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Tyrosinaemia

Tyrosinaemia type I is caused by a deficiency of fumarylacetoacetate hydroxylase resulting in severe liver and kidney failure and eventually death. The aim of dietary management is to prevent the accumulation of phenylalanine, tyrosine and sometimes methionine by means of a low protein diet. The protein requirements are met by supplementing the diet with a tyrosine, phenylalanine and/or methionine free amino acid mixture. If NTBC is prescribed then a phenylalanine and tyrosine free protein substitute is normally used. A phenylalanine, tyrosine and methionine free protein substitute is used only when the patient is unresponsive to NTBC or it is unavailable. Whilst dietary management is important in Tyrosinaemia, NTBC has significantly improved management and survival in Tyrosinaemia type I.

Tyrosinaemia type II is caused by a deficiency of tyrosine aminotransferase leading to eye lesions, skin lesions and neurological complications. The aim of dietary management is to prevent the accumulation of phenylalanine and tyrosine by means of a low protein diet. The protein requirements are met by supplementing the diet with a tyrosine and phenylalanine free amino acid mixture.

Tyrosinaemia type III is a very rare form of tyrosinaemia resulting in convulsions, ataxia and mental retardation. The aim of dietary management is to prevent the accumulation of phenylalanine and tyrosine by means of a low protein diet. The protein requirements are met by supplementing the diet with a tyrosine and phenylalanine free amino acid mixture. Guidance on amino acid intakes have been extrapolated from MRC guidelines on PKU management.

HCP Information for Tyrosinaemia

The content provided by Nutricia is for information purposes only and is in no way intended to be a substitute for medical consultation with your doctor, dietitian or healthcare professional. The information, opinions, and recommendations presented in these pages are not intended to replace the care of your own doctor, dietitian or healthcare professional. Before you make any changes in the management of your diet / treatment or any other persons diet /treatment you should always consult your doctor, dietitian or healthcare professional. Although we carefully review our content, Nutricia cannot guarantee or take responsibility for the medical accuracy of documents we publish, nor can Nutricia assume any liability for the content of any web site linked to our site. © 2016 Nutricia. All rights reserved.