dataesr/scientific-paragraphs-categorization huggingface dataset:
Statistical Analysis The statistical analyses were conducted with GraphPad Prism (Graph Pad Software, San Diego, CA, USA). Depending on the assay, we used 1-factor or 2-factor ANOVA, with Bonferroni correction. The unpaired Kruskal-Wallis test (without assuming homogeneity of variances) allowed nonparametric comparisons of CTB labeled volumes, followed by Dunn's multiple comparison test.
Acknowledgments Funded by the  Spanish Ministry of Economy and Competitiveness  under  BIO2015-70978-R , the  Spanish Ministry of Science and Innovation  under  RTI2018-101223-B-I00 ,  CIBERER  and  Fundación Ramón Areces  to L. M. Additionally,  Spanish Ministry of Science and Innovation  ( FEDER-PID2019-106230RB-I00 ,  2019 ) and  Generalitat Valenciana   IDIFEDER/2017/064 , Downloaded from iovs. arvojournals. org on  10/29/2023 A Slc38a8   Mouse Model of FHONDA Syndrome IOVS | October 2023 |   Vol. 64 | No. 13 | Article 32 | 13 2017 ,  PROMETEO/2021/024 , 2021 supported the work of  N. C. Funds  from  INSERM ,  Sorbonne Université ,  Retina France  and Genespoir supported the work of A. R. , as well as  LabEx LIFESENSES  ( ANR-10-LABX-65 ) and  IHU FOReSIGHT  ( ANR-18-IAHU-01 ) for the  Institut de la Vision , a doctoral fellowship from the  French Ministry of Education and Research  to V.
Recruiting and screening This interventional study was conducted in accordance with the ethical principles defined by the Declaration of Helsinki, approved by the University of the Aegean Ethical Committee (Approval: 13902/15. 06. 2022). The study protocol was registered in the clinicaltrials. gov database, prior to participant recruitment and enrollment, with the identifier NCT05771571. The study was carried out between 12 May, 2022 and 19 May, 2022 in the Laboratory of Nutrition and Public Health of the University of the Aegean in Lemnos, Greece. Participants were recruited via poster advertisements that was shared in social media. Between 2 and 3 weeks before prior to the clinical study, all prospective participants visited the Human Nutrition Unit, to test their eligibility. During initial screening, data on demographics, clinical history, general and nutritional habits were collected via personal interviews with the study researchers.
[23]. Preliminary in vitro studies evaluated the total antioxidant capacity and the total phenolic content of the refined olive oil and the novel refined olive oil, enhanced with 10% w/v orange peel, performing the Ferric Reducing Antioxidant Power (FRAP) assay and Folin-Ciocalteu method, respectively  [19]. The total antioxidant capacity is expressed as millimoles Fe2+ per liter (mmol Fe2+/L), and the total phenolic content of the samples are expressed as milligrams of gallic acid (GAE) per liter (mg GAE/L) of sample. In total, each meal weighed 295. 5 g. The meals that were prepared were isoenergetic (618. 53 kcal), high in fat and carbohydrates and their nutritional composition was calculated using the Diet Analysis v7. 0. 1 software (Cengage Learning, Ltd, United Kingdom). The nutritional composition of the meals is summarized in Table  1.
Data analysis The statistical analysis was performed using SPSS V21. 0 for Windows (IBM Corporation, New York, NY, United States) and Prism 9 (GraphPad Software Inc. , San Diego, CA, USA). All data were taking into consideration. The significance level was defined at 0. 05. Prior to statistical analysis, all biochemical variables were tested for the normal distribution by Kolmogorov-Smirnov normality test. Significant differences on initial population characteristics were evaluated, performing one-way analysis of variance (ANOVA). The 10-age cardiovascular risk was calculated for each volunteer, according to SCORE2 (European Society of Cardiology). All biomarkers were analysed, carrying out repeated-measures ANOVA, for the 2 factors (group, time) and their interactions. Post-hoc tests were carried out via Bonferroni test.
