bluuebunny/crossref_metadata_2025_split huggingface dataset:
Secondary Hemophagocytic Syndrome: Differences between the etiologies autoimmunes and hematological malignancies subgroup
Authors: Dubuc César Antonio Egües , Calvo-Alen Jaime , Cabrera-Miranda Lizbeth Patricia , Sola Andrea De Diego , Salsamendi José Ramon Furundarena , Lorenzo Nerea Alcorta , Jaimes Jesús Alejandro Valero , Dominguez Luis María López , Fanlo Jorge Jesús Cancio , Alonso Olga Maiz , Isacelaya Esther Uriarte , Otano Joaquín María Belzunegui 
Published: June 2020

Abstract
Background: Hemophagocytic Syndrome (HPS) is an uncommon disorder with high mortality. HPS is reported to be usually related to some underlying conditions; autoimmune (AI) and hematological malignancies (HM) are two main underlying conditions. Here, we attempted to determine the clinical characteristics/differences of HPS according to underlying conditions; AI vs. HM, based on our experience. Methods: We studied all adult patients with HPS diagnosed at our institution from 2005 to 2019. Demographic, clinical characteristics, laboratory findings, underlying disorders, hospital stay and prognosis variables were examined and related to the underlying conditions. Results: 30 patients (median age of 55 years and male/female of 14/16) met the inclusion criteria which we analyzed: HM: 12 (40%), AI: 10 (33%), and others: 8 (27%). HM group, compared with AI group, showed a significantly longer length hospital stay (HM vs AI: 61.5 vs 30.5 (median)), more severe thrombocytopenia, leukocytopenias, and neutropenia, and importantly, higher mortality (with overall mortality and HM-related HPS-mortality being 43.3 and 66.7%, respectively). Contrarily, HM group, compared with AI group, showed less severe liver abnormalities (AST; 106 vs 457 (median): ALT; 109 vs 457 (median)). Conclusions : The HM subgroup presented a greater mortality and a higher number and severe cytopenias but lower elevation of transaminases.
Dietary iron intake and anemia: Food frequency questionnaire in patients with hereditary hemorrhagic telangiectasia.
Authors: Cavalcoli Federica , Gandini Alberto , Matelloni Irene Aglaia , Catalano Francesca , Alicante Saverio , Manfredi Guido , Brambilla Gianfranco , Menozzi Fernanda , Perolini Federica , Costi Egon , Bertè Roberto , Buscarini Elisabetta 
Published: June 2020

Abstract
Background: Hereditary hemorrhagic telangiectasia (HHT) is a multisystemic inherited vascular disease characterized by a heterogeneous clinical presentation and prognosis. Dietary evaluation is relevant in HHT patients to provide adequate iron and nutrient intake. Additionally, different dietary items have been reported to precipitate epistaxis in this setting. Our primary aim was to investigate the dietary habits of HHT patients through a food-frequency questionnaire (FFQ) to evaluate the presence of precipitants and/or protective factors for epistaxis and the occurrence of possible dietary modifications. The secondary aims were to evaluate the nutritional intake of iron in HHT patients and the self-reported effect of iron treatments on epistaxis.From April 2018 to October 2018, a 138-item FFQ was provided to HHT patients followed up at the HHT Referral Center of Crema Maggiore Hospital. The relationship between food items and epistaxis was ascertained on a separate form. Daily iron intake was calculated to establish the mean iron content of food items reported in the FFQ.Results: One hundred forty-nine questionnaires were evaluated [72 females, median age 54 years (12-76). Overall, 26 (18%) patients reported dietary items that improved epistaxis (mostly blueberries and red fruits, green vegetables and legumes), while 38 (26%) reported some dietary items that exacerbated epistaxis (spices, chocolate, alcohol, strawberries and ginger). Dietary modifications were reported in up to 58% of cases. In HHT patients, the mean daily iron intake was 8.46 ± 2.78 mg, and no differences were observed in the iron intake of patients reporting a diet modification and those who did not.Conclusions: Dietary evaluation is advisable in the management of HHT patients. HHT patients should be encouraged to follow a healthy and balanced diet with increased consumption of dietary items with a high iron content.
Myocardial Disease and Ventricullar Arrhythmia in Marfan Syndrome – A Prospective Study
Authors: Mosquera Laura Muino , De Wilde Hans , Devos Daniel , Babin Danilo , Jordaens Luc , Demolder Anthony , De Groote Katya , De Wolf Daniel , De Backer Julie 
Published: June 2020

