bluuebunny/crossref_metadata_2025_split huggingface dataset:
The mask of the red death: Leadership, hubris, and the Covid-19 crisis
Authors: Villanueva Jaume , Sapienza Harry J 
Published: April 2021

Throughout history, societies have been beset by disasters that took them by surprise, often with widespread and devastating consequences. When crops, animals, or people began dying with unusual frequency, especially when the causes were not observable, people often failed to respond, responded very slowly, or responded in surprisingly misdirected ways. In this essay, we focus on the role of leadership in addressing or failing to address such crises, paying special attention to the responses to the Covid-19 crisis during 2020. Our central thesis is that leadership hubris was a central (though not the only) culprit in poor responses, and we introduce the problem of inaction as a symptom of hubris. We conclude with some reflections for practitioners and researchers, suggesting a few areas worthy of examination to better understand how societies and business organizations may construct defenses against the dangers of leadership hubris. JEL CLASSIFICATION M14; L26; D70; N00
Arterial Spin Labelling Patterns in Acute Large Vessel Occlusion Stroke
Authors: Nedunchelian Meena , Varadharajan Shriram , Keerthy S , Karthikeyan MA , Poyyamozhi Santosh , Mehta Pankaj , Cherian Mathew 
Published: April 2021

Background: Arterial spin labelling (ASL) is a noncontrast, magnetic resonance perfusion technique which can provide information about the parenchymal perfusion status and collaterals in acute stroke. Its role in the setting of large vessel occlusion (LVO) is underutilized due to the limited availability of magnetic resonance imaging (MRI) in the emergency settings. However, it might serve as an useful adjunct to other perfusion modalities in future. Objective: To study ASL perfusion patterns in anterior circulation LVO stroke and evaluate the presence of arterial transit artefacts (ATA) as a surrogate marker of collaterals. Methods: We performed a retrospective analysis of our mechanical thrombectomy database over the last 2 years for acute stroke cases in which ASL perfusion as part of MRI was performed. ASL perfusion patterns in acute LVO stroke were reviewed, with respect to presence of ATA and diffusion-weighted imaging (DWI)-ASL mismatch. Inter-rater reliability was analyzed between 2 readers of varying experience. Baseline variables were analyzed between those with and without ATA. Results: Out of 95 patients, 78 had anterior circulation occlusions, among which 27 had ASL. Type I (with ATAs) was seen in 11 patients and all of these had DWI mismatch. Type II (without ATAs) was seen in 16 patients. Of these, 15 had mismatch with DWI and only 1 had no mismatch. Inter-rater reliability for the detection of ATA on ASL was substantial (Cohen’s k—0.64). No statistical significance was noted between ATA and clinical outcomes. Conclusion: ASL patterns vary amongst patients with LVO stroke and can be classified based on the presence of ATA since resulting DWI mismatch actually indicates a pseudo-perfusion deficit.
The impact of previous cesarean section on the outcome of patients with non-adherent placenta previa
Authors: Sindiani Amer , Obeidat Nail , Abu-Azzam Omar , Hijazi Heba 
Published: April 2021

AbstractObjectiveTo evaluate the impact of previous lower segment cesarean section on maternal and fetal outcomes of patients with non-adherent placenta previaMethodsThis is a retrospective study of all patients who delivered at a tertiary referral university hospital by lower uterine cesarean section with non-adherent placenta previa, over a 10-year period. Data were obtained through hospital registry and medical records. The differences in demographic, obstetric characteristics, peripartum complications, and fetal outcome between patients with no previous cesarean and those with previous cesarean section were analyzed.ResultsNinety patients with non-adherent placenta previa were included, 54 patients with no previous cesarean and 36 patients with previous cesarean section. Patients with previous cesarean section were significantly more likely to incur inadvertent organ injury, transfusion of 4 or more units of packed red blood cells, transfusion of fresh frozen plasma, uterine brace compressive sutures, uterine artery ligation, and peripartum hysterectomy.ConclusionThere is a significant increase in the incidence of inadvertent neighboring viscus injury, transfusion of 4 units or more of packed red blood cells, transfusion of fresh frozen plasma, uterine brace compressive sutures, uterine artery ligation, and peripartum hysterectomy in patients with non-adherent placenta previa and previous cesarean section, compared to those with non-adherent placenta previa and no previous history of cesarean section, with no difference in perinatal outcome.
An age-dependent outcome analysis of microvascular decompression and percutaneous thermocoagulation in trigeminal neuralgia
Authors: Keric Naureen , Kalasauskas Darius , Kreth Sophia L. , Glaser Martin B. , Krenzlin Harald , Kantelhardt Sven R. , Ringel Florian 
Published: April 2021

