[Adult coeliac disease. II. Relationship between the severity of the disease and the extent of the histological small bowel lesions (author's transl)].
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Biomedical subjects
Publications and source records attributed to C Matuchansky.
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In a patient with occupational plumbism and a normal kidney function, EDTA therapy (1 g/24 h) resulted in a massive lead excretion (15 000 microgram/24 h) together with acute renal failure. A direct nephrotoxic effect of EDTA was excluded: after renal function returned to normal, EDTA could be subsequently readministered at progressively increasing doses (250 mg to 1 g/24 h) without any renal dysfunction. These findings strongly suggest a close relationship between the burden of lead mobilized by EDTA and the acute renal failure. This case can be classified as acute lead nephropathy of which only a few instances have been reported in the literature. The interest of monitoring lead excretion during chelation therapy is particularly emphasized.
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The slow marker perfusion technique was used in five patients with the Zollinger-Ellison syndrome in order to determine the basal and postcibal flow rates of fluids passing the duodenojejunal junction and distal ileum, and the composition of those fluids. Fecal water and electrolyte excretions were also measured. The 24-hr outputs at the ligament of Treitz were markedly increased, while fecal losses were normal or only slightly increased. Thus, the overall intestinal reabsorption of water was 96%. Fasting rates of fluid and electrolyte flow at the ligament of Treitz were also measured during a basal period, followed by a period of continuous gastric aspiration. Removal of gastric secretion had the following effects on the fluid passing through the duodenum: (1) dramatic decrease in flow rate; (2) an increase in osmolality, from hypotonicity to isotonicity; (3) rise of pH, from acid to alkaline values; (4) a decrease of PCO2, from high to normal values. No increase in fasting plasma levels of immunoreactive secretin and motilin was observed in Zollinger-Ellison syndrome, whereas normal subjects respond to acid in the duodenum by a marked rise in the circulating levels of these hormones. These facts suggest that, in Zollinger-Ellison syndrome: (1) the ability of the small bowel and colon to reabsorb water and electrolytes is normal: (2) duodenal dissipation of hydrogen ions is mainly due to intraluminal neutralization by bicarbonate; and (3) stimulation of water and electrolyte secretion by the pancreas is inadequate.
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A case of massive digestive bleeding due to an arterial abnormality of the jejunal wall is reported. Its interest is twofold: a) After a complete endoscopic and operative examination of the GI tract proved negative, an emergency superior mesenteric angiography was the only investigation which allowed to recognize the site and mechanism of the bleeding; b) from a nosologic point of view, the clinico-pathological picture was entirely similar to that of "Dieulafoy's erosion", a syndrome which has been only described, up to now, in the gastric mucosa.
Two young patients with massive intestinal bleeding resulting from a solitary submucosal arterial abnormality of the jejunum are reported. In one case, the bleeding lesion was localized only by emergency abdominal angiography, while a previous exploratory laparotomy proved negative. The vascular lesion encountered in both patients consisted of a wide caliber artery lying in the jejunal submucosa; there was no evidence of true aneurysm on serial sections. The clinicopathological picture closely resembles that of lesions previously described in the stomach under the name of "gastric submucosal aneurysms" in the English literature, or of "gastric Dieulafoy's erosion" in the French literature. It is suggested that the vascular lesions herein reported should be called "solitary large submucosal artery" or "Dieulafoy-like erosion" of the jejunum.
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The pathology of six cases of alpha-chain disease (alpha-CD), four of which were followed until complete remission or death, was studied by histologic, immunofluorescence and ultrastructural techniques. The lesions could be classified in three histopographical stages. The late stage C is an immunoblastic sarcoma probably deriving from the same clone as the initial plasmacytic stage A, stage B being a transitional one between A and C. The asynchronism of the lesions in different organs in the same patient requires a laparotomy for an accurate staging which determines the prognosis and the treatment. Complete and prolonged remissions have been observed at stage A only, sometimes with oral antibiotic treatment alone. At all stages, alpha-CD and the "Mediterranean lymphoma" share identical aetiological, clinical and pathological features. Accurate immunological studies will determine the precise-frequency of alpha-CD protein synthesis in the latter syndrome.
In a young woman with ulcerative colitis, hypoimmunoglobulinemia, and humoral immunodeficiency, lymphocyte counts vary between 600 and 1,000 per mm(3) with 0.5-1.5% bone marrow-derived (B) cells and 98-99% thymus-derived (T) cells. Anti-lymphocyte antibodies were detected by immunofluorescence and by microlymphocytotoxicity with increased reactivity at +4 degrees C. They belonged to the IgM class and were polyclonal. Studies performed with various normal lymphocyte subpopulations, several lymphoblastoid cell lines and lymphocytes from immunodeficiency patients showed that these antibodies reacted with B cells. The corresponding antigen(s) is distinct from membrane-bound immunoglobulins, is not an alloantigen, and is probably unrelated to the la-like molecules. Pokeweed mitogen stimulated B cells appear to lose this antigen. Cells from various lymphoproliferative disorders were tested. T-derived and "non T-non-B" leukemic cells did not react with the antibody. Malignant cells from B-derived lymphomas and prolymphocytic leukemias were reactive. The incidence of positivity of the leukemic cells among patients with common B chronic lymphocytic leukemia was surprisingly low (one-third of the patients). The autoantibody nature of the anti-B-cell antibodies and their pathogenic role in the genesis of the patient's hypoimmunoglobulinemia was demonstrated by the effect of removal of antibodies by massive plasmaphereses which were followed by a dramatic and transitory increase of B-cell figures. Whereas most primary immunodeficiency syndromes appear to result from an arrest in the differentiation capabilities of immunologically competent cells, autoantibodies to circulating B lymphocytes may be incriminated in the pathogenesis of some cases of hypogammaglobulinemia.
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Lymphocytotoxins (LCT) have been recently reported in the serum of patients with inflammatory disease of the bowel, but up to now these antibodies have shown no specificity for B or T lymphocyte subpopulations. A 32-year-old patient with chronic ulcerative colitis, primary hypogammaglobulinemia and a very low number (0.5 to 1.5%) of B lymphocytes in peripheral blood is described. The presence in the serum of a LCT reacting specifically with B cells was demonstrated by cytotoxicity and direct immunofluorescence experiments. Intestinal immunofluorescence studies indicated a dichotomy between blood and gut immunoglobulins, and showed a heterogeneous distribution of plasma cells of the three major classes from the jejunum to the rectum. The significance of the association of hypogammaglobulinemia, chronic ulcerative colitis, and anti-B LCT is discussed. To explain the dissociation between blood and gut immunoglobulins, it is suggested that the intestine was, in this patient, a privileged site for differentiation of B cells.
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