Her's disease pathophysiology

[candyy]

Why do people with Her's disease (liver glycogen phosphorylase deficiency) have more lactate in the blood?

[Strange]

I see no evidence they do. This seems to be an effect in Glycogen storage disease type I (von Gierke disease): https://en.m.wikipedia.org/wiki/Glycogen_storage_disease_type_I#Elevated_Lactate_and_Lactic_Acidosis

[guidoLamoto]

https://emedicine.medscape.com/article/950587-overview#a5  Her's Disease- several possible defects in the enzyme cascade that activates glycogen phosphorylase-- results in impaired production of glucose from glycogen stores..  

https://emedicine.medscape.com/article/119318-overview#a5 von Gierke Dis-- defect is in G-6-Phospahatase, so  G-6-P builds up in hepatocyte  and is metabolized to lactate. Lactate, in turn, competes with uric acid in kidney, so uric acid levels build up.