In two out of three cases of idiopathic fibrosis of the mediastinum there was also retroperitoneal fibrosis, and in one of them extensive fibrosis of the lungs. One of these, a fit middle-aged man, died suddenly with severe coronary artery stenosis due to fibrosis. The third case was that of a young man with a solitary fibrous mass around the right lower lobe bronchus mimicking a malignant tumour and producing bronchial stenosis. Of the various hypotheses about the aetiology and pathogenesis of the fibrous proliferation, that of an autoimmune process seemed the most likely in these cases.
Background-Butyrate is an important energy source for the colon and its metabolism has been reported to be defective in ulcerative colitis. One mechanism for defective butyrate metabolism in patients with ulcerative colitis could be an enzyme deficiency in the P-oxidation pathway of butyrate. Aims It is possible that abnormal butyrate oxidation may occur in the colonic epithelial cells of patients with quiescent ulcerative colitis. Such an abnormality may be of pathogenetic significance and therefore requires further study. This study set out to measure the activity of each enzyme involved in oxidation of butyrate (Fig 1).
Methods
Tissue samplesPrior to assay all tissue samples were coded so that the biochemist performing the assays was unaware of the disease group of the donor patient. It was not possible to assay all enzymes in the oxidation pathway in each (range 28-9 1) years and 39 were male. All mucosal specimens in these control patients were taken from a site at least 10 cm from any macroscopic abnormality and adjacent mucosal samples were histologically normal. Sixty of these mucosal specimens were taken at laparotomy and 13 at rigid sigmoidoscopy.Mucosal specimens were taken from 33 subjects with ulcerative proctocolitis, median age 46 (range 18-83) years, 18 were men. Adjacent mucosal specimens were taken in each case for histological examination and assessment of severity of ulcerative colitis. Mucosal specimens were taken from 13 patients at laparotomy and 20 at rigid sigmoidoscopy.Mucosal specimens were taken from 10
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