not established. Transjugular intrahepatic portosystemic shunt (TIPS) creation is the most commonly described treatment; however, the rebleed rate after TIPS is 21-37%. The purpose of the study is to determine the effectiveness of transvenous obliteration using sodium tetradecyl sulfate (STS) and to describe a new simplified technique in obliterating these varices. Four patients are presented who underwent transvenous obliteration using STS. One was obliterated using balloon occlusion from the systemic veins, the second was obliterated without balloon from a transhepatic antegrade approach, and the last two patients were obliterated using the direct antegrade technique. This simplified technique requires only a micropuncture kit (not requiring balloons or coils) and ultrasound transducer compression of the systemic draining veins, relying on high portal pressure to keep the sclerosant confined to the varices. The sclerosant is essentially trapped between the portal pressure and the ultrasound-transducer compression (10-15 min). Technical success was achieved in all four patients without procedural or postprocedural complications and no rebleeding for a mean follow-up of 17 (range 2-33) months. Transvenous obliteration of parastomal varices utilizing STS as a sclerosant is safe and effective. The newly described technique is simple, feasible, and requires minimal equipment (no balloons or coils or catheters).

Cue-elicited food seeking is eliminated with aversive outcomes following outcome devaluation.

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Eder, Andreas B; Dignath, David

2016-01-01

In outcome-selective Pavlovian-to-instrumental transfer (PIT), stimuli that are predictive of specific outcomes prime instrumental responses that are associated with these outcomes. Previous human studies yielded mixed evidence in respect to whether the PIT effect is affected by a posttraining devaluation of an outcome, with the PIT effect being preserved after a devaluation of a primary reinforcer (food, drugs) but not following the devaluation of a secondary reinforcer (money). The present research examined whether outcome-selective transfer is eliminated when the devaluation of a primary (liquid) reinforcer is strong and aversive. Experiment 1 confirmed these expectations following a devaluation with bad tasting Tween 20. However, outcome-selective transfer was still observed when the earned (devalued) outcome was not consumed immediately after each test (Experiment 2). These results suggest that the capacity of a Pavlovian cue to motivate a specific response is affected by the incentive value of the shared outcome only when the devaluation yields an aversive outcome that is consumed immediately.

A Rare Case Report of Spontaneous Resolution of Hepatic Portal ...

African Journals Online (AJOL)

Hepatic portal venous gas (HPVG) was first described ... suppurative cholangitis, peptic ulcer disease, volvulus and fecal impaction.[1-4] The prognosis is related to the underlying pathology and the coexistence of a chronic medical disease,.

Autoimmune liver disease panel

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Liver disease test panel - autoimmune ... Autoimmune disorders are a possible cause of liver disease. The most common of these diseases are autoimmune hepatitis and primary biliary cholangitis (formerly called primary biliary cirrhosis). This group of tests ...

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 ...

Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

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... National Institutes of Health (NIH) support and conduct research into many diseases and conditions. View clinical trials that are currently recruiting volunteers . Next: Definition & Facts This content is provided as a service ...

Sclerotherapy for Reticular Veins in the Lower Limbs: A Triple-Blind Randomized Clinical Trial.

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Bertanha, Matheus; Jaldin, Rodrigo Gibin; Moura, Regina; Pimenta, Rafael Elias Farres; Mariúba, Jamil Victor de Oliveira; Lúcio Filho, Carlos Eduardo Pinheiro; Alcantara, Giovana Piteri; Padovani, Carlos Roberto; Yoshida, Winston Bonetti; Sobreira, Marcone Lima

