{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Olsson B"],"funding":["European Research Council"],"pagination":["41-49"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC8743336"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["63(1)"],"pubmed_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-HGA<sub>24</sub>) and urea (u-urea<sub>24</sub>, used as an approximate protein intake measure), tyrosine and body weight were performed using paired <i>t</i> tests. Comparisons of"],"journal":["JIMD reports"],"pubmed_title":["Effects of a protein-restricted diet on body weight and serum tyrosine concentrations in patients with alkaptonuria."],"pmcid":["PMC8743336"],"funding_grant_id":["304985"],"pubmed_authors":["Milan A","Arnoux JB","Ranganath L","Imrich R","Rudebeck M","Olsson B"],"additional_accession":[]},"is_claimable":false,"name":"Effects of a protein-restricted diet on body weight and serum tyrosine concentrations in patients with alkaptonuria.","description":"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-HGA<sub>24</sub>) and urea (u-urea<sub>24</sub>, used as an approximate protein intake measure), tyrosine and body weight were performed using paired <i>t</i> tests. Comparisons of","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 Jan","modification":"2026-05-07T20:18:44.898Z","creation":"2022-02-11T16:17:00.053Z"},"accession":"S-EPMC8743336","cross_references":{"pubmed":["35028270"],"doi":["10.1002/jmd2.12255"]}}