Abstract
Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality. Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.8% women, mean age 36.3yr - range 13-70yr-) and 40 age- and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30±7months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N=55) and with previous surgery or valvular dysfunction (MFS-2; N=31). Results: Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles ((S)VES). Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion: Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Myocardial function should be evaluated in all patients with MFS, especially those with valvular disease and cardiac surgery.
Preoperative endoscopic biliary stenting before pancreatico duodenectomy: Does timing matter?
Authors: Bhatti Abu Bakar Hafeez , Jafri Roshni Zahra , Khan M Kashif , Dar Faisal Saud 
Published: June 2020

Abstract
BackgroundRole of preoperative biliary stenting (PBS) before pancreaticodoudenectomy (PD) in patients with obstructive jaundice is debatable. The objective of the current study was to assess outcomes after PD in patients who underwent upfront surgery or PBS and determine the impact of stent to surgery duration on outcomes after PD. Methods We reviewed 147 patients who underwent PD between 2011 and 2019. Patients were grouped based on whether they underwent upfront surgery (N=76) or PBS (N=71). We further assessed outcomes based on stent to surgery interval < 4 weeks or > 4 weeks. We looked at 30 and 90 day morbidity and mortality rates in these patients.Results A significant increase in wound infections (7% vs 25%)(P=0.003), overall infectious complications (22.5% vs 38.1%)(P=0.04), re admissions (0 vs 10.5%)(P=0.005) and hospital stay (9 vs 10 days)(P=0.006) was seen in the PBS group. There was no significant difference in 30 day mortality (2.8 % vs 6.5%)(P=0.4). When compared with upfront surgery group, patients with stent to surgery duration > 4 weeks had higher rates of wound infection (7% vs 29%)(P=0.009), sepsis (11.2% vs 29%)(P=0.02), overall infectious complications (22.5% vs 45.1%)(P=0.02), re admissions (0 vs 12.9%) (P=0.007) and hospital stay (9 vs 10 days)(P=0.03). The lowest rate of infectious complications was seen when PD was performed within 2 weeks (22.2%) or 6-8 weeks (12.5%) after stenting. None of the patients with stent-surgery duration < 2 weeks developed sepsis. ConclusionsPBS appears to increase infection related morbidity after PD. In patients with PBS, low morbidity is seen with early (< 2 weeks) and delayed PD (6-8weeks).
Adaptation and convergence in circadian-related genes in Iberian freshwater fish
Authors: Moreno João M , Jesus Tiago F , Sousa Vitor C , Coelho Maria M 
Published: June 2020

Abstract
BackgroundThe circadian clock is a biological timing system that improves the ability of organisms to deal with environmental fluctuations. At the molecular level it consists of a network of transcription-translation feedback loops, involving genes that activate (bmal and clock – positive loop) and repress expression (cryptochrome (cry) and period (per) – negative loop). This is regulated by daily alternations of light but can also be affected by temperature. Fish, as ectothermic, depend on the environmental temperature and thus are good models to study its integration within the circadian system. Here, we studied the molecular evolution of circadian genes in four Squalius freshwater fish species, distributed across Western Iberian rivers affected by two climatic types with different environmental conditions (e.g. light and temperature). S. carolitertii and S. pyrenaicus inhabit the colder northern region under Atlantic climate type, while S. torgalensis, S. aradensis and some populations of S. pyrenaicus inhabit the warmer southern region affected by summer droughts, under Mediterranean climate type. ResultsWe identified 16 circadian-core genes in the Squalius species using a comparative transcriptomics approach. We detected evidence of positive selection in nine of these genes using methods based on dN/dS. Positive selection was mainly found in cry and per genes of the negative loop of the cycle, with 11 putatively adaptive substitutions mostly located on protein domains. Evidence for positive selection is predominant in southern populations affected by the Mediterranean climate type. By predicting protein features we found that changes at sites under positive selection can impact protein thermostability by changing their aliphatic index and isoelectric point. Additionally, in nine genes, the phylogenetic clustering of species that belong to different clades but inhabit southern basins with similar environmental conditions indicated evolutionary convergence.ConclusionsOur results support that temperature may be a strong selective pressure driving the evolution of genes involved in the circadian system. By integrating sequence-based functional protein prediction with dN/dS-based methods to detect selection we also uncovered adaptive convergence in the southern populations, probably related to their similar thermal conditions.
The SNPs rs429358 and rs7412 of APOE gene are association with cerebral infarction but not SNPs rs2306283 and rs4149056 of SLCO1B1 gene in southern Chinese Hakka population
Authors: Wu Heming , Huang Qingyan , Yu Zhikang , Wu Hailing , Zhong Zhixiong 
Published: June 2020

