376 research outputs found
Change in acetabular version after lumbar pedicle subtraction osteotomy to correct post-operative flat back: EOS® measurements of 38 acetabula
AbstractBackgroundAbnormalities in acetabular orientation can promote the development of hip osteoarthritis, femoro-acetabular impingement, or even acetabular cup malposition. The objective of the present study was to determine whether pedicle substraction osteotomy (PSO) to correct sagittal spinal imbalance affected acetabular orientation.HypothesisPSO performed to correct sagittal spinal imbalance affects acetabular orientation by changing the pelvic parameters.Materials and methodsThis was a descriptive study in which two observers measured the acetabular parameters on both sides in 19 patients (38 acetabula) before and after PSO for post-operative flat-back syndrome. Mean time from PSO to post-operative measurements was 19months. Measurements were taken twice at a 2-week interval, on standing images obtained using the EOS® imaging system and sterEOS® software to obtain 3D reconstructions of synchronised 2D images. Acetabular anteversion and inclination were measured relative to the vertical plane. Mean pre-PSO and post-PSO values were compared using the paired t-test, and P values lower than 0.05 were considered significant. To assess inter-observer and intra-observer reproducibility, we computed the intra-class correlation coefficients (ICCs).ResultsThe measurements showed significant acetabular retroversion after PSO, of 7.6° on the right and 6.5° on the left (P<0.001). Acetabular inclination diminished significantly, by 4.5° on the right and 2.5° on the left (P<0.01). Inclination of the anterior pelvic plane decreased by 8.4° (P<0.01). Pelvic incidence was unchanged, whereas sacral slope increased by 10.5° (P<0.001) and pelvic tilt decreased by 10.9° (P<0.001). The ICC was 0.98 for both inter-observer and intra-observer reproducibility.ConclusionChanging the sagittal spinal alignment modifies both the pelvic and the acetabular parameters. PSO significantly increases sacral slope, thus inducing anterior pelvic tilt with significant acetabular retroversion. The measurements obtained using sterEOS® showed good inter-observer and intra-observer reproducibility. To our knowledge, this is the first study of changes in acetabular version after PSO
Progressive refinement rendering of implicit surfaces
The visualisation of implicit surfaces can be an inefficient task when such surfaces are complex and highly detailed. Visualising a surface by first converting it to a
polygon mesh may lead to an excessive polygon count. Visualising a surface by direct ray casting is often a slow procedure. In this paper we present a progressive refinement renderer for implicit surfaces that are Lipschitz continuous. The renderer first displays a low resolution estimate of what the final image is going to be and, as the computation progresses, increases the quality of this estimate at an interactive frame rate. This renderer provides a quick previewing facility that significantly reduces the design cycle of a new and complex implicit surface. The renderer is also capable of completing an image faster than a conventional implicit surface rendering algorithm based on ray casting
PKU dietary handbook to accompany PKU guidelines
Background: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine. Main body: In 2017 the first European PKU Guidelines were published. These guidelines contained evidence based and/or expert opinion recommendations regarding diagnosis, treatment and care for patients with PKU of all ages. This manuscript is a supplement containing the practical application of the dietary treatment. Conclusion: This handbook can support dietitians, nutritionists and physicians in starting, adjusting and maintaining dietary treatment
Meta-analysis of bone mineral density in adults with phenylketonuria
© 2024. The Author(s).BACKGROUND: Lifelong 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. METHODS: Studies 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. RESULTS: BMD-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); total body (four studies, n = 157), -0.61 (95% CI: -0.77, -0.45). The small number of observations in the subgroup analyses resulted in a high degree of uncertainty, limiting interpretation. Estimated prevalence of BMD Z-scores ≤ -2.0 was 8% (95% CI: 5%, 13%; four studies, n = 221) and < -1.0 was 42% (95% CI: 35%, 51%; five studies, n = 144). CONCLUSIONS: Adults with PKU had lower BMD Z-scores than the reference (non-PKU) population but < 1 in 10 were below the expected range for age. The low number of studies prevents identification of which population characteristics are most impacting BMD. This meta-analysis was supported by BioMarin Pharmaceutical Inc., Novato, CA and is registered with the Research Registry (reviewregistry1476).publishersversionpublishe
