Restoration efforts in the Mediterranean Basin have been changing from a silvicultural to an ecological restoration approach. Yet, to what extent the projects are guided by ecological restoration ...principles remains largely unknown. To analyse this issue, we built an on-line survey addressed to restoration practitioners.
We analysed 36 restoration projects, mostly from drylands (86%). The projects used mainly soil from local sources. The need to comply with legislation was more important as a restoration motive for European Union (EU) than for non-EU countries, while public opinion and health had a greater importance in the latter. Non-EU countries relied more on non-native plant species than EU countries, thus deviating from ecological restoration guidelines. Nursery-grown plants used were mostly of local or regional provenance, whilst seeds were mostly of national provenance. Unexpected restoration results (e.g. inadequate biodiversity) were reported for 50% of the projects and restoration success was never evaluated in 22%. Long term evaluation (>6years) was only performed in 31% of cases, and based primarily on plant diversity and cover. The use of non-native species and species of exogenous provenances may: i) entail the loss of local genetic and functional trait diversity, critical to cope with drought, particularly under the predicted climate change scenarios, and ii) lead to unexpected competition with native species and/or negatively impact local biotic interactions. Absent or inappropriate monitoring may prevent the understanding of restoration trajectories, precluding adaptive management strategies, often crucial to create functional ecosystems able to provide ecosystem services. The overview of ecological restoration projects in the Mediterranean Basin revealed high variability among practices and highlighted the need for improved scientific assistance and information exchange, greater use of native species of local provenance, and more long-term monitoring and evaluation, including functional and ecosystem services' indicators, to improve and spread the practice of ecological restoration.
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•The degree to which projects follow ecological restoration principles is unclear.•A survey was addressed to a wide range of practitioners in the Mediterranean Basin.•An ecological restoration practice index revealed high variability among practices.•More scientific assistance and use of native species of local provenance are needed.•Long-term monitoring based on ecosystems' functions and services should be promoted.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
The computer-adaptive test (CAT) of the European Organisation for Research and Treatment of Cancer (EORTC), the EORTC CAT Core, assesses the same 15 domains as the EORTC QLQ-C30 health-related ...quality of life questionnaire but with increased precision, efficiency, measurement range and flexibility. CAT parameters for estimating scores have been established based on clinical data from cancer patients. This study aimed at establishing the European Norm for each CAT domain based on general population data.
We collected representative general population data across 11 European Union (EU) countries, Russia, Turkey, Canada and the United States (n ≥ 1000/country; stratified by sex and age). We selected item subsets from each CAT domain for data collection (totalling 86 items). Differential item functioning (DIF) analyses were conducted to investigate cross-cultural measurement invariance. For each domain, means and standard deviations from the EU countries (weighted by country population, sex and age) were used to establish a T-metric with a European general population mean = 50 (standard deviation = 10).
A total of 15,386 respondents completed the online survey (n = 11,343 from EU countries). EORTC CAT Core norm scores for all 15 countries were calculated. DIF had negligible impact on scoring. Domain-specific T-scores differed significantly across countries with small to medium effect sizes.
This study establishes the official European Norm for the EORTC CAT Core. The European CAT Norm can be used globally and allows for meaningful interpretation of scores. Furthermore, CAT scores can be compared with sex- and age-adjusted norm scores at a national level within each of the 15 countries.
•This study establishes the official European Norm for the computer-adaptive test EORTC CAT Core based on 15,386 persons.•The European CAT Norm can be used globally and allows for meaningful interpretation of scores derived from EORTC CAT Core.•EORTC CAT Core scores can also be compared with sex- and age-adjusted norm scores at national levels of 15 included countries.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
We propose geographic information systems (GIS) as a framework in organization studies, particularly for scholars who consider the nuances of space and geography in various organizational contexts. ...GIS are computer-based systems that manage, store, analyze, and distribute spatial data. While more and more scholars recognize the theoretical significance of organizational space, suggestions for conducting empirical research around organizational space using alternative frameworks – such as GIS – are seldom made. We present an introduction to GIS and various spatial analyses through a case study of organizations in the reproductive healthcare field and offer future directions related to the geographic implications of understanding organizations and organizing through GIS.
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CEKLJ, IZUM, KILJ, NUK, PILJ, SAZU, UL, UM, UPUK
Abstract This international study aimed to test the measurement properties of the updated European Organisation for Research and Treatment of Cancer (EORTC) questionnaire module for colorectal ...cancer, the QLQ-CR29. The QLQ-CR29 was administered with the QLQ-C30, core questionnaire, to 351 patients from seven countries. Questionnaire scaling and reliability were established and clinical and psychometric validity examined. Patient acceptability and understanding were assessed with a debriefing questionnaire. Multi-trait scaling analyses and face validity refined the module to four scales assessing urinary frequency, faecal seepage, stool consistency and body image and single items assessing other common problems following treatment for colorectal cancer. Scales distinguished between clinically distinct groups of patients and did not correlate with QLQ-C30 scales, demonstrating construct validity. The QLQ-CR29 scores were reproducible over time in stable health. The EORTC QLQ-CR29 demonstrates sufficient validity and reliability to support its use to supplement the EORTC QLQ-C30 to assess patient-reported outcomes during treatment for colorectal cancer in clinical trials and other settings.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
The diffusion of tree crops has continuously increased during the last decades all over the world. The market boost has favored the adoption of intensive and highly mechanized cultivation, often ...triggering the degradation of the soil physical-hydrological qualities, mainly through enhanced soil erosion and compaction. Several papers have been published on soil degradation and restoration strategies in specific perennial crops and environments. This review paper collects such studies showing the sensitivity of soil under tree crops to the degradation of their physical-hydrological qualities. Then it reports the state of the art on the methodologies used for the evaluation of the physical-hydrological qualities in the field and in the laboratory, also suggesting an improved methodology for estimating the actual available water capacity. Some updated and promising experiences to recover the physical-hydrological qualities of soil are then illustrated. In particular, subsoiling and placement of drainages, spreading of organic amendments, compost, biochar, using of cover crops, and biological inoculants. A key point in applying the restoration practices is that they should not only be specific for the soil and tree rooting system, but also tailored according to the ecosystem functions that need to be improved besides plant health and yield.
Summary
Altogether 95 children with primary bone fragility were screened for variants in
PLS3
, the gene underlying X-linked osteoporosis. Two children with multiple peripheral and spinal fractures ...and low BMD had novel disease-causing
PLS3
variants. Children with milder phenotypes had no pathogenic variants.
PLS3
screening is indicated in childhood-onset primary osteoporosis.
Introduction
The study aimed to determine the role of pathogenic
PLS3
variants in children’s bone fragility and to elucidate the associated phenotypic features.
Methods
Two cohorts of children with bone fragility were screened for variants in
PLS3
, the gene underlying X-linked osteoporosis.
Cohort I
comprised 31 patients with childhood-onset primary osteoporosis of unknown etiology.
Cohort II
comprised 64 children who had sustained multiple fractures but were otherwise healthy. Clinical and radiological data were reviewed. Peripheral blood DNA was Sanger sequenced for coding exons and flanking intronic regions of
PLS3
.
Results
In two patients of
cohort I
, where other common genetic causes had been excluded, we identified two novel disease-causing
PLS3
variants.
Patient 1
was a male with bilateral femoral fractures at 10 years, low BMD (Z-score −4.1; 18 years), and multiple vertebral compression fractures. He had a novel nonsense variant in
PLS3
.
Patient 2
was a girl with multiple long bone and vertebral fractures and low BMD (Z-score −6.6 at 6 years). She had a de novo missense variant in
PLS3
; whole exome sequencing and array-CGH identified no other genetic causes. Iliac crest bone biopsies confirmed low-turnover osteoporosis in both patients. In
cohort II
, no pathogenic
PLS3
variants were identified in any of the subjects.
Conclusions
Two novel disease-causing variants in
PLS3
were identified in a boy and a girl with multiple peripheral and spinal fractures and very low BMD while no pathogenic variants were identified in children with less severe skeletal fragility.
PLS3
screening is warranted in male and female patients with childhood-onset primary osteoporosis.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
•Hyper progressive disease is a new form of progression in patients treated with immunotherapy.•The definition of HPD is yet to be consensual.•We suggest a clinical definition of patients refractory ...to immunotherapy based on the number of nivolumab injections.•20% of patients with advanced NSCLC presented with nivolumab refractory disease in our real-life cohort.•Factors associated with refractory disease were PS ≥ 2, shorter duration of treatment before nivolumab initiation.
: Immune checkpoint inhibitors (ICIs) have revolutionised cancer care especially in lung cancer. New response patterns have been described under ICIs such as pseudo-progression or hyper-progressive disease (HPD). The definition of HPD is yet to be consensual. The aim of this study was to suggest a clinical definition of nivolumab-refractory patients and find factors associated with this entity.
: We performed a multi centric retrospective study including all patients who received nivolumab for the treatment of advanced non-small cell lung cancer (NSCLC) during the French authorisation for temporary use in 2015.
