Liver transplantation for Wilson's disease: The burden of neurological and psychiatric disorders

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Effect of moderate exercise on Crohn's disease patients in remission.

Background. Physical exercise may exacerbate the disturbed homeostasis of Crohn's disease patients. Aim. To examine the effect of moderate physical exercise on gastrointestinal function in a group of Crohn's disease patients in remission. Patients and Methods, The effect of one-hour's exercise at a maximum of 60% oxygen consumption was evaluated in six males with ileal Crohn's disease in remission on orocaecal transit time (breath rest to lactulose), intestinal permeability (6-hours' urinary excretion of a sugar mixture of lactulose/mannitol), polymorphonuclear leucocytes function (peripheral blood chemiluminescence), lipoperoxidation (plasma malondialdehyde) and antioxidant trace elements (urinary and plasma zinc and copper concentrations). Six healthy age-matched subjects sewed as controls. Results. Exercise did nos elicit subjective symptoms or changes in intestinal permeability and lipoperoxidation. orocaecal transit time increased after exercise in Crohn's disease patients (72 min+/-30 vs 100 min+/-34) with no significant difference from controls (77 min+/-20 vs 83 min+/-23). Neutrophils, primed pre-exercise in Crohn's disease patients showed an increased post-exercise chemiluminescence similar to controls. Zinc urinary output significantly increased after exercise in Crohn's disease patients and remained unchanged in control subjects. Conclusions. Moderate aerobic exercise has no significant effect on the gastrointestinal parameters examined. However; basal neutrophil activation and exercise in Crohn's disease patients may trigger an excessive production of oxygen metabolites. Moreover; exercise may contribute to an increased risk of zinc deficiency

Zinc therapy increases doudenal concentrations of metallothionein and iron in Wilson's disease patients

Objective: Wilson's disease is effectively treated by zinc administration which, in vitro, increases metallothionein concentrations. To ascertain whether the latter also occurs in humans we measured metallothionein and trace element concentrations in the duodenal mucosa of 15 Wilson's disease patients: 12 treated with zinc sulphate, two treated with penicillamine, and one not yet on treatment. The control group consisted of 17 patients with dyspepsia, who underwent the same study protocol. Methods: Metallothionein and trace element concentrations were measured in duodenal mucosa biopsies according to the silver-saturation hemolysate method and atomic absorption spectrophotometry. Results: Metallothionein concentrations increased by 1500% after zinc and 150% after penicillamine in Wilson's disease patients, with respect to controls who had negative endoscopy and Wilson's disease patients who were not treated. A significant correlation was found between metallothionein and duodenal zinc concentrations. Mucosal iron concentration increased in Wilson's disease patients whether they were treated with zinc or penicillamine, Duodenum with duodenitis also had significantly increased iron levels compared with normal duodenum, Conclusions: Zinc administration increases intestinal metallothionein in Wilson's disease patients. The blockade of copper absorption and its elimination in the stools on desquamation of the intestinal cells probably explains one of the mechanisms underlying the effect of zinc treatment, Despite normal endoscopy, Wilson's disease patients present increased mucosal iron concentrations similar to those in controls with duodenitis. Metallothionein may therefore prevent oxidative damage caused by metal toxicit

Pancreaticoduodenectomy for dysplastic duodenal adenoma in a patient with familial adenomatous polyposis

Colorectal polyposis is the main feature of familial adenomatous polyposis (FAP) but benign and malignant lesions have also been described in the stomach, duodenum, small bowel, biliary tract and pancreas. There are few reports on FAP patients with duodenal polyps that developed at a younger age and even fewer oil cases with dysplastic degeneration. The progression to carcinoma usually presents quite late in the clinical history of FAP patients, typically at least 20 to 25 years after proctocolectomy. This report described the rare case of a patient presenting with duodenal adenomas with dysplastic changes and tumor infiltration as the first sign of FAP, who was treated by pancreaticoduodenectomy followed by proctocolectomy for subsequent dysplastic changes in colonic polyps

Influence of disease site and activity on peripheral neutrophil function in inflammatory bowel disease.

Reactive oxygen species, released by phagocytes, are involved in tissue injury in inflammatory bowel diseases. The aim of our study was to evaluate peripheral neutrophil function in patients with ulcerative colitis (N = 66) and Crohn's disease (N = 62) with respect to disease activity and extent, using chemiluminometry after three stimuli. Twenty-seven healthy subjects were enrolled as controls. Neutrophils from ulcerative colitis and Crohn's disease patients had a significantly higher response than those from controls following phorbol myristate acetate (86.6 +/- 6.5, 173.8 +/- 11.9, 167.5 +/- 12.2 mV, P < 0.0001), formylmethionyl-leucyl-phenylalanine (39.5 +/- 3.4, 41.3 +/- 2.7, 58.6 +/- 4.7 mV, P < 0.001), and zymosan (142.6 +/- 10.4, 223.7 +/- 8.9, 231.2 +/- 9.5 mV, P < 0.0001) administration. The increased response was observed during both active disease and remission. The highest chemiluminescence values were found in patients with active ulcerative pancolitis and ileal Crohn's disease. The activation of circulating neutrophils may indicate persistent intestinal inflammation or may be triggered by luminal factors even in the absence of symptoms

The effect of heparin on trinitrobenzene sulphonic acid-induced colitis in the rat.

Background: Reduced blood coagulability seems to protect against inflammatory bowel disease; pilot studies using heparin in patients with inflammatory bowel disease have reported positive results, Aim: To evaluate the effects of heparin treatment on microangiographic and on inflammatory parameters in experimental colitis, induced by trinitrobenzene sulphonic acid (TNBS)-ethanol, Methods: Four groups of rats: (i) controls (saline enema), TNBS-induced colitis with (ii) sham treatment (saline, s.c.), (iii) dexamethasone (0.25 mg/kg/day s.c.) and (iv) heparin (500 U/kg t.d.s., s.c.), Microangiography was performed 2 and 4 days after colitis induction, Partial thromboplastin time. colonic wet weight, macroscopic damage score and mucosal myeloperoxidase (MPO) activity were determined at day 4. Results: TNBS-induced colitis caused a reduction in visible bowel wall vessels, which was prevented by heparin (P < 0.05) but not by steroids. The macroscopic damage scores and colon wet weights were similar in all colitis groups, Compared to untreated colitis the MPO activity in heparin-treated animals was of borderline significance. Conclusions: Heparin treatment improved microangiographic features and reduced inflammation to a certain degree. Steroids delayed development of colon hypoperfusion, but were ineffective on MPO activity, It remains to be determined if the observed effects are due to the antithrombotic activity of heparin or to an antiinflammatory action