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ductopenic cholestasis

A I Musat, R M Agni, P Y Wai, J D Pirsch, D F Lorentzen, A Powell, G E Leverson, J M Bellingham, L A Fernandez, D P Foley, J D Mezrich, A M D'Alessandro, M R Lucey
The role of humoral alloreactivity in ABO-compatible liver transplantation remains unclear. To understand the significance of donor-specific HLA alloantibodies (DSA) in liver rejection, we applied the currently used strategy for detection of antibody-mediated rejection of other solid allografts. For this purpose we reviewed the data on 43 recipients of ABO identical/compatible donor livers who had indication liver biopsy stained for complement element C4d and contemporaneous circulating DSA determination. Seventeen (40%) patients had significant circulating DSA in association with diffuse portal C4d deposition (DSA+/diffuse C4d+)...
March 2011: American Journal of Transplantation
Shannon Glaser, Ian P Lam, Antonio Franchitto, Eugenio Gaudio, Paolo Onori, Billy K Chow, Candace Wise, Shelley Kopriva, Julie Venter, Mellanie White, Yoshiyuki Ueno, David Dostal, Guido Carpino, Romina Mancinelli, Wendy Butler, Valorie Chiasson, Sharon DeMorrow, Heather Francis, Gianfranco Alpini
UNLABELLED: During bile duct ligation (BDL), the growth of large cholangiocytes is regulated by the cyclic adenosine monophosphate (cAMP)/extracellular signal-regulated kinase 1/2 (ERK1/2) pathway and is closely associated with increased secretin receptor (SR) expression. Although it has been suggested that SR modulates cholangiocyte growth, direct evidence for secretin-dependent proliferation is lacking. SR wild-type (WT) (SR(+/+)) or SR knockout (SR(-/-)) mice underwent sham surgery or BDL for 3 or 7 days...
July 2010: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Daniel Gotthardt, Heiko Runz, Verena Keitel, Christine Fischer, Christa Flechtenmacher, Michael Wirtenberger, Karl Heinz Weiss, Sandra Imparato, Annika Braun, Kari Hemminki, Wolfgang Stremmel, Franz Rüschendorf, Adolf Stiehl, Ralf Kubitz, Barbara Burwinkel, Peter Schirmacher, A S Knisely, Johannes Zschocke, Peter Sauer
UNLABELLED: Cholestatic liver disease (CLD) is a major cause of progressive liver damage and liver failure. Several forms of biliary cirrhosis are caused by mutations in specific genes. We sought to identify a genetic defect in a family with CLD impossible to assign to a distinct pathogenic entity. Clinical and histopathological characterization of the family members, microarray-based single-nucleotide polymorphism genotyping, and analysis of candidate genes were performed. Among six of 11 siblings severely affected by idiopathic CLD in a family from a population isolate in Transylvania, three died of cirrhosis (aged 5, 7, and 43 years) and three had adult-onset disease with small duct cholangiopathy, including ductopenia...
October 2008: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
J L Montero, J C Pozo, P Barrera, E Fraga, G Costán, J L Domínguez, J Muntané, A Rodriguez-Ariza, M Pleguezuelo, S Rufián, P López-Cillero, M de la Mata
UNLABELLED: Pruritus is a common complication of cholestatic liver diseases or liver graft dysfunction. Current medical therapies lack efficacy. The molecular adsorbent recirculating system (MARS) represents an interesting therapeutic option. Our objective was to report our experience in the management of four patients with intractable pruritus with MARS. PATIENTS AND METHODS: The MARS treatment cycle included three consecutive treatments, each of 8 hours duration...
October 2006: Transplantation Proceedings
V J Desmet
This article reviews the histopathologic liver alterations in chronic cholestatic diseases and parenchymal changes associated with periportal ductular reaction and fibrosis, which may result in end-stage biliary cirrhosis. Adult ductopenic diseases are possible causes of such chronic cholestasis. Ductopenia is defined, and guidelines are given for its quantitation. The last section summarizes the specific histologic lesions of primary biliary cirrhosis, primary sclerosing cholangitis, their histologic mimics, and the differential diagnosis with drug-induced cholestasis and ductopenia...
May 1998: Clinics in Liver Disease
Romuald Bellmann, Ivo W Graziadei, Clemens Feistritzer, Hubert Schwaighofer, Frans Stellaard, Ekkehard Sturm, Christian J Wiedermann, Michael Joannidis
Albumin dialysis has been shown to improve the outcome in patients with cholestatic liver failure caused by chronic liver disease. This study reports 7 liver transplant recipients who were treated with albumin dialysis for intractable pruritus of different origin (ductopenic graft rejection, non-anastomotic strictures, and recurrence of hepatitis C). Treatment with histamine (H1) blockers, opioid antagonists, and cholestyramine had not been effective. The Molecular Adsorbent Recirculating System (MARS; Teraklin, Rostock, Germany) was used for albumin dialysis...
January 2004: Liver Transplantation
Alastair D Burt
Several distinct conditions are characterized by a reduction in the number of small and medium-sized intrahepatic bile ducts. These diseases are associated with progressive cholestasis, which in turn leads to biliary fibrosis and ultimately cirrhosis. The best-characterized ductopenic condition in adulthood is primary biliary cirrhosis (PBC) for which there is now strong evidence of an autoimmune cause. The antigenic targets are epitopes on proteins of the 2-oxoacid dehydrogenase complex within mitochondria...
