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OCR: Incidence: 1:100,000 (1:2000 in Quebec) Mechanism and substrate: Buildup in tyrosine in liver Clinical features Development: failure to thrive; mild to moderate mental retardation if untreated Neurologic: disturbances of coordination Skin: dry, ulcer-like blisters of hands and soles Eyes: corneal ulcers and plaques Liver: chronic hepatic cirrhosis that leads to severe or fatal liver failure Treatment: Dietary restriction of tyrosine and phenylalanine through low-protein diet and special formula ..