The Experts below are selected from a list of 756 Experts worldwide ranked by ideXlab platform
Keith D. Lindor - One of the best experts on this subject based on the ideXlab platform.
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Secondary Sclerosing Cholangitis: pathogenesis, diagnosis, and management.
Clinics in liver disease, 2012Co-Authors: Mohamad Imam, Jayant A. Talwalkar, Keith D. LindorAbstract:Secondary Sclerosing Cholangitis (SSC) is an aggressive and rare disease with intricate pathogenesis and multiple causes. Understanding the specific cause underlying each case of SSC is crucial in the clinical management of the disease. Radiologic imaging can help diagnose SSC and hence institute management in a timely manner. Management may encompass simple interventions, such as supportive therapy, antibiotics, and monitoring, or more serious measures, such as surgery, endoscopic intervention, or liver transplantation. Patients with AIDS cholangiopathy have limited therapeutic options and worsened survival. The disease should always be highly suspected in patients with primary Sclerosing Cholangitis with questionable diagnosis.
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Cholangiocarcinoma: expanding the spectrum of risk factors.
Journal of gastrointestinal cancer, 2008Co-Authors: Diaa H Elfaki, Andrea A. Gossard, Keith D. LindorAbstract:Cholangiocarcinoma (CCA) is an epithelial cancer originating from the bile ducts with features of cholangiocyte differentiation. The global incidence of this rare disease is on the rise, and it is the second most common primary hepatobiliary malignancy. We reviewed risk factors for cholangiocarcinoma development and also described potential screening strategies for this malignancy. We also report two cases in which CCA developed in patients without previously determined risk factors known for CCA. The first case is a patient with longstanding Secondary Sclerosing Cholangitis (SSC), and the second is a patient with autoimmune hepatitis (AIH). These two cases may indicate a possible association between SSC and AIH with CCA, thus, expanding the spectrum of risk factors of CCA.
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Secondary Sclerosing Cholangitis: a comparison to primary Sclerosing Cholangitis.
The American journal of gastroenterology, 2005Co-Authors: Andrea A. Gossard, Paul Angulo, Keith D. LindorAbstract:OBJECTIVES: The natural history of Secondary Sclerosing Cholangitis (SSC) is Ill-defined. In order to better determine the natural history of this condition, we retrospectively reviewed data from the Mayo Clinic in Rochester, Minnesota. We also compared the natural history of patients diagnosed with SSC to a cohort with a diagnosis of primary Sclerosing Cholangitis (PSC). METHODS: We used a computer-assisted search to identify patients with a diagnosis of SSC seen from 1992 to 2002. The diagnosis was confirmed by chart review and information about age, gender, etiology, therapy, and clinical course was sought. We excluded those presumed SSC patients who had a history of inflammatory bowel disease, those with malignancy at the time of diagnosis, and those who had undergone liver transplantation prior to the diagnosis of SSC. Patients with PSC matched for age, gender, and serum bilirubin level served as disease controls. RESULTS: We identified 31 patients, average age 57, (range 28-79). The causes of SSC included surgical trauma from cholecystectomy (13 patients), intraductal stones (12 patients), recurrent pancreatitis (4 patients), and abdominal injury (2 patients). Nine patients with SSC ultimately required liver transplantation and 4 patients have died. When compared to matched patients with PSC, the survival free of transplant was significantly shortened (p < 0.03). CONCLUSIONS: When the long-term outcome of SSC patients was compared to matched PSC controls, the SSC patients had a poorer outcome. The natural history of SSC is characterized by a shortened life expectancy.
U Seitz - One of the best experts on this subject based on the ideXlab platform.
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Magnetic resonance cholangiopancreatography in the diagnosis of primary Sclerosing Cholangitis.
