bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurodevelopmental profile and stages of regression in Phelan–McDermid syndrome
Authors: Dille Yumi , Lagae Lieven , Swillen Ann , Buggenhout Griet Van 
Published: December 2022

AbstractAimTo characterize the neurodevelopmental profile of patients with Phelan–McDermid syndrome (PMS) and describe the nature and trajectory of regression.MethodThis was a retrospective, monocentric study examining the clinical and developmental data of 24 patients (average age = 25 years 6 months, range = 6–56 years, n = 13 males) with a confirmed 22q13.3 terminal deletion carried out at the Centre for Human Genetics, University Hospital Leuven. The neurodevelopmental profile of individuals with PMS was examined, combining both cross‐sectional and longitudinal data obtained by systematic review of digital medical records.ResultsRemarkable loss of skills was present in 19 individuals affecting both language and motor skills. The first manifestations of neurodevelopmental regression occurred, on average, at the age of 7 years 6 months (range = 5–11 years). Language skills (active vocabulary) were primarily affected followed by, in order of loss, psychosocial adaptability, fine motor skills, and walking ability. The course of regression was characterized by a distinctive four‐stage pattern. The first stage often occurred around mid‐childhood and was defined by a pronounced and abrupt decline of language skills. This stage was generally followed by the second stage where a (prolonged) period of stagnation of regression was seen. The third stage was defined by acute neuropsychiatric decline (e.g. catatonia, hallucinations, psychosis). Acute events such as severe sickness, hormonal shifts, and psychosocial stress frequently preceded the fourth and final stage, which was characterized by severe neuromotor degeneration.InterpretationNeurodevelopmental regression should be considered as a key feature of PMS. We present a four‐stage model of neurodevelopmental regression, entailing language skills, fine and gross motor function, and psychosocial adaptation, which can be applied in future practice and research.
Evaluation of right ventricle systolic function after tetralogy of Fallot repair: A systematic review comparing cardiac magnetic resonance and global longitudinal strain
Authors: Oliveira Ana Laísa Andrada , de Oliveira Maria Eduarda Pereira , Guimarães Lucas Veiga , Trindade Gabrielly Machado , Chaves Guilherme Martins , Gonçalves Andreia Cristina Pernas , de Souza Talita Jaine Franchini , Moraes Letícia Santos , Lujan Victória Soares Coelho , Faria Lívia Silva de Paula , Manuel Valdano 
Published: December 2022

AbstractBackgroundMost patients who undergo tetralogy of Fallot (TOF) repair experience late right ventricle (RV) dysfunction due to pulmonary valve regurgitation (PVR). Cardiac magnetic resonance (CMR) is the gold standard method for evaluating RV during follow‐up. Global longitudinal strain (GLS) has been introduced as a novel method for the assessment of RV dysfunction. We aimed to compare the feasibility of GLS and CMR for assessing RV function after TOF repair.MethodsWe systematically reviewed the English literature using PubMed, SciELO and Google Scholar for articles published between January 1, 2015, and December 31, 2020. Articles evaluating RV function comparing by GLS and CMR after TOF repair were included.ResultsNine studies including 465 patients were analyzed. Most patients were men (280; 60%), the male:female ratio was 1.5:1, and the age range was .8 to 57.7 years. The mean follow‐up time was 6 to 32 months. The correlation between RV GLS and RV ejection fraction (EF) by CMR was negative for the articles and varied from moderate to strong (r = ‐.45, r = ‐.60, r = ‐.76).ConclusionRight ventricle GLS can be considered for routine follow‐up of TOF repair patients, even though CMR remains the noninvasive gold standard method. Using a single parameter may not allow comparison of the accuracy of 3D RV EF by using CMR and GLS. Further studies with a larger number of patients undergoing TOF repair are required to evaluate the correlation between these examinations.
Vendor independent myocardial strain values in children
Authors: Acheampong Benjamin , Parra David , Havens Corey , Jantzen David , Godown Justin , Soslow Jonathan 
Published: December 2022

AbstractBackgroundTwo‐dimensional (2D) strain imaging has become an important tool in assessing subclinical myocardial dysfunction in children. However, there are no published normal values for vendor‐independent strain software. The aim of this study was to estimate 2D strain values in a cohort of healthy children using Tomtec cardiac performance analysis (CPA), a vendor‐independent software.MethodsTransthoracic echocardiograms of healthy pediatric outpatients (0–18 years) were retrospectively analyzed from the Vanderbilt Pediatric Heart Institute using CPA. The cardiac assessment included global longitudinal strain (GLS), global longitudinal strain rate (GLSR), global circumferential strain (GCS), and global circumferential strain rate (GCSR). Mean strain values with standard deviation (SD) are reported. The Wilcoxon rank sum test, linear regression, and one‐way analysis of variance were used to assess differences among the various groups.ResultsAmong 142 children analyzed, 79 (56%) were male, and the median age was 5.5 (range, 0–18) years. The mean (SD) strain values were GLS –19.3 ± 3.4, GLSR –1.1 ± .22; GCS ‐24.7 ± 4.3, GCSR –1.5 ± .28. Age accounted for <8% of the variation in GLS, GCS, and GCSR. However, for GLSR, there was a statistically significant difference between younger and older age groups with higher GLSR in the younger age group. Age accounted for ∼25% of the variation in GLSR (R2 = .25, p < 0.001). There were no significant differences in strain based on sex.ConclusionWe report normal myocardial strain values in healthy children by age for strain using CPA. These values add to the growing body of literature on myocardial strain in children and provide necessary data for the interpretation of strain imaging.
Doubling protected land area may be inefficient at preserving the extent of undeveloped land and could cause substantial regional shifts in land use
Authors: Di Vittorio Alan V. , Narayan Kanishka B. , Patel Pralit , Calvin Katherine , Vernon Chris R. 
Published: December 2022

AbstractProjection of land use and land‐cover change is highly uncertain yet drives critical estimates of carbon emissions, climate change, and food and bioenergy production. We use new, spatially explicit land availability data in conjunction with a model sensitivity analysis to estimate the effects of additional land protection on land use and land cover. The land availability data include protected land and agricultural suitability and is incorporated into the Moirai land data system for initializing the Global Change Analysis Model. Overall, decreasing land availability is relatively inefficient at preserving undeveloped land while having considerable regional land‐use impacts. Current amounts of protected area have little effect on land and crop production estimates, but including the spatial distribution of unsuitable (i.e., unavailable) land dramatically shifts bioenergy production from high northern latitudes to the rest of the world, compared with uniform availability. This highlights the importance of spatial heterogeneity in understanding and managing land change. Approximately doubling the current protected area to emulate a 30% protected area target may avoid land conversion by 2050 of less than half the newly protected extent while reducing bioenergy feedstock land by 10.4% and cropland and grazed pasture by over 3%. Regional bioenergy land may be reduced (increased) by up to 46% (36%), cropland reduced by up to 61%, pasture reduced by up to 100%, and harvested forest reduced by up to 35%. Only a few regions show notable gains in some undeveloped land types of up to 36%. Half of the regions can reach the target using only unsuitable land, which would minimize impacts on agriculture but may not meet conservation goals. Rather than focusing on an area target, a more robust approach may be to carefully select newly protected land to meet well‐defined conservation goals while minimizing impacts to agriculture.
Relationship between index of orthodontic treatment need dental health component (IOTN‐DHC) and caries prevalence in school‐age children
Authors: Hasegawa Yuh , Batbayar Nomintsetseg , Sukhbaatar Nomingerel , Bazar Amarsaikhan , Kageyama Ikuo 
Published: December 2022

AbstractObjectivesThe objectives of the study were to investigate the relationship between caries prevalence and the Index of Orthodontic Treatment Need Dental Health Component (IOTN‐DHC) in school‐age children who live in the dentistless area and to consider the effective way to reduce the potential progression of malocclusion with the growth through the dental survey in Arbulag district, Khövsgöl province, Mongolia.Materials and MethodsThe 95 school‐age children (49 males, 46 females) who participated in the study were born in and were residents of Arbulag district, Khövsgöl province, Mongolia. The mean ages of the participants were 8.3 years (y) ± 1.7 y for males and 8.8 y ± 1.7 y for females. The Mann–WhitneyUtest was performed to determine the sex difference for the dmf/DMF index. The relationship between the IOTON‐DHC and the dmf/DMF index was statistically examined using Spearman's rank correlation coefficient.ResultsRegarding the dmf/DMF index, no sex difference was observed. There were significant, fair positive correlations between the IOTON‐DHC grade and F; between the IOTON‐DHC grade and DMF index in males. There were significant, fair positive correlations between the IOTON‐DHC grade and M; between the IOTON‐DHC grade and DMF index in females.ConclusionsSignificant, fair positive correlations were found between the caries prevalence and the grade of malocclusion. Thus, prevention and early treatment of dental caries in a mixed dentition may be effective to reduce the potential progression of malocclusions in dentistless area.
Characteristics of ulcerated and non‐ulcerated necrobiosis lipoidica
Authors: Hines Alexander , Butterfield Richard , Boudreaux Blake , Bhullar Puneet , Severson Kevin J. , McBane Robert D. , Davis Mark D. P. , Pittelkow Mark R. , Mangold Aaron R. , Alavi Afsaneh 
Published: December 2022

AbstractBackgroundNecrobiosis lipoidica (NL) is complicated by ulceration in up to 35% of cases.MethodsRetrospective study of patients with NL seen at our institution between January 1, 1992, and May 25, 2021, was conducted. Ulcerated NL (UNL, n = 83) and non‐ulcerated NL (NUNL, n = 233) groups were compared.ResultsTwenty‐six percent (83/316) of patients with NL experienced ulceration. UNL was significantly more likely to be painful (52% vs. 36%, P = 0.01), was more likely to have a lesion‐associated cutaneous malignancy (7% vs. 0%, P < 0.001), and had a larger median size (7 vs. 5 cm, P = 0.004) compared to NUNL. Vascular studies were performed on a subset of patients and revealed transcutaneous oxygen pressure (TcPO2) < 40 mm Hg in 53% and venous insufficiency in 62% with no significant differences between UNL and NUNL groups. In patients with unilateral ulceration, mean TcPO2 values (39.7 vs. 46.6 mm Hg), regional perfusion index <0.6 (29% vs. 14%), and TcPO2 < 40 mm Hg (43% vs. 14%) were worse in the ulcerated leg compared to the non‐ulcerated leg, but these differences were not statistically significant.ConclusionsUNL was more likely to be painful, develop lesion‐associated malignancy, and be larger in size compared to NUNL. There were no statistically significant differences in venous insufficiency, arterial Doppler/ankle brachial index, or TcPO2 values between UNL and NUNL patients, however, a significant portion of the cohort demonstrated abnormal vascular studies, particularly on TcPO2 and venous insufficiency testing.
Calcium lactate efficiently induces production of bacillomycin D in Bacillus subtilisNS‐174 and inhibition of spore germination of Aspergillus flavus in rice
Authors: Qian Shiquan , Li Jingwen , Xu Tiansheng , Diao Enjie , Tang Yuting , Zhou Xinrong , Liu Ying 
Published: December 2022

SummaryThe present study provided a new alternative for efficiently producing lipopeptides by calcium lactate and expanded potential applications of lipopeptides for ensuring food safety. The results indicated that the addition of calcium lactate significantly improved bacillomycin D production. When fed 150 mL of fermentation medium with 6.5 g/L calcium lactate at the batch fermentation time of 102 h, the maximum production of C14 bacillomycin D (C14BD), C15 bacillomycin D (C15BD), C16 bacillomycin D (C16BD), C17 bacillomycin D (C17BD) and total C14‐17 bacillomycin D (TBD) were 298.96, 345.43, 149.56, 216.6 and 1010.55 mg/L at 162 h, respectively. In addition, calcium lactate effectively induced biosynthesis of bacillomycin D through upregulating 2‐ketobutyric acid in the valine, leucine and isoleucine biosynthesis (VLIB) pathway that was closely related to threonine dehydratase and threonine and increasing expression of bacillomycin D synthase genes. Moreover, bacillomycin D could remarkably inhibit spore germination of Aspergillus flavus in rice.
Effects of different souring methods on phenolic constituents and antioxidant properties of non‐alcoholic gruels from sorghum and amaranth
Authors: Adeyanju Adeyemi A. , Duodu Kwaku G. 
Published: December 2022

SummaryFunctional foods have developed in modern food markets as a result of consumers' rising choice for healthier food options in recent years. This study looked at how non‐alcoholic gruels made from sorghum and amaranth were affected by lactic acid fermentation and exogenous acidification with lactic acid in terms of their phenolic content and antioxidant capacities. The phenolic compounds were identified and quantified by liquid chromatography–mass spectrometry (LC‐MS). Total phenolic content was determined by Folin–Ciocalteu method, and antioxidant properties by 2,2′‐azino‐bis(3‐ethylbenzothiazoline‐6‐sulphonicacid) (ABTS), 2,2′‐diphenyl‐1‐picrylhydrazyl (DPPH), nitric oxide (NO) assays as well as inhibition of oxidative damage of vector DNA. In comparison to cooked gruels without fermentation or acidification, both fermented and lactic acid acidified gruels (cooked) had considerably greater total phenolic acid (4%–32%), total flavonoid content (20%–30%), and radical scavenging capacities (114%–195%, 166%–208%, and 10%–28% for ABTS, DPPH and NO radical scavenging capacities respectively). Overall, fermentation and exogenous acidification enhanced the extractability of phenolic compounds and radical scavenging capacities. Therefore, souring of cereal‐based foods either by fermentation or exogenous acidification has the potential to shield the body from illnesses linked to oxidative stress.
Oil extraction from catfish (Netuma barba) waste through the supercritical fluid
Authors: da Silva Vanessa Barbosa , Igansi Andrei Vallerão , da Silva Patrick Peres , Engelmann Jenifer Inês , Cadaval Jr Tito Roberto Sant'Anna , Pinto Luiz Antonio de Almeida 
Published: December 2022

SummaryThis work aimed at oil extraction from catfish (Netuma barba) heads through the CO2 supercritical fluid (SFE‐CO2), and the ranges of supercritical conditions were extraction time (1 and 3 h), temperature (40 and 60 °C) and pressure (15 and 25 MPA). The crude oil from catfish heads presented the yield in the thermomechanical extraction was 86% compared to the Bligh and Dyer method, with concentrations of eicosapentaenoic acid (EPA) and docosahexaenoic acid DHA of approximately 9.0% and 11.9%, respectively, in the catfish oil profiles after the refining process. In the supercritical extraction, the highest yield was around 84% at 25 MPa and 40 °C in 3 h, with lower concentrations of EPA and DHA, respectively, 2.1% and 9.1%. At 25 MPa, 60 °C and 3 h, the extraction yield was around 81%, with concentrations of EPA and DHA about 3.4% and 10.0%, respectively. The highest concentration of EPA and DHA was 18.3% found in the experiment at 25 MPa, 40 °C and 1 h. The catfish head oil obtained by supercritical fluid extraction process (SFE‐CO2) showed similar characteristics to oil obtained by thermomechanical extraction and chemically refined. Thus, SFE‐CO2, besides being a promising potential for use of the fish waste, leads to sustainable food production through green technology.
External validation of yonsei nomogram predicting chronic kidney disease development after partial nephrectomy: An international, multicenter study
Authors: Abdel Raheem Ali , Landi Isotta , Alowidah Ibrahim , Capitanio Umberto , Montorsi Francesco , Larcher Alessandro , Derweesh Ithaar , Ghali Fady , Mottrie Alexander , Mazzone Elio , De Naeyer Geert , Campi Riccardo , Sessa Francesco , Carini Marco , Minervini Andrea , Raman Jay D. , Rjepaj Chris J. , Kriegmair Maximilian C. , Autorino Riccardo , Veccia Alessandro , Mir Maria Carmen , Claps Francesco , Choi Young Deuk , Ham Won Sik , Santok Glen Denmer , Tadifa John Paul , Syling Justin , Furlan Maria , Simeone Claudio , Bada Maida , Celia Antonio , Carrión Diego M. , Aguilera Bazan Alfredo , Ruiz Cristina Ballesteros , Malki Manar , Barber Neil , Hussain Muddassar , Micali Salvatore , Puliatti Stefano , Ghaith Ahmed , Hagras Ayman , Ghoneem Ayman M. , Eissa Ahmed , Alqahtani Abdulrahman , Rumaih Abdullah , Alwahabi Abdelaziz , Alenzi Mohammed Jayed , Pavan Nicola , Traunero Fabio , Antonelli Alessandro , Porcaro Antonio Benito , Illiano Ester , Costantini Elisabetta , Rha Koon Ho 
Published: December 2022

AbstractObjectiveTo externally validate Yonsei nomogram.MethodsFrom 2000 through 2018, 3526 consecutive patients underwent on‐clamp PN for cT1 renal masses at 23 centers were included. All patients had two kidneys, preoperative eGFR ≥60 ml/min/1.73 m2, and a minimum follow‐up of 12 months. New‐onset CKD was defined as upgrading from CKD stage I or II into CKD stage ≥III. We obtained the CKD‐free progression probabilities at 1, 3, 5, and 10 years for all patients by applying the nomogram found at https://eservices.ksmc.med.sa/ckd/. Thereafter, external validation of Yonsei nomogram for estimating new‐onset CKD stage ≥III was assessed by calibration and discrimination analysis.Results and limitationMedian values of patients' age, tumor size, eGFR and follow‐up period were 47 years (IQR: 47–62), 3.3 cm (IQR: 2.5–4.2), 90.5 ml/min/1.73 m2 (IQR: 82.8–98), and 47 months (IQR: 27–65), respectively. A total of 683 patients (19.4%) developed new‐onset CKD. The 5‐year CKD‐free progression rate was 77.9%. Yonsei nomogram demonstrated an AUC of 0.69, 0.72, 0.77, and 0.78 for the prediction of CKD stage ≥III at 1, 3, 5, and 10 years, respectively. The calibration plots at 1, 3, 5, and 10 years showed that the model was well calibrated with calibration slope values of 0.77, 0.83, 0.76, and 0.75, respectively. Retrospective database collection is a limitation of our study.ConclusionsThe largest external validation of Yonsei nomogram showed good calibration properties. The nomogram can provide an accurate estimate of the individual risk of CKD‐free progression on long‐term follow‐up.
Current approaches to the diagnosis and management of amyloidosis
Authors: Taylor Mark S. , Sidiqi Hasib , Hare James , Kwok Fiona , Choi Bo , Lee Darren , Baumwol Jay , Carroll Antonia S. , Vucic Steve , Neely Pat , Korczyk Dariusz , Thomas Liza , Mollee Peter , Stewart Graeme J. , Gibbs Simon D. J. 
Published: December 2022

AbstractAmyloidosis is a collection of diseases caused by the misfolding of proteins that aggregate into insoluble amyloid fibrils and deposit in tissues. While these fibrils may aggregate to form insignificant localised deposits, they can also accumulate in multiple organs to the extent that amyloidosis can be an immediately life‐threatening disease, requiring urgent treatment. Recent advances in diagnostic techniques and therapies are dramatically changing the disease landscape and patient prognosis. Delays in diagnosis and treatment remain the greatest challenge, necessitating physician awareness of the common clinical presentations that suggest amyloidosis. The most common types are transthyretin (ATTR) amyloidosis followed by immunoglobulin light‐chain (AL) amyloidosis. While systemic AL amyloidosis was previously considered a death sentence with no effective therapies, significant improvement in patient survival has occurred over the past 2 decades, driven by greater understanding of the disease process, risk‐adapted adoption of myeloma therapies such as proteosome inhibitors (bortezomib) and monoclonal antibodies (daratumumab) and improved supportive care. ATTR amyloidosis is an underdiagnosed cause of heart failure. Technetium scintigraphy has made noninvasive diagnosis much easier, and ATTR is now recognised as the most common type of amyloidosis because of the increased identification of age‐related ATTR. There are emerging ATTR treatments that slow disease progression, decrease patient hospitalisations and improve patient quality of life and survival. This review aims to update physicians on recent developments in amyloidosis diagnosis and management and to provide a diagnostic and treatment framework to improve the management of patients with all forms of amyloidosis.
Why is technocracy persistent in industrial risk? Empirical evidence from an in‐depth case study in Italy
Authors: Crivellari Paolo , Chesta Riccardo Emilio 
Published: December 2022

AbstractRisk expertise is characterised by procedures that emphasise scientific and technical aspects of risk management and downplay citizens’ concerns and lay knowledge. In this paper, we aim at accounting for some of the reasons why technocracy is persistent in industrial risk expertise. Using the conceptual tools provided by the political sociology of expertise and based on empirical research conducted with mixed qualitative techniques, we study different types of expertise about industrial risk in the petrochemical area of Porto Marghera (Venice, Italy). The first one is performed by the Agenzia Regionale per la Protezione Ambientale del Veneto (ARPAV, Regional Agency of Veneto for Environmental Protection), which is responsible for chemical risk management, and the plants of the area. The second one is performed by the Assemblea permanente contro il pericolo chimico (APCPC, Permanent Assembly Against Industrial Risk), a citizens’ association of the neighbouring community of Marghera. In this case study, we show that technocratic procedures are persistent in industrial risk due to strategic relationships between the public administration in charge of risk regulation enforcement and private chemical companies, the absence of Italian laws on inclusive participatory arenas about industrial risk, and the characteristics of the citizens’ mobilisation, which focuses on plants’ delocalisation rather than challenging the ARPAV's and plants’ technocracy.