<unk>  is the most severe form of this disorder and is caused by a shortage of the enzyme fumarylacetoacetate hydrolase (EC 3.7.1.2), encoded by the gene FAH found on chromosome number 15. Fumarylacetoacetate hydrolase is the last in a series of five enzymes needed to break down tyrosine. Symptoms of<unk>  usually appear in the first few months of life and include failure to gain weight and grow at the expected rate (failure to thrive), diarrhea, vomiting, yellowing of the skin and whites of the eyes (jaundice), cabbagelike odor, and increased tendency to bleed (particularly nosebleeds).<unk>  can lead to liver and kidney failure, problems affecting the nervous system, and an increased risk of liver cancer.
Worldwide,<unk>  affects about 1 person in 100,000. This type of tyrosinemia is much more common in Quebec, Canada. The overall incidence in Quebec is about 1 in 16,000 individuals. In the Saguenay-Lac-Saint-Jean region of Quebec,<unk>  affects 1 person in 1,846.
Type I tyrosinemia
Type II tyrosinemia
Type III tyrosinemia
Tyrosinemia
Essential fructosuria
Phenylketonuria
Glycogen storage disease type I CT
Homocystinuria