bluuebunny/crossref_metadata_2025_split huggingface dataset:
The epidemiology of amyloidosis in Queensland, Australia
Authors: Wisniowski Brendan , McLeod Donald S. A. , Adams Rebecca , Harvey Yasmin , Brown Ian , McGuire Lloyd , Armes Jane , Mollee Peter 
Published: May 2019

SummaryPublished studies on the epidemiology of amyloidosis have relied on death certificate data for case ascertainment. We estimated the incidence and mortality burden of amyloidosis among residents of the Australian state, Queensland, aged ≥20 years for the years 1999–2013 based on case ascertainment from histopathology reports. Information systems for participating laboratories were scrutinised to identify histopathology reports that documented a diagnosis of amyloidosis. Case mortality status was determined via linkage to the National Death Index. A total of 447 cases of amyloidosis were identified, with a median age at diagnosis of 66 years. A plasma cell dyscrasia was identified in 72% of patients who had paraprotein studies performed. The estimated incidence for Queenslanders aged ≥20 years was 12·1 cases per million person years. The median survival was 2·45 years. Age at diagnosis, presence of a paraprotein, earlier year of diagnosis, and inner regional location of residence (compared with residence in a major city) were independently associated with reduced survival. Our data confirms previously reported incidence data for amyloidosis of approximately 10 cases per million patient years and indicates that survival for Queensland patients with amyloidosis is improving, though it remains poor for the elderly and patients with AL amyloidosis.
Salvage high‐intensity focused ultrasound for locally recurrent prostate cancer after low‐dose‐rate brachytherapy: oncological and functional outcomes
Authors: Hostiou Thomas , Gelet Albert , Chapelon Jean‐Yves , Rouvière Olivier , Mège‐Lechevalier Florence , Lafon Cyril , Tonoli‐Catez Hélène , Badet Lionel , Crouzet Sébastien 
Published: May 2019

ObjectivesTo evaluate the oncological and functional outcomes of salvage high‐intensity focused ultrasound (S‐HIFU) for locally recurrent prostate cancer after low‐dose‐rate (LDR) brachytherapy.Patients and MethodsClinical phase II studies (2003–2015) included 50 consecutive patients with post‐brachytherapy local recurrence treated by S‐HIFU. S‐HIFU was performed with post‐external beam radiotherapy (EBRT) parameters and, since 2008, with specific post‐brachytherapy parameters. Treatments were whole‐gland ablation and, since 2009, hemi‐ablation in cases of unilateral prostate cancer. The primary objective was to assess oncological outcomes: treatment failure‐free survival, progression‐free survival (PFS), overall survival (OS), cancer‐specific survival (CSS), and metastasis‐free survival (MFS) rates. The secondary objective was to evaluate adverse events, continence, and erectile function. Kaplan–Meier analysis estimated oncological outcomes.ResultsIn all, 13 patients were treated with post‐EBRT parameters, 37 with post‐brachytherapy parameters, 35 with whole‐gland treatment, and 15 with hemi‐ablation. The median follow‐up was 4.6 years. After S‐HIFU, the median prostate‐specific antigen level was 0.3 ng/mL. At 6 years, treatment failure‐free survival, PFS, OS, CSS, and MFS rates were 41%, 45%, 93%, 98%, and 80%, respectively. Post‐brachytherapy compared with post‐EBRT parameters reduced Grade 2–3 incontinence (34% vs 62%, P = 0.015). Incontinence, bladder outlet obstruction and Grade ≥III complications were significantly reduced with hemi‐ablation compared with whole‐gland treatment (14% vs 54%, P < 0.001; 13% vs 46%, P = 0.03; 13% vs 63%, P = 0.001; respectively). Before S‐HIFU, 25 patients had a five‐item version of the International Index of Erectile Function score of ≥17, which was maintained in 48% at 12 months.ConclusionS‐HIFU for locally recurrent prostate cancer after LDR brachytherapy is associated with favourable survival rates at a price of significant morbidity. Dedicated post‐brachytherapy parameters and hemi‐ablation improve the safety of the treatment.
An exploration of care‐burden experienced by older caregivers of adults with intellectual disabilities in Ireland
Authors: Egan Caroline , Dalton Caroline Teresa 
Published: May 2019