Endoscopy, 2008Co-Authors: C Weber, R Kuhlencordt, R Grotelueschen, U Wedegaertner, T L Ang, G Adam, N Soehendra, U SeitzAbstract:Magnetic resonance cholangiopancreatography (MRCP) is a less-invasive alternative to endoscopic retrograde cholangiopancreatography (ERCP) for the diagnosis of primary Sclerosing Cholangitis (PSC). This study evaluated the diagnostic accuracy of MRCP in PSC compared with ERCP, and assessed the diagnostic accuracy of different T2w sequences. 95 patients (69 PSC, 26 controls) were evaluated using both ERCP and MRCP. Exclusion criteria included Secondary Sclerosing Cholangitis and contraindications to MRCP. The diagnosis of PSC was confirmed in 69 patients based on ERCP as the reference gold standard. MRCP was performed using a 1.5 Tesla MR unit, using breath hold, coronal and transverse half-Fourier acquisition single-shot turbo spin-echo (HASTE), coronal-oblique, fat-suppressed half-Fourier rapid acquisition with relaxation enhancement (RARE), and coronal-oblique, fat-suppressed, multisection, thin-section HASTE (TS-HASTE) sequences. The MRCP morphological criteria of PSC were evaluated and compared with ERCP. The sensitivity, specificity, and diagnostic accuracy were 86%, 77%, and 83%, respectively, using the MRCP-RARE sequence, and increased further to 93%, 77%, and 88%, respectively, by the inclusion of follow-up MRCP in 52 patients, performed at 6-12-month intervals. HASTE and TS-HASTE sequences showed significantly lower diagnostic accuracy but provided additional morphologic information. MRCP can diagnose PSC but has difficulties in early PSC and in cirrhosis, and in the differentiation of cholangiocarcinoma, Caroli's disease, and Secondary Sclerosing Cholangitis. A positive MRCP would negate some diagnostic ERCP studies but a negative MRCP would not obviate the need for ERCP.
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Magnetic resonance cholangiopancreatography in the diagnosis of primary Sclerosing Cholangitis
Endoscopy, 2008Co-Authors: C Weber, R Kuhlencordt, R Grotelueschen, U Wedegaertner, T L Ang, G Adam, N Soehendra, U SeitzAbstract:BACKGROUND AND STUDY AIMS: Magnetic resonance cholangiopancreatography (MRCP) is a less-invasive alternative to endoscopic retrograde cholangiopancreatography (ERCP) for the diagnosis of primary Sclerosing Cholangitis (PSC). This study evaluated the diagnostic accuracy of MRCP in PSC compared with ERCP, and assessed the diagnostic accuracy of different T2w sequences. PATIENTS AND METHODS: 95 patients (69 PSC, 26 controls) were evaluated using both ERCP and MRCP. Exclusion criteria included Secondary Sclerosing Cholangitis and contraindications to MRCP. The diagnosis of PSC was confirmed in 69 patients based on ERCP as the reference gold standard. MRCP was performed using a 1.5 Tesla MR unit, using breath hold, coronal and transverse half-Fourier acquisition single-shot turbo spin-echo (HASTE), coronal-oblique, fat-suppressed half-Fourier rapid acquisition with relaxation enhancement (RARE), and coronal-oblique, fat-suppressed, multisection, thin-section HASTE (TS-HASTE) sequences. The MRCP morphological criteria of PSC were evaluated and compared with ERCP. RESULTS: The sensitivity, specificity, and diagnostic accuracy were 86 %, 77 %, and 83 %, respectively, using the MRCP-RARE sequence, and increased further to 93 %, 77 %, and 88 %, respectively, by the inclusion of follow-up MRCP in 52 patients, performed at 6 - 12-month intervals. HASTE and TS-HASTE sequences showed significantly lower diagnostic accuracy but provided additional morphologic information. CONCLUSIONS: MRCP can diagnose PSC but has difficulties in early PSC and in cirrhosis, and in the differentiation of cholangiocarcinoma, Caroli’s disease, and Secondary Sclerosing Cholangitis. A positive MRCP would negate some diagnostic ERCP studies but a negative MRCP would not obviate the need for ERCP.
Mayura Kekan - One of the best experts on this subject based on the ideXlab platform.
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Secondary Sclerosing Cholangitis in localized hepatobiliary tuberculosis simulating cholangiocarcinoma: a rare case report.