Data Collection The data collected at admission were age, sex, body mass index (BMI), CHADSVASC score, medical history of dysthyroidisim, hypertension, medications known to induce hy-poMg, sepsis, dehydration, and outcome of the AF episode (resolutive vs non-resolutive). Hyperthyroidism was defined by a TSH < 0. 358 mUI/L, or if the patient was known for hyperthyroidism on the case report form (CRF). Dehydration was defined by a diastolic blood pressure < 40 mmHg or systolic blood pressure < 90 mmHg or mean blood pressure < 65 mmHg or plasmatic protein > 80 g/L or if the symptom « hypovolemia » or « dehydration » was validated by the investigator on the CRF. Sepsis was defined if temperature was >38. 5 • C or if diagnosed by the physician and reported on CRF. Hypertension was defined by a systolic blood pressure > 180 mmHg or diastolic blood pressure > 100 mmHg or if the patient had a history of high blood pressure or was treated for high blood pressure.
Information Sources This meta-analysis was based on a systematic, comprehensive search on MEDLINE via PubMed for articles published in English or French from 31 March 2016 to 31 December 2021. Due to a considerable increase in the number of studies addressing sarcopenia among cancer patients, we chose only to consult the PubMed database. The following research algorithm was used: sarcopenia AND (cancer OR tumors OR malignancies) AND (death OR overall survival OR progression-free survival OR relapse OR chemotherapy OR targeted therapy OR radiotherapy OR hormonal therapy OR surgery OR immunotherapy OR toxicity OR disability OR infection) AND human NOT review NOT letter. All articles retrieved from our previous systematic review were also included  [7].
Results The International XLH Registry was initiated in August 2017 and includes participants of all ages diagnosed with XLH, regardless of their treatment and management. At the database lock for this first interim analysis (29 March 2021), 579 participants had entered the registry before 30 November 2020 and are included in the analysis (360 children [62. 2%], 217 adults [37. 5%] and 2 whose ages were not recorded [0. 3%]; 64. 2% were female). Family history data were available for 319/345 (92. 5%) children and 145/187 (77. 5%) adults; 62. 1% had biological parents affected by XLH. Genetic testing data were available for 341 (94. 7%) children and 203 (93. 5%) adults; 370/546 (67. 8%) had genetic test results; 331/370 (89. 5%) had a confirmed PHEX mutation. A notably longer time to diagnosis was observed in adults ≥ 50 years of age (mean [median] duration 9.
Registry identification This rare-disease International XLH Registry has been registered with ClinicalTrials. gov, under the identifier NCT03193476  [23]. This registry was initiated in August 2017 and gained its first national regulatory approval in September 2017; this was in the United Kingdom  [8] , with approval in other countries following shortly after. It aims to include 1200 children and adults with a confirmed diagnosis of XLH and will run for 10 years, at which point the sponsor may decide to continue (or discontinue) the registry, in agreement with the applicable regulatory authorities  [8]. The sample size was based on evidence provided by clinical XLH experts across Europe, which suggested that 1200 participants would be sufficient to enable robust research to be conducted on the data collected in the registry.
Registry design The rationale for and description of the International XLH Registry are described elsewhere  [8]. Briefly, the International XLH Registry is an international, multicentre, non-interventional, observational collection of data in a real-world setting. It allows for the capture of clinical outcome variables and treatment details in participants with XLH; participants are followed for as long as informed consent (and assent, where applicable) and regulatory permissions are maintained  [8]. Only data collected during standard routine clinical visits and examinations are recorded within the International XLH Registry, and no specific examinations/data entries are mandated, although a core data set has been recommended to participating centres  [8]. Any drug therapy considered necessary for the participant's welfare may be administered at the discretion of the treating physician; all such treatments and any changes that occur throughout participation in the International XLH Registry can be recorded in the database  [8].
