1/7. Recanalization of a portal-vein thrombosis and partial resolution of biliary cirrhosis following cholecystectomy for cholelithiasis in an infant.cholelithiasis in infants and children is unusual and is reputedly associated with underlying hemolytic disorders, total parenteral nutrition, ileal disease, and congenital anomalies of the biliary tree. We report a case of cholecystitis with pigmented stones in a 3-month-old infant associated with portal vein thrombosis (PVT) and biliary cirrhosis without the above-mentioned causes. Recanalization of the PV and partial resolution of the cirrhosis was observed following cholecystectomy. To our knowledge, cholelithiasis associated with PVT and cirrhosis in an infant has not been reported in the literature.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/7. Prolonged cholestasis and ductopenia associated with tenoxicam.Cholestatic liver diseases leading to progressive destruction of intra-hepatic bile ducts and ductopenia encompass multiple etiologies. Pathophysiology and natural history of drug-induced cholangiopathies remain unclear. We report a case of prolonged ductopenia attributed to Tenoxicam (Tilcotil o--a non-steroidal anti-inflammatory drug of the oxicam family) ingested at therapeutic dose. A 36 year-old male patient was admitted for jaundice and Lyell syndrome starting 1 week after the ingestion of Tenoxicam. Liver biopsy showed cholestasis, non-suppurative cholangitis and polymorphous inflammatory infiltrate of the portal tracts (round cells, macrophages an eosinophils). Treatment with ursodesoxycholic acid and cholestyramine was instituted and the patient was asymptomatic 1 year after. Three years later mild biological cholestasis persisted and ductopenia was evidenced on liver biopsy. In this report we found that: (1) The toxicity of tenoxicam was probably mediated by an immunoallergic mechanism (Lyell syndrome and eosinophils on histology); (2) ductopenia was secondary to inflammatory cholangitis. Factors responsible for this chronic evolution are still unknown (genetic predisposition, vascular factors, etc.); and (3) the presence of ductopenia contrasted with the "clinical recovery" of the disease suggesting accessory bile drainage by cholangioles or partial reconstruction of the biliary tree.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
3/7. An autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver.A rare autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver is described. The patient, a 62-year-old female at the time of autopsy, presented with prolonged cholestasis of about 9 years duration. Initial (at 53 years) and second (at 59 years) liver biopsies disclosed fibrous enlargement of the portal tracts with loss of interlobular bile ducts, lymphoplasmacytic infiltration, a few epithelioid granulomas, piecemeal necrosis, atypical ductular proliferation and deposition of copper granules. hypergammaglobulinemia with elevated IgM was also noted. These clinicopathological features resembled primary biliary cirrhosis. However, no florid duct lesions were found, and absence of antimitochondrial antibodies and cholangiographic demonstration of a beaded biliary tree favored a diagnosis of primary sclerosing cholangitis. The autopsied liver disclosed sclerosis and cholangioectases of the intra- and extrahepatic biliary tree in addition to biliary cirrhosis. The histology of the biliary tree disclosed nonspecific fibrosing inflammation in the extra- and intrahepatic biliary tree. Other autopsy findings included chronic thyroiditis, sialoadenitis and pancreatitis.- - - - - - - - - - ranking = 4keywords = tree (Clic here for more details about this article) |
4/7. Chronic active hepatitis with histological features of primary biliary cirrhosis.A 51-year-old woman was evaluated because of Raynaud's phenomenon, sjogren's syndrome, and general malaise. There was neither skin itching nor jaundice. Endoscopic retrograde cholangiopancreatography showed a normal extrahepatic as well as intrahepatic biliary tree. serum GOT and GPT fluctuated with episodes of marked increases. The alkaline phosphatase was slightly increased and total cholesterol was normal. There were marked increases of IgG and IgM. AMA was positive at a titer of 1:320, which was measured by an indirect immunofluorescence method. PBC-specific AMA (anti-M2) was positive, but mixed-form AMA (anti-M4) negative. An LE-cell test, ASMA, ANA, and anti-dna antibody were all positive on several repeated tests. A wedge liver biopsy tissue showed mixed features of PBC and CAH. A diagnosis of CAH was made on the basis of the clinical, serological, and morphologic findings. The patient responded well to prednisolone treatment with prominent improvement of her symptoms and liver function tests. Subsequently, AMA fell to undetectable levels by indirect immunofluorescence method.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
5/7. Familial intrahepatic cholestatic cirrhosis in young adults.Two siblings with intrahepatic cholestatic cirrhosis and their brother, who had a potentially related disease at the time of accidental death, are presented. The onset of disease occurred during adolescence in all 3 cases. The initial sign was mild jaundice or portal hypertension. There was no abnormality in the countenance, cardiovascular system, or vertebral column. Except for the brother who died from an accident, jaundice gradually increased. death followed due to cirrhosis. Liver biopsy specimens of these 2 patients showed diminution of interlobular bile ducts with no significant cholangitis. At autopsy, the livers of the 2 patients showed biliary cirrhosis without extrahepatic biliary obstruction. In both cases there was an accessory lobe on the right hepatic lobe. Histologically, septal bile ducts showed pronounced papillary proliferations of the epithelium; there was also a decrease in the number of small interlobular bile ducts. Excess copper accumulation in the liver was ascertained. It is suggested that the disease in the 2 autopsied cases is intrahepatic cholestatic cirrhosis due to hypoplasia of the intrahepatic biliary trees.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
6/7. Florid duct lesions and extensive bile duct loss of the intrahepatic biliary tree in chronic liver diseases other than primary biliary cirrhosis.Intrahepatic biliary tree with either florid duct lesions or a moderate to severe degree of the duct loss in four livers with chronic hepatic diseases other than primary biliary cirrhosis were studied with histometric and serial section observations. Florid duct lesions, distributed segmentally in the liver, were found in one case with incomplete septal cirrhosis and one case with idiopathic portal hypertension. The florid duct lesions including marked plasma cell infiltration and occasional periductal granulomas, were not associated with any bile duct loss in the two cases. The duct lesions were reversible in one case during a long clinical course. On the other hand, a moderate to severe bile duct loss with biliary epithelial degeneration and necrosis was associated with no or little periductal inflammatory cell infiltration in one other case with chronic intrahepatic cholestasis, probably drug-induced, and in one case with idiopathic portal hypertension. Although florid duct lesions and bile duct loss were important diagnostic features of primary biliary cirrhosis, one of them was observed to develop independently in severely diseased livers, not consistent with a diagnosis of primary biliary cirrhosis, sclerosing cholangitis or intrahepatic bile duct paucity syndrome.- - - - - - - - - - ranking = 5keywords = tree (Clic here for more details about this article) |
7/7. Percutaneous transhepatic cholangiography with the "skinny" needle. A rapid, simple, and accurate method in the diagnosis of cholestasis.One hundred thirty-one cases of cholestasis were evaluated by "skinny" needle percutaneous transhepatic cholangiography. The biliary tree was seen in 129 patients (98.5%). When the biliary tree was dilated, a success rate of 100% was obtained. In the 45 patients with nondilated ducts, visualization was achieved in 95.6%. There was no mortality, early operation, or transfusion requirement associated with the procedure. This form of cholangiography is a simple, reliable, and extremely high-yield technique for evaluating patients with cholestasis.- - - - - - - - - - ranking = 2keywords = tree (Clic here for more details about this article) |