BMC gastroenterology, 2017Co-Authors: Aleena Jain, Rachana Chaturvedi, Chetan Kantharia, Amita Joshi, Mangesh Londhe, Mayura KekanAbstract:Background Hepatobiliary tuberculosis includes miliary, tuberculous hepatitis or localized forms. The localised form is extremely uncommon and can mimic malignancy. Still rarer is its presentation as Sclerosing Cholangitis.
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Secondary Sclerosing Cholangitis in localized hepatobiliary tuberculosis simulating cholangiocarcinoma: a rare case report
BMC, 2017Co-Authors: Aleena Jain, Rachana Chaturvedi, Chetan Kantharia, Amita Joshi, Mangesh Londhe, Mayura KekanAbstract:Abstract Background Hepatobiliary tuberculosis includes miliary, tuberculous hepatitis or localized forms. The localised form is extremely uncommon and can mimic malignancy. Still rarer is its presentation as Sclerosing Cholangitis. Case presentation A 50 year male presented with acute onset jaundice, significant weight loss and elevated liver enzymes with clinico-radiological suspicion of cholangiocarcinoma. A left hepatectomy was done and dilated bile ducts filled with caseous necrotic material were seen intra-operatively. Histopathology suggested localized hepatobiliary tuberculosis with features of Secondary Sclerosing Cholangitis. Conclusion Localised hepatobiliary tuberculosis can cause diagnostic difficulties and its possibility should be considered especially in endemic areas
Cornelia M. Gelbmann - One of the best experts on this subject based on the ideXlab platform.
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Outcome of patients with ischemic-like cholangiopathy with Secondary Sclerosing Cholangitis after liver transplantation.
Scandinavian journal of gastroenterology, 2010Co-Authors: Gabriele I. Kirchner, Petra Ruemmele, Jürgen Schölmerich, Marcus N. Scherer, Aiman Obed, Reiner Wiest, Matthias Froh, Martin Loss, Hj Schlitt, Cornelia M. GelbmannAbstract:Abstract Background and aims. Sclerosing Cholangitis in critically ill patients (SC-CIP) with sepsis and acute respiratory distress syndrome (ARDS) is a cholestatic liver disease with a rapid progression to liver cirrhosis and hepatic failure. Data on outcome of these patients after liver transplantation (LT) are sparse. Patients and methods. Eleven patients (46 ± 12 years; mean labMELD-score: 27 ± 7) with SC-CIP underwent LT. Six patients had severe polytrauma with multiple bone fractures, sepsis and ARDS. Five non-traumatic patients acquired SC-CIP during long-term intensive-care-unit stays due to sepsis and ARDS. Time to diagnosis, the microbiologic results and the survival rates after LT were evaluated. Results. SC-CIP was diagnosed by endoscopic retrograde cholangiopancreatography (ERCP) within 3 ± 1 months after manifestation of cholestasis and histologically confirmed in explanted livers. The predominant microorganisms isolated in bile were: Enterococcus and Candida albicans. Mean follow-up after L...
