A 3 year child presents with hepatomegaly and recurrent episodes of vomiting, jaundice and hypoglycemia, beginning from the later pa of infancy. These episodes seem to have no temporal relation with the intake of milk or milk products. The mother also gives a history that the child has an aversion to sweet food. Which of the following enzymes may be deficient in this child?
HEREDITARY FRUCTOSE INTOLERANCE Aldolase B deficiency Symptoms appear on intake of fructose Aversion to sweet food C/F: Hypoglycemia Hepatomegaly Jaundice Vomiting Reducing substance in urine: POSITIVE - BENEDICT'S TEST T/t- Avoid fructose
Glucose-6-phosphatase
Aldolase B
Branching enzyme
Galactose 1 phosphate uridyl transferase
Disorders of Carbohydrate Metabolism