2017-12-01

Reticular veins are subdermal veins located in the lower limbs and are mainly associated with aesthetic complaints. Although sclerotherapy is the treatment of choice for reticular veins in the lower limbs, no consensus has been reached regarding to the optimal sclerosant. To compare the efficacy and safety of 2 sclerosants used to treat reticular veins: 0.2% polidocanol diluted in 70% hypertonic glucose (HG) (group 1) vs 75% HG alone (group 2). Prospective, randomized, triple-blind, controlled, parallel-group clinical trial with patients randomly assigned in a 1:1 ratio between the 2 treatment groups from March through December 2014, with 2 months' follow-up. The study was conducted in a single academic medical center. Eligible participants were all women, aged 18 to 69 years, who had at least 1 reticular vein with a minimum length of 10 cm in 1 of their lower limbs. The patients underwent sclerotherapy in a single intervention with either 0.2% polidocanol plus 70% HG or 75% HG alone to eliminate reticular veins. The primary efficacy end point was the disappearance of the reticular veins within 60 days after treatment with sclerotherapy. The reticular veins were measured on images obtained before treatment and after treatment using ImageJ software. Safety outcomes were analyzed immediately after treatment and 7 days and 60 days after treatment and included serious adverse events (eg, deep vein thrombosis and systemic complications) and minor adverse events (eg, pigmentation, edema, telangiectatic matting, and hematomas). Ninety-three women completed the study, median (interquartile range) age 43.0 (24.0-61.0) years for group 1 and 41.0 (27.0-62.0) years for group 2. Sclerotherapy with 0.2% polidocanol plus 70% HG was significantly more effective than with 75% HG alone in eliminating reticular veins from the treated area (95.17% vs 85.40%; P vein pigmentation length for group 1 and 7.09% for group 2, with no significant difference between the groups (P = .09

Seladelpar (MBX-8025), a selective PPAR-δ agonist, in patients with primary biliary cholangitis with an inadequate response to ursodeoxycholic acid: a double-blind, randomised, placebo-controlled, phase 2, proof-of-concept study.

Science.gov (United States)

Jones, David; Boudes, Pol F; Swain, Mark G; Bowlus, Christopher L; Galambos, Michael R; Bacon, Bruce R; Doerffel, Yvonne; Gitlin, Norman; Gordon, Stuart C; Odin, Joseph A; Sheridan, David; Wörns, Markus-Alexander; Clark, Virginia; Corless, Linsey; Hartmann, Heinz; Jonas, Mark E; Kremer, Andreas E; Mells, George F; Buggisch, Peter; Freilich, Bradley L; Levy, Cynthia; Vierling, John M; Bernstein, David E; Hartleb, Marek; Janczewska, Ewa; Rochling, Fedja; Shah, Hemant; Shiffman, Mitchell L; Smith, John H; Choi, Yun-Jung; Steinberg, Alexandra; Varga, Monika; Chera, Harinder; Martin, Robert; McWherter, Charles A; Hirschfield, Gideon M

2017-10-01

Many patients with primary biliary cholangitis have an inadequate response to first-line therapy with ursodeoxycholic acid. Seladelpar is a potent, selective agonist for the peroxisome proliferator-activated receptor-delta (PPAR-δ), which is implicated in bile acid homoeostasis. This first-in-class study evaluated the anti-cholestatic effects and safety of seladelpar in patients with an inadequate response to ursodeoxycholic acid. The study was a 12-week, double-blind, placebo-controlled, phase 2 trial of patients with alkaline phosphatase of at least 1·67 times the upper limit of normal (ULN) despite treatment with ursodeoxycholic acid. Patients, recruited at 29 sites in North America and Europe, were randomly assigned to placebo, seladelpar 50 mg/day, or seladelpar 200 mg/day while ursodeoxycholic acid was continued. Randomisation was done centrally (1:1:1) by a computerised system using an interactive voice-web response system with a block size of three. Randomisation was stratified by region (North America and Europe). The primary outcome was the percentage change from baseline in alkaline phosphatase over 12 weeks, analysed in the modified intention-to-treat (ITT) population (any randomised patient who received at least one dose of medication and had at least one post-baseline alkaline phosphatase evaluation). This study is registered with ClinicalTrials.gov (NCT02609048) and the EU Clinical Trials Registry (EudraCT2015-002698-39). Between Nov 4, 2015, and May 26, 2016, 70 patients were screened at 29 sites in North America and Europe. During recruitment, three patients treated with seladelpar developed fully reversible, asymptomatic grade 3 alanine aminotransferase increases (one on 50 mg, two on 200 mg), ranging from just over five to 20 times the ULN; as a result, the study was terminated after 41 patients were randomly assigned. The modified ITT population consisted of 12 patients in the placebo group, 13 in the seladelpar 50 mg group, and 10 in the

