Clinical Findings in Tyrosinemia	
		
Liver crises (in the first 2 years of life)	Intercurrent viral infection and anorexia, irritability, vomiting	
	Increased liver size, ascites, anasarca and hemorrhagic diathesis	
	Jaundice	
	Laboratory: prolongation of the coagulation time, transaminase levels initially only mildly elevated, serum tyrosine, methionine and phenylalanine elevated and generalized aminoaciduria, serum a-fetoprotein estremely high 	
Cirrhosis	Liver decompensation, neurologic crises, renal involvement, hepatocarcinoma	
Hepatocellular carcinoma 		
Neurological crises	Painful paresthesias, extreme hyperextension of the trunck and neck, tonic convulsions	
	Hypertension and tachycardia; 	
	Weakness or paralysis, risk of respiratory insufficiency	
Renal disease	Range from no evidence of symptoms (with fibrosis on biopsy) to renal failure 	
Other	Pancreatic islet hypertrophy  with hypoglycemia; insulin-dependent diabetes mellitus; hypertrophic cardiomyopathy.