Accessible summaryOlder family carers of an adult with an Intellectual disability were asked about how they sometimes feel when supporting another person.Most carers felt a little stressed or strained.Older carers felt less stressed than younger carers.The value of supports that help carers was identified.AbstractBackgroundPeople with intellectual disabilities are experiencing increased longevity, and in parallel, their family caregivers are also ageing. The literature identifies that these caregivers are at risk of burden. The aim of this study was to measure the level of caregiver burden among older carers of adults with intellectual disabilities in an Irish sample and to analyse the effect of socio‐demographic factors upon experiences of caregiver burden.Materials and MethodsThirty caregivers completed a survey questionnaire. Data were collected based upon participants’ self‐reports of burden using the Zarit Burden Interview (ZBI) and a socio‐demographic questionnaire. Data were analysed using SPSS version 24.ResultsOver 57% of carers indicated a mild‐to‐moderate level of burden. Analysis indicated that younger caregivers experience significantly higher levels of burden, when compared to older caregivers.ConclusionsThis study contributes to our understanding of burden among an Irish population of older caregivers supporting an adult with an intellectual disability. It identified that carers do experience burden. The importance of proactive assessments and supports for these caregivers was revealed. This study highlights a lack of Irish research in this area and may pave the way for future research which could build upon its findings.
Deciphering the canonical blockade of activated Hageman factor (FXIIa) by benzamidine in the coagulation cascade: A thorough dynamical perspective
Authors: Salifu Elliasu Y. , Agoni Clement , Olotu Fisayo A. , Dokurugu Yussif M. , Soliman Mahmoud E. S. 
Published: May 2019

AbstractThe experimental inhibitory potency of benzamidine (BEN) paved way for further design and development of inhibitors that target β‐FXIIa. Structural dynamics of the loops and catalytic residues that encompass the binding pocket of β‐FXIIa and all serine proteases are crucial to their overall activity. Employing molecular dynamics and post‐MD analysis, this study sorts to unravel the structural and molecular events that accompany the inhibitory activity of BEN on human β‐FXIIa upon selective non‐covalent binding. Analysis of conformational dynamics of crucial loops revealed prominent alterations of the original conformational posture of FXIIa, evidenced by increased flexibility, decreased compactness, and an increased exposure to solvent upon binding of BEN, which could have in turn interfered with the essential roles of these loops in enhancing their procoagulation interactions with biological substrates and cofactors, altogether resulting in the consequential inactivation of FXIIa. A sustained interaction of the catalytic triad residues and key residues of the autolysis loop impeded their roles in catalysis which equally enhanced the inhibitory potency of BEN toward β‐FXIIa evidenced by a favorable binding. Findings provide essential structural and molecular insights that could facilitate the structure‐based design of novel antithrombotic compounds with enhanced inhibitory activities and low therapeutic risk.
The crosstalk between microbiome and asthma: Exploring associations and challenges
Authors: Abdel‐Aziz Mahmoud I. , Vijverberg Susanne J. H. , Neerincx Anne H. , Kraneveld Aletta D. , Maitland‐van der Zee Anke H. 
Published: May 2019

AbstractWith the advancement of high‐throughput DNA/RNA sequencing and computational analysis techniques, commensal bacteria are now considered almost as important as pathological ones. Understanding the interaction between these bacterial microbiota, host and asthma is crucial to reveal their role in asthma pathophysiology. Several airway and/or gut microbiome studies have shown associations between certain bacterial taxa and asthma. However, challenges remain before gained knowledge from these studies can be implemented into clinical practice, such as inconsistency between studies in choosing sampling compartments and/or sequencing approaches, variability of results in asthma studies, and not taking into account medication intake and diet composition especially when investigating gut microbiome. Overcoming those challenges will help to better understand the complex asthma disease process. The therapeutic potential of using pro‐ and prebiotics to prevent or reduce risk of asthma exacerbations requires further investigation. This review will focus on methodological issues regarding setting up a microbiome study, recent developments in asthma bacterial microbiome studies, challenges and future therapeutic potential.
Retracted: Increased maternal serum placental growth hormone variant in pregnancies complicated by otosclerosis
Authors: Liao Shutan , Lu Shan , Li Guangliang , Chen Ruiying 
Published: May 2019