Evaluation of clinical value of serum CA19-9

International Nuclear Information System (INIS)

Lv Haifeng; Lin Zhiyu; Lu Xiaozhuo; Chen Yini

2003-01-01

The article is to study the clinical significance of serum CA19-9 in diagnosing malignant tumor occurred in digestive system and to select cut off values for differentiating diagnosis of a pancreatic neoplasms and pancreatitis. Using chemiluminescence immunoassay, serum CA19-9 level of below subjects were analyzed: control group (n=21); digestive system neoplasm group (n=125, with 7 cases conformed as pancreatic cancer); non-neoplastic disease group (n=387, with 15 cases conformed as pancreatitis secondary to destructive cholangitis). Receiver operating characteristic (ROC) curve was used for analyzing results and selecting cut off values. When cut off value was 18.4 kU/L, sensibility for conforming a digestive system neoplasm was 60.8%, while its related specificity against control group and non-neoplastic disease group was 95. 2% and 68.2%, respectively. Sensibility for diagnosing pancreatic neoplasm was 85.7%, while its related specificity against control group and non-pancreatic origin tumor group was 95.0% and 63.1%, respectively. When 37 kU/L was chosen as cut off value, specificity for differentiating diagnosis of pancreatic neoplasm and pancreatitis secondary to destructive cholangitis rose from 13.3% to 46.7%. Serum CA19-9 could be used as an aid in detecting digestive system neoplastic disease; it is a reliable marker for pancreatic neoplasm. Raising cut off value may help to differentiate pancreatic neoplasm and pancreatitis secondary to destructive cholangitis

Infiltration of peritumoural but tumour-free parenchyma with IgG4-positive plasma cells in hilar cholangiocarcinoma and pancreatic adenocarcinoma.

Science.gov (United States)

Resheq, Yazid J; Quaas, Alexander; von Renteln, Daniel; Schramm, Christoph; Lohse, Ansgar W; Lüth, Stefan

2013-10-01

Recently, new guidelines for diagnosing IgG4-associated cholangitis have been published devaluing the diagnostic significance of IgG4-positive plasma cells and steroid trials. We sought to evaluate the utility of IgG4-positive plasma cells in discriminating IgG4-associated cholangitis from hilar cholangiocarcinoma and autoimmune pancreatitis from pancreatic adenocarcinoma under conditions when malignancy is likely to be missed. Resection specimens obtained from patients with hilar cholangiocarcinoma, pancreatic adenocarcinoma or hepatocellular carcinoma were re-evaluated for IgG4-positivity. Histological analysis focussed on peritumoural but tumour-free sections. Perioperative biochemical and clinical data were reviewed. Nineteen patients with hilar cholangiocarcinoma and 29 patients with pancreatic adenocarcinoma were eligible for histological re-evaluation. Six of 19 (32%) patients with hilar cholangiocarcinoma and 5 of 29 (17%) patients with pancreatic adenocarcinoma were IgG4-positive (≥20 IgG4-positive plasma cells per high power field). Patients with IgG4-positive hilar cholangiocarcinoma showed significantly higher levels of serum total bilirubin (3.6mg/dl vs. 1.8mg/dl; Philar cholangiocarcinoma. IgG4-positive plasma cells are of limited utility especially in distinguishing hilar cholangiocarcinoma from IgG4-associated cholangitis even when combined with clinical parameters and may be misleading under conditions when malignancy is missed. Copyright © 2013 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Biliary Tract Disorders, Gallbladder Disorders, and Gallstone Pancreatitis

Science.gov (United States)

... cholangitis may also occur. Figure 1: Anatomy of liver, bile duct, pancreas duct and sphincter of Oddi. Note that ... This test uses sound waves to examine the bile ducts, liver and pancreas. It is not invasive and is ...

