Effects of a protein-restricted diet on body weight and serum tyrosine concentrations in patients with alkaptonuria.

ABSTRACT: In an open-label, controlled study of nitisinone in alkaptonuria (SONIA 2), patients were advised to lower dietary protein intake to reduce serum tyrosine (s-Tyr) levels and the risk of keratopathy. A body weight increase was observed in the nitisinone-treated patients but not in the control group. To investigate the effectiveness and consequence of protein restriction in patients with alkaptonuria, a post-hoc analysis of SONIA 2 was performed. One hundred and thirty-eight patients were randomised (nitisinone: n = 69, controls: n = 69). Comparison of baseline and Month 12 data on 24-h urinary excretion of HGA (u-HGA₂₄) and urea (u-urea₂₄, used as an approximate protein intake measure), tyrosine and body weight were performed using paired t tests. Comparisons of

SUBMITTER: Olsson B 

PROVIDER: S-EPMC8743336 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature