The purpose of this thesis was to conduct an overview of the literature on tyro-sinemia type 1 in order to determine the symptoms and complications of the disease and what the patients should pay special attention in their diet to ensure the best possible quality of life and survival. Tyrosinemia type 1 is a congenital disease of catabolism of the amino acid tyrosine due to decreased or absent activity of the en-zyme fumarylacetoacetate hydrolase (FAH), which in the course of the disease manifests itself as progressive hepatic impairment, renal tubular dysfunction and porphyria-like metabolic crisis. According to research, it has been established that patients should start treatment with nitisinone and urgent treatment of acute liver failure, if necessary. In parallel with these measures, restrictions on dietary protein intake (restriction of tyrosine and phenylalanine) are also introduced. In this thesis, we came to the conclusion that every patient should be regularly monitored by a specialised doctor who is focused on working with congenital metabolic diseases and clinical dietitian. Undoubtedly, the patient must take nitisinone for life and fol-low a strict diet.
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