TY - CHAP M1 - Book, Section TI - Glycogen Storage Disease Type I (GSD I) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - A severe inborn liver dysfunction caused by an almost total deficiency of hepatic glucose-6-phosphatase (Type Ia) or a defect in intracellular transport of the enzyme substrate (Type Ib), resulting in severe acute hypoglycemia. Seizures, cyanosis, apnea, and hypotonia are associated with this medical condition. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/11/08 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164071022 ER -