cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.5129023
title: Supplementary Material for: Integration of Intention and Outcome for Moral Judgment in Frontotemporal Dementia: Brain Structural Signatures

description: <b><i>Background:</i></b> Moral judgment has been proposed to rely on a distributed brain network. This function is impaired in behavioral variant frontotemporal dementia (bvFTD), a condition involving damage to some regions of this network. However, no studies have investigated moral judgment in bvFTD via structural neuroimaging. <b><i>Methods:</i></b> We compared the performance of 21 bvFTD patients and 19 controls on a moral judgment task involving scenarios that discriminate between the contributions of intentions and outcomes. Voxel-based morphometry was used to assess (a) the atrophy pattern in bvFTD patients, (b) associations between gray matter (GM) volume and moral judgments, and (c) structural differences between bvFTD subgroups (patients with relatively preserved moral judgment and patients with severer moral judgment impairments). <b><i>Results:</i></b> Patients judged attempted harm as more permissible and accidental harm as less permissible than controls. The groups' performance on accidental harm was associated with GM volume in the precuneus. In controls, it was al- so associated with the ventromedial prefrontal cortex (VMPFC). Also, both groups' performance on attempted harm was associated with GM volume in the temporoparietal junction. Patients exhibiting worse performance displayed smaller GM volumes in the precuneus and temporal pole. <b><i>Conclusions:</i></b> Results suggest that moral judgment abnormalities in bvFTD are associated with impaired integration of intentions and outcomes, which depends on an extended brain network. In bvFTD, moral judgment seems to critically depend on areas beyond the VMPFC.
DOI: 10.6084/m9.figshare.5129011
title: Supplementary Material for: The Sustained-Exposure Dexamethasone Formulation OTO-104 Offers Effective Protection against Noise-Induced Hearing Loss

description: The otoprotective effects of OTO-104 were investigated both prior to and following acute acoustic trauma. Guinea pigs received a single intratympanic injection of OTO-104 and were assessed in a model of acute acoustic trauma. Doses of at least 2.0% OTO-104 offered significant protection against hearing loss induced by noise exposure when administered 1 day prior to trauma and up to 3 days thereafter. Otoprotection remained effective even with higher degrees of trauma. In contrast, the administration of a dexamethasone sodium phosphate solution did not protect against noise-induced hearing loss. Activation of the classical nuclear glucocorticoid and mineralocorticoid receptor pathways was required for otoprotection by OTO-104. The sustained exposure properties of OTO-104 were also superior to a steroid solution.
DOI: 10.6084/m9.figshare.5129014
title: Supplementary Material for: The Sustained-Exposure Dexamethasone Formulation OTO-104 Offers Effective Protection against Cisplatin-Induced Hearing Loss

description: The otoprotective effects of OTO-104 were investigated following both acute and chronic administration of cisplatin. The acute administration of cisplatin to guinea pigs resulted in profound hearing loss (70-80 dB SPL) across all frequencies tested. A single intratympanic injection of 6% OTO-104, but not of lower doses, almost completely protected against cisplatin ototoxicity. In contrast, a dexamethasone solution administered under the same experimental conditions offered no otoprotection. OTO-104 was also very effective in protecting against the progressive hearing loss observed with the chronic administration of cisplatin (3 injections at a weekly interval). The otoprotection was found to be dependent upon the activation of dexamethasone-dependent classical nuclear receptor pathways.
DOI: 10.6084/m9.figshare.5129023.v1
title: Supplementary Material for: Integration of Intention and Outcome for Moral Judgment in Frontotemporal Dementia: Brain Structural Signatures