Learning Outcomes Report

NARCIS (Netherlands)

Stoyanov, Slavi; Spoelstra, Howard; Burgoyne, Louise; O’Tuathaigh, Colm

2018-01-01

Aim of the study The learning outcomes study, conducted as part of WP3 of the BioApp project, has as objectives: (a) generating a comprehensive list of the learning outcomes; (b) reaching an agreement on the scope and priority of the learning outcomes, and (c) making suggestions for the further

Rare presentation of pancreatic schwannoma: a case report

Directory of Open Access Journals (Sweden)

Tofigh Arash

2008-08-01

Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.

A Rare Reason of Ileus in Renal Transplant Patients With Peritoneal Dialysis History: Encapsulated Peritoneal Sclerosis.

Science.gov (United States)

Gökçe, Ali Murat; Özel, Leyla; İbişoğlu, Sevinç; Ata, Pınar; Şahin, Gülizar; Gücün, Murat; Kara, V Melih; Özdemir, Ebru; Titiz, M İzzet

2015-12-01

Encapsulating peritoneal sclerosis is a rare complication of long-term peritoneal dialysis ranging from moderate inflammation of peritoneal structures to severe sclerosing peritonitis and encapsulating peritoneal sclerosis. Complicated it, ileus may occur during or after peritoneal dialysis treatment or after kidney transplant. We sought to evaluate 3 posttransplant encapsulating peritoneal sclerosis through clinical presentation, radiologic findings, and outcomes. We analyzed 3 renal transplant patients with symptoms of encapsulating peritoneal sclerosis admitted posttransplant to our hospital with ileus between 2012 and 2013. Conservative treatment was applied to the patients whenever necessary to avoid surgery. One patient improved with medical therapy. Surgical treatment was delayed and we decided it as a last resort, in 2 cases with no response to conservative treatment for a long time. Finally, patients with peritoneal dialysis history should be searched carefully before renal transplant for intermittent bowel obstruction story.

Sclerosis and the Nd:YAG, Q-switched laser with multiple frequency for treatment of telangiectases, reticular veins, and residual pigmentation.

Science.gov (United States)

Cisneros, J L; Del Rio, R; Palou, J

1998-10-01

The combination of low concentrations of sclerosing solution and the Nd:YAG, Q-switched laser with multiple (quadruple) frequency provides good results in the treatment of telangiectases and reticular varicose veins of the lower extremities, as well as pigmentation that may appear during sclerotherapy. This paper is based on a series of patients with telangiectases and reticular veins who were treated with sclerotherapy and the Nd:YAG, Q-switched laser with quadruple frequency. Patients with telangiectases and reticular veins received two or three treatment sessions with polydocanol and the Nd:YAG, Q-switched laser with quadruple frequency. Then, they were assessed a clinical score corresponding to the level of improvement achieved. Residual hematic pigmentation lesions were also eliminated with the laser. Excellent improvement was evident in 90% of the patients with minimal residual lesions. The combined technique of sclerosing solution and the Nd:YAG laser with multiple frequency is a valid alternative for the elimination of telangiectases and reticular veins of the lower limbs. This technique has several advantages, such as the use of low concentrations of sclerosing solution, high patient acceptance levels due to minimal disturbances, and the fact that local anesthesia is unnecessary. Good results are obtained without complications and minimal residual pigmentation. These mild pigmentation can be treated with the Nd:YAG laser.

Physiochemical properties and reproducibility of air-based sodium tetradecyl sulphate foam using the Tessari method.

Science.gov (United States)

Watkins, Mike R; Oliver, Richard J

2017-07-01

Objectives The objectives were to examine the density, bubble size distribution and durability of sodium tetradecyl sulphate foam and the consistency of production of foam by a number of different operators using the Tessari method. Methods 1% and 3% sodium tetradecyl sulphate sclerosant foam was produced by an experienced operator and a group of inexperienced operators using either a 1:3 or 1:4 liquid:air ratio and the Tessari method. The foam density, bubble size distribution and foam durability were measured on freshly prepared foam from each operator. Results The foam density measurements were similar for each of the 1:3 preparations and for each of the 1:4 preparations but not affected by the sclerosant concentration. The bubble size for all preparations were very small immediately after preparation but progressively coalesced to become a micro-foam (foams developed liquid more rapidly when made in a 1:3 ratio (37 s) than in a 1:4 ratio (45 s) but all combinations took similar times to reach 0.4 ml liquid formation. For all the experiments, there was no statistical significant difference between operators. Conclusions The Tessari method of foam production for sodium tetradecyl sulphate sclerosant is consistent and reproducible even when made by inexperienced operators. The best quality foam with micro bubbles should be used within the first minute after production.