Abstract
Background: Apolipoprotein E (ApoE) and solute carrier organic anion transporter family member 1B1 (SLCO1B1) are involved in regulating lipid metabolism. However, the relation between genetic polymorphisms of APOE and SLCO1B1 and cerebral infarction (CI) remains unclear.Methods: A total of 945 CI patients and 1,028 control participants were included in the study. The single nucleotide polymorphisms (SNPs) rs429358, rs7412 of APOE gene and SNPs rs2306283, rs4149056 of SLCO1B1 gene were analyzed by fluorescence polymerase chain reaction (PCR) method.Results: The genotype ɛ3/ɛ3 is the most common genotype of APOE gene, in which ɛ3 is the allele with greatest frequency, followed by ɛ4 and ɛ2. There were statistically significant differences of genotype ɛ2/ɛ2 (c2=4.718, P=0.030), ɛ2/ɛ3 (c2=18.076, P<0.001), ɛ3/ɛ4 (c2=18.714, P<0.001), ɛ4/ɛ4 (c2=4.710, P=0.046) in CI patients comparing with controls. The SLCO1B1 genotype *1b/*1b and haplotype *1b have greatest frequency in the study sample. There was no statistically significant difference in frequencies of SLCO1B1 genotypes and haplotypes in CI patients comparing with controls. Moreover, the ε4 carriers had significantly higher LDL-C, Apo-B, and lower Apo-A1/Apo-B than the other groups, while the ε2 carriers showed the opposite results in the CI group and controls. Logistic regression analysis indicated that participants with ε4 allele had a significantly higher risk of CI in males (OR 3.508, 95% CI 2.186-5.629, P<0.001) and females (OR 2.723, 95% CI 1.428-5.194, P=0.002).Conclusions: The study indicated that the SNPs rs429358 and rs7412 of APOE may be associated with susceptibility to cerebral infarction, in southern Chinese Hakka population.
Novel non-specific lipid-Transfer Protein (TdLTP4) isolated from Durum wheat: Antimicrobial activities to control pathogen and spoilage bacteria and Anti-Inflammatory properties in lipopolysaccharide (LPS)-stimulated RAW 264.7 macrophages
Authors: Hsouna Anis Ben , Saad Rania Ben , Dhifi Wissal , Khaled Marwa , Mnif Wissem , Brini Faiçal 
Published: June 2020