description: <b><i>Background:</i></b> Moral judgment has been proposed to rely on a distributed brain network. This function is impaired in behavioral variant frontotemporal dementia (bvFTD), a condition involving damage to some regions of this network. However, no studies have investigated moral judgment in bvFTD via structural neuroimaging. <b><i>Methods:</i></b> We compared the performance of 21 bvFTD patients and 19 controls on a moral judgment task involving scenarios that discriminate between the contributions of intentions and outcomes. Voxel-based morphometry was used to assess (a) the atrophy pattern in bvFTD patients, (b) associations between gray matter (GM) volume and moral judgments, and (c) structural differences between bvFTD subgroups (patients with relatively preserved moral judgment and patients with severer moral judgment impairments). <b><i>Results:</i></b> Patients judged attempted harm as more permissible and accidental harm as less permissible than controls. The groups' performance on accidental harm was associated with GM volume in the precuneus. In controls, it was al- so associated with the ventromedial prefrontal cortex (VMPFC). Also, both groups' performance on attempted harm was associated with GM volume in the temporoparietal junction. Patients exhibiting worse performance displayed smaller GM volumes in the precuneus and temporal pole. <b><i>Conclusions:</i></b> Results suggest that moral judgment abnormalities in bvFTD are associated with impaired integration of intentions and outcomes, which depends on an extended brain network. In bvFTD, moral judgment seems to critically depend on areas beyond the VMPFC.
DOI: 10.6084/m9.figshare.5129005
title: Supplementary Material for: Measures to Assure that Ebola Guidelines Are Correctly Applied

description: <b><i>Objective:</i></b> The purpose of this study was to assure the application, in our primary care health district, of the World Health Organisation and Spanish Ebola virus disease (EVD) guidelines, and to identify and resolve gaps, in order to protect professionals from the risks associated with attending to patients with Ebola. <b><i>Materials and Methods:</i></b> A checklist was drawn up and an audit procedure of the structural, functional and operational conditions was designed for managing a self-presenting patient suspected with EVD in general practice. This audit was applied twice (before and after assessment) in 9 health centres of a health district in Spain. The checklist was structured according to 9 categories (51 elements in total). This tool was designed to avoid errors in the management of EVD patients that are considered very serious in view of their potential consequences. <b><i>Results:</i></b> Of the 51 elements on the checklist, 7-26 (14-51%) required direct intervention to rectify deficits in the first audit. The following elements requiring improvement were identified: the incorporation of memory aids to ensure the correct application of the EVD protocol, defining what information should be provided to the patient and (where applicable) anyone accompanying them as well as incorporating advice on how to deal with any individuals with whom they might have come into contact. A second audit assured the adequacy of the proposed solutions. <b><i>Conclusion:</i></b> In this study, the tool achieved the successful application of EVD protocol, assuring that primary care centres were able to handle an Ebola suspect patient safely.
DOI: 10.6084/m9.figshare.5129002
title: Supplementary Material for: Reproductive Outcome of Infertile Patients with Fibroids Based on the Patient and Fibroid Characteristics; Optimal and Personalized Management

description: <b><i>Aims:</i></b> To analyze the detailed clinical course of infertile patients with uterine fibroids and to identify optimal and personalized treatment based on the patient or fibroid characteristics. <b><i>Methods:</i></b> Retrospective analysis of a case series was performed on 176 infertile patients with fibroids. The patients were classified into different groups according to different treatments (conservative infertility treatment, myomectomy and non-myomectomy surgery). Patient or fibroid characteristics for different groups were analyzed for a possible correlation with the reproductive outcome. <b><i>Results:</i></b> The cumulative pregnancy rates by conservative treatment plateaued in 1 year. Myomectomy improved the reproductive outcome in patients who did not conceive with conservative infertility treatments. The most important determinant of the reproductive outcome in patients by conservative treatment prior to surgery was a past patient history of pregnancy. The most important determinant of the reproductive outcome after myomectomy was patient age. <b><i>Conclusion:</i></b> Myomectomy should be considered when infertile patients with fibroids do not conceive within 1 year of conservative infertility treatments. The most important determinant of reproductive outcome after myomectomy is patient age. Therefore, for patients younger than 40, the treatment schedule should be carefully considered so that the patients can sufficiently benefit from myomectomy and assisted reproductive technology.
DOI: 10.6084/m9.figshare.5129008
title: Supplementary Material for: Liver Resection for Metastatic Disease; A Population-Based Analysis of Trends