Long-term follow-up sonography of benign cystic thyroid nodules after a percutaneous ethanol injection: the incidence of malignancy-mimicking nodules

International Nuclear Information System (INIS)

Park, Ji Sung; Kim, Dong Wook; Eun, Choong Ki; Choi, Seok Jin; Rho, Myung Ho

2008-01-01

To evaluate the incidence of malignancy-mimicking sclerosed thyroid nodules, from long-term follow-up ultrasonography (US) after an US-guided percutaneous ethanol injection (PEI). We examined 86 benign cystic thyroid nodules from 80 patients. The nodules were classified into two groups based on whether an aspiration (Group A, n = 26) or non-aspiration (Group B, n 60) of infused ethanol was performed. The final follow-up US over 12 months was performed in all patients. Of the 86 nodules, the cystic portion of 82 (95.3%) cases, from 76 patients, completely disappeared subsequent to the first follow-up US (Group A, n = 24, Group B, n = 58) (Chi-square test, ρ > 0.05). Moreover, 46 sclerosed thyroid nodules showed two or more of the 'five sonographic criteria' upon a follow-up US (Group A, n = 13, 50.0%, Group B, n = 32, 53.3%). A higher ratio of the cystic portion of the nodules was associated with a higher incidence of the 'five sonographic criteria' for malignancies detected via a follow-up US (ρ < 0.01; Student's t-test). For the long-term follow-up US, the 'five sonographic criteria' were observed in half the patients who received US-guided PEI. Also, by acknowledging the possibility that sonographic findings mimic a malignancy, since the sclerosed thyroid nodule, patients may avoid an unnecessary biopsy

Alternative outcomes create biased expectations regarding the received outcome: Evidence from event-related potentials.

Science.gov (United States)

Marciano, Déborah; Bentin, Shlomo; Deouell, Leon Y

2018-05-01

After choosing between uncertain options, one might get feedback on both the outcome of the chosen option and the outcome of the unchosen option (the alternative). Behavioral research has shown that in such cases people engage in outcome comparison, and that the alternative outcome influences the way one evaluates his own received outcome. Moreover, this influence differs whether one was responsible or not for the choice made. In two studies, we looked for the electrophysiological correlates of outcome comparison. Subjects chose one of two boxes shown on the screen, each box contained a gain or a loss. The alternative outcome was always revealed first, followed by the received outcome. In half of the trials the software picked one box instead of subjects. We tested whether the feedback-related negativity (FRN) and the P3 elicited by the received reflect outcome comparison. As expected, we found that the FRN and P3 were more positive when the received outcome was a gain (vs. a loss). The FRN and P3 were also sensitive to the value of the alternative outcome, but contrary to our predictions, they were more positive when the alternative outcome was a gain (vs. a loss). As the FRN and P3 are sensitive to expectations, we hypothesized that our findings might result from subjects' biased expectations: subjects might have wrongly believed that a good (bad) alternative outcome signaled a bad (good) received outcome. This hypothesis, coined as the Alternative Omen Effect, was confirmed in parallel in a series of behavioral experiments: people see an illusory negative correlation between the uncorrelated outcomes of choice options (reported in Marciano-Romm et al. (2016)). A challenge for future research will be to disentangle the effects of expectation from those of outcome comparison. Copyright © 2018 Elsevier Ltd. All rights reserved.

Biliær papillomatose er en sjælden årsag til intermitterende obstruktiv ikterus

DEFF Research Database (Denmark)

Veedfald, Simon; Vainer, Ben; Wettergren, André

2011-01-01

Over a five-year period a 67 year-old male had been experiencing recurring bouts of biliary obstruction with occasional superimposed cholangitis. Renewed endoscopic retrograde cholangiopancreatography revealed amorphous filling defects and excessive mucinous discharge from the papilla of Vater. S...