Abstract
Background: Lipid transfer proteins (LTP) are members of the family of pathogenesis-related proteins (PR-14) that play a key role in plant defense mechanisms.Methods: In this study, a novel gene TdLTP4 encoding an antifungal protein from wheat (cv. Om Rabiaa) was subcloned, overexpressed in Escherichia coli BL-21 (DE3) and enriched using ammonium sulfate fractionation. The TdLTP4 fusion protein was then tested against a panel of pathogens, food-borne and spoilage bacteria and fungi in order to evaluate the antimicrobial properties. Our protein was applied to 0.5 µg/mL LPS-induced RAW 264.7 macrophages in vitro at different concentrations (5, 10, 20, 50 and 100 µg/ml). Levels of nitric oxide (NO), pro-inflammatory cytokines interleukin (IL)-1β (IL-1 β), interleukin (IL)-6 (IL-6), tumor necrosis factor (TNF-α) and anti-inflammatory cytokine IL-10 in the supernatant fraction were measured using enzyme-linked immunosorbent assay (ELISA). Expression of cyclooxygenase-2 (COX-2) and inducible nitric oxide synthase (iNOS) were detected via Western blot. Results: The inhibition zones and minimal inhibitory concentration (MIC) values of bacterial strains were in the range of 14-26 mm and 62.5-250 µg/mL, respectively. Moreover, a remarkable activity against several fungal strains was revealed. TdLTP4 (5–100 µg/mL) decreased the production of NO (IC50= 4.32 µg/mL), IL-6 (IC50= 11.52 µg/mL), IL-1β (IC50= 7.87 µg/mL) and TNF-α (IC50 =8.66 µg/mL) by lipopolysaccharide (LPS)-stimulated RAW 264.7 macrophages. Our protein could modulate the macrophages inflammatory mode by causing reduction in iNOS and COX2.Conclusion: According to these findings, LTP fusion protein could be used as natural anti-inflammatory and antimicrobial agent in food preservation and human health.
Oxidized Low-Density Lipoprotein (Ox-LDL) and Malondialdehyde (MDA) and Menopause: Loss of Protective Effect of Premenopuasl Status in Type 2 Diabetes Mellitus
Authors: Arani Reyhane Hizomi , Rabizadeh Soghra , Rajab Armin , Heidari Firuzeh , Esteghamati Alireza , Mirmiranpour Hossein , Hajmiri Minoosadat , Nakhjavani manouchehr 
Published: June 2020

Abstract
Background: A network of factors suggested to influence oxidative modification of serum low-density lipoprotein (ox-LDL) concentrations and it plays critical role in diabetic copmlications.This study aimed to assess the joint effect of Type 2 diabetes mellitus (T2DM), gender and menopausal status on ox-LDL and Malondialdehyde (MDA) levels.Material and Method: A total of 594 female and male participants were divided into six groups: 189 postmenopausal women with T2DM, 79 premenopausal women with T2DM, 54 premenopausal women without T2DM, 53 postmenopausal women without T2DM, 186 men with T2DM and 33 men without T2DM. Laboratory and anthropometric measurements, metabolic syndrome (MetS) and the 10-year risk score for atherosclerotic cardiovascular disease (ASCVD) were assessed. Results: Ox-LDL in women with T2DM was three times higher than women without T2DM. However, ox-LDL in men with T2DM was 1.3 times higher than men without T2DM. In non-T2DM participants, postmenopausal women had higher 10-year ASCVD risk score, concentrations of ox-LDL and MDA and higher prevalence of MetS compared to premenopausal women. However, this difference disappeared in participants with T2DM. In The interaction analysis did not show an additional effect of menopausal status and T2DM for ox-LDL and MDA serum levels in female participants (respectively p= 0.310, p= 0.922). In multivariate analysis, TG was independently associated with ox-LDL concentrations (OR: 1.27, p ≤0.001). In T-test analysis, participants with MetS, higher risk of ASCVD had higher levels of ox-LDL.Conclusion: The magnitude of the increase in ox-LDL and MDA levels in women with T2DM was significantly higher than men with T2DM. The differences between pre- and postmenopausal women with and without T2DM in circulating ox-LDL and MDA mostly were dependent on T2DM, regardless of menopausal status. T2DM override the effect of menopausal status, gender and age on ox-LDL and MDA concentrations. Having MetS, increased TG levels and higher 10-year ASCVD risk score suggested to be associated with higher levels of ox-LDL.
The Risks of Advancing Parental Age on Neonatal Morbidity and Mortality are U- or J-Shaped for Both Maternal and Paternal Ages.
Authors: Thompson James 
Published: June 2020

