1/9. Combination therapy with mycophenolate mofetil and ursodeoxycholic acid for primary biliary cirrhosis.Evidence of autoimmunity in primary biliary cirrhosis (PBC) provides a rationale for treatment with an immunosuppressant. Such a drug should be sufficiently free from serious toxicities to enable patients with asymptomatic, but progressive, disease to be treated longterm. Mycophenolate mofetil (MMF) mediates immunosuppression by selectively and reversibly inhibiting lymphocyte function, and has a more acceptable safety profile than other immunosuppressants that have been used in the treatment of this disease. Two patients, whose response to long-term treatment with ursodeoxycholic acid (UDCA) had been inadequate, were treated with a combination of MMF 2 g daily and UDCA 1 g daily for 12 months. In both patients this regimen was associated with no clinically significant adverse events, a decrease in elevated serum alkaline phosphatase levels to values close to the upper limit of normal, and an almost complete disappearance of the chronic inflammatory cell infiltrate that had been present in pre-combination treatment liver biopsies. MMF may be an appropriate immunosuppressive drug for use in the long-term treatment of patients with PBC, including asymptomatic patients.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/9. Primary sclerosing cholangitis and ulcerative colitis.This is a report on a 36-year-old male patient presenting with a rare combination of ulcerative colitis and primary sclerosing cholangitis. The disease of the biliary tract was suspected on the basis of the endoscopic retrograde representation of the common bile duct, and serologically differentiated from a chronic destructive, non-supperative cholangitis on the basis of a lack of antimitochondrial antibodies. Subsequently, a hepaticojejunostomy was carried out to normalize the bile flow.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
3/9. turner syndrome associated with acquired von Willebrand disease, primary biliary cirrhosis, and inflammatory bowel disease.We report a unique case of turner syndrome associated with acquired von Willebrand disease (AvWD), primary biliary cirrhosis (PBC), and inflammatory bowel disease (IBD). During 7 years of close follow-up, the patient presented with multiple major episodes of upper and lower gastrointestinal bleeding caused by different pathogenic mechanisms, such as IBD, AvWD, gastric varices, and thrombocytopenia. AvWD mimicking familial vWD type III on laboratory testing was most probably triggered by autoimmune mechanism associated with PBC. Therapy of PBC with ursodeoxycholic acid (UDCA) resulted in significant decrease of liver enzymes followed by normalization of vWF and FVIII levels. Portosystemic shunt placement with ligation of gastric varices improved hypersplenism and severe thrombocytopenia and led to clinical stability for more than 24 months. The clinicopathological features of these disorders and of the recurrent bleeding episodes are discussed in the text along with a review of the literature.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
4/9. A 38-year-old African-American woman with an unusually rapid progression of "Primary Biliary Cirrhosis": a missed opportunity!The case discussed is of a 38-year-old African-American woman who developed upper abdominal symptoms and liver test abnormalities. She underwent cholecystectomy for presumed gallstone disease. This was followed by a worsening of her condition, with the development of jaundice in the next 2 weeks. Results of reevaluation included transaminases around 1000 IU/L with minimal elevation of alkaline phosphatase (ALP), an antimitochondrial antibody (AMA) titer of 1:320, and an elevated immunoglobulin m (IgM). The antinuclear antibodies (ANA) level was positive, but titers were not obtained. There was no suggestion of bile duct obstruction. Liver biopsy findings were believed to be consistent with primary biliary cirrhosis (PBC). She was therefore started on, but failed treatment with, ursodeoxycholic acid. She was transferred to a transplant center 8 weeks later after a brief episode of encephalopathy and hypoglycemia. The clinical findings were consistent with subfulminant hepatic failure secondary to autoimmune hepatitis (AIH) with an ANA titer of 1:1280, an anti-smooth muscle antibody (SMA) titer of 1:40, and an elevated IgG. review of the biopsy showed panlobular inflammation and bridging necrosis consistent with severe AIH. On imaging, she had ascites and a nodular appearance of the liver. An immediate drop in transaminases followed corticosteroid therapy, but her disease was already irreversible, and she underwent successful liver transplantation. The explanted liver was shrunken and noncirrhotic with massive hepatocellular collapse and contained multiple regenerating nodules, explaining the ultrasonographic appearances. The inflammatory component had greatly diminished compared with the earlier biopsy. The case illustrates the importance of knowledge of the natural course of a specific disease and the careful interpretation of clinical data, including autoimmune markers. PBC would rarely cause liver failure in a young woman; it is not a rapidly progressive disease. The original clinical diagnosis was unduly swayed by a positive AMA, which can be seen in up to 20% of patients with AIH. Markedly elevated transaminases with minimal elevation of ALP and positive ANA in a young woman should have pointed toward AIH at an earlier stage. The academic discussion of AMA-positive AIH versus PBC/AIH overlap syndrome remains intriguing, but prompt institution of aggressive immunosuppressive therapy aimed at the AIH component should not be deferred. In retrospect, an opportunity was missed.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
