Page 170 - BIOCHEMISTRY II
P. 170
The 2 main types of Pompe’s
disease
1) Infantile Pompe
• Classic infantile Pompe is the most severe that ends
with rapidly progressive cardiomegaly (enlarged heart)
hepatomegaly (enlarged liver) weakness and hypotonia.
• This leads to early death usually in the first year of
life.
2) Juvenile Pompe (Late onset)
• Presents later than infancy.
• typically does not include severe cardiomyopathy.
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