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Secondary Sclerosing Cholangitis
Nature Reviews Gastroenterology & Hepatology, 2009Co-Authors: Petra Ruemmele, Ferdinand Hofstaedter, Cornelia M. GelbmannAbstract:Secondary Sclerosing Cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation and progressive destruction of the biliary tree that leads to biliary cirrhosis. SSC is thought to develop as a consequence of known injuries or Secondary to pathological processes of the biliary tree. The most frequently described causes of SSC are longstanding biliary obstruction, surgical trauma to the bile duct and ischemic injury to the biliary tree in liver allografts. SSC may also follow intra-arterial chemotherapy. Sclerosing Cholangitis in critically ill patients is a largely unrecognized new form of SSC, and is associated with rapid progression to liver cirrhosis. The mechanisms leading to cholangiopathy in critically ill patients are widely unknown; however, the available clinical data indicate that ischemic injury to the intrahepatic biliary tree may be one of the earliest events in the development of this severe form of Sclerosing Cholangitis. Therapeutic options for most forms of SSC are limited, and patients with SSC who do not undergo transplantation have significantly reduced survival compared with patients with primary Sclerosing Cholangitis. Sclerosing Cholangitis in critically ill patients, in particular, is associated with rapid disease progression and poor outcome. Secondary Sclerosing Cholangitis (SSC) may be caused by various insults to the biliary tree; main causes are chronic biliary obstruction, infectious or toxic Cholangitis, immunological causes and ischemic cholangiopathies SSC is a progressive disease characterized by fibrosis and destruction of the biliary tree, which leads to biliary cirrhosis; in most cases, the benefits of therapeutic interventions are limited Sclerosing Cholangitis in critically ill patients (SC–CIP) is a new entity of Sclerosing cholangiopathy and is associated with a particularly rapid progression of the disease The hallmark of SC–CIP is the early formation of biliary casts; the etiology of SC–CIP may involve early ischemic bile duct injury; however other factors are probably also involved The clinical signs during the initial phase of SC–CIP are not specific; a diagnosis of SC-CIP is often overlooked and, therefore, the frequency of SC–CIP is probably underestimated Aside from transplantation, there are no effective treatment options for SC–CIP; the median survival of patients with SC–CIP who do not undergo liver transplantation is only about 13 months Secondary Sclerosing Cholangitis is a chronic cholestatic biliary disease, which unlike primary Sclerosing Cholangitis, is thought to develop as a consequence of known injuries. The presence of Sclerosing Cholangitis in critically ill patients is a new entity that is increasingly recognized. This Review discusses the features, diagnosis, pathogenesis and treatment options for Secondary Sclerosing Cholangitis and Sclerosing Cholangitis in critically ill patients.
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Secondary Sclerosing Cholangitis
Nature reviews. Gastroenterology & hepatology, 2009Co-Authors: Petra Ruemmele, Ferdinand Hofstaedter, Cornelia M. GelbmannAbstract:Secondary Sclerosing Cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation and progressive destruction of the biliary tree that leads to biliary cirrhosis. SSC is thought to develop as a consequence of known injuries or Secondary to pathological processes of the biliary tree. The most frequently described causes of SSC are longstanding biliary obstruction, surgical trauma to the bile duct and ischemic injury to the biliary tree in liver allografts. SSC may also follow intra-arterial chemotherapy. Sclerosing Cholangitis in critically ill patients is a largely unrecognized new form of SSC, and is associated with rapid progression to liver cirrhosis. The mechanisms leading to cholangiopathy in critically ill patients are widely unknown; however, the available clinical data indicate that ischemic injury to the intrahepatic biliary tree may be one of the earliest events in the development of this severe form of Sclerosing Cholangitis. Therapeutic options for most forms of SSC are limited, and patients with SSC who do not undergo transplantation have significantly reduced survival compared with patients with primary Sclerosing Cholangitis. Sclerosing Cholangitis in critically ill patients, in particular, is associated with rapid disease progression and poor outcome.
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Ischemic-like cholangiopathy with Secondary Sclerosing Cholangitis in critically ill patients.
The American journal of gastroenterology, 2007Co-Authors: Cornelia M. Gelbmann, Petra Rümmele, M. Wimmer, Ferdinand Hofstädter, Björn Göhlmann, Esther Endlicher, Frank Kullmann, Julia Langgartner, Jürgen SchölmerichAbstract:Ischemic-Like Cholangiopathy With Secondary Sclerosing Cholangitis in Critically Ill Patients
Tim O. Lankisch - One of the best experts on this subject based on the ideXlab platform.
