Best Answer: Sucrose (common name: table sugar, also called saccharose) is a disaccharide (glucose + fructose) with the molecular formula C12H22O11. Its systematic name is Î±-D-glucopyranosyl-(1â2)-Î²-D-fructofuran...
The classic form of infantile-onset Pompe disease begins within a few months of birth. Infants with this disorder typically experience muscle weakness (myopathy), poor muscle tone (hypotonia), an enlarged liver (hepatomegaly), and heart defects.
The infantile form of Pompe disease is characterized by a total lack of alpha glucosidase activity and by a rapid buildup of glycogen in skeletal muscle and in the heart. Late Onset Forms. The time of presentation of late onset Pompe disease varies from the first to the seventh decade.