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Ménétrier's disease is a protein-losing gastropathy that is uncommon in childhood. Its symptoms are unspecific, with abdominal pain, vomiting, and edema. Blood tests show hypoproteinemia and hypoalbuminemia, and upper digestive endoscopy reveals giant gastric folds. In children, cytomegalovirus has been identified as a possible cause. Here we describe two sisters presenting with Ménétrier's disease, 2 years apart. This diagnosis should be considered in the presence of hypoalbuminemia in children when a nephrotic syndrome is excluded.

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BACKGROUND: Aminosalicylates are first-choice treatment for mild-to-moderately active ulcerative colitis (UC); however, multi-dosing regimens are inconvenient. AIM: To compare the efficacy and safety of once- (OD) vs. twice- (BD) daily prolonged-release mesalazine (Pentasa, Ferring, Saint-Prex, Switzerland) for active mild-to-moderate UC in a non-inferiority study. METHODS: Eligible patients (n = 206) were randomised to 8 weeks of mesalazine (4 g/day), either OD with two sachets of 2 g mesalazine granules in the morning (n = 102) or BD with one 2 g sachet in the morning and one in the evening (n = 104). Patients also received 4 weeks of mesalazine enema 1 g/day. Disease activity was assessed at randomisation, weeks 4, 8 and 12 using the UC Disease Activity Index (UC-DAI). Clinical and endoscopic remission (primary endpoint) was assessed after 8 weeks. Patients recorded stool frequency and rectal bleeding in a daily diary. RESULTS: The primary endpoint, non-inferiority in clinical and endoscopic remission with OD vs. BD mesalazine at 8 weeks, was met (intent-to-treat population: 52.1% vs. 41.8%, respectively, 95% confidence interval -3.4, 24.1; P = 0.14). Improvement of UC-DAI score (92% vs. 79%; P = 0.01) and mucosal healing (87.5% vs. 71.1%; P = 0.007) were significantly better, time to remission significantly shorter (26 vs. 28 days; P = 0.04) and safety similar with OD vs. BD dosing. CONCLUSIONS: When combined with mesalazine enema, prolonged-release mesalazine once-daily 4 g is as effective and well tolerated as 2 g twice-daily for inducing remission in patients with mild-to-moderately active ulcerative colitis (Clinicaltrials.gov: NCT00737789).

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BACKGROUND AND STUDY AIMS: The mortality rate from upper gastrointestinal bleeding (UGIB) remains high, at 5 % - 10 %. The aim of the current study was to describe the epidemiological characteristics, prognostic factors, and actual practice in a cohort of patients with UGIB admitted to French general hospitals. METHODS: From March 2005 to February 2006, a prospective multicenter study was conducted at 53 French hospitals. A total of 3298 patients admitted for UGIB were enrolled consecutively. Patient data were collected up to the date of discharge from hospital. RESULTS: Data were available for 2130 men and 1073 women (mean age 63 ± 18 years), one-third of whom were taking drugs that would increase the risk of UGIB. The two main causes of bleeding were peptic ulcers (38 %) and esophagogastric varices (EGV) or portal hypertensive gastropathy (24.5 %). Mean Rockall score was 5.0 ± 2.3. Endoscopy was performed on 96 % of patients (within 24 hours in 79 %), and 66 % of those with ulcers and 62.5 % of the EGV patients underwent hemostatic therapy when indicated. Rebleeding occurred in 9.9 % of the patients, and 8.3 % died. Independent predictors of rebleeding were: need for transfusion (odds ratio [OR] 19.1; 95 % confidence interval [95 %CI] 10.1 - 35.9); hemoglobin < 10 g/dL (OR: 1.7; 95 %CI 1.1 - 3.3); Rockall score (OR: 1.4 for each 1 point score increase; 95 %CI 1.0 - 1.9), systolic blood pressure < 100 mmHg (OR: 1.9; 95 %CI 1.4 - 2.5), and signs of recent bleeding (OR: 2.4; 95 %CI 1.7 - 3.5). Independent predictors of mortality were: Rockall score (OR: 2.8; 95 %CI 2.0 - 4.0), co-morbidities (OR: 3.6 for each additional co-morbidity; 95 %CI 2.0 - 6.3), and systolic blood pressure < 100 mmHg (OR: 2.1; 95 %CI 1.8 - 2.8). Rockall score, blood pressure and co-morbidities were taken as continuous variables meaning that the OR was 1.4 for every point increase, it was the same for blood pressure. CONCLUSION: UGIB still occurs mainly as a result of peptic ulcers and portal hypertension in France, and causes significant rates of mortality. There is scope for improvement via better prevention (better use of UGIB-facilitating drugs), endoscopic therapy, and management of co-morbidities.

