Tyrosinemia: the Quebec experience


Fig. 3: Outcome in 36 patients with tyrosinemia evaluated before liver transplantation (as of June 1995). Patients are classified according to their main presenting feature (liver nodule son imaging, neurological crises, lack of symptoms, liver failure or renal failure). NTBC therapy was offered as of 1993. These patients do not make up the entire population with tyrosinemia in the province of Quebec. **This patient's serum creatinine level decreased from 200 to 140 mmol/L in the first year of NTBC therapy. ***This patient's liver failure resolved rapidly after NTBC therapy was started, and the patient's condition is now stable. VDBS = vanishing bile duct syndrome.

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