Abstract
Background
Trigeminal neuralgia (TN) is a severe pain condition and the most common facial neuralgia. While microvascular decompression (MVD) presents an excellent treatment in neurovascular compression cases, percutaneous thermocoagulation (PT) of the ganglion Gasseri is an alternative option. This study aimed to evaluate post-operative complication rate and outcome of both treatment strategies related to the patient’s age.

Methods
The medical records of all patients with the diagnosis of trigeminal neuralgia undergoing an MVD or PT of the ganglion Gasseri (between January 2007 and September 2017) were reviewed to determine the efficacy and the complication rate of both methods in regard to the patient’s age.

Results
Seventy-nine patients underwent MVD surgery and 39 a PT. The mean age of patients in the MVD group was 61 years and 73 years in the PT group. There were 59 (50%) female patients. Nerve-vessel conflict could be identified in 78 (98.7%) MVD and 17 (43.6%) PT patients on preoperative MRI. Charlson comorbidity index was significantly higher in PT group (2.4 (1.8) versus 3.8 (1.8) p < 0.001). The Barrow pain score (BPS) at the last follow-up demonstrated higher scores after PT (p = 0.007). The complication rate was markedly higher in PT group, mostly due to the facial hypesthesia (84.6% versus 27.8%; p < 0.001). Mean symptom-free survival was significantly shorter in the PT group (9 vs. 26 months, p < 0.001). It remained statistically significant when stratified into age groups: (65 years and older: 9 vs. 18 months, p = 0.001).
Duration of symptoms (OR 1.005, 95% CI 1.000–1.010), primary procedure (OR 6.198, 95% CI 2.650–14.496), patient age (OR 1.033, 95% CI 1.002–1.066), and postoperative complication rate (OR 2.777, 95% CI 1.309–5.890) were associated with treatment failure.

Conclusion
In this patient series, the MVD is confirmed to be an excellent treatment option independent of patient’s age. However, while PT is an effective procedure, time to pain recurrence is shorter, and the favorable outcome (BPS 1 and 2) rate is lower compared to MVD. Hence MVD should be the preferred treatment and PT should remain an alternative in very selected cases when latter is not possible but not in the elderly patient per se.
Perinatal outcomes of neonates born from different endometrial preparation protocols after frozen embryo transfer: a retrospective cohort study
Authors: Li Cheng , He Yi-Chen , Xu Jing-Jing , Wang Yu , Liu Han , Duan Chen-Chi , Shi Chao-Yi , Chen Lei , Wang Jie , Sheng Jian-Zhong , Huang He-Feng , Wu Yan-Ting 
Published: April 2021

Abstract
Background
Previous studies have focused on pregnancy outcomes after frozen embryo transfer (FET) performed using different endometrial preparation protocols. Few studies have evaluated the effect of endometrial preparation on pregnancy-related complications. This study was designed to explore the association between different endometrial preparation protocols and adverse obstetric and perinatal complications after FET.

Methods
We retrospectively included all FET cycles (n = 12,950) in our hospital between 2010 and 2017, and categorized them into three groups, natural cycles (NC), hormone replacement therapy (HRT) and ovarian stimulation (OS) protocols. Pregnancy-related complications and subsequent neonatal outcomes were compared among groups.

Results
Among all 12,950 FET cycles, the live birth rate was slightly lower for HRT cycles than for NC (HRT vs. NC: 28.15% vs. 31.16%, p < 0.001). The pregnancy loss rate was significantly higher in OS or HRT cycles than in NC (HRT vs. NC: 17.14% vs. 10.89%, p < 0.001; OS vs. NC: 16.44% vs. 10.89%, p = 0.001). Among 3864 women with live birth, preparing the endometrium using OS or HRT protocols increased the risk of preeclampsia, and intrahepatic cholestasis of pregnancy (ICP) in both singleton and multiple deliveries. Additionally, OS and HRT protocols increased the risk of low birth weight (LBW) and small for gestational age (SGA) in both singletons and multiples after FET.

Conclusion
Compared with HRT or OS protocols, preparing the endometrium with NC was associated with the decreased risk of pregnancy-related complications, as well as the decreased risk of LBW and SGA after FET.
Midwives’ experiences of cultural competency training and providing perinatal care for migrant women a mixed methods study: Operational Refugee and Migrant Maternal Approach (ORAMMA) project
Authors: Fair Frankie , Soltani Hora , Raben Liselotte , van Streun Yvonne , Sioti Eirini , Papadakaki Maria , Burke Catherine , Watson Helen , Jokinen Mervi , Shaw Eleanor , Triantafyllou Elena , van den Muijsenbergh Maria , Vivilaki Victoria 
Published: April 2021

Abstract
Background
The number of international migrants continues to increase worldwide. Depending on their country of origin and migration experience, migrants may be at greater risk of maternal and neonatal morbidity and mortality. Having compassionate and culturally competent healthcare providers is essential to optimise perinatal care. The “Operational Refugee and Migrant Maternal Approach” (ORAMMA) project developed cultural competence training for health professionals to aid with providing perinatal care for migrant women. This presents an evaluation of ORAMMA training and explores midwives’ experiences of the training and providing care within the ORAMMA project.

Methods
Cultural competence was assessed before and after midwives (n = 35) received ORAMMA compassionate and culturally sensitive maternity care training in three different European countries. Semi-structured interviews (n = 12) explored midwives’ experiences of the training and of caring for migrant women within the ORAMMA project.

Results
A significant improvement of the median score pre to post-test was observed for midwives’ knowledge (17 to 20, p < 0.001), skills (5 to 6, p = 0.002) and self-perceived cultural competence (27 to 29, p = 0.010).
Exploration of midwives’ experiences of the training revealed themes of “appropriate and applicable”, “made a difference” and “training gaps” and data from ORAMMA project experiences identified three further themes; “supportive care”, “working alongside peer supporters” and “challenges faced”.

Conclusions
The training improved midwives’ knowledge and self-perceived cultural competence in three European countries with differing contexts and workforce provision. A positive experience of ORAMMA care model was expressed by midwives, however clearer expectations of peer supporters’ roles and more time within appointments to assess the psychosocial needs of migrant women were desired. Future large-scale research is required to assess the long-term impact of the ORAMMA model and training on practice and clinical perinatal outcomes.
Patterns of surveillance for late effects of BCR-ABL tyrosine kinase inhibitors in survivors of pediatric Philadelphia chromosome positive leukemias
Authors: Smith Stephanie M. , Sabnis Himalee S. , Lewis Rebecca Williamson , Effinger Karen E. , Bergsagel John , Patterson Briana , Mertens Ann , Sakamoto Kathleen M. , Schapira Lidia , Castellino Sharon M. 
Published: April 2021

Abstract
Background
Targeted anticancer therapies such as BCR-ABL tyrosine kinase inhibitors (TKIs) have improved outcomes for chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph + ALL). However, little is known about long-term risks of TKIs in children. Exposure-based survivorship guidelines do not include TKIs, thus surveillance practices may be variable.

Methods
We retrospectively examined surveillance for cardiac and endocrine late effects in children receiving TKIs for Ph + leukemias, diagnosed at < 21 years between 2000 and 2018. Frequency of echocardiogram (ECHO), electrocardiogram (EKG), thyroid stimulating hormone (TSH), dual-energy x-ray absorptiometry (DXA), and bone age testing were abstracted. Descriptive statistics were stratified by leukemia type.

Results
66 patients (CML n = 44; Ph + ALL n = 22) met inclusion criteria. Among patients with CML, ≥1 evaluation was done: ECHO (50.0%), EKG (48.8%), TSH (43.9%), DXA (2.6%), bone age (7.4%). Among patients with Ph + ALL, ≥1 evaluation was done: ECHO (86.4%), EKG (68.2%), TSH (59.1%), DXA (63.6%), bone age (44.4%). Over a median 6.3 and 5.7 years of observation, respectively, 2% of patients with CML and 57% with Ph + ALL attended a survivorship clinic.