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AIMS: Inflammatory bowel diseases (IBD) have a negative impact on patients' quality of life. The aims of this survey were to learn more about patients' concerns, and to compare their feelings with the beliefs of their close relations and physicians. PATIENTS AND METHODS: A specific questionnaire, including the RFIPC and the MFI-20, was used. Patients' answers were compared with those given by their close relations and attending physicians. RESULTS: This national survey included 2424 French patients. At the time of diagnosis, 73% of patients expressed having fears, but were also relieved to understand their symptoms. IBD was responsible for fatigue and weariness, and had a negative impact on daily, occupational, leisure, family and personal life. The main fears concerned unpredictable flare-ups followed by need for an ostomy bag and risk of surgery. The answers provided by close relations and physicians matched those of the patients, but physicians overestimated the patient's knowledge and underestimated disease impact. CONCLUSION: IBD has a deleterious effect on quality of life. Close relations of the patient realize the impact the disease has on the patients' life, but attending physicians still tend to minimize patients' symptoms.

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Managing irritable bowel syndrome (IBS) is difficult and often a source of dissatisfaction for the patient, explaining the increasingly frequent recourse to alternative treatments. These highly varied treatments are often associated. They can be classed into four categories: reflexology methods, interventions on the psyche, biological therapies, and treatments using certain forms of energy. Although some studies show interesting results, currently there are not sufficient scientific arguments to recommend one or another of these alternative treatments. Multicenter controlled studies are needed to better evaluate the strategies that appear to be cost-effective.

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BACKGROUND: Non-organ-specific autoantibodies (NOSAs) are frequently found in the sera of patients with Hepatitis C Virus (HCV) infection. However, no conclusive answers have been produced concerning the clinical relevance of these antibodies. AIM: To determine whether a relationship might exist between the presence of NOSA and the severity of liver disease in chronic hepatitis C. METHODS: 186 treatment-naïve chronic hepatitis C patients were studied consecutively for autoantibodies. Liver biopsies were analyzed according to the Metavir score. RESULTS: NOSAs were present in 75 patients (40%). Anti-nuclear antibodies were found in 32% of patients (speckled pattern), anti-smooth muscle in 15% without F-actin specificity, anti-mitochondria in 0.5%, and anti-LKM1 in 0.5%, respectively. No liver-cytosol1 or soluble liver antigen antibodies were detected. There was a highly significant correlation between the positivity of NOSA and the degree of inflammation and hepatocellular injury (p = 0.001) and also with the degree of fibrosis (p < 0.0001). The presence of NOSA was associated with higher aspartate aminotransferase, gamma-glutamyl-transpeptidase, gamma-globulin and immunoglobulin G levels. By contrast, no differences were observed regarding age, gender, route of infection, duration of disease, HCV genotypes or viral load. CONCLUSION: NOSAs were associated with the most severe forms of chronic HCV infections.

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Genomic imprinting at the H19/Igf2 locus is governed by a cis-acting Imprinting-Control Region (ICR), located 2 kb upstream of the H19 gene. This region possesses an insulator function which is activated on the unmethylated maternal allele through the binding of the CTCF factor. It has been previously reported that paternal transmission of the H19(SilK) deletion, which removes the 3' portion of H19 ICR, leads to the loss of H19 imprinting. Here we show that, in the liver, this reactivation of the paternal H19 gene is concomitant to a dramatic decrease in Igf2 mRNA levels. This deletion alters higher-order chromatin architecture, Igf2 promoter usage and tissue-specific expression. Therefore, when methylated, the 3' portion of the H19 ICR is a bi-functional regulatory element involved not only in H19 imprinting but also in 'formatting' the higher-order chromatin structure for proper tissue-specific expression of both H19 and Igf2 genes.

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BACKGROUND: Cystic dystrophy of aberrant pancreas, characterized by cystic formations in ectopic pancreatic tissue, produces variable signs. Treatment is difficult. CASE REPORT: Our patient presented signs of acute pancreatitis and angiocholitis due to cystic dystrophy of an aberrant pancreas situated in the duodenal wall associated with chronic alcoholic pancreatitis. Prolonged medical treatment was unsuccessful. Duodenopancreatectomy with preservation of the tail led to complete regression of the clinical signs. DISCUSSION: Signs of cystic dystrophy of an aberrant pancreas vary greatly. Diagnosis is generally established on the basis of echoendoscopic findings. After failure of medical treatment, duodenopancreatectomy with preservation of the tail could be the most appropriate surgical treatment, hypothesizing that the disorders result from obstruction of the Wirsung and the lower main bile ducts.

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Mesenteric panniculitis is a rare disease of the adipose tissue of mesentery. Ascites is an unusual presentation. We report a case of panniculitis whose initial presentation was an obesity-hypoventilation syndrome with pulmonary hypertension. Respiratory disorders were related to abundant ascites. Panniculitis cure and ascites disappearance allowed return to normal pulmonary function and pulmonary artery pressure.