Participants in the first interim analysis The International XLH Registry comprises people of all ages diagnosed with XLH, regardless of their sex, treatment or management  [8]. This first interim analysis of the data from the International XLH Registry (database lock: 29 March 2021) includes data from an ITT population who had been entered into the registry prior to 30 November 2020 and who had signed a consent form allowing for their data to be analysed prior to 29 March 2021. At this data cut, participants' data had been entered from hospital sites in 16 countries: Belgium, Bulgaria, Denmark, France, Germany, Ireland, Israel, Italy, the Netherlands, Norway, Portugal, Slovakia, Slovenia, Spain, Sweden and the United Kingdom. Of note, participant data from Germany was taken from a single centre as part of an existing country-wide Kyowa Kirin International-sponsored investigator-initiated study (IIS).
Data capture and processing Data capture and data processing included results from genetic testing and other methods of diagnosis, patient demographics, clinical presentation of XLH, length/ height, weight and BMI. Analyses of data were descriptive; length/height, weight and BMI were analysed by Z-score (WHO 2007/CDC reference values were used to calculate Z-scores). Data processing was carried out by the contracted research organisation IQVIA (Durham, NC, US). The authors contributed to the statistical analysis plan and interpretation of the data.
Registry participant demographics At the data cut, 753 participants had been screened, of whom 579 (76. 9%) were eligible for inclusion in the analysis in accordance with the last date of inclusion (i. e. , 'last patient in' on 30 November 2020) and the presence of a signed valid consent form at the time of database lock on 29 March 2021 ('enrolled participants') (Fig. 1 ). Participants were from 81 hospital sites in 16 countries. Demographics and baseline characteristics of the enrolled population are presented in Table  1. In total, 374 (64. 6%) of this population were female; the population comprised 360 (62. 2%) children (< 18 years of age), 217 (37. 5%) adults and 2 (0. 3%) female participants of unknown age, with a mean (standard deviation [SD]) age at registry enrolment of 9. 5 (4. 5) years in children and 41.
Of those participants tested (n = 368), 24 did not have a confirmed mutation (6. 5%). At the time of database lock, 2 results had not been entered into the database. Where a genetic test was unavailable at a hospital site, in this hereditary X-linked dominant disease, an unambiguous and obvious family history, together with clinical and biochemical manifestations and radiographic imaging, could appropriately confirm a diagnosis of XLH. Family history data were available for 319/345 (92. 5%) pediatric participants and 145/187 (77. 5%) adult participants. In children, the biological mother was reported to be affected in 164/319 (51. 4%); the biological father was affected in 49/317 (15. 5%); the mean number of siblings and other family members affected were 0. 7 and 1. 3, respectively. In adults, 56/140 (40.
For example, burosumab was initially only approved in patients aged 1 year and older and adolescents with a growing skeleton, and burosumab may be more commonly prescribed in patients with more severe symptoms. These differences will need to be considered in future analyses of this registry. This first interim analysis of the registry data included a relatively high number of participants from the UK and France (234 and 141, respectively). This could be explained by the higher number of participating sites in those countries, possibly as a consequence of increased centralization of services in those countries, higher hospital site motivation and/or larger patient populations. As this may result in the registry being more representative of clinical practice and burosumab reimbursement in these countries than of all the participating countries as a whole, it will be important to consider differences in clinical practice in the countries included in this registry in future analyses.
For example, burosumab reimbursement was not uniformly available, and burosumab treatment may be limited to patients with more severe forms of XLH in some regions. The large data set and number of participating countries and hospital sites in the International XLH Registry make these data more generalizable to larger populations than previous smaller studies. There are many potential explanations for the differences in recruitment between different centres and countries (e. g. , ethical board approval, healthcare systems, centralization of care for rare diseases, specific interests and connections of physicians/investigators); Germany, for example, already has its own ongoing pediatric XLH study, which may partially explain why fewer hospital sites in Germany have enrolled in this International XLH Registry.
In this analysis, more children than adults had a genetic test for XLH documented in the data collection form. This result could be partially explained by an increased availability and awareness of the importance of genetic testing in these younger children to confirm the diagnosis, provide an assessment of patient outcome, support treatment decisions and help family genetic counselling. In contrast, there was a relative lack of availability of genetic testing when many older adults were initially diagnosed, and the older population may have been more often diagnosed through clinical, biochemical, radiological and family history. In total, 15 of 579 participants in this first interim analysis of the International XLH Registry had confirmed non-PHEX mutations. It must be stressed that the International XLH Registry comprises an ITT population of all enrolled participants with suspected XLH and includes real-world data available at the time of interim analysis.
For this current analysis, the percentage of participants with a confirmed non-PHEX mutation is small (2. 6%) and as such is unlikely to affect the overall results. Future analyses will exclude this patient population. In this analysis, more children had family history data collected than adults. This may be explained by the increased awareness of the disease and the importance of these data for accurate diagnosis. Family history can be used as a diagnostic tool and to help guide decisions about genetic testing for the participant and at-risk family members. A full genetic pedigree can also capture details about the health of multiple generations. This information can be important in diagnosing an inherited condition such as XLH, revealing the pattern of inheritance and informing clinical decisions regarding genetic testing and management of affected participants.
Dental abscesses were a common complaint of children in this registry. This is certainly consistent with the data published by Nguyen et al. , who found that dental abscesses were a cause of intense pain, altered smile and problematic chewing-all contributing factors to the reduced quality of life of participants with XLH. This highlights the need for specialized care by dental experts aware of the role of XLH in dental conditions and for early intervention to reduce later complications  [30]. Absolute length or height of registry participants tracked consistently below the WHO normal reference values from infancy throughout childhood and into adulthood for both males and females. However, an outlier to this pattern was observed in a single male participant of 18 years of age, whose height approached the WHO reference range.
0% reported as being overweight and 30. 0% as obese  [35]. However, data collected in this registry have shown that children and adults combine shorter stature with 'normal' weight, leading to an elevated BMI. Another potential contributor to elevated BMI could be due to the participant's proportionally shorter lower limbs: since the torso is heavier than the legs, it would be expected that the torso would contribute a greater proportional weight. In addition, XLH has an effect on mobility, fatigue, pain and muscle weakness; this may result in reduced mobility and activity, which could in turn lead to a more sedentary lifestyle. In consideration of all the possible contributory factors, these length/height, weight and BMI findings reinforce the need for more research in patients with XLH to determine whether traditional methods of measuring BMI are appropriate and whether alternative methods could be explored.
High BMI may contribute to increased mortality in XLH  [26] , and it would be relevant to conduct more research into why patients with XLH do develop weightand obesity-related metabolic complications. Several XLH registries are ongoing, but, to our knowledge, no results have been published in peer-reviewed journals to date. SUNFLOWER (Japan/South Korea)  [36]  and the Disease Monitoring Program (United States/ Latin America)  [37]   This registry does have some limitations. As with all observational registries, there were no requirements for mandatory data completion, and some registry data were not yet recorded for some parameters at the time of data cut. Some countries and hospitals did not record all variables as part of their standard clinical practice, and these data, as per the 'entry' in the Electronic Data Capture database, would have been recorded as 'Missing' data.
This highlights the need to refine the Electronic Data Capture database: differentiation is needed to determine the exact nature of data not entered, for example 'Data not reported yet' versus 'Data missing (i. e. , lost/irretrievable)'. This 'Missing' data is a potential source of bias and could limit the generalizability of the findings; for example, if the data are not missing at random but are due to data availability/data collection practices from individual countries or clinical sites. It will be necessary to report and consider missing data, including any patterns present, when conducting future analyses. Nonetheless, useful conclusions can still be drawn from such a large volume of data. An additional limitation of the registry is that the diagnosis of clinical conditions is based on the local practice of expert physicians.
Although strict inclusion criteria were specified in the International XLH Registry, participants were entered into the database by healthcare professionals in good faith that participants had a confirmed XLH diagnosis. The interim analysis of the International XLH Registry described here includes the ITT population of all participants who signed initial consent forms. A genetic test was not a mandated inclusion criterion. When the data were analysed, we found a small number of participants with confirmed non-PHEX mutations. Although we must accept the possibility of misdiagnosis in a real-world setting, the inclusion in the registry of a small number of participants who had a confirmed non-PHEX mutation emphasizes the need to perform a full family and genetic history of each affected participant to ensure correct diagnosis.
All participants will be followed up as part of the registry (unless they withdraw consent) and will be reported in subsequent analyses of patients with 'confirmed' XLH on conventional therapy or burosumab. The baseline data from such a large data set corroborate other studies and observations  [33]  and also show that, even with early diagnosis and medical management, children with XLH are still not thriving as they should. Questions do still remain, such as whether high BMI translates into morbidity or shortened survival compared with the general population [40], and how the chronicity of problems affect participants' life courses, as previous studies have suggested that patients with XLH do have a shortened survival  [26]. The long-term data that this registry will supply will hopefully provide more information to help answer these questions, thus will be useful in helping to address the paucity of disease natural history and treatment data in XLH.
Conclusions We describe here the first interim analysis of the baseline data from the International XLH Registry, which holds the largest data set of participants with XLH in the world to date. The demographic and clinical characteristics of this data set are consistent with those reported in other XLH studies, case series and non-interventional programmes, all of which add to the general validity and generalizability of these results. A good proportion of participants have a strong family history and/or genetic diagnosis, especially children. This is a robust data set that will mature and increase over time, growing and extending in terms of numbers, countries, hospital sites and prospective follow-up data. Future analyses will seek to provide new data on the progressive, accumulative nature of this disease as well as new insights describing the impact of the different treatment options for XLH that are now in the current treatment armamentarium.
XLH Registry from Medialis Ltd to a new clinical research organization in January 2019, the sponsor updated the core documents to refer to the burosumab post-authorization safety study as a sub-study and reassigned the role of data controller from the patient to the sponsor. J Tonge, A Dillon and J MacLeod, as employees of Medialis Ltd, served as study managers during the implementation of the International XLH Registry. GA, FE and EL are members of the European Reference Network for Rare Kidney Diseases Author contributions GA, SSBN, AMB, MLB, KB, CLC, FE, SG, DH, RK, EL, OM, MZM, ON, DS, LTS, MCZ are members of the International XLH Registry Steering Committee and were involved in the conception, design and coordination of the study, and were involved in reviewing and revising the manuscript.
Instrumental textural attributes Three different parameters (hardness, extensibility and extension area) were measured after performing the aforementioned uniaxial extensibility method (Table  1 ). The coefficient of variation of the mean (CV mean ) varied from 5-14% for hardness, 4-19% for extensibility and 8-23% for the extensional area. The highest values for the three parameters were observed in Laboko and Kratchi, while the lowest were observed in Dodo except for extensibility where Irindou showed the lowest value (2. 4 mm). Preliminary assays for the LSF method were carried out on four genotypes in order to determine the best compression speed for determination of BEV. A biaxial strain of 1 (dimensionless) was selected based on the low standard deviation and steady state achieved by the applied stress (data not shown).
Sensory analysis and consumer overall liking of pounded yam Sensory and overall liking results show that the six selected studied genotypes can be considered as contrasting genotypes in terms of stretchability, moldability and overall liking. These results were considered a good dataset to evaluate if instrumental textural methods can be used to predict sensory properties and consumer preferences. Relation between instrumental textural properties, sensory properties and consumer preferences Table  4  shows the correlations between instrumental uniaxial extensibility attributes (extensional area, hardness and extensibility), sensory attributes and consumers' overall liking. Note here that these results were obtained without taking into account the Aga genotype due to the impossibility of this dough to form a sheet for measurement with the uniaxial extensibility method.