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Liver transplantation for critically Ill patients with Secondary Sclerosing Cholangitis: Outcome and complications.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation S, 2015Co-Authors: Torsten Voigtländer, Michael P. Manns, Elmar Jaeckel, Frank Lehner, Tim O. LankischAbstract:Secondary Sclerosing Cholangitis in critically ill patients (SSC-CIP) is a destructive cholangiopathy with a poor prognosis. Liver transplantation (LT) is an established therapeutic option in end-stage liver disease but is insufficiently evaluated in patients with SSC-CIP. Our aim was the retrospective analysis of the outcome and complications of patients with SSC-CIP undergoing LT between 2002 and 2012. Demographic characteristics, laboratory, transplantation, and follow-up data were compared to sex- and age-matched patients undergoing LT because of other reasons. Quality of life (QoL) before and after LT was assessed in a retrospective telephone interview. LT was performed in 21 patients with SSC-CIP. The main causes for intensive care unit admission comprised cardiothoracic surgery interventions (10/21, 48%), polytrauma (6/21, 29%), and pneumonia (3/21, 14%). Median follow-up period after LT was 82 months (interquartile range [IQR], 37-129) for patients with SSC-CIP and 83 months (IQR, 55-104) for control patients. Biopsy-proven rejection episodes in patients with SSC-CIP (4/21, 19%) were similar compared to control patients (12/60, 20%; P = 0.93). Cytomegalovirus infections were equal in both groups (10/21, 48% versus 25/60, 42%; P = 0.64). The 1-, 3-, and 5-year survival rates of patients with SSC-CIP versus control patients were 100% versus 98%, 86% versus 92%, and 76% versus 87%, respectively (P > 0.05). The QoL improved significantly after LT in SSC-CIP. In conclusion, LT is a valid option for patients with SSC-CIP with excellent long-term outcome and improvement of QoL. Liver Transpl 21:1295-1299, 2015. © 2015 AASLD.
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Microbiological analysis of bile and its impact in critically ill patients with Secondary Sclerosing Cholangitis
Journal of Infection, 2015Co-Authors: Torsten Voigtländer, Ralf-peter Vonberg, Michael P. Manns, Sebastian Suerbaum, Ensieh Leuchs, Philipp Solbach, Tim O. LankischAbstract:Summary Objectives 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. Methods 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. Results 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 Conclusions 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.
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Secondary Sclerosing Cholangitis following liver transplantation: a rare cause for graft failure
Zeitschrift fur Gastroenterologie, 2013Co-Authors: Torsten Voigtländer, T Alten, F Lehner, C P Strassburg, M P Manns, Tim O. LankischAbstract:We report on a 25-year-old female patient who presented with recurrent cholestasis following liver transplantation due to primary Sclerosing Cholangitis. Abdominal ultrasound and computed tomography showed intrahepatic bile duct dilatation and stenosis of the common hepatic artery with flow acceleration and decreased resistance index. The patient developed a severe Secondary Sclerosing Cholangitis (SSC) with biliary casts - despite interventional stent placement of the common hepatic artery - thus requiring retransplantation. After prolonged intensive care unit treatment the patient was discharged in a good general condition. This case report describes SSC as a rare cause for graft failure. In unclear cholestasis after liver transplantation SSC has to be considered as the underlying cause.
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Secondary Sclerosing Cholangitis in critically ill patients: model of end-stage liver disease score and renal function predict outcome.
Endoscopy, 2012Co-Authors: Torsten Voigtländer, Michael P. Manns, Ahmed A. Negm, Andrea Schneider, Christian P. Strassburg, Jochen Wedemeyer, Tim O. LankischAbstract:Secondary Sclerosing Cholangitis in critically ill patients (SSC – CIP) is an underdiagnosed emerging disease. The aim of this study was to characterize clinical features and prognostic factors for mortality in SSC – CIP. This retrospective study included 54 patients who were diagnosed via endoscopic retrograde cholangiopancreatography (ERCP) after cardiothoracic surgery (n = 21), sepsis (n = 13), polytrauma (n = 11), and others (n = 9). In total, 33 patients who either died (n = 27) or needed liver transplantation (n = 6) were compared with surviving patients (n = 21). The model for end-stage liver disease (MELD) score and need for renal replacement therapy were independent risk factors for mortality. Compared with ERCP, accuracy was 30 % for ultrasound and 36 % for liver biopsies. As a result of microbiological bile analysis, 28 % of patients required a change in antibiotic treatment. SSC – CIP is frequently a fatal disease. ERCP should be considered in selected patients to establish the diagnosis and hence provide useful clinical information.