description: <b><i>Objective:</i></b> The study aims to evaluate all patients who underwent liver resection for metastatic disease for demographics, characteristics of the primary tumor and metastasis, volume of liver resection specimens per pathology laboratory and to describe trends in surgical treatment. <b><i>Methods:</i></b> Data were prospectively collected using the Dutch nationwide pathology network. All pathology reports containing details on liver resections for metastatic disease between January 2001 and December 2010 were evaluated. <b><i>Results:</i></b> A total of 3,916 liver resections were performed in 3,699 patients with a median age of 63 years (range 1-91). The primary tumor was mainly colorectal (n = 3,256; 88.0%). The number of ‘high volume liver centers' increased from 2 to 12 in the study period, whereas the number of ‘low volume centers' decreased. The number of liver resections increased from 224 to 596 per year (p ≤ 0.0001). A significant increase was demonstrated in elderly patients, patients with multiple metastases, liver resections for smaller metastases and minor liver resections. <b><i>Conclusion:</i></b> Although the majority of patients were young and had solitary metastasis, indications for liver resection are expanding as indicated by increasing numbers of elderly and patients with multiple liver metastases. Patients with non-colorectal liver metastases were seldom candidates for resection.
DOI: 10.6084/m9.figshare.5128999
title: Supplementary Material for: Novel Nonsense Mutation in the <b><i>NLRP7</i></b> Gene Associated with Recurrent Hydatidiform Mole

description: <b><i>Aim:</i></b> This study aimed to clarify the genetic and epigenetic features of recurrent hydatidiform mole (RHM) in Japanese patients. <b><i>Methods:</i></b> Four Japanese isolated RHM cases were analyzed using whole-exome sequencing. Villi from RHMs were collected by laser microdissection for genotyping and DNA methylation assay of differentially methylated regions (DMRs). Single nucleotide polymorphisms of <i>PEG3</i> and <i>H19</i> DMRs were used to confirm the parental origin of the variants. <b><i>Results:</i></b> A novel homozygous nonsense mutation in <i>NLRP7</i> (c.584G&gt;A; p.W195X) was identified in 1 patient. Genotyping of one of her molar tissue revealed that it was biparental but not androgenetic in origin. Despite the fact that the RHM is biparental, maternally methylated DMRs of <i>PEG3</i>, <i>SNRPN</i> and <i>PEG10</i> showed complete loss of DNA methylation. A paternally methylated DMR of <i>H19</i> retained normal methylation. <b><i>Conclusions:</i></b> This is the first Japanese case of RHM with a novel homozygous nonsense <i>NLRP7</i> mutation and a specific loss of maternal DNA methylation of DMRs. Notably, the mutation was identified in an isolated case of an ethnic background that has not previously been studied in this context. Our data underscore the involvement of <i>NLRP7</i> in RHM pathophysiology and confirm that DNA methylation of specific regions is critical.
DOI: 10.6084/m9.figshare.5129005.v1
title: Supplementary Material for: Measures to Assure that Ebola Guidelines Are Correctly Applied

description: <b><i>Objective:</i></b> The purpose of this study was to assure the application, in our primary care health district, of the World Health Organisation and Spanish Ebola virus disease (EVD) guidelines, and to identify and resolve gaps, in order to protect professionals from the risks associated with attending to patients with Ebola. <b><i>Materials and Methods:</i></b> A checklist was drawn up and an audit procedure of the structural, functional and operational conditions was designed for managing a self-presenting patient suspected with EVD in general practice. This audit was applied twice (before and after assessment) in 9 health centres of a health district in Spain. The checklist was structured according to 9 categories (51 elements in total). This tool was designed to avoid errors in the management of EVD patients that are considered very serious in view of their potential consequences. <b><i>Results:</i></b> Of the 51 elements on the checklist, 7-26 (14-51%) required direct intervention to rectify deficits in the first audit. The following elements requiring improvement were identified: the incorporation of memory aids to ensure the correct application of the EVD protocol, defining what information should be provided to the patient and (where applicable) anyone accompanying them as well as incorporating advice on how to deal with any individuals with whom they might have come into contact. A second audit assured the adequacy of the proposed solutions. <b><i>Conclusion:</i></b> In this study, the tool achieved the successful application of EVD protocol, assuring that primary care centres were able to handle an Ebola suspect patient safely.
DOI: 10.6084/m9.figshare.5129008.v1
title: Supplementary Material for: Liver Resection for Metastatic Disease; A Population-Based Analysis of Trends