Conclusions
Despite common exposure to TKIs in survivors of Ph + leukemias, patterns of surveillance for late effects differed in CML and Ph + ALL, with the latter receiving more surveillance likely due to concomitant chemotherapy exposures.
Targeted therapies such as TKIs are revolutionizing cancer treatment, but surveillance for late effects and referral to survivorship clinics are variable despite the chronicity of exposure. Evidence based guidelines and longer follow-up are needed.
MACE-Seq-based coding RNA and TrueQuant-based small RNA profile in breast cancer: tumor-suppressive miRNA-1275 identified as a novel marker
Authors: Majed Sevan Omer , Mustafa Suhad Asad 
Published: April 2021

Abstract
Introduction
Disruption of cellular processes in the breast by abnormally expressed miRNA is characterized to develop cancer. We aimed to identify the differential expression of small RNAs (sRNAs) and mRNAs in formalin-fixed paraffin-embedded (FFPE) tissue of the breast cancer (BC) and normal adjacent tissue (NAT). Another aim is to determine the differential expression of miR-1275 as a novel biomarker for BC and also identify its target genes.

Methods
TrueQuant method for analysis of sRNA expression and MACE-sequencing method for analysis of gene expression were used analyzing. The RT-qPCR technique was used to confirm miR-1275 down expression. Target genes of miR-1275 were computationally identified using target prediction sites and also the expression level of them was experimentally determined among the expressed genes.

Results
TrueQuant findings showed that 1400 sRNAs were differentially expressed in the FFPE tissue of two Kurdish cases with BC, as compared to NAT. Among the sRNAs, 29 small RNAs were shown to be significantly downregulated in BC cells. The RT-qPCR results confirmed that miR-1275 was significantly down-expressed in 20 Kurdish cases with BC compared to NAT. However, Overall survival (OS) analysis revealed that the correlation between the expression level of miR-1275 and clinical significance was highly corrected in cases with BC (OS rate: P = 0.0401). The MACE-seq results revealed that 26,843 genes were differentially expressed in the BC tissue compared to NAT, but 7041 genes were displayed in a scatter plot. Furthermore, putative target genes (DVL3, PPP2R2D, THSD4, CREB1, SYT7, and PRKACA) were computationally identified as direct targets of miR-1275 in several target predicted sites. The MACE-seq results revealed that the expression level of these targets was increased in BC tissue compared to NAT. The level of these targets was negatively associated with miR-1275 expression. Finally, the role of down-regulated miR-1275 on its targets in biological mechanisms of BC cells was identified; including cell growth, proliferation, movement, invasion, metastasis, and apoptosis.

Conclusion
Down-expressed miR-1275, a tumor suppressor, is a novel biomarker for early detection of BC. DVL3, PPP2R2D, THSD4, CREB1, SYT7, and PRKACA are newly identified to be targeted by miR-1275.
USH2A gene variants cause Keratoconus and Usher syndrome phenotypes in Pakistani families
Authors: Ahmed Asif Naveed , Tahir Raheel , Khan Niamat , Ahmad Mushtaq , Dawood Muhammad , Basit Abdul , Yasin Muhammad , Nowshid Maha , Marwan Muhammad , Sultan Komal , Saleha Shamim 
Published: April 2021

Abstract
Background
Retinitis pigmentosa (RP) is the most common inherited retinal dystrophy, affecting approximately 1 in 4000 individuals worldwide. The most common form of syndromic RP is Usher syndrome (USH) accounting for approximately 20–30 % of RP cases. Mutations in the USH2A gene cause a significant proportion of recessive non-syndromic RP and USH type II (USH2). This study aimed to determine the causative role of the USH2A gene in autosomal recessive inherited ocular diseases and to establish genotype-phenotype correlation associated with USH2A variants.

Methods
We performed direct Sanger sequencing and co-segregation analysis of the USH2A gene to identify disease causing variants in a non-syndromic RP family, two USH2 families and two Keratoconus (KC) families.

