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?
Correct Answer: Specific enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase
Description: Patient is suffering from type V Glycogen Storage Disease (McArdle Disease).
Treatment:
Avoidance of strenuous exercise.
Glucose or sucrose given before exercise or injection of glucagon can markedly improve tolerance in these patients.
Vitamin B6 supplementation reduces exercise intolerance and muscle cramps.
Note - Specific enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (Myozyme) is available for treatment of Pompe disease.
Reference- Harper’s illustrated biochemistry. 30th edition page no: 179
Category:
Biochemistry
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