description: <b><i>Objective:</i></b> The study aims to evaluate all patients who underwent liver resection for metastatic disease for demographics, characteristics of the primary tumor and metastasis, volume of liver resection specimens per pathology laboratory and to describe trends in surgical treatment. <b><i>Methods:</i></b> Data were prospectively collected using the Dutch nationwide pathology network. All pathology reports containing details on liver resections for metastatic disease between January 2001 and December 2010 were evaluated. <b><i>Results:</i></b> A total of 3,916 liver resections were performed in 3,699 patients with a median age of 63 years (range 1-91). The primary tumor was mainly colorectal (n = 3,256; 88.0%). The number of ‘high volume liver centers' increased from 2 to 12 in the study period, whereas the number of ‘low volume centers' decreased. The number of liver resections increased from 224 to 596 per year (p ≤ 0.0001). A significant increase was demonstrated in elderly patients, patients with multiple metastases, liver resections for smaller metastases and minor liver resections. <b><i>Conclusion:</i></b> Although the majority of patients were young and had solitary metastasis, indications for liver resection are expanding as indicated by increasing numbers of elderly and patients with multiple liver metastases. Patients with non-colorectal liver metastases were seldom candidates for resection.
DOI: 10.6084/m9.figshare.5128993
title: Supplementary Material for: Orbital Extension of an Unsuspected Choroidal Melanoma Presumably through an Aqueous Tube Shunt

description: <b><i>Aims:</i></b> To report a patient with unrecognized small macular melanoma, who gradually developed neovascular glaucoma that was treated with an Ahmed valve. We presume that tumor cells gained access to the orbit through this aqueous drainage device. <b><i>Methods:</i></b> The medical records pertaining to 5 different centers, the results of imaging studies, the clinical course of the patient and histopathological findings were retrospectively reviewed. <b><i>Results:</i></b> A 61-year-old man presented with a blind and painful proptotic right eye. Retinal hemorrhage had been suspected 3 years earlier and followed accordingly thereafter. Yearly performed MRI studies demonstrated the steady growth of the hemorrhagic lesion/tumor. Meanwhile, he developed neovascular glaucoma first treated unsuccessfully with cyclocryotherapy then with implantation of an Ahmed valve. Right proptosis became evident within a year, and a final MRI study revealed a large retrobulbar mass. Orbital exenteration was performed and histopathological examination showed intraocular and orbital epithelioid cell melanoma. Tumor cells were also found within the reservoir of the Ahmed valve. The patient died of widespread metastases 1 year later. <b><i>Conclusions:</i></b> Ahmed valve implantation in an eye with unsuspected macular choroidal melanoma may ultimately be associated with extraocular extension and orbital tumor formation.
DOI: 10.6084/m9.figshare.5128996
title: Supplementary Material for: Factors Associated with Tremor Changes during Sedation with Dexmedetomidine in Parkinson's Disease Surgery

description: <b><i>Introduction:</i></b> Dexmedetomidine is an α<sub>2</sub>-agonist recently proposed as a potentially ideal drug for sedation during the surgical treatment of Parkinsonʼs disease (PD). This report documents the incidence of changes in motor symptoms (especially tremor) in PD patients sedated with dexmedetomidine for deep brain stimulation or ablation procedures. <b><i>Methods:</i></b> We reviewed a retrospective cohort of 22 patients who underwent surgery for PD with dexmedetomidine sedation at a single institution from 2010 to 2014. A logistic regression analysis was performed to analyze possible confounding factors. <b><i>Results:</i></b> 14 cases of tremor reduction or suppression were recorded (cumulative incidence: 63.6%; 95% CI: 40.7-82.8). No association could be identified between loading dose, β-blocker use and preoperative total Unified Parkinson's Disease Rating Scale III, with tremor changes. The maintenance dose of dexmedetomidine was higher in patients who did not experience changes [median and range for patients with and without tremor alteration 0.75 (0.2-1.0) and 1.0 µg × kg<sup>-1</sup> × h<sup>-1</sup> (0.7-1.4), respectively; p = 0.021]. <b><i>Conclusion:</i></b> Dexmedetomidine provides adequate sedation during surgery for PD, but it might affect motor signs making intraoperative testing difficult or even impossible. Dosage appears not to be the determining factor in motor changes, whose cause remains unclear.
DOI: 10.6084/m9.figshare.5128990
title: Supplementary Material for: The Influence of Patient Characteristics on Anticholinergic Events in Older People

