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Kaplan Qbank USMLE



Author4 Posts
  #1

A 17 year old boy is brought to the emergency department because of severe cramps in his calf muscles that began after he ran a 1-mile race. His urine is a burgundy color. Physical examination shows tender calf muscles. Laboratory studies show an increased serum creatine kenase activity and myoglobinuria. Following exercise of an ischemic left forearm, his serum lactic acid concentration does not increase in blood obtained from the vein proximal to the exercised extremity. This patient most likely has a deficiency of which of the following enzymes in muscle?

1.) Debranching enzyme
2.) Glycogen Synthase
3.) Glucose-6-phosphatase
4.) Fructose-1,6 bisphosphatase
5.) Glycogen phosphorylaze

  #2

McArddle's disease

Muscle glycogen phophorylase deficiency

5 is the answer.




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  #3

5

  #4

yes, the others would be:

1)Cori"s disase

2) is hinting that he CANT synthesis glycogen

3)Glycogen storage disease type I-(Von Gieke)

4)essential enzyme in gluconeogenesis


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