Branching enzyme deficiency is seen in: (A) Andersen disease, (B) McArdle's syndrome, (C) Cori disease, (D) Von Gierke disease
Answer is A. Type Name Enzyme Deficiency Clinical Features IV Amylopectinosis, Andersen disease Branching enzyme Hepatosplenomegaly Accumulation of polysaccharide with few branch points Death from hea or liver failure before age 5 Reference: Harper; 30th edition; Table: 18-2; Page no: 179