AbstractObjectiveTo investigate the potential role of maternal serum concentrations of placental growth hormone variant (GH‐V), insulin‐like growth factor (IGF) 1 and 2, and IGF‐binding proteins (IGFBP) 1 and 3 in pregnancies complicated by otosclerosis.MethodOtosclerosis cases (n = 22) and age, ethnicity‐matched controls (n = 22) were selected in a nested case‐control study. Maternal serum hormone concentrations at 26 and 34 weeks of gestation were determined by enzyme‐linked immunosorbent assay (ELISA).ResultsConcentrations of all measured hormones except IGFBP‐1 were increased as gestation progressed. Maternal serum GH‐V concentrations at 26 and 34 weeks of gestation were significantly increased in the otosclerosis group, when compared to the control group (2.53 ± 0.17 ng/ml vs. 1.78 ± 0.19 ng/ml, P = 0.036; 4.34 ± 0.31 ng/ml vs. 3.12 ± 0.18 ng/ml, P < 0.001, respectively). Maternal serum IGF‐1 concentrations at 34 weeks in otosclerotic patients were significantly higher than in the controls (589.1 ± 21.4 ng/ml vs. 499.7 ± 17.8 ng/ml, P < 0.001). The increase of IGF‐1 was not observed at 26 weeks of gestation. Serum IGF‐2, IGFBP‐1 and IGFBP‐3 at either 26 or 34 weeks were unaltered between the two groups.ConclusionMaternal serum concentrations of GH‐V and IGF‐1 were altered in pregnancy complicated by otosclerosis, suggesting that the GH‐IGF axis may contribute to the development of this condition during pregnancy.
Loss of ZNF750 in ocular and cutaneous sebaceous carcinoma
Authors: North Jeffrey P. , Solomon David A. , Golovato Justin , Bloomer Michele , Benz Stephen C. , Cho Raymond J. 
Published: May 2019

AbstractBackgroundSebaceous carcinoma (SeC) is an uncommon malignancy arising from sebaceous glands of the conjunctiva and skin. Recurrent mutations in the ZNF750 were recently identified in ocular SeC. We assessed whether ZNF750 loss is a specific feature of ocular SeC or a general feature of sebaceous tumors.MethodsImmunostaining for ZNF750 expression was performed in 54 benign and malignant sebocytic proliferations. Staining for ZNF750 was scored on a three‐tier scale: positive (>75%), partially positive (5%‐74%), and negative (<5%).ResultsZNF750 expression was negative in 4/11 ocular SeC, and partially positive in 4/11 ocular SeC and 6/13 cutaneous SeC. No extraocular tumors were negative. No loss was found in sebaceous adenoma or sebaceous hyperplasia. In nine previously sequenced ocular SeCs, two lacked detectable somatic mutations in ZNF750, but showed complete loss of staining, indicating non‐mutational inactivation of ZNF750.ConclusionWe show complete loss of the ZNF750 epidermal differentiation regulator in about half of ocular SeC, highlighting the most common genetic defect in this cancer type. Loss of ZNF750 expression is seen even in tumors without truncating mutations and reduced in many of the remaining ocular and cutaneous SeC. In contrast, no ZNF750 loss was detected in benign sebaceous proliferations.
Review of genodermatoses with characteristic histopathology and potential diagnostic delay
Authors: Ko Christine J. , Atzmony Lihi , Lim Young , McNiff Jennifer M. , Craiglow Brittany G. , Antaya Richard J. , Choate Keith A. 
Published: May 2019

AbstractAdvances in human genetics have enabled discovery of new genes for inherited skin diseases and cutaneous malformations as well as refined categorization of genodermatoses. Careful phenotyping has been central to genetic discoveries, and it provides critical clues for clinical diagnoses, particularly when the skin disorder is not congenital. This article will review several lesser‐known genodermatoses that often present after infancy with recognizable histopathologic features.
Two‐year analysis of dermatological out‐of‐hours consultations at Frankfurt University Hospital
Authors: Osowski Sebastian A. , Wagner Niklas , Osowski Andreas C. , Ochsendorf Falk , Kaufmann Roland , Weberschock Tobias 
Published: May 2019

SummaryBackgroundIn recent years, emergency consultations have become more common in all medical disciplines. In Germany, dermatological out‐of‐hours consultations are handled by emergency practices, emergency departments and tertiary care providers. Little information is available on the reasons for these dermatological consultations.ObjectivesThe aim of this study was to analyze patient characteristics, diagnoses and admission rates resulting from these consultations.MethodsWe conducted a retrospective study covering two years of out‐of‐hours consultations at a dermatological tertiary referral center.ResultsA total of 3635 patients presented at the referral center. The most frequent outpatient diagnoses were acute urticaria (13.8 %) and bacterial infections (12.3 %). 83 % of the outpatient diagnoses required the most advanced competence level according to the new German curriculum for undergraduate education of medical students. 405 (11.01 %) patients did not require dermatological treatment, and 430 patients (13.6 %) were admitted to hospital. Most admissions were due to bacterial infections and herpes zoster. Advanced age, pain and fever were associated with a relatively high risk of admission.ConclusionsAdmission rates at the dermatological tertiary referral center were substantially lower than at interdisciplinary emergency departments. A few diagnoses accounted for more than half of all consultations. These diagnoses are well represented within the new German curriculum.
Two‐dimensional strain echocardiography‐derived left ventricular ejection fraction, volumes, and global systolic dyssynchrony index: Comparison with three‐dimensional echocardiography
Authors: Vaidya Gaurang Nandkishor , Salgado Benjamin C. , Badar Faraz , John Anub , Stoddard Marcus F. 
Published: May 2019