description: <b><i>Aims:</i></b> To examine patient characteristics that predict adverse anticholinergic-type events in older people. <b><i>Methods:</i></b> This retrospective population-level study included 2,248 hospitalised patients. Individual data on medicines that are commonly associated with anticholinergic events (delirium, constipation and urinary retention) were identified. Patient characteristics examined were medicines with anticholinergic effects (ACh burden), age, sex, non-anticholinergic medicines (non-ACM), Charlson comorbidity index scores and ethnicity. The Akaike information criterion was used for model selection. The data were analysed using logistic regression models for anticholinergic events using the software NONMEM. <b><i>Results:</i></b> ACh burden was found to be a significant independent predictor for developing an anticholinergic event [adjusted odds ratio (aOR): 3.21, 95% CI: 1.23-5.81] for those taking an average of 5 anticholinergic medicines compared to those taking 1. Both non-ACM and age were also independent risk factors (aOR: 1.41, 95% CI: 1.31-1.51 and aOR: 1.08, 95% CI: 1.05-1.10, respectively). <b><i>Conclusion:</i></b> To our knowledge, this is the first study that has examined population-level data in a nonlinear model framework to predict anticholinergic-type adverse events. This study evaluated the relationship between important patient characteristics and the occurrence of anticholinergic-type events. These findings reinforce the clinical significance of reviewing anticholinergic medicines in older people.
DOI: 10.6084/m9.figshare.5128993.v1
title: Supplementary Material for: Orbital Extension of an Unsuspected Choroidal Melanoma Presumably through an Aqueous Tube Shunt

description: <b><i>Aims:</i></b> To report a patient with unrecognized small macular melanoma, who gradually developed neovascular glaucoma that was treated with an Ahmed valve. We presume that tumor cells gained access to the orbit through this aqueous drainage device. <b><i>Methods:</i></b> The medical records pertaining to 5 different centers, the results of imaging studies, the clinical course of the patient and histopathological findings were retrospectively reviewed. <b><i>Results:</i></b> A 61-year-old man presented with a blind and painful proptotic right eye. Retinal hemorrhage had been suspected 3 years earlier and followed accordingly thereafter. Yearly performed MRI studies demonstrated the steady growth of the hemorrhagic lesion/tumor. Meanwhile, he developed neovascular glaucoma first treated unsuccessfully with cyclocryotherapy then with implantation of an Ahmed valve. Right proptosis became evident within a year, and a final MRI study revealed a large retrobulbar mass. Orbital exenteration was performed and histopathological examination showed intraocular and orbital epithelioid cell melanoma. Tumor cells were also found within the reservoir of the Ahmed valve. The patient died of widespread metastases 1 year later. <b><i>Conclusions:</i></b> Ahmed valve implantation in an eye with unsuspected macular choroidal melanoma may ultimately be associated with extraocular extension and orbital tumor formation.
DOI: 10.6084/m9.figshare.5128987
title: Supplementary Material for: Current Status of the Management of Hereditary Breast and Ovarian Cancer in Asia: First Report by the Asian BRCA Consortium

description: <b><i>Background:</i></b><i>BRCA1/BRCA2</i> mutations are associated with an increased lifetime risk for hereditary breast and ovarian cancer (HBOC). Compared with the Western developed countries, genetic testing and risk assessment for HBOC in Asia are less available, thus prohibiting the appropriate surveillance, clinical strategies and cancer management. <b><i>Methods:</i></b> The current status of HBOC management in 14 Asian countries, including genetic counselling/testing uptakes and clinical management options, was reviewed. We analysed how economic factors, healthcare and legal frameworks, and cultural issues affect the genetic service availability in Asia. <b><i>Results:</i></b> In 2012, only an estimated 4,000 breast cancer cases from 14 Asian countries have benefited from genetic services. Genetic testing costs and the absence of their adoption into national healthcare systems are the main economic barriers for approaching genetic services. Training programmes, regional accredited laboratories and healthcare professionals are not readily available in most of the studied countries. A lack of legal frameworks against genetic discrimination and a lack of public awareness of cancer risk assessment also provide challenges to HBOC management in Asia. <b><i>Conclusions:</i></b> The Asian BRCA Consortium reports the current disparities in genetic services for HBOC in Asia and urges the policy makers, healthcare sectors and researchers to address the limitations in HBOC management.