Some cases of type III glycogen storage disease

Rosenfeld, E.L.; Popova, I A; Chibisov, Irina

doi:10.1016/0009-8981(76)90250-3

Cited by 10 publications

(2 citation statements)

References 11 publications

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“…(6) Type I11 Glycogenosis is caused by inactivity of the debranching enzyme, amylo-1, 6-glucosidase (72)(73)(74)(75). The glycogen content of the liver is greatly increased, but only the outermost 1,4 chains can be released as glucose (73).…”

Section: Hypoglycemia Caused By Decreased Output Of Glucose From the mentioning

confidence: 99%

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Hypoglycemia: A pathophysiologic approach

Senior

Sadeghi‐Nejad

1989

Acta Paediatrica

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An exploration of the factors that sustain glucose levels in the normal fasting subject reveals that the single major component is conservation of glucose rather than gluconeogenesis. Conservation is achieved by recycling of glucose carbon as lactate, pyruvate and alanine, and a profound decrease in the oxidation of glucose by the brain brought about by the provision and use of ketones. What glucose continues to be oxidized is for the most part formed from glycerol. Gluconeogenesis from protein plays little part in the process.Fasting hypoglycemia results from disorders affecting either one of the two critical sustaining factors-the recycling process or the availability and use of ketones.Individual hypoglycemic entities are examined against this background. B . Senior and A . Sadephi-NeiadActa Paediatr Scand Suppl 352 LNER Ghlcero'/ \I -\I -r l COP A 6 Fig. 1 . Schematic representation of fuel provision and utilization in fed (A) and fasting (B) states. Note recycling of glucose carbon through the Con and alanine cycles. FFA = free fatty acid, RBC = red blood cells.

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Section: Hypoglycemia Caused By Decreased Output Of Glucose From the mentioning

confidence: 99%

“…Virtually all of the liver glycogen remains sequestered from the fuel economy of the body. Recyciing, conversion of glycerol to glucose and ketogenesis are all intact (76), but the inability to produce glucose from the stored glycogen results in fasting hypoglycemia during childhood (72,74).…”

Section: Hypoglycemia Caused By Decreased Output Of Glucose From the mentioning

confidence: 99%