A 1-year-old child presents with severe hypoglycemia, hepatomegaly, and hyperuricemia. Blood tests show elevated lactate and triglycerides. A deficiency in glucose-6-phosphatase is suspected. This clinical picture is most consistent with which glycogen storage disease?

The correct answer to the question "A 1-year-old child presents with severe hypoglycemia, hepatomegaly, and hyperuricemia. Blood tests show elevated lactate and triglycerides. A deficiency in glucose-6-phosphatase is suspected. This clinical picture is most consistent with which glycogen storage disease?" is Option D: Von Gierke disease (Type I).

The other options — A) McArdle disease (Type V), B) Pompe disease (Type II), C) Cori disease (Type III) — are incorrect for this USMLE Exam Prep question. As explained above: Von Gierke disease is caused by a deficiency in glucose-6-phosphatase, preventing the release of free glucose from glycogen and gluconeogenesis, leading to profound hypoglycemia, hepatomegaly, and hy…