In the context of glycogen storage diseases, mild hypoglycemia, hepatomegaly, and decreased free glucose after an epinephrine challenge is characteristic of which disorder?

The combination of mild hypoglycemia, hepatomegaly, and decreased free glucose after an epinephrine challenge is characteristic of Cori's disease, also known as Glycogen Storage Disease type III. Cori's disease is caused by a deficiency of the enzyme debranching enzyme, which is essential for the complete breakdown of glycogen. This deficiency leads to an accumulation of short, branched chains of glycogen in the liver and muscles.

In patients with Cori's disease, the liver cannot adequately release glucose into the bloodstream during fasting or stress, such as an epinephrine challenge. This results in a diminished ability to mobilize glycogen stores, which explains the observed hypoglycemia. Hepatomegaly occurs due to the accumulation of abnormal glycogen in the liver. The failure to release free glucose after stimulation does reflect the impaired breakdown of glycogen into usable glucose, highlighting the specific enzymatic dysfunction present in this disorder.

Understanding the metabolic pathways and the enzymatic roles is crucial in distinguishing between various glycogen storage diseases, as they present with overlapping symptoms but have distinct underlying mechanisms. For instance, other glycogen storage diseases like Von Gierke's involve glucose-6-phosphatase deficiency, leading to severe