Inborn Error of Carbohydrate Metabolism Level 3

InbornError of
Carbohydrate Metabolism
What is it?
*Defective gene- prevents carbohydrate (CHO) metabolism
-happens in infancy, childhood with Low Blood Sugar,
 Poor Physical Growth
Facts:
*Galactosemia- Diet to Prevent physical/Mental Retardation,
Cataracts, Cirrhosis, Portal Hypertension
*Fructosemia- no enzyme to convert fructose to glucose
-rare and can cause Gastrointestinal Discomfort, Growth Failure,
Nausea, Malaise
-omit Sugar, Maple Syrup, Molasses, Honey, Fructose, Sucrose,
Sorbitol
*Hepatic glycogen storage diseases (GSD)- can’t convert
Glycogen to Glucose in the Liver; Increase Protein intake, small/
frequent feedings, if steroids need 2 gram Sodium Diet
-Avoid- lactose and sucrose, concentrated sweets may be avoided
*Sucrose intolerance- occurs after Irritable Bowel or
Gastrointestinal Flu, eliminate Carbohydrates to prevent Diarrhea
What can I do?
*See your Physician- about vitamins, supplements
*Work with a RD- to eliminate the nutrient not digested
*See FFF RD- on line for help with meal planning, Read All Labels