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Biomedical subjects

A Lageron

Publications and source records attributed to A Lageron.

At least 55 records · Page 3Linked to original sources

[Diffuse polymorphous inclusions in a patient treated with perhexiline maleate (author's transl)].

Case report of polyneuritis and liver dysfunction induced by perhexiline maleate in a 64 years old male patient. The ultrastructural study of nerve, liver, muscle and skin biopsies shows polymorphous, membrane bound, often multilamellar, lysosome-like inclusions, the content of which is probably complex lipids. The histochemical study of liver reveals a lipid storage, consisting mainly of triglycerides and of smaller amount of phospholipids and free fatty acids, the pattern of which is abnormal. The biochemical study of nerve tissue shows a decrease of phospholipids levels and some qualitative disturbances in gangliosides. These changes, some of which are similar to those reported in amphiphilic drug intoxications, are prevalent in high lipid metabolism cells such as hepatic and Schwann cells.

Biopsy↗

[Hepatic overload of gangliosides after perhexiline maleate. Morphological and biochemical course (author's transl)].

A 72 years old man treated with perhexiline maleate (400 mg during 15 months) complained of skin rash, sensitive neuropathy, liver damage and mild renal insufficiency. Clinical status improved when the treatment was stopped, but liver size and renal status remained inchanged. Liver biopsy were performed at 0,6 and 15 months after the end of treatment. Inflammation and hepatocyte necrosis, seen on the first specimen, disappeared latter. Steatosis and voluminous, swollen, foamy liver seen were present in the three biopsies. Sclerosis had increased in the last one in spite of treatment arrest. Histochemical study exhibited liver storage of triglycerides, fatty acids, phospholipids, and gangliosides. Cytoplasmic glycogen was increased and some nuclei were glycogenated. Enzymatic pattern suggests a minor form of type I glycogen storage or increased neoglycogenesis. Perhexiline relation to this complex liver storage is discussed.

Aged↗

[Adult-type Gaucher's disease: an histochemical study of one case (author's transl)].

Spleen of adult-type Gaucher's disease has been investigated by histochemical technics. As usually, an overload of cerebroside (revealed by the PAS reaction for glycolipids) with beta-glucosidase deficiency has been demonstrated. But many particularities must be emphasized in this case: increase of acid MPS, high activities of pentose pathway--and glycolysis enzymes in the overload cells.

Adult↗

[Adult Niemann-Pick disease: a 26 years follow-up. Report of a case with isolated visceral involvement, excess of tissue sphingomyelin, and deficient sphingomyelinase activity (author's transl)].

The case report of Niemann-Pick disease, in a 26 years old woman whose first symptoms appeared when she was 17 months old, is described. The disease, involving considerable hepatosplenomegaly and pulmonary infiltration, was diagnosed by the presence of lipid laden macrophages (resembling foam cells, sea blue histiocytes and kidney intermediate forms) in the bone marrow, liver and kidney, and an excess of tissue sphingomyelin and cholesterol, and a decrease in sphingomyelinase in circulating leucocytes. The results of ultrastructural, histochemical and biochemical studies on hepatic and renal lipids are reported. The relationship of the case to the sea blue histiocyte syndrome is discussed.

Adult↗

Influence of carbon tetrachloride or riboflavin on liver carcinogenesis with a single dose of aflatoxin b1.

Liver carcinogenesis with a single dose of aflatoxin B1 (7 mg/kg body weight) has been investigated in a group of female Wistar strain rats by repeated biopsies and necropsies. Another group received a subsequent intoxication with carbon tetrachloride by inhalation (approximately 200 doses) and another one was overloaded with riboflavin (25 parts/10(6) in drinking water). The frequency of hepatomata was almost equal in the aflatoxin and aflatoxin-carbon tetrachloride group. It was lowere in the riboflavin-aflatoxin group. In these 3 groups cirrhosis was never present in neoplastic livers. Megalocytosis was the first lesion observed. All tumoral livers had previous or concomitant megalocytosis. This modification was about as frequent, intense and widespread in aflatoxin-CCl4 and aflatoxin groups but appeared much earlier, as did the first hepatoma, in the aflatoxin-CCl4 group. It was less frequent, less intense and less widespread in the riboflavin-aflatoxin group than in the aflatoxin group. There was also a lower frequency of hepatomata in the riboflavin-aflatoxin group, but the difference was not significant due to the too small number of animals involved. The facts are not a proof of the existence of an obligatory link between megalocytosis and carcinogenesis since a slight megalocytosis was observed in the riboflavin group not affected by the neoplastic process. However, the simplest explanation of our results would be to consider that the potential tumour cells are located among the megalocytic cells, without admitting that every megalocyte is obligatorily a precancerous cell. CCl4 seems to act in shortening the time of appearance of megalocytosis. The protective effect of riboflavine should be regarded with more caution.

Aflatoxins↗