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What is A child was brought to the hospital was found to have hypoglycemia, hepatomegaly and accumulation of highly branched glycogen called limit dextrins. He is likely to be suffering from: and explain in detail?
Ans. 'D' Cori's diseaseCori's disease is also called Limit dextrinosis. It is a Type IIa glycogen storage disorder.It occurs due to the deficiency of the enzyme liver and muscle debranching enzyme.The clinical features are fasting hypoglycemia, hepatomegaly in infancy, accumulation of characteristic branched polysaccharide (limit dextrin) and muscle weakness.