[Hepatic granulomatosis associated with a circulating anticoagulant, disclosing Q fever].
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Biomedical subjects
Publications and source records attributed to J F Devars du Mayne.
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Acute hepatic porphyrias are genetic diseases of heme synthesis with severe prognosis due to strong or acute abdominal pain and neurological complications. Clinical evolution is characterized by acute attacks frequently induced by either forbidden drugs, or infections, alcohol intake or often unknown factors. Modern treatment is perfusion of hematin, which is a stable form of heme. Hematin will induce again delta-aminolévulinic (ALA)-synthase synthesis repression. Its tolerance is perfect whereas clinical and biochemical efficiency is absolute in our experience, if initiated very early. This drug is now considered as the treatment of acute intermittent porphyria crises.
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From 1985 through 1988, 52 patients aged 16 to 85 years and referred for acute diarrhea underwent routine clinical, microbiological, endoscopic and histopathological examinations. Enteropathogens were isolated in 50 percent of patients, mostly from stool samples rather than from biopsy samples, though results were sometimes dissociated. Significantly lower digestive endoscopic abnormalities were seen in 60 percent of patients. Upper gastrointestinal endoscopy was not contributive. Rectal and colonic biopsies showed histological abnormalities in nearly all cases half of which were polymorphonuclear infiltrates and crypt abscesses. Glandular distortion was not found. An analysis of clinical, endoscopic and histological data showed that major abnormalities were mostly, but not constantly, related to infections due to enteroinvasive bacteria. As in other studies, a high rate of negative stool cultures was observed. Based on these results, we suggest to perform either further and more sophisticated microbiological investigations, or to conduct a routine search for viral infections whose incidence, among adult patients with acute diarrhea, is actually unknown.
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We report the seventeenth case of primary hepatic lymphoma. Its originality is an association with one cryptogenetic cirrhosis, which had never been described but does not seen fortuitous. We discussed the significance of such an association, one's being cellular immunity anomalies.
The effectiveness of 2 hematins administered by intravenous infusion was compared in acute intermittent porphyria. Judging from subjective symptoms (abdominal pain), clinical improvement was complete and constant. There was a rapid decrease in urinary excretion of porphyrins precursors, with a clearer response of delta-aminolevulinic acid than of porphobilinogen. Urine levels of uroporphyrin often returned to normal after two infusions. The drugs were very well tolerated. Provided it is administered early, before neurological complications develop, treatment with hematin completely relieves abdominal symptoms and suppresses most of the biochemical changes associated with hepatic porphyria.
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Liver biopsies were systematically performed in 20 patients with evidence of an acquired immunodeficiency syndrome (18 with opportunistic infections, 9 with Kaposi's sarcoma). Hepatitis, related to hepatitis-B virus in 3 cases and to cytomegalovirus in 3 cases, was present in 6 of them. All patients had at least one of the three following lesions: non-specific portal inflammatory infiltration (6 cases), granulomatous lesions (12 cases), vascular abnormalities (12 cases). Among hepatic granulomatous lesions: 3 were due to acid-fast bacilli and 1 to Cryptococcus. Sinusoidal abnormality included proliferation of Küpffer cells (9 cases), sinusoidal dilatation (2 cases), peliosis (4 cases) and Kaposi's sarcoma (1 case). A relationship between these abnormalities cannot be excluded.
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