Clinical Findings

The clinical features are very similar to those in GSD V; however, hemolytic anemia can also occur. Along with glycogen accumulation, there is also an accumulation of fine fibrillar polysaccharide material resembling amylo-pectin, as what is seen in GSD IV.

An infantile form of phosphofructokinase deficiency has also been reported. These patients present with limb weakness, seizures, cortical blindness, and corneal clouding. Death occurs before 4 years of age from respiratory failure.

Biochemical enzyme analysis or histochemical demonstration of the enzymatic defect in the muscle is required to establish the diagnosis.

Getting Started With Dumbbells

Getting Started With Dumbbells

The use of dumbbells gives you a much more comprehensive strengthening effect because the workout engages your stabilizer muscles, in addition to the muscle you may be pin-pointing. Without all of the belts and artificial stabilizers of a machine, you also engage your core muscles, which are your body's natural stabilizers.

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