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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six jaundiced patients with primary sclerosing cholangitis and a dominant biliary stricture were managed by endoscopic placement of endoprostheses. Five showed considerable improvement within weeks of stenting: their serum bilirubin concentration fell from mean (range) 266 mumol/l (63-681) to 65 mumol/l (10-280) after one month. One patient required a liver transplant at five months because of continued deterioration in hepatic function. Follow up of 12-49 months in the remaining five patients shows sustained biochemical improvement, with repeat cholangiograms indicating doubling of the minimum calibre of the extrahepatic bile duct in four patients and considerable shortening of stricture length in three. Three patients developed sepsis at the time of the initial endoprosthesis insertion: surgical drainage was necessary in one. Endoscopic methods of improving biliary-enteric drainage in jaundiced patients with primary sclerosing cholangitis may be preferable to surgical and percutaneous methods, which may complicate subsequent liver transplantation.
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PMID:Improving biliary-enteric drainage in primary sclerosing cholangitis: experience with endoscopic methods. 175 70

A further extension of the U-tube technique is described in the treatment of six patients with primary sclerosing cholangitis who developed progressive jaundice and recurrent biliary sepsis. All six patients had operative intrahepatic duct dilatation and U-tube placement. Three patients in addition had a Roux-en-Y hepaticojejunal anastomosis. Five patients are improved and are well after a median follow-up period of 56 months. Two patients have had the U-tube removed electively. Three patients with progressive disease required further percutaneous catheter dilatation of intrahepatic strictures via the U-tube tract. Application of the technique permits evaluation of the biliary system by tube cholangiography and provides access in complex cases for repeated therapeutic intrahepatic stricture dilatation.
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PMID:Primary sclerosing cholangitis: biliary drainage and duct dilatation. 382 36

In spite of an improved understanding of the etiology of primary sclerosing cholangitis, which supports the use of immunosuppressive therapy with steroids and azathioprine, these pharmacologic manipulations have not altered the ultimate outcome of the disease. Drainage remains the most accepted mode of therapy when possible. Recent advances in diagnosis with ERCP and treatment with transhepatic biliary drainage may change the time-honored surgical approach to this disease as these techniques become more widely available. Until then, however, proper surgical management depends upon a high index of suspicion at the time of laparotomy so that irrevocable damage to the biliary tree will not be done prior to the establishment of the correct diagnosis. Simple drainage of the biliary tree will provide symptomatic relief in some patients; unfortunately, most patients will succumb to progressive biliary cirrhosis or sepsis in spite of all treatment.
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PMID:Primary sclerosing cholangitis. 702 3

The clinical course of 37 patients who underwent 46 liver transplantations for primary (n = 33) and secondary (n = 4) sclerosing cholangitis was reviewed. The median follow-up was 37 months. The patient and graft survivals for patients with primary sclerosing cholangitis at 1, 2, and 5 years were 96.9%, 91.6%, 87.9%, and 83.1%, 74.2%, 65.2%, respectively. In the patients with primary sclerosing cholangitis (PSC), prior surgery except for simple cholecystectomy was associated with significantly greater operative time and blood loss. No cholangiocarcinoma was identified at the time of transplantation. Human leukocyte antigen typing for PSC patients was heavily weighed toward B8 (58.8%) compared with control (11.8%). Sixty-two percent of patients with PSC also had inflammatory bowel disease. Moderate or severe rejection requiring OKT3, "rescue therapy" with FK506, or retransplantation was relatively higher in patients with inflammatory bowel disease (70%) versus patients without inflammatory bowel disease (36.4%) and a matched control group (37.5%). Progressive inflammatory bowel disease was seen in 6 of 19 patients, with 3 developing cancer and a dysplasia. Two patients in the entire group died of sepsis and 3 of colon cancer (2 recurrent and 1 primary). These data demonstrate that excellent survival results can be achieved in this group of patients. Rejection is frequent and often severe and steroid refractory. Colon cancer represents the most frequent cause of death in PSC patients after liver transplantation and demands constant attention.
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PMID:Liver transplantation for sclerosing cholangitis. 763 12

Biliary obstruction is a common and potential fatal condition. Its pathological effects include depressed immunity, impaired phagocytic activity and reduced Kupffer cell function with consequent endotoxemia, septicemia and renal failure. Over the last decade however, non-surgical biliary drainage procedures performed with radiologic or endoscopic guidance emerged as alternative to surgical of therapy in many situations, particularly palliation of malignant strictures because of their lower morbidity and mortality rates. Endoscopic stent placement is preferred over percutaneous transhepatic catheter drainage in general. If endoscopic procedure is not possible or fails percutaneous transhepatic biliary drainage or combined radiological-endoscopic procedure should be employed. Surgery is currently reserved only for curative resection/palliative drainage in young and fit patients. Preoperative biliary drainage aimed at reducing post-operative morbidity and mortality is not universally accepted and needs further study. Benign strictures are increasingly being dilated non-surgically with temporary stenting, especially in patients with failed surgery, recurrent strictures, contraindication to surgery and pre-liver transplant strictures eg primary sclerosing cholangitis. A cohesive team consisting of a surgeon, a radiologist and an endoscopist is required to achieve the best possible results.
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PMID:Nonsurgical drainage for biliary obstruction. 782 40

Three patients with primary sclerosing cholangitis and Hodgkin's disease, a previously unrecognized association, are reported. All three patients were men, and one patient had Crohn's disease of the colon. Primary sclerosing cholangitis was diagnosed 2, 11 and 17 yr before diagnosis of Hodgkin's disease in the three patients, and all three had advanced biliary cirrhosis prompting referral for liver transplantation. The symptoms of Hodgkin's disease were often masked by similar manifestations of primary sclerosing cholangitis, particularly symptoms of recurrent biliary sepsis. Hodgkin's disease is another disorder that may occur in patients with primary sclerosing cholangitis, particularly in the setting of advanced disease, and may be masked by the underlying hepatobiliary disease.
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PMID:Primary sclerosing cholangitis and Hodgkin's disease. 822 18

