2 doctors weighed in:

What's the pathophysiology of lactic acidosis in von gierki's disease?

2 doctors weighed in
Dr. David Rosenfeld
Pain Management
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In brief: See below

La arises from impairment of gluconeogenesis.
Lactic acid is generated both in the liver and muscle and is oxidized to pyruvic acid and then converted via the gluconeogenenic pathway to g6p. Accumulation of g6p inhibits conversion of lactate to pyruvate. The Lactic Acid level rises during fasting as glucose falls. In people with gsd i, it may not fall entirely to normal even when gluc levels r nml.

In brief: See below

La arises from impairment of gluconeogenesis.
Lactic acid is generated both in the liver and muscle and is oxidized to pyruvic acid and then converted via the gluconeogenenic pathway to g6p. Accumulation of g6p inhibits conversion of lactate to pyruvate. The Lactic Acid level rises during fasting as glucose falls. In people with gsd i, it may not fall entirely to normal even when gluc levels r nml.
Dr. David Rosenfeld
Dr. David Rosenfeld
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