SynthesisJournal of inherited metabolic disease2014
A systematic review of bone mineral density and fractures in phenylketonuria.
Synthesis in Journal of inherited metabolic disease, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04969809 (Comparison of Atherogenic Risk Factors and Efficacy of Nutritional Treatment Among Adult Phenylketonuria Patients), which is not on this map. Cited by 20 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Comparison of Atherogenic Risk Factors and Efficacy of Nutritional Treatment Among Adult Phenylketonuria Patients
Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 40 citations in OpenAlex.
- Bone health in phenylketonuria: a systematic review and meta-analysis.Orphanet journal of rare diseases · 2015Pooled it
- Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial.The American journal of clinical nutrition · 2016Trial
- Evaluation of bone mineral density and biochemical markers in pediatric patients with phenylketonuria.Molecular genetics and metabolism reports · 2024Article
- Association of serum levels of phenylalanine and tyrosine with hip fractures and frailty in older adults: The cardiovascular health study.Archives of osteoporosis · 2024Article
- Creatine energy substrate increases bone density in the PahMolecular genetics and metabolism reports · 2023Article
- Low bone mineralization in phenylketonuria may be due to undiagnosed metabolic acidosis.Molecular genetics and metabolism reports · 2023Article
- Glutamine energy substrate anaplerosis increases bone density in the PahJIMD reports · 2022Article
- Article
- A New View of Bone Loss in Phenylketonuria.Organogenesis · 2021Article
- Mesenchymal stem cell energy deficit and oxidative stress contribute to osteopenia in the PahMolecular genetics and metabolism · 2021Article
- Anthropometry and bone mineral density in treated and untreated hyperphenylalaninemia.Endocrine connections · 2020Article
- Sex effects of dietary protein source and acid load on renal and bone status in the PahPhysiological reports · 2019Article
- A bone mineralization defect in the PahMolecular genetics and metabolism · 2018Article
- Sex differences in body composition and bone mineral density in phenylketonuria: A cross-sectional study.Molecular genetics and metabolism reports · 2018Article
- Bone health in patients with inborn errors of metabolism.Reviews in endocrine & metabolic disorders · 2018Review
- The complete European guidelines on phenylketonuria: diagnosis and treatment.Orphanet journal of rare diseases · 2017Review
- 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
- Amino Acid Medical Foods Provide a High Dietary Acid Load and Increase Urinary Excretion of Renal Net Acid, Calcium, and Magnesium Compared with Glycomacropeptide Medical Foods in Phenylketonuria.Journal of nutrition and metabolism · 2017Article
- Impact of Dietary Intake on Bone Turnover in Patients with Phenylalanine Hydroxylase Deficiency.JIMD reports · 2017Article
- Glycomacropeptide is a prebiotic that reduces Desulfovibrio bacteria, increases cecal short-chain fatty acids, and is anti-inflammatory in mice.American journal of physiology. Gastrointestinal and liver physiology · 2015Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 2 countries.
Funding
Abstract
introductionOur objective was to systematically review and analyze published data on bone mineral density (BMD) and fracture rates in patients with phenylketonuria (PKU), and relationships between BMD and phenylalanine levels. METHODOLOGY: We searched PubMed, CINAHL, and Cochrane databases from January 1966 to November 2013 for studies of spine BMD or fracture in PKU and control subjects. We excluded studies assessing skeletal health by ultrasound or peripheral quantitative computer tomography. Both authors reviewed abstracts for inclusion, and read full text papers to extract data.
resultsSixteen studies met eligibility criteria. Meta-analysis of three studies found that spine BMD was 0.100 g/cm(2) lower (95% CI, -0.110, -0.090 g/cm(2)) in 67 subjects with PKU, compared to 161 controls. Among six studies, 20% (53 of 263) of PKU subjects experienced clinical fractures. In the single study with controls, the fracture rate was 2.6 fold higher (95% CI, 1.1-6.1) after age 8 in PKU subjects, compared to healthy sibling controls. When considering a total of 12 studies in 412 subjects, nine or 75% of studies representing 71% of studied subjects reported no association between phenylalanine levels and BMD. Spine BMD is lower in PKU than control subjects, but only one study controlled for smaller body size. Existing studies suggest a clinical fracture rate of 20% among PKU subjects, but fracture rates in controls are lacking. Finally, existing data shows no consistent relationship between phenylalanine levels and BMD. Future studies are needed to clarify the etiology and health consequences of low BMD in PKU.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.