pediatrics
Other Carbohydrate Metabolism Disorders
Introduction
Phosphoenolpyruvate carboxykinase deficiencyimpairs gluconeogenesis and results in symptoms and signs similar to the hepatic forms of glycogen storage disease but without hepatic glycogen accumulation. include those of glycolytic enzymes or enzymes in the pentose phosphate pathway. Common examples are pyruvate kinase deficiency ( see Glycolytic Pathway Defects ) and See Overview of Carbohydrate Metabolism Disorders . See also table See also Approach to the Patient With a Suspected Inherited Disorder of Metabolism . 