RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164070991 T1 Glycogen Storage Disease Type 0 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=1164070991 RD 2022/08/18 AB Inborn error of metabolism of glucose caused by decreased glycogen synthetase activity and characterized by fasting hypoglycemia, high blood ketones, increased free fatty acids, and low levels of alanine and lactate beginning in early infancy. Conversely, feeding results in hyperglycemia and hyperlactatemia. Unlike other GSDs, GSD 0 does not result in tissue accumulation of normal or abnormal glycogen. Although glycogen synthase deficiency does not result in storage of extra glycogen in the liver, it is often classified as a glycogen storage disease because it is another defect of glycogen storage and can cause similar problems.