Hereditary tyrosinemia type I: A new clinical classification with difference in prognosis on dietary treatment
Francjan J. Van Spronsen, Yolande Thomasse, G. Peter A. Smit, James V. Leonard, Peter T. Clayton, Vaclav Fidler, Ruud Berger, Hugo S. A. Heymans – 1 November 1994 – Hereditary tyrosinemia type I (McKusick 27670) is a heterogeneous disease with poor prognosis, yet there are few reports of the long‐term prognosis. It is therefore difficult to decide on the treatment for individual patients. We have conducted an international survey of patients with tyrosinemia type I and examined the probability of survival on dietary treatment and the causes of death in 108 patients with tyrosinemia type I.