Why would you have lactic acidosis in von gierke's disease?

Question

why?

Answer 1

To put it very simply: we know with Von Gierke's disease the individual cannot convert glycogen back into glucose. And normally, when blood glucose levels drop, the stores of glycogen in the liver and muscles are steadily converted back into glucose but when there is no more glycogen to convert or it cannot be converted, the body will begin to break down muscle tissue into glucose, (glucose, by the way, is the fuel used to create ATP and ATP is the ultimate fuel for our cells) and a byproduct of muscle tissue breakdown is lactic acid.

Now, imagine a person with Von Gierke's eats breakfast and then a couple hours go by and the blood glucose levels need replenishing but the body can't convert the glycogen to glucose due to the disease. You might intuitively think that the next on the list to convert to glucose would be the fat reserves but that is not the case. In fact, the next stop is the muscle tissue. So if the time between meals is more than a few hours, then blood glucose levels will drop to the point that the body is going to do do something about bringing them back up to normal. It's going to to convert muscle tissue and there's going to be lactic acid released from that catabolism. This could happen several times a day and more. Each time, there's more and more lactic acid production.

Answer 2

Impaired gluconeogenesis results in elevations of lactic acid (4-10 mM) even when the child is well. In an episode of metabolic decompensation, lactic acid levels abruptly rise and can exceed 15 mM, producing severe metabolic acidosis. Uric acid, ketoacids, and free fatty acids further increase the anion gap. Manifestations of severe metabolic acidosis include vomiting and hyperpnea, which can exacerbate hypoglycemia by reducing oral intake. Repeated episodes of vomiting with hypoglycemia and dehydration may occur in infancy and childhood, precipitated by (or mimicking) infections such as gastroenteritis or pneumonia.