cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.23626833
title: Supplementary Material for: Deep Brain Stimulation for Pediatric Dystonia: Clinicians’ Perspectives on the Most Pressing Ethical Challenges

description: INTRODUCTION. Pediatric deep brain stimulation (pDBS) is commonly used to manage treatment-resistant primary dystonias with favorable results, and more frequently used for secondary dystonia to improve quality of life. There has been little systematic empirical neuroethics research to identify ethical challenges and potential solutions to ensure responsible use of DBS in pediatric populations. METHODS. Clinicians (n=29) who care for minors with treatment-resistant dystonia were interviewed for their perspectives on the most pressing ethical issues in pediatric DBS. RESULTS: Using thematic content analysis to explore salient themes, clinicians identified four pressing concerns: 1) uncertainty about risks and benefits of pDBS (22/29; 72%) that poses a challenge to informed decision making; 2) ethically navigating decision-making roles (15/29; 52%), including how best to integrate perspectives from diverse stakeholders (patient, caregiver, clinician) and how to manage surrogate decisions on behalf of pediatric patients with limited capacity to make autonomous decisions; 3) information scarcity effects on informed consent and decision quality (15/29; 52%) in the context of patient and caregivers’ expectations for treatment; and 4) narrow regulatory status and access (7/29; 24%) such as the lack of FDA approved indications that contribute to decision-making uncertainty and liability and potentially limit access to DBS among patients who may benefit from it. CONCLUSION. These results suggest that clinicians are primarily concerned about ethical limitations of making difficult decisions in the absence of informational, regulatory and financial supports. We discuss two solutions already underway, including supported decision making to address uncertainty, and further data sharing to enhance clinical knowledge and discovery.
DOI: 10.6084/m9.figshare.23684169
title: Supplementary Material for: A Rare Case of Direct Spontaneous Carotid-Cavernous Fistula In a 6-month-old Infant and Review of literature

description: Direct or Type A CCFs are the direct connection between the cavernous segment of the Internal carotid artery and the cavernous sinus. While most direct CCFs are caused by trauma, spontaneous direct CCFs are extremely rare in infants. We herein report a case of a 6-month-old child who came to our OPD with bulging in the right eye since 20 days after birth. On examination, there was right eye abduction limitation with no deviation, associated with proptosis. Bruit was present on auscultation. CEMRI showed an enlarged right cavernous sinus with dilatation of the superior ophthalmic vein suggestive of a carotid-cavernous fistula. The patient was referred to advance center where he was advised patch therapy to prevent amblyopia was kept under observation by neurosurgeon until the age of 3 years, after which he was scheduled to undergo trans arterial coiling.
DOI: 10.6084/m9.figshare.23694570
title: Supplementary Material for: Hypertensive Retinopathy as a presenting sign of pheochromocytoma with malignant hypertension: a child case

description: A 13-year-old was admitted to our clinic complaining about a vision loss of over two weeks. Bilateral optic disc edema, peripapillary flame-shaped hemorrhages, macular star pattern exudates and cotton wool spots were found in fundoscopic examination. The OCT exam showed bilaterally serous retinal detachments in sub-foveal region with intraretinal exudates. A pediatric examination found a high systemic hypertension of 230/140 mmHg and laboratory tests revealed increased levels of plasmatic and urinary catecholamines. An abdominal MRI showed a left suprarenal pheochromocytoma and the child underwent urgently an adrenalectomy. Informed patient consent was obtained from legal guardians to publish clinical images.Malignant Hypertension (MHT) as a result of a pheochromocytoma is an extremely rare condition in childhood. MHT crisis represents a potential life-threatening event and an immediate treatment can prevent a multi-organ failure, including the eyes. An early diagnosis of MHT by fundus examination can lead to a completely favorable ophthalmological evolution and entirely functional recovery.
DOI: 10.6084/m9.figshare.23798688
title: Supplementary Material for: Left Main Coronary Artery Revascularization in Patients with Impaired Renal Function; Percutaneous Coronary Intervention vs. Coronary Artery Bypass Grafting

description: Introduction: The evidence about the optimal revascularization strategy in patients with left-main coronary artery (LMCA) disease and impaired renal function is limited. Thus, we aimed to compare the outcomes of LMCA disease revascularization (PCI vs. CABG) in patients with and without impaired renal function. Methods: This retrospective cohort study included 2138 patients recruited from 14 centers between 2015 to 2019. We compared patients with impaired renal function who had PCI (n= 316) to those who had CABG (n= 121) and compared patients with normal renal function who had PCI (n= 906) to those who had CABG (n= 795). The study outcomes were in-hospital and follow-up major adverse cardiovascular and cerebrovascular events (MACCE). Results: Multivariable logistic regression analysis showed that the risk of in-hospital MACCE was significantly higher in CABG compared to PCI in patients with impaired renal function [OR: 8.13 (95% CI: 4.19- 15.76), P&lt;0.001] and normal renal function [OR: 2.59 (95% CI: 1.79- 3.73); P&lt;0.001]. There were no differences in follow-up MACCE between CABG and PCI in patients with impaired renal function [HR: 1.14 (95% CI: 0.71- 1.81), P= 0.585] and normal renal function [HR: 1.12 (0.90- 1.39), P= 0.312]. Conclusions: PCI could have an advantage over CABG in revascularization of LMCA disease in patients with impaired renal function regarding in-hospital MACCE. The follow-up MACCE was comparable between PCI and CABG in patients with impaired and normal renal function.
DOI: 10.6084/m9.figshare.23807337
title: Supplementary Material for: Optic Neuritis Following Second Administration of COVID-19 Vaccine