Abstract
Background: The biologic implications of delayed parenthood have been blamed for a major public health crisis in the United States, that includes high rates of neonatal morbidity and mortality (NMM). The objective of this study was to evaluate the risk of parent age on NMM and to provide results that can serve as a starting point for more specific mediation modeling. Methods: Data containing approximately 15,000,000 birth records were obtained from the United States Natality database for the years 2014 to 2017. A Bayesian modeling approach was used to estimate the both the total effect and the risk adjusted for confounding between parent ages and for mediation by chromosomal disorders including Down syndrome. Outcomes included neonatal mortality and nine measures of neonatal morbidity.Results: For paternal age, seven NMM (preterm birth, very preterm birth, low Apgar score, treatment with antibiotics, treatment with surfactant, prolonged ventilation, neonatal mortality) had U-shaped risk patterns, two NMM (small for gestational age, admission to neonatal intensive care) had J-shaped risk patterns, one NMM (seizures) was not significantly related to paternal age. For maternal age, three NMM (low Apgar score, treatment with antibiotics and neonatal mortality) had U-shaped risk patterns, four NMM (preterm delivery, very preterm delivery, admission to neonatal intensive care, treatment with surfactant) had J-shaped risk patterns, one NMM (small for gestational age) had a risk declining with age, one NMM (prolonged ventilation) had a risk increasing with age and one NMM (seizures) was not significantly related to maternal age. Conclusions. Under the assumptions of a specific causal model, both advancing maternal and paternal ages had U- or J-shaped risk patterns for neonatal morbidity and mortality.
Constraining PERMANOVA and LDM to Within-Set Comparisons by Projection Improves the Efficiency of Analyses of Matched sets of Microbiome Data
Authors: Zhu Zhengyi , Satten Glen A , Mitchell Caroline , Hu Yi-Juan 
Published: June 2020

Abstract
Background: Matched-set data arise frequently in microbiome studies. For example, we may collect pre- and post-treatment samples from a set of individuals, or use important confounding variables to match data from case participants to one or more control participants. Thus, there is a need for statistical methods for data comprised of matched sets, to test hypotheses against traits of interest (e.g., clinical outcomes or environmental factors) at the community level and/or the OTU (operational taxonomic unit) level. Optimally, these methods should accommodate complex data such as those with unequal sample sizes cross sets, confounders varying within sets, as well as continuous traits of interest. Methods: PERMANOVA is a commonly used distance-based method for testing hypotheses at the community level. We have also developed the linear decomposition model (LDM) that unifies the community-level and OTU-level tests into one framework. Here we present a new strategy that can be used with both PERMANOVA and the LDM for analyzing matched-set data. We propose to include an indicator variable for each set as covariates, so as to constrain comparisons between samples within a set, and also permute traits within each set, which can account for exchangeable sample correlations. The flexible nature of PERMANOVA and the LDM allows discrete or continuous traits or interactions to be tested, within-set confounders to be adjusted, and unbalanced data to be fully exploited. Results: Our simulations indicate that our proposed strategy outperformed alternative strategies, including the commonly-used one that utilizes restricted permutation only, in a wide range of scenarios. Using simulation, we also explored optimal designs for matched-set studies. The flexibility of PERMANOVA and the LDM for a variety of matched-set microbiome data is illustrated by the analysis of data from two real studies. Conclusions: Including set indicator variables and permuting within sets when analyzing matched-set data with PERMANOVA or the LDM is a strategy that performs well and is capable of handling the complex data structures that frequently occur in microbiome studies.
Probabilistic Tsunami Hazard Model of Building Inundation Following a Subaqueous Volcanic Explosion Based on the 1716 Tsunami Scenario in Taal Lake, Philippines
Authors: Pakoksung Kwanchai , Suppasri Anawat , Imamura Fumihiko 
Published: June 2020

Abstract
A probabilistic hazard analysis of a tsunami generated by a subaqueous volcanic explosion is performed for Taal Lake in the Philippines. The Taal volcano in Taal Lake is an active volcano on Luzon Island in the Philippines, and its eruption would have a strong impact on humans around the coastal area of the lake. This study aims to develop a probabilistic tsunami hazard model of inundated buildings for tsunami mitigation in future scenarios. To develop the probabilistic tsunami hazard model, different explosion diameters were used to generate tsunamis of different magnitudes in the TUNAMI-N2 model. The initial water level in the tsunami model was estimated based on the explosion energy as a function of the explosion diameter. The tsunami-induced inundation from the TUNAMI-N2 model was overlaid on the distribution of buildings. The statistical distribution of inundated buildings can be modeled with the lognormal distribution, which exhibits the best fit among nine candidate statistical distributions. The tsunami hazard analysis is explained by using the conditional hazard curve and map. These products were used to calculate the probability of building inundation given the occurrence of a subaqueous explosion. The results from this study can be used for future tsunami mitigation in the case of a tsunami generated by a subaqueous volcanic explosion.