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This case-report studies the clinical, radiological, anatomopatholo-gical and therapeutic aspects of peripheral primitive neuroectodermal tumours (PNET). PNET are neoplasms with a similar histology to tumours in the Ewing family. Diagnosis requires histopathology, immunohistochemistry and cytogenetic studies. To our knowledge, this rare tumour has never been reported in the mesocolon (our case). The treatment, which is usually similar to that for Ewing's sarcoma is complex and has not yet been codified, which is another difficult aspect of this disease with a poor prognosis.

The association of congenital absence of the portal vein and hepatocellular carcinoma has been described, but is rare. It is most frequently discovered fortuitously in children. The absence of intrahepatic portal circulation may predispose to the development of hepatocarcinoma.

Objective: The aim of this study was to assess the performance of magnetic resonance cholangiography (MRC) in the preoperative diagnosis of choledocholithiasis., Methods: A total of 147 consecutive patients underwent MRC for clinical and biological signs of common bile duct stones. ERCP was then carried out in 101 patients in whom there was a past history of cholecystectomy. The remaining 46 patients without a past history of biliary surgery underwent cholecystectomy and intraoperative cholangiography (IOC). The diagnosis obtained by MRC was compared with the final diagnosis established after endoscopic or surgical removal of calculi., Results: A total of 113 patients had choledocholithiasis (single or multiple, including 15 cases of microlithiasis). There were no false-positive results with MRC. The false-negative results were caused mainly by small stones <3 mm in diameter, and to a lesser extent, cholangitis. Overall, the sensitivity was 93% and the specificity 100% for MRC in detecting common bile duct stones. The sensitivity and specificity of ERCP were respectively 94% and 100%, versus 93.5% and 93.3% for IOC. There was no statistically significant difference, however, between MRC and the other techniques., Conclusion: MRC is a key technique in the preoperative diagnosis of choledocholithiasis. Its diagnostic value is comparable to ERCP, but it appears to be more specific than IOC. Nevertheless, its diagnostic capability remains limited in cases of microlithiasis and cholangitis.

Objectives: To describe the characteristics of in-patients with alcoholic liver disease in Hepatogastroenterology and to evaluate whether geographic location was a risk factor for cirrhosis., Methods: A French, national, multicenter, prospective investigation was performed in the last quarter of 1997. To be included in the study, patients had to have drunk at least 50 g of alcohol per day for the past year or to have cirrhosis., Results: Seventeen centers included 802 patients, 20% had histologically proven cirrhosis or probable cirrhosis. Thirty-five percent had undergone liver biopsy. Twenty five percent of these patients had cirrhosis without acute alcoholic hepatitis and 37% had cirrhosis with acute alcoholic hepatitis. After dividing France along a Bordeaux-Strasbourg axis, there was more histologically proven or probable cirrhosis in the North (46%) than in the South (36%) (P<0.005) while daily alcohol intake was greater the South (150 +/- 6 g) than in the North (129 +/- 4 g) (P<0.0001). When the six variables (age, sex, daily consumption of alcohol over the past 5 years, presence of hepatitis B surface antigen and antibodies to hepatitis C virus, total duration of alcohol abuse) were considered together in stepwise logistic regression analysis, geographic location changed the prediction of cirrhosis. The odds ratio for cirrhosis in patients living to the North of the Bordeaux-Strasbourg axis was 1.9 (95% confidence interval range 1.1-3.2) (P<0.02), suggesting the role of nutritional factors.

Amprenavir (141W94) is extensively metabolized by P450 cytochromes, specifically, CYP3A4. Because hepatic insufficiency reduces P450-mediated metabolism, the concentrations in plasma of drugs metabolized through this pathway are often increased in subjects with liver disease. Following administration of a single, oral dose of 600 mg of amprenavir, pharmacokinetic parameters were determined for 10 subjects with severe cirrhosis, 10 subjects with moderate cirrhosis, and 10 healthy volunteers. Model-independent methods for determining the area under the plasma concentration-time curve (AUC) from time zero to infinity (AUC(0-infinity)) showed an increase in amprenavir AUC(0-infinity) of 2.5-fold in the group with moderate cirrhosis and 4.5-fold in the group with severe cirrhosis compared with that in the control group of healthy volunteers (P < 0.05). AUC(0-infinity) was linearly related to the severity of liver disease, as assessed by the Child-Pugh score. Of the laboratory data used to calculate the Child-Pugh score, only the mean total bilirubin concentration showed a significant relationship with AUC(0-infinity). The relationship between the total bilirubin concentration and the AUC(0-infinity) of amprenavir was well characterized by a simple E(max) model, suggesting that the total bilirubin concentration may be a useful parameter for predicting the amprenavir AUC in subjects with hepatic insufficiency. Finally, the sera of cirrhotic subjects showed significant decreases in the levels of alpha(1)-acid glycoprotein, the primary plasma binding protein for amprenavir. On the basis of the results of this study, for an exposure equivalent to a clinical dose of 1,200 mg twice daily in subjects without cirrhosis, subjects with Child-Pugh scores of 5 to 8 should receive a twice-daily 450-mg dose of amprenavir, and subjects with Child-Pugh scores of 9 to 15 should receive a twice-daily 300-mg dose of amprenavir.

