The cellular distribution of lipososmes in the liver of newborn rats.
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Biomedical subjects
Publications and source records attributed to T de Barsy.
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A case of an infantile type III glycogenosis (Forbes disease), confirmed by morphologic and biochemical studies, had light-microscopic, histochemical, and electron-microscopic evidence of multicore structures and type 1 fiber predominance with hypotrophy. This association is discussed with relation to the unusual clinical findings. The authors conclude that two distinct disease entities--Forbes disease and multicore myopathy--may coexist.
Muscular glycogenosis is a disease resulting from genetic abnormalities altering an enzyme which is involved in glycogen metabolism. In addition to disorders of glycogenolysis and glycolysis, there are other pathological processes such as acid maltase (alpha-glucosidase) deficiency and diseases associated with abnormal glycogen structure. Glycolysis is the only metabolic pathway that can produce ATP in the absence of oxygen. It is then easy to understand that any disturbance in this energy pathway can result in dysfunction of the muscle machine and in a number of symptoms which are common to these abnormalities. An overall review of the various diseases know to exist on the glycogenolytic and glycolytic pathway will enable the reader to acquire a better knowledge of their particular features.
Regional brain glucose metabolism has been studied in a case of mitochondrial encephalomyopathy with ragged red fibers, using positron emission tomography with fluorodeoxyglucose as the tracer. A marked decrease in glucose utilization was found in all gray structures, with a preponderance of metabolic alterations in posterior cortical regions and thalamus and a relative sparing of anterior cortical areas and basal nuclei.