TY - CHAP M1 - Book, Section TI - Glycogen Storage Disease Type I (GSD I) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Marciniak, Bruno A1 - Dalens, Bernard J. PY - 2006 T2 - Syndromes: Rapid Recognition and Perioperative Implications AB - 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 hypoglycemia and its consequences (seizures, cyanosis, apnea). SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/28 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=58078006 ER -