GSD type VI (Hers disease) — Flashcards

Flashcard 1: Which glycogen storage disease has an increased risk for gout? _____

Answer: Von Gierke disease

Flashcard 2: Glucocorticoids increase hepatic _____ storage

Answer: glycogen

Flashcard 3: Which two organs produce nascent HDL? _____

Answer: Liver, Intestines

Flashcard 4: What glycolysis enzyme deficiency causes hemolytic anemia in a newborn?_____

Answer: Pyruvate kinase deficiency

Flashcard 5: Which glycogen storage disorder is associated with accumulation of limit dextrin-like structures? _____

Answer: Cori disease

Flashcard 6: Which glycogen storage disorder is associated with increased glycogen in muscle? _____

Answer: McArdle disease

Flashcard 7: glycogen synthesis

Answer: glycogen synthase

Flashcard 8: glycogenolysis

Answer: glycogen phosphorylase

Flashcard 9: McArdle's disease (GSD type V)

Answer:

Extra: painful muscle cramps, myoglobinuria with strenuous exerciseglycogen accumulation in muscleARskeletal muscle glycogen phosphorylasemnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)

Flashcard 10: Cori's disease (GSD type III)

Answer:

Extra: hepatomegalyfasting hypoglycemia, increased hepatic glycogen stores, normal blood lactate levelsARglycogen debranching enzymemnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)