Biomedical subjects
Publications and source records attributed to J P Igual.
Choledochal cysts have an increased risk of biliary tract malignancy, induced by reflux of pancreatic juice and anomalous pancreatobiliary ductal junction. We report one case of congenital choledochal cyst in a 68-year-old female whose malignancy was revealed by history of pain and jaundice. Pancreatico-duodenectomy was performed and the patient was alive 6 months later.
Hemangioendothelioma is seldom seen in adults. Its severe evolution is due, not so much to the exceptional transformation into hemangiosarcoma, but mainly to haemorrhagic complications by rupture or consumption coagulopathy and to severe cardiac insuffficiency secondary to arteriovenous shunts. The case reported here concerns a 64 year-old woman presenting pain in the left hypochondrium and splenomegaly. A splenectomy was performed and the histological findings were compatible with the diagnosis of hepato-splenoganglionic hemangioendothelioma. The evolution was unfavorable. The patient died a few months later in a picture of haemorrhagic syndrome and cardiac insufficiency. Histological findings on autopsy specimens indicated a cavernous hemangioma. The treatment of these diffuse hemangiomas is a difficult one. Hepatic artery ligation has been advocated in certain desperate situations. Nevertheless, because of a collateral circulation, recurrences are frequent.
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An outbreak of trichinosis following meals in a rural area of Provence is reported. The epidemiologic parameters reconstitute the contamination cycle (man-pig: fox-rat) which indicated indisputably the diagnosis, the appropriate therapy, and also evidenced a rural zoonosis of trichinosis in Provence. The clinical picture among twenty-one people included morbilliform rash in 38% of cases and an enanthema in 24% of cases, uncommon findings, with respect to their frequency, and the other usual signs. Early laboratory diagnosis was made possible by blood analysis for hypereosinophilia and immunoassay (ELISA) for antigen-specific IgE. Treatment with benzimidazoles was quite effective.
With reference to an observation of porphyria variegata, which was complex and unusual as all such observations are, the direct responsibility of rifampicin is underscored. Porphyria variegata should be considered in two situations. The first of these is a dramatic acute neuroabdominal picture: the diagnosis of acute hepatic porphyria is established by the measurement of urinary porphyrins; the second step is to distinguish between the three acute hepatic porphyrias by looking for cutaneous manifestations and determining the respective proportions of coproporphyrins and protoporphyrins in the stools. In porphyria variegata, fecal protoporphyrins are significantly increased. The second situation is cutaneous involvement suggestive of late-onset cutaneous porphyria: porphyria variegata as well as hereditary coproporphyria in the cutaneous phase must be considered. Diagnosis can be established only through measuring fecal porphyrins. Porphyria variegata is a genetic enzymatic disorder inherited on an autosomal dominant basis. A study of the family is required in all cases, but the conventional methods for detecting heterozygotes for porphyria variegata are not satisfactory. Carriers will be unequivocally distinguished from healthy subjects only through measuring the defective enzyme activity.
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The authors discuss the various recent discoveries and fundamental data on absorption from the digestive tube and the digestive hormones. The practical applications of these data in oral feeding are underlined.