Liver Transplantation in Tyrosinaemia Type I: the Groningen Experience

Dietary treatment cannot prevent a lethal outcome in many patients with hereditary tyrosinaemia type I (van Spronsen et al 1994). Therefore, until the discovery of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) as a potential drug (Lindstedt et al 1992), orthotopic liver transplantation (OLT) was the only definitive treatment in patients with this disease (van Spronsen 1989). We report our experiences with OLT in 9 patients with tyrosinaemia type I.