A 7-month-old girl presented with recurrent chest infection, chocking during feeding

(C). Specific enzyme replacement therapy (ERT) with recombinant human acid a-glucosidase is available for treatment of Pompe disease and is capable of preventing deterioration or reversing abnormal cardiac and skeletal muscle functions. A high-protein diet and exercise therapy may also be beneficial. Nocturnal ventilatory support, when indicated, should be used. It has been shown to improve the quality of life and is particularly beneficial during a period of respiratory decompensation