Objective
Early treatment of phenylketonuria (ET-PKU) prevents mental retardation, but many patients still show cognitive and mood problems. In this study, it was investigated whether ET-PKU-patients ...have specific phenylalanine (Phe-)related problems with respect to social-cognitive functioning and social skills.
Methods
Ninety five PKU-patients (mean age 21.6 ± 10.2 years) and 95 healthy controls (mean age 19.6 ± 8.7 years) were compared on performance of computerized and paper-and-pencil tasks measuring social-cognitive abilities and on parent- and self-reported social skills, using multivariate analyses of variance, and controlling for general cognitive ability (IQ-estimate). Further comparisons were made between patients using tetrahydrobiopterin (BH4,
N
= 30) and patients not using BH4. Associations with Phe-levels on the day of testing, during childhood, during adolescence and throughout life were examined.
Results
PKU-patients showed poorer social-cognitive functioning and reportedly had poorer social skills than controls (regardless of general cognitive abilities). Quality of social-cognitive functioning was negatively related to recent Phe-levels and Phe-levels between 8 and 12 years for adolescents with PKU. Quality of social skills was negatively related to lifetime phenylalanine levels in adult patients, and specifically to Phe-levels between 0 and 7, and between 8 and 12 years. There were no differences with respect to social outcome measures between the BH
4
and non-BH
4
groups.
Conclusion
PKU-patients have Phe-related difficulties with social-cognitive functioning and social skills. Problems seem to be more evident among adolescents and adults with PKU. High Phe-levels during childhood and early adolescence seem to be of greater influence than current and recent Phe-levels for these patients.
Context.
The Solar Orbiter spacecraft will be equipped with a wide range of remote-sensing (RS) and in situ (IS) instruments to record novel and unprecedented measurements of the solar atmosphere and ...the inner heliosphere. To take full advantage of these new datasets, tools and techniques must be developed to ease multi-instrument and multi-spacecraft studies. In particular the currently inaccessible low solar corona below two solar radii can only be observed remotely. Furthermore techniques must be used to retrieve coronal plasma properties in time and in three dimensional (3D) space. Solar Orbiter will run complex observation campaigns that provide interesting opportunities to maximise the likelihood of linking IS data to their source region near the Sun. Several RS instruments can be directed to specific targets situated on the solar disk just days before data acquisition. To compare IS and RS, data we must improve our understanding of how heliospheric probes magnetically connect to the solar disk.
Aims.
The aim of the present paper is to briefly review how the current modelling of the Sun and its atmosphere can support Solar Orbiter science. We describe the results of a community-led effort by European Space Agency’s Modelling and Data Analysis Working Group (MADAWG) to develop different models, tools, and techniques deemed necessary to test different theories for the physical processes that may occur in the solar plasma. The focus here is on the large scales and little is described with regards to kinetic processes. To exploit future IS and RS data fully, many techniques have been adapted to model the evolving 3D solar magneto-plasma from the solar interior to the solar wind. A particular focus in the paper is placed on techniques that can estimate how Solar Orbiter will connect magnetically through the complex coronal magnetic fields to various photospheric and coronal features in support of spacecraft operations and future scientific studies.
Methods.
Recent missions such as STEREO, provided great opportunities for RS, IS, and multi-spacecraft studies. We summarise the achievements and highlight the challenges faced during these investigations, many of which motivated the Solar Orbiter mission. We present the new tools and techniques developed by the MADAWG to support the science operations and the analysis of the data from the many instruments on Solar Orbiter.
Results.
This article reviews current modelling and tool developments that ease the comparison of model results with RS and IS data made available by current and upcoming missions. It also describes the modelling strategy to support the science operations and subsequent exploitation of Solar Orbiter data in order to maximise the scientific output of the mission.
Conclusions.
The on-going community effort presented in this paper has provided new models and tools necessary to support mission operations as well as the science exploitation of the Solar Orbiter data. The tools and techniques will no doubt evolve significantly as we refine our procedure and methodology during the first year of operations of this highly promising mission.