Objective: The aim of this study was to evaluate magnetic resonance cholangiography (MRC) in the diagnosis of biliary tree obstruction., Methods: Fifty-eight consecutive patients underwent MRC (GYROSCAN ACS II 1.5 Tesla, TSE T2 axial/coronal-MIP sequences) for clinical and biochemical signs of main bile duct obstruction. MRC images were interpreted by two radiologists and consensus was established according to presence or absence of main bile duct dilation, choledocholithiasis, and malignant or benign stricture. MRC was compared to a final diagnosis established by ultrasound and CT in 19 cases, endoscopic retrograde cholangiopancreatography (ERCP) in 25, intraoperative cholangiography and exploration in 14, and clinical, biochemical, and histological presentation when relevant. Included were single or multiple choledocholithiasis (28, including 11 < or = 3 mm), malignant (10) and benign (12) strictures, and intrahepatic cholestasis (9)., Results: Overall, MRC was sensitive (94%) and specific (92%) in detecting main bile duct dilation and choledocholithiasis (86 % and 97 %), but was less sensitive (64%) for small stones < or = 3 mm. Sensitivity for stones > 3 mm was 100%. For benign and malignant strictures, MRC was less sensitive (67% and 80%) but remained specific (98% and 96%). In the detection of normal main bile duct, MRC was highly sensitive (100%) and specific (94%). Diagnostic accuracy ranged from 91% to 98%., Conclusion: MRC appears to be specific for choledocholithiasis and sensitive except for small stones. Results for biliary stricture are less satisfactory, but remain specific. Our data confirm that MRC can be useful in the diagnostic workup of main bile duct obstruction.

Objectives: The aim of this study was to determine whether a medium and long chain triglyceride mixture for parenteral nutrition is used in accordance with indications and contraindications in hospital practice., Methods: Patient data recorded in 30 consecutive patients included illness, nutritional status, laboratory findings before nutrition as well as indications and contraindications for parenteral nutrition., Results: When expressed in g.kg-1.day-1 maximal recommended doses of the mixture were exceeded in 32% but there was no excess when expressed in g.kg-1.hr-1. Serious hepatic insufficiency was present in 11% of the patients, 38% had hypertriglyceridemia and one had serious coagulopathy. There were 3 contraindications for the mixture., Conclusion: Indications for using this emulsion were respected, but there were contraindications in 45% of the cases. These contraindications are however questionable as is the daily dosage. Because the mixture seems better for use in many cases of parenteral nutrition, it would appear best to discuss the prescription case by case.

Digestive epilepsy is a rare disease, poorly recognized by gastroenterologists. Its diagnosis requires a compatible clinical presentation, the absence of concomitant organic digestive disease, and an effective and long-lasting response to specific anticonvulsant agents. We report a case of digestive epilepsy due to a meningioma of the right parietal lobe in a 79-year-old woman suffering from headaches, vertigo, sweating and abdominal pain for at least 14 years. Initial diagnosis was irritable bowel syndrome. A meningal syndrome led to neurological work-up showing cerebral meningioma. The recurrent paroxysmal abdominal pain was interpreted as manifestations of digestive epilepsy, and effective and long-lasting treatment was obtained with carbamazepine. After analysis of the determining elements in this case, the epidemiology, pathophysiology, diagnostic work-up, therapy, and differential diagnosis of digestive epilepsy are discussed.

The aim of this study was to assess the ultrasonographic abnormalities of the rectal wall and surrounding structures in patients with cirrhosis and to correlate these findings with endoscopy. From November 1992 to May 1993, 53 cirrhotic patients and 30 control subjects were examined by transrectal ultrasonography and rectoscopy. In addition to rectoscopy and transrectal ultrasonography, patients with cirrhosis underwent an upper gastrointestinal endoscopy. Ultrasonography abnormalities of the rectum were detected in 32 cases of cirrhosis (60.5%): a) in 21 cases the rectal wall thickness was greater than 5 mm and the sub-mucosa was thickened ( > or = 2 mm) and dissected by thin echo-free elements; b) the remaining 11 patients presented, in addition to the previously described abnormalities, rounded elongated echo-free structures surrounding the rectum which were not found in the control group. All these 11 patients had rectal varices at rectoscopy. In the group of cirrhotic patients, rectoscopy showed abnormal dilated veins in 23 cases (43.5%) and rectal varices in 11 cases. Ultrasonographic and endoscopic abnormalities of the rectum were more often visualized in the group of cirrhotic patients than in the control group (P < 0.001). In patients with cirrhosis, the demonstration of rectal varices was associated with transrectal ultrasound abnormalities (P < 0.01), and in particular peri-rectal vascular formations. In cases of cirrhosis the rectal wall abnormalities were not associated with gastric varices at endoscopic inspection but were linked with large esophageal varices or gastric mucosal abnormalities demonstrated on endoscopy (P < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