: 303 patients were included in the cohort and 292 had details on the number of nivolumab injections received. 57 patients (20%) were nivolumab-refractory. These patients had worse PS at nivolumab initiation (p < 0.0001), shorter duration of treatment before nivolumab (p = 0.028) and had dramatically shorter nivolumab overall survival (p < 0.0001) than patients who did not present with refractory disease.
: Nivolumab-refractory disease can affect up to 20% of patients treated with nivolumab for advanced NSCLC with dramatically shortened survival rates. Further studies are needed to understand the precise mechanisms leading to refractory disease as well as its management.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
The evolution of biogenic amines from must to wine has been studied in seven different grape cultivars before and after malolactic fermentation. Alcoholic and malolactic fermentations have been ...carried out using selected yeasts and bacteria that, in a previous study, were unable to produce biogenic amines. The study has been performed under aseptic conditions to exclude possible interferences due to uncontrolled contaminating microorganisms present in grapes and/or in the environment. The goal of this work was to investigate the influence of grape on biogenic amines content of the wine. The results obtained showed that grape variety is related to the presence of some biogenic amines in wines and that, climatic conditions also affect the accumulation of these compounds in grapes.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
IMPORTANCE: Atypical parkinsonian syndromes (APS), including progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and multiple system atrophy (MSA), may be difficult to distinguish in ...early stages and are often misdiagnosed as Parkinson disease (PD). The diagnostic criteria for PSP have been updated to encompass a range of clinical subtypes but have not been prospectively studied. OBJECTIVE: To define the distinguishing features of PSP and CBS subtypes and to assess their usefulness in facilitating early diagnosis and separation from PD. DESIGN, SETTING, PARTICIPANTS: This cohort study recruited patients with APS and PD from movement disorder clinics across the United Kingdom from September 1, 2015, through December 1, 2018. Patients with APS were stratified into the following groups: those with Richardson syndrome (PSP-RS), PSP-subcortical (including PSP-parkinsonism and progressive gait freezing subtypes), PSP-cortical (including PSP-frontal and PSP-CBS overlap subtypes), MSA-parkinsonism, MSA-cerebellar, CBS–Alzheimer disease (CBS-AD), and CBS–non-AD. Data were analyzed from February 1, through May 1, 2019. MAIN OUTCOMES AND MEASURES: Baseline group comparisons used (1) clinical trajectory; (2) cognitive screening scales; (3) serum neurofilament light chain (NF-L) levels; (4) TRIM11, ApoE, and MAPT genotypes; and (5) volumetric magnetic resonance imaging measures. RESULTS: A total of 222 patients with APS (101 with PSP, 55 with MSA, 40 with CBS, and 26 indeterminate) were recruited (129 58.1% male; mean SD age at recruitment, 68.3 8.7 years). Age-matched control participants (n = 76) and patients with PD (n = 1967) were included for comparison. Concordance between the antemortem clinical and pathologic diagnoses was achieved in 12 of 13 patients with PSP and CBS (92.3%) undergoing postmortem evaluation. Applying the Movement Disorder Society PSP diagnostic criteria almost doubled the number of patients diagnosed with PSP from 58 to 101. Forty-nine of 101 patients with reclassified PSP (48.5%) did not have the classic PSP-RS subtype. Patients in the PSP-subcortical group had a longer diagnostic latency and a more benign clinical trajectory than those in PSP-RS and PSP-cortical groups. The PSP-subcortical group was distinguished from PSP-cortical and PSP-RS groups by cortical volumetric magnetic resonance imaging measures (area under the curve AUC, 0.84-0.89), cognitive profile (AUC, 0.80-0.83), serum NF-L level (AUC, 0.75-0.83), and TRIM11 rs564309 genotype. Midbrain atrophy was a common feature of all PSP groups. Eight of 17 patients with CBS (47.1%) undergoing cerebrospinal fluid analysis were identified as having the CBS-AD subtype. Patients in the CBS-AD group had a longer diagnostic latency, relatively benign clinical trajectory, greater cognitive impairment, and higher APOE-ε4 allele frequency than those in the CBS–non-AD group (AUC, 0.80-0.87; P < .05). Serum NF-L levels distinguished PD from all PSP and CBS cases combined (AUC, 0.80; P < .05). CONCLUSIONS AND RELEVANCE: These findings suggest that studies focusing on the PSP-RS subtype are likely to miss a large number of patients with underlying PSP tau pathology. Analysis of cerebrospinal fluid defined a distinct CBS-AD subtype. The PSP and CBS subtypes have distinct characteristics that may enhance their early diagnosis.
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