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Cardiac and respiratory complications are treated symptomatically. Physical therapy may be beneficial for some patients. Alterations in diet may provide temporary improvement but will not alter the course of the disease. Genetic counseling can provide families with information regarding risk in future pregnancies.
On April 2006 the united state Food and Drug Administration approved a Biologic License Application (BLA) the first treatment for patients with Pompe disease. This was based on enzyme replacement therapy using biologically active recombinant human alglucosidase alfa produced in Chinese Hamster Ovary cells.