Glycogen Storage Disorders

Which enzyme deficiency characterizes Glycogen Storage Disease Type I (Von Gierke disease)?

All of the following glycogen storage disorders present with hepatomegaly and hypoglycemia EXCEPT:

A 6-year-old boy from Delhi presents with severe fasting hypoglycemia, hepatomegaly, lactic acidosis, and hyperuricemia. Enzyme assay confirms glucose-6-phosphatase deficiency. All of the following are true regarding his condition EXCEPT:

A 3-year-old boy from rural Maharashtra presents with severe hypoglycemia (blood glucose 35 mg/dL), hepatomegaly, and lactic acidosis (pH 7.2, lactate 8 mmol/L) after a 6-hour fast. His parents report recurrent episodes of seizures triggered by fasting. Liver biopsy shows excessive glycogen accumulation with normal structure. Enzyme assay confirms glucose-6-phosphatase deficiency. What is the most appropriate immediate management?

A 5-year-old girl from Delhi presents with progressive muscle weakness, myalgia, and dark urine after running in a school sports day 3 days ago. On examination, she has mild hepatomegaly and proximal muscle weakness. Serum creatine kinase is 8,500 U/L (normal <200), and urine myoglobin is positive. Her blood glucose during fasting is 85 mg/dL (normal). A forearm ischemic exercise test shows no rise in venous lactate but a normal rise in ammonia. Which glycogen storage disorder is most likely?