Which of the following metabolic disorders cause post-prandial hypoglycemia?
Post Prandial here means after ingestion of fruit juices (typically in child after weaning when fruit juices are staed in the diet). The fructose in fruit juice will sequester all Pi in the form of Fructose -1-phosphate but cannot conve it to energy by glycolysis. So body tries to switch to glycogen degradation for energy. But, Glycogenolysis gets inhibited also due to pi sequestration hence no glucose from glycogenolysis causing hypoglycemia (see fig) Hereditary fructose intolerance: Autosomal recessive Deficiency of Aldolase B Glycogen storage disorders are associated with fasting hypoglycemia. Fanconi-Bickel disease: Glycogen storage disease Type XI similar to Von-Gierke's disease. Mutation in GLUT2
Glycogen storage disease type I
Glycogen storage disease type III
Fanconi-Bickel syndrome
Hereditary fructose intolerance
Glycogen