Considered to be chronic form of tyrosinemia type 1 or to occur in survivors of acute form of syndrome. Caused by deficiency of fumaryl acetoacetase. Most commonly seen in French Canadian population in Quebec. Marked by nodular cirrhosis, kidney edema and variable hyperplasia of islets of Langerhans. Usu. patients develop hypophosphatemic rickets, Fanconi syndrome and altered electrolyte metabolism. Urine develops a cabbage-type odor. Patients should be restricted to diet that is low in phenylalanine and tyrosine.