Membrane glycoprotein defects in congenital platelet disorders.
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Biomedical subjects
Publications and source records attributed to D Dupuis.
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This is a case report of a megacolon in a 26 years old male patient having typical von Recklinghausen's disease. Exploratory laparotomy shows no neurofibroma on digestive tract. Myenteric plexus study of colic resection specimen following B. Smith technique reveals marked alterations. These alterations are both quantitative (hyperhypoganglionosis) and qualitative (ganglion cells are irregular in shape and size, dendritic processes are hypertrophic, irregular; schwannosis; no inflammatory infiltration). The patient suffers no more transit disorders after adequate resection of this megacolon.
Platelets from a patient with eosinophilic leukaemia were not aggregated by ristocetin. The defect was not corrected by normal human plasma and was due to a platelet abnormality. The patient's platelets also showed a diminished sensitivity to aggregation by bovine factor VIIIVWF. The defect was not associated with a prolonged bleeding time. No abnormalities were detected in ADP, collagen or thrombin-induced platelet aggregation. Biochemical studies showed that the platelets were deficient in sialic acid. This deficiency was associated with a reduced staining for glycoprotein I following SDS-polyacrylamide gel electrophoresis. The results suggest an acquired platelet surface abnormality.
Male albinos rats were fed diets containing parathion-methyl or Zineb so that the average daily intake is 1/10 or 1/20 lethal dose 50, for a period of 4 or 8 weeks. The activity of hepatic microsomal enzymes, namely :aminopyrine N-demethylase, BHT oxidase, aniline hydroxylase, Butter Yellow & Amaranth reductases, NADPH-cyt. c reductase, G6P phosphatase, were measured. The activities of G6P & 6PG deshydrogenases were also determined. With the application of the parathion-methyl doses, the only effect showed is the lowering of the microsomal protein concentration.