TY - CHAP M1 - Book, Section TI - Glycogen Storage Disease Type 0 A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164070991 ER -