Clinical Findings

Classical branching enzyme deficiency most frequently presents during the first few months of life, with hepatosplenomegaly and failure to thrive. Progressive liver cirrhosis, with portal hypertension, ascites, esopha-geal varices, and death, usually occurs before 5 years of age. However, there are patients who have survived without apparent progressive liver disease. The neuromuscular system may also be involved. Severe cardiomyopathy as the predominant symptom has also been reported.[20] The diagnosis of type IV is established by demonstration of abnormal glycogen (an amylopectin-like polysaccharide) and a deficiency of glycogen branching enzyme in liver, muscle, or fibroblasts.

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.

Get My Free Ebook


Post a comment