AbstractBackgroundThree‐dimensional (3D) echocardiography is the most accurate echocardiographic method for ventricular chamber quantification. It is unclear how two‐dimensional (2D) techniques perform against 3D technology and whether 2D methods can be extrapolated to obtain 3D data.MethodsRetrospective review of transthoracic echocardiography was performed, with comparison of ejection fraction (EF), end‐diastolic volume (EDV), end‐systolic volume (ESV), and 2D strain‐derived global longitudinal strain (GLS) and synchrony index.ResultsOne‐hundred patients were identified. Using 3D echocardiography as reference standard, good correlation was noted with 2D strain‐derived EF (r = 0.89, P < 0.01) and with 2D standard biplane EF (r = 0.90, P < 0.01) and similarly for EDV (r = 0.84 and r = 0.81, respectively, both P < 0.01). Two‐dimensional strain‐derived EDV by 8% and 2D biplane‐derived EDV underestimated by 8% (P < 0.01). In relation to 3D EF, 2D strain underestimated by 2% and 2D standard biplane overestimated by 2% (P < 0.01). There was a negative correlation between GLS and 3D EF (r = 0.84, P = 0.001). On multivariate analysis, 3D EF could be derived from 2D strain [3D EF = 34.345 + (0.125 * EDV) + (−0.289 * ESV) + (−1.141 * GLS)]. Three‐dimensional echocardiography‐derived synchrony parameter (ie, standard deviation from mean time to minimum systolic volume from 16 subvolumes) did not correlate with 2D strain‐derived synchrony index (r = 0.171).ConclusionsTwo‐dimensional standard biplane and 2D strain EF and EDV strongly correlate with 3D EF and EDV. Although 2D methods are predictive of 3D findings, over‐ and underestimations may occur. Three‐dimensional echocardiography should be used when available.
Mechanisms, pathophysiology, and diagnostic imaging of left ventricular outflow tract obstruction following mitral valve surgery and transcatheter mitral valve replacement
Authors: Silbiger Jeffrey J. , Lee Samantha , Christia Panagiota , Perk Gila 
Published: May 2019

AbstractLeft ventricular outflow tract obstruction is a serious complication of mitral valve surgery (repair and replacement) and transcatheter mitral valve replacement. An appreciation of the various mechanisms which cause outflow obstruction in these settings is critical to avoiding this complication and to initiating appropriate treatment. This article discusses the mechanisms, pathophysiology, and imaging of left ventricular outflow tract obstruction which can arise following insertion of a variety of mitral valve prosthetics.
Elastic aortic properties in cystic fibrosis adults without cardiovascular risk factors: A case‐control study
Authors: Vizzardi Enrico , Sciatti Edoardo , Bonadei Ivano , Menotti Elisa , Prati Francesco , Scodro Marta , Dallapellegrina Lucia , Berlendis Marialma , Poli Piercarlo , Padoan Rita , Metra Marco 
Published: May 2019