Of the multiple treatment options available to physicians managing patients with primary sclerosing cholangitis (PSC), no single form of therapy has proven to be efficacious or to improve the clinical outcome of affected patients. Surgical and radiological methods require multiple procedures which are associated with increased morbidity, the most serious being sepsis. Endoscopic therapy is safer and easier to perform, and has been shown to provide symptomatic as well as objective improvement in affected patients. It, too, is associated with some complications, although less serious than other alternative treatments, but it is not effective in protracted disease. The potential of overlooking or missing cholangiocarcinoma is more apparent when nonsurgical techniques are employed. In this review we will discuss the various endoscopic treatment modalities utilized in PSC disease, and include the results that have been obtained with these techniques since they were incorporated into our armamentarium in 1983.
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PMID:Endoscopic management of primary sclerosing cholangitis: review, and report of an open series. 824 63

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are the most common cholestatic disorders in adulthood requiring hepatic transplantation. Although they run similar courses, they may have different problems before and after transplantation. The aim of this study was to compare pre- and post-transplant complications and outcomes in these two similar but distinct patient groups. One hundred and seventeen adult patients underwent liver transplantation at our institution over a 6 year period, including 19 with PSC and 20 with PBC. Pre-transplant there were no significant differences in age, liver biochemistry, haematology or Child-Pugh scores between the two groups. The mean duration of disease before transplant was longer in PSC patients (11.7 vs 6.5 years; P < 0.05). The prevalence of septic cholangitis was greater in PSC (58 vs 5%; P < 0.01) as was the requirement for surgical or radiological interventional procedures, excluding cholecystectomy (53 vs 0%; P < 0.01). At transplantation, four patients with PSC had previously unrecognized cholangiocarcinoma. In the pre-transplant period these four patients had uncontrolled biliary sepsis at the time of transplant vs five of 15 PSC patients without cholangiocarcinoma. Postoperatively, PSC patients had a greater prevalence of intra-abdominal sepsis requiring surgical or radiological intervention (42 vs 5%; P < 0.05). In comparison, patients with PBC had a high prevalence of skeletal complications (30 vs 10%; P < 0.05) particularly avascular necrosis (15 vs 0%). The prevalence of chronic rejection was similar in both groups (15%). Overall survival was higher in PBC patients (85 vs 63%; P < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver transplantation for primary sclerosing cholangitis versus primary biliary cirrhosis: a comparison of complications and outcome. 851 94

Over the past 30 years, liver transplantation has evolved from an experimental therapy to a routine procedure and most pathology textbooks have now a section dedicated to the pathology of liver transplant. Although there remain problems of biopsy interpretation due to the numerous post-transplant complications which can occur singly or in association, the major changes have been well characterized and are reviewed here, a particular attention being given to those features which are unique to or distinctive of the liver allograft. These include the outcome of donor fatty liver, reperfusion damage, massive haemorrhagic necrosis and the patterns of rejections, in particular the rarity and delayed onset of hyperacute rejection and the selective involvement of the small interlobular bile ducts and vascular endothelia in both acute and chronic graft rejection. "Functional" cholestasis with hepatocyte ballooning, cholangiolar cholestasis associated with sepsis and lesions of the larger bile ducts which may result from preservation, immune and/or ischaemic injury and closely resemble the changes observed in primary sclerosing cholangitis are also considered. Later in the post-transplant course, changes due to de novo or recurrent hepatitis have to be distinguished from those of late cellular rejection, protracted biliary complications, lymphoproliferative disorders, disease recurrence, in particular autoimmune chronic hepatitis, primary biliary cirrhosis and sclerosing cholangitis, differential diagnoses which often require interpretation of allograft changes in conjunction with all laboratory and clinical data available.
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PMID:Pathology and biopsy diagnosis of the transplanted liver. 860 Jun 91

In post-infancy, multinucleated giant cell hepatitis is rare. Various conditions and diseases associated with post-infantile giant cell hepatitis have been described, but the pathogenesis remains unknown. In this paper we review the case reports of four patients (3 male, 1 female; aged 22 to 32 years) with primary sclerosing cholangitis and autoimmune hepatitis. The follow-up ranges from five to seven years. All patients showed cholestasis and repeated elevation of hepatic transaminases. Patients with viral infections, metabolic disorders and toxic influences were excluded. Histopathology of liver tissue in all four patients revealed giant cell formation with up to 20 nuclei in 20-70% of all hepatocytes. Post-infantile giant cell hepatitis was defined histopathologically. The clinical course of all four patients markedly improved after immunosuppressive treatment. Further improvement was observed with the addition of ursodeoxycholic acid. Follow-up liver biopsies during treatment showed reduced inflammation and a decreased number of giant cells. One patient, who initially was admitted to the hospital with liver cirrhosis died five years later due to a sepsis. The clinical course of the other three patients remained stable during the observation period, and no progression of liver fibrosis was recorded as long as immunosuppressive treatment was continued. Cholestasis and autoimmunity seem to be two important mechanisms triggering hepatic giant cell formation in post-infancy. In the reported cases long-lasting cholestasis in primary sclerosing cholangitis together with features of autoimmune hepatitis seem to have triggered the formation of syncytial hepatic giant cells.
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PMID:Post-infantile giant cell hepatitis in patients with primary sclerosing cholangitis and autoimmune hepatitis. 887 1


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