5/9. Acquired factor v inhibitor complicated by Hashimoto's thyroditis, primary biliary cirrhosis and membranous nephropathy.A 59-year-old man diagnosed as having Hashimoto's thyroditis, primary biliary cirrhosis (PBC) and membranous nephropathy (MN) showed consciousness disturbance, convulsions of the upper part of his body, and rapid progression of anemia, which seemed to be derived from subdural and retroperitoneal hemorrhage, respectively. He had been diagnosed as having eosinophilia about 6 weeks before the attack. Coagulation tests revealed a prolonged activated partial thromboplastin time and prothrombin time, which could not be normalized by mixing with normal plasma. factor v (FV) activity was severely decreased and the purified immunoglobulin g of the patient inhibited normal plasma FV activity in a dose-dependent manner, suggesting the presence of antibody-mediated circulating inhibitors specific for FV. Treatment with steroids and azathioprine as well as plasmapheresis led to improvement of his clinical symptoms, normalization of the coagulation tests, and disappearance of eosinophilia. However, the inhibitor reappeared about 7 months later in association with eosinophilia, which was also improved by steroid therapy. To our knowledge, this is the first report of the co-existence of these three kinds of immune-mediated disorders, and the first report concerning the association between acquired FV inhibitors and PBC with MN. A new unknown immune mechanism, which causes eosinophilia, may be involved in the development of the FV inhibitor in this patient.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
6/9. Overlap syndrome between autoimmune hepatitis and primary biliary cirrhosis complicated by hepatocellular carcinoma.The risk of hepatocellular carcinoma superimposed in the evolution of autoimmune hepatitis or primary biliary cirrhosis is low, even in patients with long-standing cirrhosis. We report a case of hepatocellular carcinoma occurring in a 46 year old woman with liver cirrhosis following overlap syndrome between autoimmune hepatitis and primary biliary cirrhosis, routinely followed while on the waiting list for liver transplantation. The patient had combined biochemical (elevated aminotransferases, alkaline phosphatase and gamma-glutamyl-transpeptidase in the range of 2-3 times above the upper limit of the normal) and serological (anti-smooth muscle antibody > 1/80 and anti-mitochondrial antibody anti-M2 > 1/40) criteria of autoimmune hepatitis and primary biliary cirrhosis. Hepatocellular carcinoma was diagnosed in the setting of chronic liver disease by the combination of two concordant imaging technics (Doppler ultrasound and magnetic resonance imaging) showing a hepatic nodule with arterial hypervascularization and elevated serum levels of alpha-fetoprotein up to 950 ng/ml. liver transplantation is the best treatment both for the solitary nodule less than 5 cm and underlying autoimmune cirrhosis. Using the new Model for End-Stage Liver Disease allocation system our patient was placed in a prior position for liver transplantation (MELD 29). Unfortunately, a sudden fulminant liver failure complicated with intravascular disseminated coagulopathy was fatal for our patient while awaiting liver transplantation.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
7/9. Cutaneous lesions as presenting symptoms of primary biliary cirrhosis: an undifferentiated connective tissue disease-like onset.We describe two patients with primary biliary cirrhosis (PBC) who presented with specific symptoms mimicking an undifferentiated connective tissue disease (arthromyalgia, fatigue, cutaneous lesions either morbillous-like or urticarial, the latter with an eosinophil infiltrate of upper dermis). Subsequent detection firstly of eosinophilia in the blood and secondarily of antimitochondrial antibodies with results of liver biopsy allowed a diagnosis of asymptomatic PBC. In our cases, a peculiar sign of early stage of PBC was represented also by the eosinophilia in the liver.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
8/9. Biliary liver cirrhosis secondary to cystic fibrosis: a rare indication for liver transplantation.As more effective therapies prolong the lives of patients with cystic fibrosis, there are now more patients in this population diagnosed with liver diseases. Secondary biliary cirrhosis is not a rare complication of mucoviscidosis. It is diagnosed in 20% of patients with mucoviscidosis; in 2% it is accompanied by portal hypertension. On average patients with portal hypertension and its complications are 12 years old. liver transplantation is an accepted method of treatment for children with cystic fibrosis and portal hypertension. It eliminates the cause of the portal hypertension, decreases life-threatening medical conditions, and improves their nutritional status and quality of life. Despite immunosuppressive treatment they do not seem to beat increased risk of upper respiratory tract infections. On the contrary improved respiratory function and status are generally observed. We present our first case of orthotopic liver transplantation performed in a 29-year-old man with cystic fibrosis. The donor was a 42-year-old woman who died of a ruptured cerebral aneurysm. The surgery was performed in September 2004. The patient received immunosuppression based on steroids, basiliximab, tacrolimus, and mycophenolic acid due to renal insufficiency. Antibiotic (meropenem) and antiviral prophylaxis (gancyclovir) were used. A 6-month period of observation confirmed the clinical data from the pediatric population-a good prognosis with improved nutritional status, respiratory function, and quality of life.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
9/9. Idiopathic biliary ductopenia in adults: a report of five cases.The clinical and pathological findings of five adult cases of idiopathic nonsyndromatic paucity of interlobular bile ducts are reported. patients were 18-32 years old at the onset of the disease; four presented with pruritus and/or jaundice and one with bleeding of the esophageal varices. Two patients were siblings. serum alkaline phosphatase counts ranged from 1 to 16 times the upper normal value, and total bilirubin counts ranged from 0.6 to 8.8 mg/dL (10 to 150 mumol/L). Initial liver biopsy showed portal and periportal fibrosis with cholangiolar proliferation and reduction in the number of interlobular bile ducts. Antimitochondrial antibodies were absent, and bile ducts were normal after opacification. The patients were observed for 3-11 years. Repeated liver biopsies in the five patients showed progression of the lesions, with development of biliary type cirrhosis in four. Two of the four patients with cirrhosis died of hepatic failure 3 and 11 years after onset of the disease. In the two other cases, liver transplantation was performed successfully. These cases suggest that chronic cholestasis with marked ductopenia resembling the nonsyndromatic paucity described in infancy and childhood may reveal itself at an adult age. This disorder, possibly familial, may rapidly progress to severe and even fatal liver disease and could be a new indication for liver transplantation.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |