bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Lysosomal Diseases Testing Laboratory: A review of the past 47 years
Authors: Wenger David A. , Luzi Paola 
Published: April 2020

SummaryLysosomal disorders are diseases that involve mutations in genes responsible for the coding of lysosomal enzymes, transport proteins, activator proteins and protein processing enzymes. These defects lead to the storage of specific metabolites within lysosomes resulting in a great variety of clinical features depending on the tissues with the storage, the storage products and the extent of the storage. The methods for rapidly diagnosing patients started in the late 1960's when the enzyme defects were identified eliminating the need for tissue biopsies. The first requests for diagnostic help in this laboratory came in 1973. In that year, patients with Krabbe disease and Niemann‐Pick type A were diagnosed. Since that time samples from about 62 000 individuals have been received for diagnostic studies, and 4900 diagnoses have been made. The largest number of diagnosed individuals had metachromatic leukodystrophy and Krabbe disease because of our research interest in leukodystrophies. A number of new disorders were identified and the primary defects in other disorders were clarified. With new methods for diagnosis, including newborn screening, molecular analysis, microarrays, there is still a need for biochemical confirmation before treatment is considered. With new treatments, including gene therapy, stem cell transplantation, enzyme replacement used alone or in combination becoming more available, the need for rapid, accurate diagnosis is critical.
Thermal‐ and collision‐induced dissociation studies of functionalized imidazolium‐based ionic liquid cations
Authors: De Silva Maleesha , Brown Andrew C. , Patrick Amanda L. 
Published: April 2020

AbstractIonic liquids are now used in applications ranging from chemical synthesis to spacecraft propulsion. With this comes the need to characterize new syntheses, identify environmental contamination, and determine eventual fate in terrestrial and space environments. This work investigates the effects of source conditions, particularly capillary temperature, on the observed mass spectrum and determines the collision‐induced dissociation (CID) patterns of imidazolium‐based ionic liquid cations as a function of their substituent types. Experiments were carried out on a Thermo LTQ‐XL ion‐trap mass spectrometer and a Bruker microTOF‐Q II mass spectrometer. Dissociation of the imidazolium cations occurred predominantly via substituent losses, except in benzyl‐substituted systems, for which the neutral loss of the imidazole was exclusively observed. Several of these dissociation pathways were studied in greater depth using complementary quantum chemical calculations. The nature of the neutral losses from the substituents was found to be highly dependent upon the nature of the substituent, as would be expected, establishing bases for characterization.
Enhanced winter and summer trend difference of Madden–Julian Oscillation intensity since 1871
Authors: Wang Ziyue , Li Tim , Gao Jianyun , Peng Melinda 
Published: April 2020

AbstractLong‐term winter and summer Madden–Julian Oscillation (MJO) trends in the past 138 years (1871–2008) were examined using National Oceanic and Atmospheric Administration (NOAA) 20th Century Reanalysis V2c dataset. It is found that MJO shows a distinctive different trend between boreal winter and summer. While the MJO intensity in both boreal winter and summer has a rising trend, the winter trend is much greater than the summer trend. As a result, the winter–summer difference shows a significant increasing trend. The distinctive winter and summer trends are attributed to the difference of atmospheric background circulation (such as vertical velocity) and static stability responses to the global warming between boreal winter and summer over equatorial eastern Indian Ocean. In boreal winter, both the surface moistening and strengthened inter‐tropical convergence zone convection contribute to an increase of MJO activity. This is in contrast to boreal summer when a greater static stability and anomalous subsidence tend to offset the moistening effect, leading to a relatively weaker increase of the MJO activity.
Guest editorial: Clarifications on the use of the new classification of periodontitis
Authors: Sanz Mariano , Papapanou Panos N. , Tonetti Maurizio S. , Greenwell Henry , Kornman Ken 
Published: April 2020

AbstractThis editorial provides clarifications on the application of the Stage and Grade classification of periodontitis. In particular it describes: (1) how to apply the extent criterion to the defined Stage of the disease; and (2) how to calculate tooth loss because of periodontitis in Stage III and IV cases presenting with evidently hopeless (irrational to treat) teeth with a clinical definition of such teeth.
A comparison of the nutritional value of Einkorn, Emmer, Khorasan and modern wheat: whole grains, processed in bread, and population‐level intake implications
Authors: Van Boxstael Frank , Aerts Hanna , Linssen Sarah , Latré Joos , Christiaens Anneline , Haesaert Geert , Dierickx Isabelle , Brusselle Joeri , De Keyzer Willem 
Published: April 2020

AbstractBACKGROUNDInterest in alternatives to the traditional wheat Triticum aestivum among farmers, millers, bakers, and consumers is increasing. The Altergrain project aimed to compare the Belgian‐soil cultivated Einkorn (1K), Emmer (EMM), Khorasan (KH), and modern wheat (MW) with respect to nutritional values of kernels, breads made from these cereals, and population‐level nutrient intake implications.RESULTSAncient wheats 1K, EMM, and KH contain lower total carbohydrate content than MW. Further, ancient wheats are higher in both protein and crude ash content. Vitamin E levels in breads prepared using 1K and EMM were higher than those in MW, but those prepared from KH had lower vitamin E levels than MW. Breads prepared using ancient wheats have higher total phenol content (TPC) than those from MW. Baking caused a decrease in vitamin E and TPC in bread prepared from ancient wheat, the exception being the one prepared using KH, which had a higher TPC than MW. When replacing bread made from MW with those made from ancient grains, no differences were observed with respect to conformance with the Belgian Recommend Daily Requirements.CONCLUSIONAncient wheats from Belgian soil are as nutritive as MWs even after being processed into bread. At the kernel level, nutritional differences are present, but only small differences are present in terms of nutritional intake when nutrition parameters are calculated for consumed bread. © 2020 Society of Chemical Industry
Fermentation with mono‐ and mixed cultures of Lactobacillus plantarum and L. casei enhances the phytochemical content and biological activities of cherry silverberry (Elaeagnus multiflora Thunb.) fruit
Authors: Lizardo Rona Camille M , Cho Hyun Dong , Won Yeong Seon , Seo Kwon Il 
Published: April 2020

AbstractBACKGROUNDLactic acid fermentation has been widely used to improve the nutritional and functional properties of food products. Cherry silverberry (Elaeagnus multiflora Thunb.) is considered as an invasive plant species with known medicinal and functional properties. In this study, improvement of the biological activity and health benefits of cherry silverberry fruit through lactic acid fermentation was investigated.RESULTSExtracts of cherry silverberry fruits fermented by pure cultures of Lactobacillus plantarum KCTC 33131 and L. casei KCTC 13086 exhibited favorable physicochemical properties and enhanced phytochemical content, antioxidant properties (DPPH radical scavenging activity, reducing power, superoxide dismutase‐like property and hydrogen peroxide scavenging activity) and α‐glucosidase and tyrosinase enzyme inhibitory activity as compared with unfermented fruits. Despite a decrease in the specific phenolic acid contents among the fermented samples, the cherry silverberry fruit fermented by mixed cultures of L. plantarum and L. casei contained superior total polyphenols (3.78 ± 0.22 mg GAE g−1) and total (0.66 ± 0.12 mg QE g−1) and individual flavonoid contents in comparison with fruits fermented by single cultures and unfermented ones. Multivariate analysis also showed strong association among total phytochemical contents and biological activities.CONCLUSIONSThis work has elucidated the effect of fermentation with L. plantarum KCTC 33131 and L. casei KCTC 13086 on the improvement of the physicochemical properties and biological activity of cherry silverberry fruit. It also revealed the potential application of fermented cherry silverberry in the production of food materials beneficial for health. © 2020 Society of Chemical Industry
A long‐term follow‐up study of extracorporeal irradiated autografts in limb salvage surgery for malignant bone and soft tissue tumors: A minimum follow‐up of 10 years after surgery
Authors: Outani Hidetatsu , Takenaka Satoshi , Hamada Kenichiro , Imura Yoshinori , Kakunaga Shigeki , Tamiya Hironari , Wakamatsu Toru , Naka Norifumi , Ueda Takafumi , Araki Nobuhito 
Published: April 2020

AbstractBackground and ObjectivesThe aim of this study is to assess the survival, function, radiographic appearance, and modes of failure of extracorporeal irradiated (ECI) autografts in a long‐term setting.MethodsWe retrospectively reviewed 87 patients who were treated for bone and soft tissue tumors using ECI autografts between 1988 and 2009.ResultsThe 56 patients had a minimum follow‐up of 10 years, and the median follow‐up period was 16.5 years. The reimplantation procedures included 24 osteoarticular grafts, 16 intercalary grafts, 10 autograft‐prosthetic composite grafts, and 6 hemicortical grafts. The 15‐year graft and event‐free survival rates were 76.8% and 47.9%, respectively. Infection and structural failure were the most common reasons for additional surgery. The time for additional surgery was significantly longer in patients with composite grafts (P < .01). The median Musculoskeletal Tumor Society score and the International Society of Limb Salvage score were 80% and 84%, respectively.ConclusionsECI autografts are a durable option for reconstruction after resection of musculoskeletal tumors and provide good function over more than 15 years. Most graft failures occurred within 5 years of the index surgery. However, composite grafts showed a tendency to fail more than 10 years after the surgery.
Photo‐stimulated “turn‐on/off” molecularly imprinted polymers based on magnetic mesoporous silicon surface for efficient detection of sulfamerazine
Authors: Xu Wanzhen , Gao Minmin , Yin Xifeng , Zhang Liming , Cao Yunfei , Zhang Yiyun , Huang Weihong 
Published: April 2020

AbstractIn this study, novel photo‐stimulated molecularly imprinted polymers based on magnetic mesoporous carrier surface were developed for selective identification and intelligent separation of sulfamerazine in complex samples. The photosensitive monomer of the molecularly imprinted polymers was azobenzene derivative 5‐[(4‐(methacryloyloxy)phenyl) diazenyl] isophthalic acid with stimulus reaction mechanisms, which has photoisomerization between trans and cis for N=N bonds. Further, the properties of the photo‐stimulated molecularly imprinted polymers were further evaluated through several sets of adsorption experiments. It illustrated that the maximum adsorption amount is 0.45 mmol/L. By ultraviolet spectrophotometry, the material reaches typical characteristic peaks of photo sensitivity, and the cycle time is 16 min. Three adsorption and desorption processes were repeated, the adsorption rate reached 34.4%. Overall, the photo‐stimulated molecularly imprinted polymers can enrich and separate determine sulfamerazine with high selectivity, which have good recovery for real samples.
EAT‐10 Scores and Fiberoptic Endoscopic Evaluation of Swallowing in Head and Neck Cancer Patients
Authors: Florie Michelle , Pilz Walmari , Kremer Bernd , Verhees Femke , Waltman Ghislaine , Winkens Bjorn , Winter Naomi , Baijens Laura 
Published: April 2020

ObjectiveThe purpose of this study was to determine the relationship between patient‐reported symptoms of oropharyngeal dysphagia (OD) using the Eating Assessment Tool (EAT)‐10 and the swallowing function using a standardized fiberoptic endoscopic evaluation of swallowing (FEES) protocol in head and neck cancer (HNC) patients with confirmed OD.MethodsFifty‐seven dysphagic HNC patients completed the EAT‐10 and a FEES. Two blinded clinicians scored the randomized FEES examinations. Exclusion criteria consisted of presenting with a concurrent neurological disease, scoring below 23 on a Mini‐Mental State Examination, being older than 85 years, having undergone a total laryngectomy, and being illiterate or blind. Descriptive statistics, linear regression, sensitivity, specificity, and predictive values were calculated.ResultsThe majority of the dysphagic patients (N = 38; 66.7%) aspirated after swallowing thin liquid consistency. A large number of patients showed postswallow pharyngeal residue while swallowing thick liquid consistency. More specifically, 42 (73.0%) patients presented postswallow vallecular residue, and 39 (67.9%) patients presented postswallow pyriform sinus residue. All dysphagic patients had an EAT‐10 score ≥ 3. Linear regression analyses showed significant differences in mean EAT‐10 scores between the dichotomized categories (abnormal vs. normal) of postswallow vallecular (P = .037) and pyriform sinus residue (P = .013). No statistically significant difference in mean EAT‐10 scores between the dichotomized categories of penetration or aspiration was found (P = .966).ConclusionThe EAT‐10 questionnaire seems to have an indicative value for the presence of postswallow pharyngeal residue in dysphagic HNC patients, and a value of 19 points turned out to be useful as a cutoff point for the presence of pharyngeal residue in this study population.Level of Evidence: 2b
Evaluating Common Cavity Cochlear Deformities Using CT Images and 3D Reconstruction
Authors: Weiss Nora M. , Langner Soenke , Mlynski Robert , Roland Peter , Dhanasingh Anandhan 
Published: April 2020

ObjectivesThe aim of this study is to compare the common cavity (CC) with the normal anatomy inner ear in order to evaluate whether the cavity is representing both the cochlear and the vestibular parts of the inner ear and to revisit CC deformity from a three‐dimensional (3D) perspective.MethodsHigh‐resolution computed tomography image datasets of 17 temporal bones initially identified as CC were evaluated with 3D reconstruction and multiplanar image analysis using a free available software for 3D segmentation of the inner ear. All 3D images of CC were compared to a normal inner ear. Maximum and minimum diameter of the CC were correlated with the circumference of the CC in an axial plane.ResultsIn 13 cases (76%), CC represented only the vestibular part of the inner ear and did not represent CC as defined here and by Sennaroglu, Kontorinis, and Khan. True CC was correctly diagnosed in only one case (6%). In three cases (18%), a rudimentary part of the cochlear portion could be identified. The axes' length of the elliptical cavity showed a strong positive linear relation to the circumference of the cavity (long axis: r = 0.94; P < .0001; short axis: r = 0.68; P = .0029).ConclusionThis study supports the assumption that many reported CC cases only represent the vestibular part of the inner ear and are therefore cases of cochlear aplasia. 3D segmentation and systematic analysis of CT‐imaging add clinical value to the comprehension of the morphology of the anatomical structures of the inner ear.Level of Evidence2C Laryngoscope, 131:386–391, 2021
Urban and agricultural soils in Southern California are a reservoir of carbapenem‐resistant bacteria
Authors: Lopez Nicolas V. , Farsar Cameron J. , Harmon Dana E. , Ruiz Cristian 
Published: April 2020

AbstractCarbapenems are last‐resort β‐lactam antibiotics used in healthcare facilities to treat multidrug‐resistant infections. Thus, most studies on identifying and characterizing carbapenem‐resistant bacteria (CRB) have focused on clinical settings. Relatively, little is still known about the distribution and characteristics of CRBs in the environment, and the role of soil as a potential reservoir of CRB in the United States remains unknown. Here, we have surveyed 11 soil samples from 9 different urban or agricultural locations in the Los Angeles–Southern California area to determine the prevalence and characteristics of CRB in these soils. All samples tested contained CRB with a frequency of <10 to 1.3 × 104 cfu per gram of soil, with most agricultural soil samples having a much higher relative frequency of CRB than urban soil samples. Identification and characterization of 40 CRB from these soil samples revealed that most of them were members of the genera Cupriavidus, Pseudomonas, and Stenotrophomonas. Other less prevalent genera identified among our isolated CRB, especially from agricultural soils, included the genera Enterococcus, Bradyrhizobium, Achromobacter, and Planomicrobium. Interestingly, all of these carbapenem‐resistant isolates were also intermediate or resistant to at least 1 noncarbapenem antibiotic. Further characterization of our isolated CRB revealed that 11 Stenotrophomonas, 3 Pseudomonas, 1 Enterococcus, and 1 Bradyrhizobium isolates were carbapenemase producers. Our findings show for the first time that both urban and agricultural soils in Southern California are an underappreciated reservoir of bacteria resistant to carbapenems and other antibiotics, including carbapenemase‐producing CRB.
A Novel DBS Paradigm for Axial Features in Parkinson's Disease: A Randomized Crossover Study
Authors: Karl Jessica A. , Ouyang Bichun , Goetz Steven , Metman Leo Verhagen 
Published: April 2020

AbstractBackgroundHigh‐frequency (130–185 Hz) deep brain stimulation (DBS) of the subthalamic nucleus is more effective for appendicular than axial symptoms in Parkinson's disease (PD). Low‐frequency (60–80 Hz) stimulation (LFS) may reduce gait/balance impairment but typically results in worsening appendicular symptoms. We created a “dual‐frequency” programming paradigm (interleave‐interlink, IL‐IL) to address both axial and appendicular symptoms. In IL‐IL, 2 overlapping LFS programs are applied to the DBS lead, with the overlapping area focused on the optimal cathode. The nonoverlapping area (LFS) is thought to reduce gait/balance impairment, whereas the overlapping area (high‐frequency stimulation, HFS) aims to control appendicular symptoms.MethodsWe performed a randomized, double‐blind crossover trial comparing patients’ previously optimized IL‐IL and conventional HFS paradigms. Each arm was 2 weeks in duration. The primary outcome measure was the patient/caregiver Modified Clinical Global Impression Severity (CGI‐S). Secondary outcome measures included blinded motor evaluations, timed tests, patient/caregiver questionnaires, and Personal KinetiGraphs (PKG).ResultsTwenty‐five patients were enrolled, and 20 completed. The patient/caregiver CGI‐S for gait/balance (P = 0.01) and appendicular symptom control (P = 0.001), and the blinded rater MDS‐UPDRS‐III (−5.22, P = 0.02), CGI‐S gait/balance (P = 0.01), and CGI‐S speech (P = 0.02) were better while on IL‐IL. Scores on Parkinson's Disease Quality of Life (P = 0.002) and Freezing‐of‐Gait Questionnaires (P = 0.04) were better on IL‐IL. The Timed‐Up‐and‐Go was 9.8% faster (P = 0.01), with 11.8% reduction in steps (P = 0.001) on IL‐IL. There was no difference in PKG bradykinesia (P = 0.18) or tremor (P = 0.23) between paradigms.ConclusionsOur results prompt consideration of this novel programming paradigm (IL‐IL) for PD patients with axial symptom impairment as a new treatment option for both axial and appendicular symptoms. © 2020 International Parkinson and Movement Disorder Society
Microvascular coupler device versus hand‐sewn venous anastomosis: A systematic review of the literature and data meta‐analysis
Authors: Maruccia Michele , Fatigato Giusy , Elia Rossella , Ragusa Luigi Amerigo , Vestita Michelangelo G. , Nacchiero Eleonora , Robusto Fabio , Nicoli Fabio , Pedro Ciudad  , Giudice Giuseppe 
Published: April 2020

AbstractBackgroundSuccessful vascular anastomosis is essential for the survival of free tissue transfer. The aim of the study is to review the current literature and perform a meta‐analysis to assess the potential advantages of a mechanical anastomosis coupler device (MACD) over the hand‐sewn (HS) technique for venous anastomoses.MethodsA systematic Medline search was performed to gather all reports of articles related to MACD from 1984 until now. The following data were extracted: first author and publication date, study design, number of patients and anastomosis, coupler size, site and type of reconstruction, venous anastomotic time, flap failure. A meta‐analysis was performed on articles that met the following inclusion criteria: studies comparing MACD and HS technique in venous anastomosis, reporting anastomotic time, and postoperative complications.ResultsThirty‐three studies were included for the analysis. Twenty‐four were retrospective case series and nine were retrospective comparative studies. A total of 12,304 patients were enrolled with a mean age of 49.23 years (range 31–72). A total of 13,669 flaps were accomplished. The thrombosis rate recorded with MACD was 1.47%. The meta‐analysis revealed that MACD significantly decreased anastomotic time (standard difference in means = −0.395 ± 0.105; Z = −3.776; p < .001) and postoperative flap failure risk (odds ratio [OR] = 0.362, 95% confidence interval [CI] = 0.218–0.603, Z = −3.908, p < .001), but it did not decrease postoperative venous thrombosis risk (OR = 0.504, 95% CI = 0.255–1.129, Z = −1.666, p = .096).ConclusionsMACDs are a safe and effective alternative to traditional anastomosis. The anastomotic coupler is easier, much faster, and requires less technical skills than a HS microvascular anastomosis.
The Role of Dietary Advanced Glycation End Products in Metabolic Dysfunction
Authors: Sergi Domenico , Boulestin Hakim , Campbell Fiona M. , Williams Lynda M. 
Published: April 2020

AbstractAdvanced glycation end products (AGEs) are a heterogeneous group of molecules produced, non‐enzymatically, from the interaction between reducing sugars and the free amino groups of proteins, nucleic acids, and lipids. AGEs are formed as a normal consequence of metabolism but can also be absorbed from the diet. They have been widely implicated in the complications of diabetes affecting cardiovascular health, the nervous system, eyes, and kidneys. Increased levels of AGEs are also detrimental to metabolic health and may contribute to the metabolic abnormalities induced by the Western diet, which is high in processed foods and represents a significant source of AGEs. While increased AGE levels are a consequence of diabetic hyperglycaemia, AGEs themselves activate signaling pathways, which compromise insulin signaling and pancreatic β‐cell function, thus, contributing to the development of type 2 diabetes mellitus (T2DM). Furthermore, AGEs may also contribute to the obesogenic effects of the Western diet by promoting hypothalamic inflammation and disrupting the central control of energy balance. Here, the role of dietary AGEs in metabolic dysfunction is reviewed with a focus on the mechanisms underpinning their detrimental role in insulin resistance, pancreatic β‐cell dysfunction, hypothalamic control of energy balance, and the pathogenesis of T2DM and obesity.