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Bone mineral density in French adults with early-treated phenylketonuria
| dc.rights.license | open | en_US |
| dc.contributor.author | DYBAL, Elisa | |
| dc.contributor.author | MAILLOT, Francois | |
| dc.contributor.author | FEILLET, Francois | |
| dc.contributor.author | FOUILHOUX, Alain | |
| dc.contributor.author | ASTUDILLO, Leonardo | |
| dc.contributor.author | LAVIGNE, Christian | |
| dc.contributor.author | ARNOUX, Jean-Baptiste | |
| dc.contributor.author | ODENT, Sylvie | |
| dc.contributor.author | GAY, Claire | |
| dc.contributor.author | SCHIFF, Manuel | |
| dc.contributor.author | MAZODIER, Karin | |
| dc.contributor.author | KUSTER, Alice | |
| hal.structure.identifier | Bordeaux population health [BPH] | |
| dc.contributor.author | RIGALLEAU, Vincent
IDREF: 069788146 | |
| dc.contributor.author | THAUVIN-ROBINET, Christel | |
| dc.contributor.author | LEGUY-SEGUIN, Vanessa | |
| dc.contributor.author | DOUILLARD, Claire | |
| dc.contributor.author | CHARRIERE, Sybil | |
| dc.date.accessioned | 2025-04-24T08:50:09Z | |
| dc.date.available | 2025-04-24T08:50:09Z | |
| dc.date.issued | 2025-03 | |
| dc.identifier.issn | 1096-7206 | en_US |
| dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/206390 | |
| dc.description.abstractEn | Phenylketonuria (PKU) treatment requires a low-phenylalanine (Phe) diet limiting natural protein intake, using medical low-protein foods and Phe-free amino acids (AA) supplements along with micronutriments' supplies. Current recommendations suggest maintaining this diet for life to prevent neuro-psychological effects of high Phe concentrations. The long-term consequences of such a diet are poorly understood, particularly on bone health. Our study aimed to assess the prevalence of low bone mineral density (BMD) (Z-score ≤ -2, for vertebral and/or femoral site) in adults with PKU and to investigate associated risk factors, in the French ECOPHEN cohort. The study included 171 patients with 67.3 % of women and a median age of 27 years old. The median femoral and vertebral Z-scores of BMD were both - 0.6. Only 11.4 % of patients had a low BMD. Compared to patients with normal BMD, patients with low BMD had a lower body mass index (BMI) (median 20.4 versus 24.4 kg/m(2), p = 0.002), and were more likely to have never stopped their diet (58.8 % versus 31.8 %, p = 0.029). They also had higher total protein intake (1.1 versus 0.9 g/kg/day, p = 0.015), with more proteins from AA supplements (0.80 vs 0.53 g/kg/day, p = 0.010). In multivariate analysis, younger patients born after 1990 and who never stopped diet had a 4.7 times risk to have low BMD (p = 0.005), after adjustment on age, sex, BMI. In summary, our study identified a subgroup of PKU adult patients with low BMD and showed that prolonged low natural protein diet is associated with low BMD. | |
| dc.language.iso | EN | en_US |
| dc.rights | Attribution-NonCommercial 3.0 United States | * |
| dc.rights.uri | http://creativecommons.org/licenses/by-nc/3.0/us/ | * |
| dc.subject.en | Adult | |
| dc.subject.en | Bone mineral density | |
| dc.subject.en | Fracture | |
| dc.subject.en | Low-phenylalanine diet | |
| dc.subject.en | Phenylketonuria | |
| dc.title.en | Bone mineral density in French adults with early-treated phenylketonuria | |
| dc.title.alternative | Mol Genet Metab | |
| dc.type | Article de revue | en_US |
| dc.identifier.doi | 10.1016/j.ymgme.2025.109044 | en_US |
| dc.subject.hal | Sciences du Vivant [q-bio]/Santé publique et épidémiologie | en_US |
| dc.identifier.pubmed | 39919675 | en_US |
| bordeaux.journal | Molecular Genetics and Metabolism | en_US |
| bordeaux.page | 109044 | en_US |
| bordeaux.volume | 144 | en_US |
| bordeaux.hal.laboratories | Bordeaux Population Health Research Center (BPH) - UMR 1219 | en_US |
| bordeaux.issue | 3 | en_US |
| bordeaux.institution | Université de Bordeaux | en_US |
| bordeaux.institution | INSERM | en_US |
| bordeaux.team | LEHA_BPH | en_US |
| bordeaux.peerReviewed | oui | en_US |
| bordeaux.inpress | non | en_US |
| hal.popular | non | en_US |
| hal.audience | Internationale | en_US |
| hal.export | false | |
| dc.rights.cc | Pas de Licence CC | en_US |
| bordeaux.COinS | ctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Molecular%20Genetics%20and%20Metabolism&rft.date=2025-03&rft.volume=144&rft.issue=3&rft.spage=109044&rft.epage=109044&rft.eissn=1096-7206&rft.issn=1096-7206&rft.au=DYBAL,%20Elisa&MAILLOT,%20Francois&FEILLET,%20Francois&FOUILHOUX,%20Alain&ASTUDILLO,%20Leonardo&rft.genre=article |