Results
Disease causing variants in the USH2A gene were identified in two families displayed KC and USH2 phenotypes. A novel variant c.4029T > G, p.Asn1343Lys in the USH2A gene was detected in a Pakistani family with KC phenotype. In addition, a missense variant (c.7334 C > T, p. Ser2445Phe) in the USH2A gene was found segregating in another Pakistani family with USH2 phenotype. Homozygosity of identified missense USH2A variants was found associated with autosomal recessive inherited KC and USH2 phenotypes in investigated families. These variants were not detected in ethnically matched healthy controls. Moreover, the USH2A variants were predicted to be deleterious or potentially disease causing by PolyPhen-2, PROVEAN and SIFT.

Conclusions
This study provided first evidence for association of a novel USH2A variant with KC phenotype in a Pakistani family as well as established the phenotype-genotype correlation of a USH2A variant (c.7334 C > T, p. Ser2445Phe) with USH2 phenotype in another Pakistani family. The phenotype-genotype correlations established in present study may improve clinical diagnosis of affected individuals for better management and counseling.
Clinical and genetic analysis of a case with centronuclear myopathy caused by SPEG gene mutation: a case report and literature review
Authors: Zhang Gang , Xu Min , Huang Tingting , Lin Wenxin , Chen Jinglin , Chen Wangyang , Chang Xingzhi 
Published: April 2021

Abstract
Background
Centronuclear myopathy (CNM), a subtype of congenital myopathy (CM), is a group of clinical and genetically heterogeneous muscle disorders. Since the discovery of the SPEG gene and disease-causing variants, only a few additional patients have been reported.

Case presentation
The child, a 13-year-old female, had delayed motor development since childhood, weakness of both lower extremities for 10 years, gait swinging, and a positive Gower sign. Her distal muscle strength of both lower extremities was grade IV. The electromyography showed myogenic damage and electromyographic changes. Her 11-year-old sister had a similar muscle weakness phenotype. Gene sequencing revealed that both sisters had SPEG compound heterozygous mutations, and the mutation sites were c.3715 + 4C > T and c.3588delC, which were derived from their parents. These variant sites have not been reported before. The muscle biopsy showed the nucleic (> 20% of fibers) were located in the center of the cell, the average diameter of type I myofibers was slightly smaller than that of type II myofibers, and the pathology of type I myofibers was dominant, which agreed with the pathological changes of centronuclear myopathy.

Conclusions
The clinical phenotypes of CNM patients caused by mutations at different sites of the SPEG gene are also different. In this case, there was no cardiomyopathy. This study expanded the number of CNM cases and the mutation spectrum of the SPEG gene to provide references for prenatal diagnosis and genetic counseling.
Association between healthy lifestyle practices and life purpose among a highly health-literate cohort: a cross-sectional study
Authors: Hirooka Nobutaka , Kusano Takeru , Kinoshita Shunsuke , Aoyagi Ryutaro , Hidetomo Nakamoto 
Published: April 2021

Abstract
Background
The national health promotion program in the twenty-first century Japan (HJ21) correlates life purpose with disease prevention, facilitating the adoption of healthy lifestyles. However, the influence of clustered healthy lifestyle practices on life purpose, within the context of this national health campaign remains uninvestigated. This study assessed the association between such practices and life purpose, in line with the HJ21.

Methods
We performed a nationwide cross-sectional survey on certified specialists in health management. Participants’ demographic information, lifestyle, and purpose in life were measured using a validated tool. The cohort was median-split into two groups based on their clustered health-related lifestyle score. The values for health-related lifestyle and purpose were compared between the two groups and the correlation between health-related lifestyle and purpose in life was measured.

Results
Data from 4820 participants were analyzed. The higher-scoring health-related lifestyle group showed a significantly higher life purpose than the lower group (35.3 vs 31.4; t = 23.6, p < 0.001). There was a significant association between the scores of clustered healthy lifestyle practices and life purpose (r = 0.401, p < 0.001). The higher-scoring health-related lifestyle group achieved a higher life purpose than the lower-scoring group. This association between healthy lifestyle practices and life purpose denotes a positive and linear relationship.

Conclusions
Our results suggest that individuals who have a better health-related lifestyle gain a higher sense of life purpose. In other words, a healthy lifestyle predicts a purpose in life. Our findings posit that examining the causal relationship between healthy lifestyle and purpose in life may be a more efficient approach toward health promotion.