1/124. Primary sclerosing cholangitis mimicking choledocal cyst type 1 in a young patient.A choledochal cyst type I was diagnosed in a 12-year-old boy in 1984. The diagnosis was made using ultrasound and confirmed using computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP). Instead of the usual surgical treatment, endoscopic balloon dilatation of the sphincter of oddi and the distal common bile duct was carried out using an endoscopic procedure. The patient experienced relief of symptoms, gained weight and felt healthy again. An ERCP performed in 1990, because of increasing levels of liver enzymes and clinical features of abdominal pain and fatigue, revealed typical cholangiographic findings associated with primary sclerosing cholangitis, including bile duct irregularities with diffuse narrowing and twisting of the bile ducts with localized ectatic and strictured areas. Percutaneous needle liver biopsy confirmed the diagnosis. We conclude that primary sclerosing cholangitis should be considered when interpreting ERCP films from patients who are supposed to have choledochal cysts type I.- - - - - - - - - - ranking = 1keywords = bile duct, bile, duct (Clic here for more details about this article) |
2/124. Recurring fibro-obliterative venopathy in liver allografts.Recurrent diseases in liver allografts are not uncommon. These occur most frequently in those transplanted for viral hepatitis b and C. We report an unusual case of recurrent process in two consecutive liver allografts received by a 37-year-old woman, who previously had an unremarkable past medical history but developed a rapidly progressive cholestatic liver failure. Histopathologic examination of the native liver showed fibroocclusive lesions of both terminal hepatic venules and portal vein branches. The exuberant fibroobliterative process created dense fibrosis with whorled appearance, and broad fibrous septa connecting adjacent central areas, and sometimes bridging portal to central areas. Dense portal fibrosis resulted in compression atrophy and loss of bile ducts. The first allograft, which failed within 3 months, showed histopathologic findings similar to that of the native liver. A liver biopsy that was performed 20 months after the second liver transplant again showed similar histopathology. The histopathologic features and clinical presentation of this patient suggest an unusual form of recurring progressive fibroobliterative venopathy causing liver failure.- - - - - - - - - - ranking = 0.33333333333333keywords = bile duct, bile, duct (Clic here for more details about this article) |
3/124. Sclerosing cholangitis associated to cryptosporidiosis in liver-transplanted children.Three children of a series of 461 pediatric liver transplant recipients developed diffuse cholangitis associated with intestinal cryptosporidium carriage. All three received immunosuppression consisting of tacrolimus and prednisone. Cryprosporidium carriage was treated with paramomycin, while immunosuppression was decreased according to graft tolerance. No other infectious pathogens were found, and no vascular problems were detected. Bile duct anastomosis was reoperated in all three, but biliary cirrhosis developed in one patient, requiring retransplantation. All three patients are alive and well, and free of intestinal parasites on follow-up. CONCLUSION: cryptosporidium intestinal infection may play a role in some cases of otherwise unexplained cholangiopathies in pediatric liver transplant recipients. This may lead to significant morbidity, including need for retransplantation.- - - - - - - - - - ranking = 0.014921756862085keywords = duct (Clic here for more details about this article) |
4/124. Clinicopathologic findings of recurrent primary sclerosing cholangitis after orthotopic liver transplantation.Whether primary sclerosing cholangitis (PSC) occurs after orthotopic liver transplantation is controversial, largely because the pre-transplant diagnosis of PSC is based on nonspecific radiological and histological findings. We reviewed clinical, radiological, and histological records of 53 patients who underwent liver transplantation for PSC between 1985 and 1998. Three patients with patent hepatic arteries and no evidence of chronic rejection had radiological and histological findings that may have been due to recurrent PSC. Bile duct stricturing in these patients proved permanent and progressive and affected both the quality of life and graft survival. The first patient, who is 110 months after transplantation, has had repeated episodes of cholangitis for the last year. The second patient underwent excision of a strictured hepatic duct 45 months after transplantation and was ultimately retransplanted 95 months after initial transplantation. The third patient underwent left hemihepatectomy of an atrophied lobe 50 months after transplantation. Although the patient population assessed in this study is limited, putative recurrent PSC in the allografts has led either to graft loss or to clinically significant hepatobiliary complications of the graft.- - - - - - - - - - ranking = 0.029843513724171keywords = duct (Clic here for more details about this article) |
5/124. A case of segmental primary sclerosing cholangitis.A 74-year-old man was admitted to the Yokohama City University School of medicine for investigation of high values of ALP and Y-GTP. Radiographic examinations, including abdominal computed tomography and percutaneous transhepatic cholangiography, strongly suggested bile duct cancer in the hepatic hilus. After left lobectomy, pathological examination disclosed segmental primary sclerosing cholangitis. Clinical examination cannot always distinguish primary sclerosing cholangitis from cancer. We report a case of segmental primary sclerosing cholangitis and discuss the diagnosis and the treatment of this disease.- - - - - - - - - - ranking = 0.33333333333333keywords = bile duct, bile, duct (Clic here for more details about this article) |