Measurement of residual disease following neoadjuvant chemotherapy that accurately predicts long-term survival in locally advanced breast cancer (LABC) is an essential requirement for clinical trials ...development. Several methods to assess tumor response have been described. However, the agreement between methods and correlation with survival in independent cohorts has not been reported.
We report survival and tumor response according to the measurement of residual breast cancer burden (RCB), the Miller and Payne classification and the Response Evaluation Criteria in Solid Tumors (RECIST) criteria, in 151 LABC patients. Kappa Cohen's coefficient (К) was used to test the agreement between methods. We assessed the correlation between the treatment outcome and overall survival (OS) and relapse-free survival (RFS) by calculating Harrell's C-statistic (c).
The agreement between Miller and Payne classification and RCB classes was very high (К = 0.82). In contrast, we found a moderate-to-fair agreement between the Miller and Payne classification and RECIST criteria (К = 0.52) and RCB classes and RECIST criteria (К = 0.38). The adjusted C-statistic to predict OS for RCB index (0.77) and RCB classes (0.75) was superior to that of RECIST criteria (0.69) (P = 0.007 and P = 0.035, respectively). Also, RCB index (c = 0.71), RCB classes (c = 0.71) and Miller and Payne classification (c = 0.67) predicted better RFS than RECIST criteria (c = 0.61) (P = 0.005, P = 0.006 and P = 0.028, respectively).
The pathological assessment of tumor response might provide stronger prognostic information in LABC patients.
Current evidence for the effectiveness of post-stroke cognitive rehabilitation is weak, possibly due to two reasons. First, patients typically express cognitive deficits in several domains. Therapies ...focusing on specific cognitive deficits might not address their interrelated neurological nature. Second, co-occurring psychological problems are often neglected or not diagnosed, although post-stroke depression is common and related to cognitive deficits. This pilot trial aims to test a rehabilitation program in virtual reality that trains various cognitive domains in conjunction, by adapting to the patient's disability and while investigating the influence of comorbidities.
Thirty community-dwelling stroke patients at the chronic stage and suffering from cognitive impairment performed 30 min of daily training for 6 weeks. The experimental group followed, so called, adaptive conjunctive cognitive training (ACCT) using RGS, whereas the control group solved standard cognitive tasks at home for an equivalent amount of time. A comprehensive test battery covering executive function, spatial awareness, attention, and memory as well as independence, depression, and motor impairment was applied at baseline, at 6 weeks and 18-weeks follow-up.
At baseline, 75% of our sample had an impairment in more than one cognitive domain. The experimental group showed improvements in attention (Formula: see text (2) = 9.57, p < .01), spatial awareness (Formula: see text (2) = 11.23, p < .01) and generalized cognitive functioning (Formula: see text (2) = 15.5, p < .001). No significant change was seen in the executive function and memory domain. For the control group, no significant change over time was found. Further, they worsened in their depression level after treatment (T = 45, r = .72, p < .01) but returned to baseline at follow-up. The experimental group displayed a lower level of depression than the control group after treatment (Ws = 81.5, z = - 2.76, r = - .60, p < .01) and (Ws = 92, z = - 2.03, r = - .44, p < .05).
ACCT positively influences attention and spatial awareness, as well as depressive mood in chronic stroke patients.
The trial was registered prospectively at ClinicalTrials.gov (NCT02816008) on June 21, 2016.
Background
Crohn’s disease (CD) and ulcerative colitis (UC) are inflammatory bowel diseases (IBD) that contributes in part to irreversible bowel damage and long-term complications, reduced quality of ...life, invalidity, and economic burden. Suboptimal control of IBD is associated with higher healthcare resource utilization (HCRU), impaired quality of life (QoL), and reduced work productivity.
Aims
The IBD-PODCAST study aimed to assess the proportion of IBD patients with suboptimal control and its associated impact.
Methods
IBD-PODCAST is a cross-sectional, multicenter study that aimed to characterize the CD and UC population with optimal or suboptimal control according to the STRIDE-II criteria and patient- and physician-reported measures. Here we present the results of the Spanish cohort (
n
= 396).