May 2002: Clinics in Liver Disease
F P Vleggaar, H R van Buuren, P E Zondervan, F J ten Kate, W C Hop
The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. Patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia...
August 2001: Gut
A C Jmelnitzky, M Guidi, A Bologna, M Viola, C Soccini, R Barbero, P Belloni, M Apraiz
To assess epidemiological and clinical significance of drug hepatotoxicity in the setting of liver diseases consultation, ten thousand and three hundred forty two prospectively designed clinical records from patient cared for in our Liver Unit in the period 1988-1998 were incorporated into the study; 58 out of 10,342 (prevalence = 5.6%) fulfilled at least the first three of the following causality requirements: 1.--Liver injury associated in time to drug exposition; 2.--Negative evaluation of more common other etiologies; (alcohol, viruses, immunologic, metabolic, etc) 3...
2000: Acta Gastroenterologica Latinoamericana
S A Taga, M K Washington, N Terrault, T L Wright, K A Somberg, L D Ferrell
Some liver allograft recipients with hepatitis C virus (HCV) infection develop hyperbilirubinemia, which might be the result of a cholestatic variant of hepatitis C. We evaluated all liver biopsy samples from 6 liver transplant recipients who had polymerase chain reaction-positive HCV infection and histologic evidence of hepatitis and jaundice and compared them with liver biopsy samples from a control group of transplant recipients with HCV hepatitis without jaundice. Patients with known ductopenic rejection, biliary obstruction, or co-infection with hepatitis A or B were excluded from the study...
July 1998: Liver Transplantation and Surgery
D Rodríguez Martínez, C M Fernández Rodríguez, I Rodríguez Prada, S Pereira Bueno, M Butrón, F Colina
We describe a 56 years old male patient with long-term chronic liver disease of unknown etiology presenting with esophageal varices rupture. Prophylaxis of re-bleeding with propranolol and endoscopic sclerotherapy failed to prevent further haemorrhagic events and the placement of a transjugular intrahepatic portosystemic shunt (TIPS) was needed. The portal hemodynamic data revealed sinusoidal portal hypertension and the liver biopsy displayed ductopenic cholestasis. The patient met all criteria of idiopathic ductopenia...
January 1998: Gastroenterología y Hepatología
K R Reddy, E R Schiff
Several antibiotics are known to be hepatotoxic. Apart from subtle, reversible, and often inconsequential abnormalities, cholestasis is the predominant presentation that has a protracted and disabling course. Hepatitis, like illness, is characteristic of some drugs, and chronic liver disease may evolve. It is important to recognize if a patient has an adverse reaction to a drug because continuing use of the drug in the face of hepatitis can have disastrous consequences. Chronic liver disease may ensue and progress onto cirrhosis; this has typically been seen following use of isoniazid and nitrofurantoin...
December 1995: Gastroenterology Clinics of North America
M Sebagh, O Farges, A Kalil, D Samuel, H Bismuth, M Reynes
Sclerosing cholangitis defined by cholangiographic criteria may occur after orthotopic liver transplantation. In this retrospective study, we analyzed failed grafts and antecedent serial biopsies of 24 patients who developed this type of nonanastomotic biliary strictures. Sclerosing cholangitis was histologically diagnosed if there was a combination of periductal fibrosis and features of large bile duct obstruction. The condition was observed in all but one available failed allografts. This later showed ischemic-type lesions without periductal fibrosis...
January 1995: American Journal of Surgical Pathology
P C van de Meeberg, F H Wolfhagen, K J van Erpecum, G P van Berge Henegouwen
Based on proceedings of the 75th Falk Symposium in Maastricht (The Netherlands) on cholestasis and gallstone disease, recent developments in this field are highlighted. New ideas on bile and gallstone formation as well as effect of cholestasis on the handling of bilirubin and bile salts are presented. Special attention is paid to the latest data concerning ductopenic liver diseases with emphasis on therapy with ursodeoxycholic acid (UDCA).
July 1995: Netherlands Journal of Medicine
E M Ward, M J Kiely, T P Maus, R H Wiesner, R A Krom
Nonanastomotic hilar bile duct strictures developed in 16 of 152 patients who underwent liver transplantation. The type of pretransplantation liver disease did not significantly affect the likelihood of hilar stricture formation. Possible causes of hilar biliary strictures include hepatic artery occlusion, ductopenic arteriopathic rejection, and cytomegalovirus infection; however, five of the 16 patients had hilar strictures without these complications. Hilar strictures developed within 3 months after transplantation in 11 of the 16 patients...
October 1990: Radiology
K B Noack, R H Wiesner, K Batts, B van Hoek, J Ludwig
No abstract text is available yet for this article.
February 1991: Transplantation Proceedings
R H Wiesner, J Ludwig, B van Hoek, R A Krom
Hepatic allograft rejection is presently classified into acute and chronic rejection based on histological features, timing and reversibility. However, because features of both types of rejection can occur at any time, and in many combinations, the terms "acute" and "chronic" seem inappropriate in some instances. Thus the term "cellular rejection" better defines the histological features of portal hepatitis, nonsuppurative destructive cholangitis and endotheliitis, which are independent of time and response to therapy...
October 1991: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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