GSD type I (von Gierke disease)Downloads

A 4-month-old boy is brought to his pediatrician for a well-child visit. His parents have noticed that he has had poor growth compared to his older siblings. The boy was delivered vaginally after a normal pregnancy. His temperature is 98.8°F (37.1°C), blood pressure is 98/68 mmHg, pulse is 88/min, and respirations are 20/min. On exam, his abdomen appears protuberant, and the boy appears to have abnormally enlarged cheeks. A finger stick reveals that the patient’s fasting blood glucose is 50 mg/dL. On further laboratory testing, the patient is found to have elevated blood lactate levels, as well as no response to a glucagon stimulation test. What enzymatic defect is most likely present?

While walking through a park with his wife, a 51-year-old man with type 2 diabetes mellitus develops nausea, sweating, pallor, and palpitations. For the past 3 weeks, he has been trying to lose weight and has adjusted his diet and activity level. He eats a low-carb diet and swims 3 times a week. The man returned home from a training session 2 hours ago. Current medications include basal insulin and metformin. Shortly before his wife returns from their car with his emergency medication kit, he becomes unconscious. Administration of which of the following is the most appropriate next step in management?