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Biomedical subjects

A Richard

Publications and source records attributed to A Richard.

At least 145 records · Page 8Linked to original sources

[Clinical, ultrastructural and biochemical study of a case of GM1 type 2 gangliosidosis].

Clinical, histological, ultrastructural and biochemical studies have been performed in a living 20-month-old infant with GM1-gangliosidosis type 2. Rectum, brain and liver biopsies were done. The histological and ultrastructural examination revealed the presence of cytoplasmic membranous bodies in the nervous system and a vacuolisation of the visceral parenchymatous cells, particularly histiocytes. The diagnosis was established by the finding of a generalized beta-galactosidase deficiency and an accumulation of GM1-ganglioside in brain. In leukocytes, the activity of p-nitrophenyl-beta-galactosidase was below 5%, and that of GM1-ganglioside beta-galactosidase below 1% of values obtained in controls. In cerebral tissue, GM1 ganglioside constituted 80% of total gangliosides; its concentration was 15 times that in age-matched controls. No accumulation of GM1 could be evidence in liver. Enzymatic examination of leukocytes obtained from the consanguineous parents revealed heterozygote values.

Brain↗

[Pulmonary edema due to inhalation of gas and vapors].

In 1975, France will have manufactured 3.2 million tons of plastic materials which by pyrolysis, liberate chlorine, methyl chloride, hydrocyanic acid, hydrofluoric acid and other toxic gases. Now these materials are burnt in fires in "modern", buildings or vehicles , associated with fats (acrolein) and other gases and toxic industrial or household fumes which attack the lung. At sufficient dosage these agressive agents have first of all a "suffocating" action when in case of survival, a caustic and corrosive action. Pulmonary edema of the lesional type is met with at two stages in these cases. They are due to a direct action on the bronchial epithelium and an indirect action by disturbance of surfactant metabolism. It is an edema which is interstitial above all, and secondarily endo-alveolar. Study of the clinical, radiological, bronchoscopic and hemodynamic symptomatology. Differential diagnosis is not always easy. The possibility of burns of the respiratory pathways, blast, must be looked at. These elements can moreover be associated with lesions due to toxic inhalation. In case of survival the course is one of fibrosis with a restrictive syndrome.

Air Pollutants↗