Tyrosinemia Type I

Treatment

Is there a treatment(s) for Tyrosinemia Type I?

The treatment regimen that is recommended for people with Tyrosinemia is a low-protein diet (low phenylalanine and low tyrosine), nitisinone (a drug that stops the breakdown of tyrosine to prevent succinylacetone production), and medical formula that supplements all amino acids other than phenylalanine and tyrosine.

To find a metabolic dietitian specializing in diets for people with tyrosinemia, visit the GMDI Find a Metabolic Dietitian webpage.

References
  • Lisa Sniderman King, MSc, CGC, Cristine Trahms, MS, RD, and C Ronald Scott, MD
  • http://www.ncbi.nlm.nih.gov/books/NBK1515/
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Are there other organ or symptom specific treatments for Tyrosinemia Type I?

Are there other organ or symptom specific treatments for Tyrosinemia Type I?

Before the development of nitisinone as a treatment, liver transplantation was the only effective treatment for Tyrosinemia Type I. Now, liver transplantation should be reserved for children who have severe liver when they are diagnosed or do not respond to nitisinone or people who have been diagnosed with malignant (cancerous) changes in their liver.

References
  • Lisa Sniderman King, MSc, CGC, Cristine Trahms, MS, RD, and C Ronald Scott, MD
  • http://www.ncbi.nlm.nih.gov/books/NBK1515/

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