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7386 Biochemistry

A 15 year old female patient presents with pain in the calf muscles on exercise. Routine investigation reveals burgundy-colored urine (sample obtained after exercise). Histopathology of mucle shows deposits of glycogen in an excess amount. Which of the following treatment modality should not be followed in this patient?

AAvoidance of strenuous exercise
BGlucose or sucrose given before exercise or injection of glucagon can markedly improve tolerance in these patients
CVitamin B6 supplementation
DSpecific enzyme replacement therapy (ERT) with recombinant human acid Ξ±-glucosidase (Correct Answer)