| Title: |
Meta-analysis of bone mineral density in adults with phenylketonuria |
| Authors: |
Rocha, Júlio César; Hermida, Álvaro; Jones, Cheryl J.; Wu, Yunchou; Clague, Gillian E.; Rose, Sarah; Whitehall, Kaleigh B.; Ahring, Kirsten K.; Pessoa, André L. S.; Harding, Cary O.; Rohr, Fran; Inwood, Anita; Longo, Nicola; Muntau, Ania C.; Sivri, Serap; Maillot, François |
| Source: |
http://lobid.org/resources/99370675069506441#!, 19(1):338. |
| Publication Year: |
2024 |
| Collection: |
Publisso (ZB MED-Publikationsportal Lebenswissenschaften) |
| Subject Terms: |
Female [MeSH]; Systematic Reviews as Topic [MeSH]; Phenylketonuria; Phenylketonurias/physiopathology [MeSH]; Adult [MeSH]; Humans [MeSH]; Meta-analysis; Z-score; Diet; Diet adherence; Osteoporosis; Phenylalanine/blood [MeSH]; Bone; Osteopenia; Bone Density/physiology [MeSH]; Male [MeSH]; Phenylalanine; Research; Bone mineral density |
| Description: |
AbstractBackgroundLifelong management of phenylketonuria (PKU) centers on medical nutrition therapy, including dietary phenylalanine (Phe) restriction in addition to Phe-free or low-Phe medical foods/protein substitutes. Studies have reported low bone mineral density (BMD) in mixed-age PKU populations, possibly related to long-term Phe restriction. Therefore, a meta-analysis investigating BMD specifically in adults with PKU was conducted.MethodsStudies reporting BMD-related outcomes were identified from a systematic literature review evaluating somatic comorbidities experienced by adults with PKU on a Phe-restricted diet (searched February 1, 2022, updated November 1, 2023). Risk of study bias was assessed (Scottish Intercollegiate Guidelines Network checklists). The primary outcome of the meta-analysis was pooled mean BMD Z-scores of different bones. Secondary outcomes were the prevalence of low BMD Z-scores at pre-specified thresholds. Subgroup analyses of mean BMD Z-scores (decade of study publication, controlled versus uncontrolled blood Phe levels, gender) were conducted.ResultsBMD-related data from 4097 individuals across 10 studies rated as at least acceptable quality were included. Mean BMD Z-scores were statistically significantly lower compared with an age-matched control or reference (non-PKU) population, across bones, but still within the expected range for age (> -2.0): lumbar spine (seven studies,n = 304), -0.63 (95% confidence interval (CI): -0.74, -0.52); femoral neck (four studies,n = 170), -0.74 (95% CI: -1.25, -0.22); radius (three studies,n = 114), -0.77 (95% CI: -1.21, -0.32); ... |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
https://repository.publisso.de/resource/frl:6505212; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11391789/ |
| DOI: |
10.1186/s13023-024-03223-9 |
| Availability: |
https://repository.publisso.de/resource/frl:6505212; https://doi.org/10.1186/s13023-024-03223-9; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11391789/ |
| Rights: |
https://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.B3D24275 |
| Database: |
BASE |