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BACKGROUND: Cyclosporin is used for the treatment of corticosteroid-resistant inflammatory bowel disease. Secondary liver disease is a risk. CASE REPORT: Acute hepatitis with predominant major transaminase elevation occurred in a patient treated with cyclosporin for corticosteroid-resistant Crohns disease. No viral, alcoholic, autoimmune or metabolic cause could be incriminated. Complete cure was achieved after withdrawal of cyclosporin. DISCUSSION: Only one case of cholestatic hepatitis has been reported in chronic inflammatory bowel disease. Cyclosporin was the probable cause in our case as other causes of acute hepatitis were ruled out and withdrawal led to cure. Cyclosporin can induce abnormal liver tests in 25% of cases. If reducing dose does not lead to improvement, it may be necessary to discontinue cyclosporin. Regular liver tests would thus be required for patients given cyclosporin for chronic inflammatory bowel disease.

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Lymphocytic colitis is a clinico-pathological syndrome characterized by chronic diarrhea, normal endoscopy, diffuse colonic mucosal inflammatory changes. Collagenous colitis is defined by a thickening of the collagen plate. The etiology is unknown but immune disorders have been frequently associated with it and it has been linked with the taking of certain drugs such as nonsteroïd anti-inflammatory drugs or veinotonics. We are reporting a case of microscopic colitis associating both lymphocytic and collagenous colitis, wich induced chronic diarrhea in a 65-year-old man. It appeared after he had taken ticlopidine. Diarrhea stopped after he had discontinuated ticlopidine, and recurred after he resumed taking the drug. Histological damages from lymphocytic colitis improved six month after he had stopped taking ticlopidine. Chronic diarrhea induced by ticlopidine might be caused by lymphocytic colitis.

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Amplification of the 16S rRNA gene of Tropheryma whippelii was performed in eight patients with Whipple's disease and 34 control patients to confirm a diagnosis of Whipple's disease and to monitor the course of disease. Polymerase chain reaction (PCR) tests were positive before treatment in 13 of 15 tissue samples from Whipple's disease patients (gut 8/8; lymph nodes 2/2; bone marrow 1/2; peripheral blood 2/3), in contrast to none of 54 tissue samples from controls. PCR tests converted to negative within 4-6 months in six of the Whipple's disease patients undergoing therapy. These results show that PCR is a reliable and useful tool for diagnosis of Whipple's disease and for monitoring bacterial elimination during antibiotic therapy.

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We report five cases of herpetic esophagitis in human immunodeficiency virus non-infected patients. Herpetic esophagitis is a frequent infection in immunocompromised patients. However, sixty four cases of herpetic esophagitis have been reported in immunocompetent patients. The infection presents non-specific esophageal symptoms. Diagnosis relies on esophageal histology and viral cultures. Treatment by acyclovir is recommended to prevent severe complications. This infection is probably underestimated in immunocompetent hosts but seems to be more frequent in patients with severe illness that can be responsible for transitory immunodeficiency. Biopsies of esophageal lesions should be systematically performed in these patients.

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BACKGROUND/AIMS: Lipiodol chemoembolization is a widely used method of treatment in patients with unresectable hepatocellular carcinoma, but its efficacy is still debated. The aim of our study was to assess the efficacy of lipiodol chemoembolization in patients with unresectable hepatocellular carcinoma. METHODS: Seventy-three patients with unresectable hepatocellular carcinoma, but without severe liver disease or portal vein occlusion, were randomly assigned to receive either repeated lipiodol chemoembolization (lipiodol, cisplatin (2 mg/kg), lecithin, and gelatin sponge injected into the hepatic artery) plus tamoxifen (40 mg) or tamoxifen alone. The main end-point was survival. RESULTS: The 37 patients in the lipiodol chemoembolization group received 104 courses (median 3 per patient). By 1 September 1996, 58 patients had died: 30 in the lipiodol chemoembolization group and 28 in the tamoxifen group. There was no difference in survival between the two groups (p=0.77). The relative risk of death in the lipiodol chemoembolization plus tamoxifen group as compared to the tamoxifen group was 0.92 (95% confidence interval 0.55 to 1.56). At 1 year, survival was 51% and 55%, respectively. An objective tumoral response was more frequently observed in the lipiodol chemoembolization group than in the tamoxifen group (24 versus 5.5%, respectively, p=0.046). Lipiodol chemoembolization caused two deaths and induced signs of liver failure in 51% of the patients assigned to this treatment. CONCLUSION: In our randomized study, lipiodol chemoembolization did not improve the survival of patients with unresectable hepatocellular carcinoma treated with tamoxifen.

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Hepatitis C is frequently associated with immune-mediated diseases, such as cryoglobulinemia. Guillain-Barré syndrome is an acute demyelinating neuropathy of probable immune pathogenesis. We describe two patients with Guillain-Barré syndrome, and associated chronic hepatitis C, the second one previously treated with interferon. The link between both conditions may be hepatitis C being the trigger of this immune polyneuropathy. Guillain-Barré syndrome should be added to the list of conditions associated with hepatitis C.

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