Systematic literature review of the somatic comorbidities experienced by adults with phenylketonuria
Publisher Copyright: © The Author(s) 2024.Background: Phenylketonuria (PKU) is an inborn error of phenylalanine (Phe) metabolism that, if untreated, causes Phe accumulation in the brain leading to neurophysiologic alterations and poor outcomes. Lifelong management centers on dietary Phe restriction, yet long-term complete metabolic control is unachievable for many adults. High blood Phe levels or chronic Phe and intact protein restriction in the diet may lead to somatic comorbidities. A systematic literature review was conducted to evaluate somatic comorbidities experienced by adults with PKU. Methods: Clinical and observational studies reporting somatic comorbidities experienced by individuals with PKU aged ≥ 16 years (or classified as adults) evaluating a Phe-restricted diet with or without pharmacologic therapy versus no therapeutic intervention (including healthy controls), or pharmacologic therapy versus a Phe-restricted diet alone, were identified. PubMed® was searched (February 1, 2022 and updated November 1, 2023), using a pre-defined search strategy, followed by two-stage screening and data extraction. Included studies were grouped by PKU population comparison. Results: 1185 records were screened; 51 studies across 12,602 individuals were extracted. Bone-related abnormalities were the most reported outcome (n = 21); several outcome measures were used. Original study groupings included: Phe-restricted diet versus healthy controls or reference values (n = 40); treatment-adherent versus those non-adherent (n = 12). Additional groups added as part of a protocol amendment included: different Phe-restricted diets (n = 4); severe versus less severe disease (n = 5). Vote counting indicated a higher burden of ≥ 1 comorbidity (or outcome measure) for the Phe-restricted diet group by 37 of 38 studies included in the analysis of Phe-restricted diet versus healthy controls; higher burden in healthy controls was reported in 12 studies. Vote counting was similar between those treatment adherent (n = 7) versus non-adherent (n = 10). Conclusions: Adults with PKU have a higher comorbidity burden than a non-PKU population. More robust studies are needed to better understand the relationship between effective metabolic control and comorbidity burden, using consistent outcome measures. This SLR was supported by BioMarin Pharmaceutical Inc., Novato, CA, and is registered with the Research Registry (reviewregistry1476).publishersversionpublishe
Physical activity to improve cognition in older adults: can physical activity programs enriched with cognitive challenges enhance the effects? A systematic review and meta-analysis
: EPHPP quality rating scores (DOCX 38 kb
Extracellular DNA release, quorum sensing, and PrrF1/F2 small RNAs are key players in Pseudomonas aeruginosa tobramycin-enhanced biofilm formation
Biofilms are structured microbial communities that are the leading cause of numerous chronic infections which are difficult to eradicate. Within the lungs of individuals with cystic fibrosis (CF), Pseudomonas aeruginosa causes persistent biofilm infection that is commonly treated with aminoglycoside antibiotics such as tobramycin. However, sublethal concentrations of this aminoglycoside were previously shown to increase biofilm formation by P. aeruginosa, but the underlying adaptive mechanisms still remain elusive. Herein, we combined confocal laser scanning microscope analyses, proteomics profiling, gene expression assays and phenotypic studies to unravel P. aeruginosa potential adaptive mechanisms in response to tobramycin exposure during biofilm growth. Under this condition, we show that the modified biofilm architecture is related at least in part to increased extracellular DNA (eDNA) release, most likely as a result of biofilm cell death. Furthermore, the activity of quorum sensing (QS) systems was increased, leading to higher production of QS signaling molecules. We also demonstrate upon tobramycin exposure an increase in expression of the PrrF small regulatory RNAs, as well as expression of iron uptake systems. Remarkably, biofilm biovolumes and eDNA relative abundances in pqs and prrF mutant strains decrease in the presence of tobramycin. Overall, our findings offer experimental evidences for a potential adaptive mechanism linking PrrF sRNAs, QS signaling, biofilm cell death, eDNA release, and tobramycin-enhanced biofilm formation in P. aeruginosa. These specific adaptive mechanisms should be considered to improve treatment strategies against P. aeruginosa biofilm establishment in CF patients’ lungs
- …