RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164071080 T1 Glycogen Storage Disease Type III (GSD III) T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164071080 RD 2024/03/28 AB Inherited metabolic disease resulting in accumulation of abnormal glycogen in different tissues of the body. Glycogen storage disease Type III (Cori Disease) is an autosomal recessive metabolic disorder and inborn error of metabolism characterized by a deficiency in glycogen debranching enzymes. There are four different types based on the clinical presentation. It presents during infancy with hypoglycemia and failure to thrive. Clinical examination usually reveals hepatomegaly, muscular disease, hypotonia, and often severe cardiomyopathy usually occurring later. The hepatomegaly and splenomegaly regress at adolescence.