6/124. Immunological similarities between primary sclerosing cholangitis and chronic sclerosing sialadenitis: report of the overlapping of these two autoimmune diseases.Primary sclerosing cholangitis (PSC) is characterized by destructive inflammation and fibrosis affecting the bile ducts. The etiology of PSC is still unknown, although lymphocytic infiltration in the portal areas suggests an immune-mediated destruction of the bile ducts. patients with one autoimmune disease often suffer from one or more other autoimmune diseases. It is well known that there is a close relationship between PSC and inflammatory bowel disease, particularly ulcerative colitis(UC). However, the pathological findings in UC and other overlap diseases do not resemble those of PSC. In the present study, we report a patient with chronic sclerosing sialadenitis (Kuttner's tumor) and PSC. It is compared the sclerosing changes in both salivary glands and bile ducts histologically. In addition, the expression pattern of mast cell tryptase, b-FGF, and HLA-DR were examined in both tissues immunohistochemically. Histological features of sclerosing change in both salivary and bile ducts were quite similar. Marked mast cell infiltration and b-FGF expression were seen in the sclerosing areas in both tissues. In active inflammatory areas of the salivary glands, HLA-DR expression was also seen. We hypothesized that similar immune reactions occur in both the salivary gland and bile ducts and are responsible for the fibrosis that follows.- - - - - - - - - - ranking = 1.6666666666667keywords = bile duct, bile, duct (Clic here for more details about this article) |
7/124. A case of sclerosing cholangitis managed by a percutaneous approach.In 1992, a 61-year-old man who complained of recurrent episodes of fever and jaundice was diagnosed as having sclerosing cholangitis. In the three years that followed, the clinical picture progressively worsened; and, in 1995, the patient was hospitalized again for biliary obstruction. A liver transplantation was excluded because of concomitant severe coronary heart disease. A percutaneous transhepatic cholangiogram showed several critical strictures of the intrahepatic biliary tree and a temporary internal-external biliary drainage was placed to relieve the obstruction. After 40 days, a two-step percutaneous biliary balloon dilation was performed followed by topical steroid treatment through the catheter. After 45 days, the catheter was removed and steroid treatment tapered orally. In the three years that followed, the patient was well. He experienced only about 1-2 episodes of ascending cholangitis per year requiring antimicrobial therapy. Laboratory analysis showed a gradual improvement in hepatic chemistry, serum bilirubin, and erythrocyte sedimentation rate (ESR). In our patient, the association of percutaneous balloon dilation and topical steroid treatment improved both the clinical and radiological picture, without significant side-effects. This approach should be considered a valuable and cost-effective option in primary sclerosing cholangitis, mainly for patients not eligible for liver transplantation.- - - - - - - - - - ranking = 0.00033880914711517keywords = obstruction (Clic here for more details about this article) |
8/124. Inflammatory pseudotumor of the liver with primary sclerosing cholangitis.Inflammatory pseudotumor (IPT) of the liver is a rare benign variant of hepatic masses, and its exact etiology has not been elucidated. We report a case of IPT associated with primary sclerosing cholangitis (PSC). The patient was a 50-year-old man admitted to our hospital because of jaundice. Abdominal ultrasonography (US) and computed tomography showed multiple dilations of the intrahepatic bile ducts and multiple masses in the liver. On magnetic resonance imaging, the masses were slightly hypointense on T1-weighted images and slightly hyperintense on T2-weighted images. On T1-weighted images after the bolus infusion of Gd chelate, the masses had no contrast enhancement, and they were hypointense in the arterial phase and portal venous phase. However, they were slightly enhanced and became almost isointense relative to the surrounding normal liver parenchyma in the delayed phase. Endoscopic retrograde cholangiography demonstrated multiple irregular strictures and dilations of the intrahepatic bile ducts. Angiography demonstrated no abnormal findings, but, interestingly, subsequent dynamic CO2-enhanced US showed a strongly hyperechoic string, indicating that an artery had penetrated through the hypoechoic mass. A US-guided percutaneous needle biopsy revealed that the lesions were morphologically comparable to IPT. After cholangiography and microscopic analysis of the tumor, the final diagnosis was determined to be IPT of the liver with PSC. A number of previous reports have suggested a possible relationship between IPT and PSC, based on pathological findings. This report confirmed, based on clinical findings, that PSC is one of the causes of hepatic IPT.- - - - - - - - - - ranking = 0.66666666666667keywords = bile duct, bile, duct (Clic here for more details about this article) |
9/124. Hypereosinophilic sclerosing cholangitis: findings using half-Fourier magnetic resonance imaging.Hypereosinophilic sclerosing cholangitis is a rare disease caused by eosinophilic infiltration of the gallbladder and biliary tract seen in the idiopathic hypereosinophilic syndrome. We report a 42-year-old woman who presented with symptoms of cholecystitis and obstructive cholangitis. Imaging with magnetic resonance cholangiography using a half-Fourier spinecho sequence, we were able to visualize rapidly and non-invasively a severely abnormal gallbladder, evidence of liver parenchymal inflammation, and biliary duct dilatation.- - - - - - - - - - ranking = 0.014921756862085keywords = duct (Clic here for more details about this article) |
10/124. Sequential occurrence of primary sclerosing cholangitis and autoimmune hepatitis type III in a patient with ulcerative colitis: a follow up study over 14 years.In 1983, a female patient born in 1963 presented with symptoms of ulcerative colitis and typical clinical and histological signs of primary sclerosing cholangitis (PSC). At this time only pANCA were positive while other marker antibodies for autoimmune liver disorders could not be detected. In summer 1987 the clinical picture changed and was replaced by laboratory and histological signs typical of autoimmune hepatitis (AIH). Thus, IgG levels increased considerably and cholestatic enzymes became normal. For the first time, anti-liver-pancreas antibodies (LP), a diagnostic marker for AIH type III could be detected. In the following years several relapses occurred also induced by repeated discontinuation of immunosuppressive therapy. Symptoms of colitis persisted but signs of cholestasis remained absent for the following ten years. In 1997, colitis exacerbated again and colectomy had to be performed together with liver transplantation. Surprisingly, histology of the explanted liver now showed the typical features of PSC stage III/IV while the significant criteria for AIH were now lacking. Thus, progression to cirrhosis was, probably, mainly induced by the biliary destructive and fibrotic process although biochemical and serological data were clearly indicative of an autoimmune, i.e. AIH-related manifestation.- - - - - - - - - - ranking = 0.23726184691519keywords = cholestasis (Clic here for more details about this article) |
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