Tyrosenemia

Tyrosinemia
What is it?
*Heredity disorder, deficiency of parahydroxyphenylpyruvic acid oxidase blocks the conversion of tyrosine to homogentisic acid
*Tyrosinemia Type I- recessive hepatorenal disease involving enzyme deficiency of
FAH (fumaryllacetoacetate hydrolase)
-increases methionine, phenylalanine, tyrosine
-acute, often death within first year of life
Signs and Symptoms
*Liver failure or severe nodular cirrhosis with renal tubular involvement
*Can be aggravated by Vitamin C deficiency, liver immaturity, High Protein diet
Recommend:
*Restrict tyrosine and phenylalanine in the diet
*Normal growth/development for age
*Adequate Vitamin C for age
*Initially feed phenylalanine/tyrosine hydrolysate to infants, add small
amounts of milk to provide minimum requirements
What can I do?
*See your Physician
-if blood methionine levels elevated- try Product 80056/Mead Johnson/Norvartis
-use CHO (Carbohydrate) supplements as polycose, plus minerals/vitamins
*See FFF RD on line for help
*See a RD for help with diet plan
-Calories and nutrients for age
-Phenylalanine and tyrosine for age, body size