Cori's Glycogen Storage Disease Type 3 for USMLE 

Glycogen Storage Disease Type 3 also known as Cori's, Forte's and Limit Dextranosis for Medical Students and USMLE. Focusing on Pathophysiology, Signs, Symptoms, Clinical, Diagnosis, Treatment and Management
PATHOPHYSIOLOGY
Branching chain of glycogen is made up of 1,4 and 1,6 glycogen bonds. 1,4 is broken by different bond than 1,6. So if there is a deficiency of 1,6 glucosidase bond than you are left with a limit dextran molecule which begins to accumulate in liver and muscle.
There are two types known as Glycogen Storage Disease IIIa affecting muscle and liver. And Glycogen Sotrage Disease IIIb which affects only the liver.
CLINICAL
It leads to neonatal hypoglycemia, hyoptonia and cardiomyopathy. The liver may also be involved leading to hepatomegaly. During adoelscents the liver pathology decreases and in adults there is cirrhosis and hepatocellular carcinoma
TREATMENT
Treated similarly to Glycogen Storage Disease 1 (Von Gierke). So focus on providing corn starch to maintain the level of glucose level and helps prevent hypoglycmia. ALso they may require liver transplant.