We undertook a multicenter randomized trial to compare the efficacy of terlipressin combined with transdermal nitroglycerin and that of octreotide in the emergency control of acute variceal hemorrhage in cirrhosis. Over 16 mo, 87 patients with endoscopically proved active bleeding from esophageal or cardiac varices were enrolled in five centers in France and randomly assigned to receive intravenous terlipressin (2 mg and then 1 mg/4 hr over 24 hr) and transdermal nitroglycerin (10 mg/12 hr over 24 hr) (group 1) or octreotide (continuous intravenous infusion of 25 micrograms/hr over 12 hr and then 100 micrograms at hr 12 and hr 18 subcutaneously) (group 2). Initial control of bleeding was assessed at the end of 12 hr of treatment on the basis of stability of blood pressure and hematocrit level with no further transfusion requirements. At 12 hr, bleeding was controlled in 59% (24 of 41) in group 1 and 78% (36/46) of group 2 patients (Fisher's exact test, p = 0.064). Mean transfusion requirements over this 12-hr period were significantly greater in group 1 (three blood units; range = 0 to 13) than in group 2 (one blood unit; range = 0 to 5) (p = 0.002). After the first 12 hr, 20% of patients (5 of 24) had repeat bleeding in group 1 compared with 27% (10 of 36) in group 2. During the first 48-hr period, five patients (12%) died in group 1, compared with 3 (6%) in group 2. Few side effects were noted in either group. However, in group 1 two patients experienced severe bradycardia; it resulted in death in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)

The deposits obstructing 12 biliary endoprostheses were examined by scanning electron microscopy and analyzed quantitatively by Fourier transform infrared spectroscopy. Calcium palmitate and bilirubinate associated with proteins were found in all the deposits. Apart from the small amount of cholesterol, this composition closely resembled that of brown pigment gallstones and suggested an infectious cause. Facultative aero-anaerobic organisms were found on bacteriological investigation. beta-Glucuronidase activity was only detected in one sample, suggesting that the calcium bilirubinate may have been formed through tissue glucuronidases. In our study, deposit formation was thought to have been caused by the action of bacterial phospholipases (although not measured) rather than beta-glucuronidases.

We report the case of a child suffering from a graft versus host disease after an allogenic bone marrow transplantation. He developed intestinal, hepatic and cutaneous symptoms. The outcome after treatment was favorable with a total normalization.

We report the case of a young man presenting clinical features of portal hypertension of unknown origin. The ultrasonographic examination showed suprahepatic blockage signs leading to the diagnosis of Budd-Chiari syndrome caused by incomplete diaphragm of the inferior vena cava. The color Doppler examination suggested caval obstruction, showing an abrupt shift from a laminar to a bosterous flux. Secondly cavograms and surgery later confirmed the diagnosis.

A randomized trial was undertaken to determine the efficacy of nitroglycerin in addition to terlipressin infusion to improve the control of acute variceal hemorrhage compared with balloon tamponade. Forty-seven bleeding episodes in 34 cirrhotic patients were included, with terlipressin plus sublingual nitroglycerin in 23 episodes (group I) and balloon tamponade in 24 episodes (group II). At the end of the 12-hr period, hemorrhage had been equally controlled in both groups (18 of 23; 78% in group I and 19 of 24; 79% in group II). When attempted, balloon tamponade appeared more efficient in the failures of group I (4 of 4) than did terlipressin plus nitroglycerin in the failures of group II (0 of 3), although this difference was not significant. Major complications were rare (one in each group) and never required cessation of therapy. Thus terlipressin and nitroglycerin were as effective as balloon tamponade in controlling variceal hemorrhage and were associated with few minor complications.

The authors report the case of a 44 year old man with a giant leiomyoma of the lower third of the esophagus. The patient presented with chest pain and the tumour was detected by echocardiography. The diagnosis was confirmed by computerised tomography and histological examination of the surgical specimen weighing 501.5 g. The surgeon performed a large esophago-gastric resection and reestablished the continuity of the digestive track by interposing a section of colon. A good result was obtained with a follow-up of 4 years. The authors underline the potential value of a simple barium swallow during cardiological assessment.

We report the case of a 65 year-old woman treated by radiation therapy alone for Hodgkin's disease. She developed febricule, nonicteric cholestasis, and inflammatory syndrome two months after irradiation. The clinical record, biological and histological results allowed us to dismiss another etiology especially an hepatic location of Hodgkin's disease. Out come after a twenty months predominance of pathohistological abnormalities in the centrilobular region were in favor of a radiation-induced hepatitis.

The authors report the case of a patient with acute alithiasic cholecystitis associated with viral B hepatitis revealing periarteritis nodosa. Histopathological results showed signs of focal arteritis in the gallbladder and liver. Because of the negativity of the viral DNA in serum and the lack of histopathological necrosis in hepatic specimen, the patient was treated by steroid therapy only with a rapid regression of signs of vasculitis and the disappearance of the hepatitis markers.

The authors report about one case of degenerated adenoma of the liver without any inducing hormonal therapy in a male patient. This case seems to be the first known to date. The authors discuss its aspects according to the data given by the literature.