ArticleJournal of inherited metabolic disease2010
Fatty acid profile in patients with phenylketonuria and its relationship with bone mineral density.
Article in Journal of inherited metabolic disease, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 6 of them syntheses that pooled it.
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Who cites it
23 citing papers in PubMed, 6 syntheses or guidelines pooled it, 61 citations in OpenAlex.
- Meta-analysis of bone mineral density in adults with phenylketonuria.Orphanet journal of rare diseases · 2024Pooled it
- Systematic literature review of the somatic comorbidities experienced by adults with phenylketonuria.Orphanet journal of rare diseases · 2024Pooled it
- Burden of phenylketonuria in Latin American patients: a systematic review and meta-analysis of observational studies.Orphanet journal of rare diseases · 2022Pooled it
- Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis.Orphanet journal of rare diseases · 2018Pooled it
- Bone health in phenylketonuria: a systematic review and meta-analysis.Orphanet journal of rare diseases · 2015Pooled it
- A systematic review of bone mineral density and fractures in phenylketonuria.Journal of inherited metabolic disease · 2014Pooled it
- Effects ofThe British journal of nutrition · 2025Article
- Evaluation of bone mineral density and biochemical markers in pediatric patients with phenylketonuria.Molecular genetics and metabolism reports · 2024Article
- Nutrient Status and Intakes of Adults with Phenylketonuria.Nutrients · 2024Article
- Creatine energy substrate increases bone density in the PahMolecular genetics and metabolism reports · 2023Article
- Glutamine energy substrate anaplerosis increases bone density in the PahJIMD reports · 2022Article
- Phenylketonuria oxidative stress and energy dysregulation: Emerging pathophysiological elements provide interventional opportunity.Molecular genetics and metabolism · 2022Review
- Effect of nutritional intervention on nutritional status among children with disorders of amino acid and nitrogen metabolism (AANMDs): A scoping review.Intractable & rare diseases research · 2021Article
- Mesenchymal stem cell energy deficit and oxidative stress contribute to osteopenia in the PahMolecular genetics and metabolism · 2021Article
- Article
- Bone mineral density is within normal range in most adult phenylketonuria patients.Journal of inherited metabolic disease · 2020Article
- A bone mineralization defect in the PahMolecular genetics and metabolism · 2018Article
- Analysis of the functional muscle-bone unit of the forearm in patients with phenylketonuria by peripheral quantitative computed tomography.Journal of inherited metabolic disease · 2017Article
- Micronutrients, Essential Fatty Acids and Bone Health in Phenylketonuria.Annals of nutrition & metabolism · 2017Article
- Impact of Dietary Intake on Bone Turnover in Patients with Phenylalanine Hydroxylase Deficiency.JIMD reports · 2017Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPatients with phenylketonuria (PKU) undergo a restrictive vegan-like diet, with almost total absence of n-3 fatty acids, which have been proposed as potential contributors to bone formation in the healthy population. The PKU diet might lead these patients to bone mass loss and, consequently, to the development of osteopenia/osteoporosis. Therefore, we proposed to analyze their plasma fatty acid profile status and its relationship with bone health.
methodsWe recruited 47 PKU patients for this cross-sectional study and divided the cohort into three age groups (6-10 years, 11-18 years, 19-42 years). We measured their plasma fatty acid profile and bone mineral density (BMD) (both at the femoral neck and the lumbar spine). Seventy-seven healthy controls also participated as reference values of plasma fatty acids.
resultsDocosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA) and total n-3 fatty acids were significantly diminished in PKU patients compared with healthy controls. DHA, EPA, and total n-3 fatty acids were also positively associated with bone mineral density (r = 0.83, p = 0.010; r = 0.57, p = 0.006; r = 0.73, p = 0.040, respectively). There was no association between phenylalanine (Phe), Index of Dietary Control (IDC), calcium, 25-hydroxivitamin D concentrations, daily calcium intake, and BMD.
conclusionOur results suggest a possible influence of essential fatty acids over BMD in PKU patients. The lack of essential n-3 fatty acids intake in the PKU diet might affect bone mineralization. Further clinical trials are needed to confirm the effect of the n-3 essential fatty acids on bone accrual in a cohort of PKU patients.
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