Results
A total of 104/196 (53.1%) CD and 83/200 (41.5%) UC patients were found to have suboptimal disease control. Long-term treatment targets according to STRIDE-II were applied in 172 (87.8%) CD and 181 (90.5%) UC patients. 125 of 172 (72.7%) CD and 74 of 181 (40.9%) UC patients were currently treated with targeted immunomodulators. Patients with CD and UC and suboptimal disease control showed impaired QoL, higher HCRU and direct costs, and also loss of work productivity compared to those with optimal control.
Conclusion
Despite a high rate of targeted immunomodulator therapy, a substantial proportion of IBD patients show suboptimal disease control according to the STRIDE II criteria. Those patients with suboptimal disease control exhibit impaired QoL, less work productivity, and higher HCRU, suggesting that there is considerable need for better treatment approaches in IBD.
Abstract Background Cytoreductive surgery with peritonectomy procedures and hyperthermic intraperitoneal chemotherapy (CRS + HIPEC) represents a radical therapeutic approach to achieve complete ...cytoreduction in ovarian peritoneal carcinomatosis. The aim of the present study was to analyze the outcomes obtained by the application of these procedures in a single center with extensive experience treating peritoneal carcinomatosis. Patients and methods A series of 218 consecutive patients diagnosed with peritoneal carcinomatosis from primary or recurrent ovarian cancer (FIGO stage IIIC–IV) and treated with CRS + HIPEC between January 1996 and June 2012 were included in this observational study. Results Peritoneal carcinomatosis was treated primarily in 56% (124/218) of the cases and recurrently in 43% (94/218). A total of 42/218 patients (19%) presented with FIGO stage IV. Compared to recurrent cases, patients with primary ovarian carcinomatosis were older and presented higher Peritoneal Cancer Index (PCI) and percentage of FIGO stage IV; however, no significant differences in survival (5-year overall survival in patients with R0 cytoreduction, 63% and 56%, respectively) were observed. Cytoreduction score, PCI, lymphatic involvement and surgical morbidity ≥Grade III were statistically significant prognostic factors for survival in both univariate and multivariate analysis. Conclusions CRS + HIPEC treating macroscopic and microscopic disease is currently an excellent surgical approach to achieve high rates of complete cytoreduction and improve survival in patients with peritoneal carcinomatosis from ovarian cancer. In order to minimize the high potential morbidity of these procedures, CRS + HIPEC should be performed in highly experienced centers.
The assembly of biological communities depends on deterministic and stochastic processes whose influence varies across spatial and temporal scales. Although ectomycorrhizal (ECM) fungi play a key ...role in forest ecosystems, our knowledge on ECM community assembly processes and their dependency on spatial scales is still scarce. We analysed the assembly processes operating on ECM fungal communities associated with
Cistus albidus
L. and
Quercus
spp. in Mediterranean mixed forests (Southern Spain), for which root tip ECM fungi were characterized by high-throughput sequencing. The relative contribution of deterministic and stochastic processes that govern the ECM fungal community assembly was inferred by using phylogenetic and compositional turnover descriptors across spatial scales. Our results revealed that stochastic processes had a significantly higher contribution than selection on root tip ECM fungal community assembly. The strength of selection decreased at the smallest scale and it was linked to the plant host identity and the environment. Dispersal limitation increased at finer scales, whilst drift showed the opposite pattern likely suggesting a main influence of priority effects on ECM fungal community assembly. This study highlights the potential of phylogeny to infer ECM fungal community responses and brings new insights into the ecological processes affecting the structure and dynamics of Mediterranean forests.
The application of probiotics on aquatic animals is increasing for a better fish welfare status as well as an environment-friendly activity which are actual demands of modern aquaculture industry. A ...bacterium from skin mucus of healthy gilthead seabream (
Sparus aurata
L.) has been isolated and identified as
Shewanella putrefaciens
Pdp11. Different studies have been done to know its application as probiotic in the Senegalese sole and gilthead seabream farming. This article reviews the studies carried out with this probiotic microorganism focusing on the current knowledge of its in vitro and in vivo mechanisms of action. The results suggested that the probiotic
S. putrefaciens
Pdp11, due to its beneficial effects, could be used in the aquaculture activity of both species.
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