AbstractIntroductionThe presence of CFTR in smooth muscle and endothelial cells, systemic inflammation, and oxidative stress could explain vascular alterations in cystic fibrosis. Aortic elastic properties are determinants of left ventricular function by means of ventriculo‐arterial coupling and indicators of cardiovascular risk.ObjectivesThe purpose of the present study was to compare clinically stable patients affected by cystic fibrosis without overt pulmonary hypertension with controls to evaluate aortic tissue Doppler elastic properties, such as distensibility, stiffness, and strain.MethodsA total of 22 adults affected by cystic fibrosis, and 24 healthy volunteers matched for age and sex were enrolled. None had known cardiovascular risk factors, secondary diabetes, neither aortic stenosis nor regurgitation. All people underwent blood pressure measurement and transthoracic echocardiography.ResultsAortic diameter measured at Valsalva sinuses was significantly higher in patients with cystic fibrosis than healthy people, median 32.0 (interquartile range 29.8–35.0) vs 24.3 (22.2–30.0) mm; P < 0.001. Aortic distensibility was significantly lower among patients than controls, being 2.4 (1.3–3.3) vs 5.6 (3.4–8.3) per mm Hg (P < 0.001), while stiffness higher, 7.7 (6.0–14.8) vs 3.7 (2.9–6.7); P < 0.001. Finally, M‐mode strain of ascending aorta was lower in patients, 4.1 (3.4–7.3)% than in controls, 13.4 (7.7–19.4)%; P < 0.001.ConclusionFor the first time in humans, we demonstrated subclinical alterations in aortic elastic properties in young adults affected by cystic fibrosis without pulmonary hypertension or secondary diabetes. This phenomenon could influence left ventricular function earlier by means of ventriculo‐arterial coupling and may be a tool to identify patients who benefit from a closer follow‐up.
Successful biventricular cardiac resynchronization therapy in a failing Fontan patient: Implications of ventriculo‐ventricular interdependency in single ventricle physiology
Authors: Udink ten Cate Floris E. A. , Germund Ingo , Goossens Stijn S. , Bennink Gerardus , Sreeram Narayanswami 
Published: May 2019

AbstractWe describe a Fontan patient with severe heart failure who was successfully treated with biventricular cardiac resynchronization therapy (CRT). Our case shows that strain imaging might play a crucial role in guiding placement of pacing leads and in characterizing the electromechanical substrate associated with a favorable CRT response. Furthermore, we demonstrate for the first time that ventriculo‐ventricular interdependency seems an important mechanical concept, which can be utilized to augment cardiac performance in failing Fontan patients with a functional hypoplastic ventricle.
Comparative responses of termite functional and taxonomic diversity to land‐use change
Authors: Liu Shengjie , Lin Xiaobing , Behm Jocelyn E. , Yuan Hao , Stiblik Petr , Šobotník Jan , Gan Jianmin , Xia Shangwen , Yang Xiaodong 
Published: May 2019

1. While it is clear that land‐use change significantly impacts the taxonomic dimension of soil biodiversity, how the functional dimension responds to land‐use change is less well understood.2. This study examined how the transformation of primary forests into rubber tree monocultures impacts individual termite species and how this change is reflected in termite taxonomic and functional α‐diversity (within site) and β‐diversity (among sites).3. Overall, individual species responded strongly to land‐use change, whereby only 11 of the 27 species found were able to tolerate both habitats. These differences caused a 27% reduction in termite taxonomic richness and reduced taxonomic β‐diversity in rubber plantations compared with primary forests. The study also revealed that the forest conversion led to a shift in some termite species with smaller body size, shorter legs and smaller mandibular traits. Primary forests exhibited higher functional richness and functional β‐diversity of termite species, indicating that functional traits of termite species in rubber plantations are more evenly distributed.4. The present study suggests that forest conversion does not merely decrease taxonomic diversity of termites, but also exerts functional trait filtering within some termite species. The results affirm the need for biodiversity assessments that combine taxonomic and functional indicators when monitoring the impact of land‐use change.
When and where plant‐soil feedback may promote plant coexistence: a meta‐analysis
Authors: Crawford Kerri M. , Bauer Jonathan T. , Comita Liza S. , Eppinga Maarten B. , Johnson Daniel J. , Mangan Scott A. , Queenborough Simon A. , Strand Allan E. , Suding Katharine N. , Umbanhowar James , Bever James D. 
Published: May 2019

AbstractPlant‐soil feedback (PSF) theory provides a powerful framework for understanding plant dynamics by integrating growth assays into predictions of whether soil communities stabilise plant–plant interactions. However, we lack a comprehensive view of the likelihood of feedback‐driven coexistence, partly because of a failure to analyse pairwise PSF, the metric directly linked to plant species coexistence. Here, we determine the relative importance of plant evolutionary history, traits, and environmental factors for coexistence through PSF using a meta‐analysis of 1038 pairwise PSF measures. Consistent with eco‐evolutionary predictions, feedback is more likely to mediate coexistence for pairs of plant species (1) associating with similar guilds of mycorrhizal fungi, (2) of increasing phylogenetic distance, and (3) interacting with native microbes. We also found evidence for a primary role of pathogens in feedback‐mediated coexistence. By combining results over several independent studies, our results confirm that PSF may play a key role in plant species coexistence, species invasion, and the phylogenetic diversification of plant communities.