description: A 28-year-old woman presented to eye casualty with signs and symptoms suggestive of optic neuritis following a recent COVID-19 vaccination (The Moderna mRNA-1273 vaccine). The best corrected visual acuity was 6/15 in the right eye and 6/6 in the left eye with a relative afferent pupillary defect in the right eye. Following examination and investigation, she was found to fit the McDonald Criteria for Multiple Sclerosis and was commenced on disease-modifying therapy. Demyelinating events have been identified to occur following COVID-19 vaccinations. In this case we have found that The Moderna mRNA-1273 vaccine could have contributed to the development of optic neuritis following a second dose of the vaccine.
DOI: 10.6084/m9.figshare.24279595
title: Supplementary Material for: Efficacy of therapeutic apheresis for cryoglobulinemic vasculitis patients with renal involvement: a systematic review

description: Introduction: Therapeutic apheresis (TA) is commonly used for cryoglobulinemic vasculitis (CV) patients, but its efficacy remains uncertain. This systematic review aimed to assess the efficacy of different TA modalities, such as plasma exchange (PE), plasmapheresis (PP), and cryofiltration (CF), in treating CV patients with renal involvement. Methods: Literature search of MEDLINE, EMBASE and Cochrane Databases was conducted up to December 2022. Studies that reported the outcomes of TA in adult CV patients with renal involvement were assessed. The protocol for this systematic review has been registered with PROSPERO (no. CRD42023417727). The quality of each study was evaluated by the investigators using the validated methodological index for non-randomized studies (minors) quality score. Results: 154 patients who encountered 170 episodes of serious events necessitating TA were evaluated across 76 studies. Among them, 51% were males, with a mean age ranging from 49 to 58 years. The CV types included 15 type I, 97 type II, and 13 type III, while the remaining patients exhibited mixed (n=17) or undetermined CV types (n=12). Among the treatment modalities, PE, PP and CF were performed in 85 (56%), 52 (34%), and 17 patients (11%), respectively, with no identical protocol for TA treatment. The overall response rate for TA was 78%, with response rates of 84%, 77%, and 75% observed in type I, II, and III patients respectively. Most patients received steroids, immunosuppressants, and treatment targeting the underlying causative disease. The overall long-term renal outcome rate was 77%, with type I, II and III patients experiencing response rates of 89%, 76% and 90%, respectively. The renal outcomes in patients receiving PE, PP and CF were comparable, with rates of 78%, 76% and 81%, respectively. Conclusions: This study presents compelling evidence that combination of TA with other treatments, especially immunosuppressive therapy, is a successful strategy for effectively managing severe renal involvement in CV patients. Among the TA modalities studied, including PE, PP, and CF, all demonstrated efficacy, with PE being the most frequently employed approach.
DOI: 10.24432/c58g7v
title: Character Trajectories
DOI: 10.5281/zenodo.3752137
title: Raw LFP data from Gothard Lab multisensory processing project

description: Data set contains LFPs collected from two adult male rhesus macaques (foz and blu). There are 41 total sessions. Data were collected with a 16-channel linear electrode array that was acutely lowered into the amygdala and surrounding tissue.
DOI: 10.5281/zenodo.5550075
title: Principles of RNA recruitment to viral ribonucleoprotein condensates in a segmented dsRNA virus

description: <strong>Rotaviruses transcribe eleven distinct protein-coding RNAs that must be stoichiometrically co-packaged prior to their replication to make an infectious virion. During infection, </strong><strong>rotavirus transcripts accumulate in cytoplasmic ribonucleoprotein (RNP) condensates, termed viroplasms. </strong><strong>Understanding the mechanisms of viroplasm assembly and RNA enrichment within is crucial to gaining greater insight into their function and stoichiometric assortment of individual transcripts.</strong> <strong>We analysed the subcellular distribution of individual RV transcripts and viroplasm transcriptome by combining multiplexed DNA-barcoded single-molecule RNA FISH of infected cells. Using DNA-PAINT microscopy, we provide evidence of the early onset of viral transcript oligomerisation that occurs prior to the formation of viroplasms. We demonstrate that viral sequences lacking the conserved terminal regions fail to undergo enrichment in rotavirus RNP condensates. We show that individual viral transcripts exhibit variable propensities to partition into viroplasms, irrespective of their absolute numbers in cells, suggesting a selective RNA enrichment mechanism distinct from other known cellular RNP granules. </strong><strong>We suggest that rotavirus replication factories represent unique RNP condensates enriched in eleven types of cognate transcripts that may facilitate the assembly of a multi-segmented RNA genome.</strong>
DOI: 10.5281/zenodo.7470075
title: Additional dataset for 'Principles of RNA recruitment to viral ribonucleoprotein condensates in a segmented dsRNA virus'

description: Additional dataset - RNA FISH data set for Figure 6b
DOI: 10.6084/m9.figshare.19576117
title: Multiple sequence alignments and HMMs of the four core virion proteins of eukaryotic bamfordviruses

description: Multiple sequence alignments of the major capsid proteins, minor capsid proteins, protease and ATPase found in the bamfordviruses that infect eukaryotes. These include a concatenated alignment and an alignment with <em>E. coli </em>phage PRD1 as an outgroup. <br> Files also include 38 HMM models used to search for protein homologoues.
DOI: 10.6084/m9.figshare.22240717
title: Supplementary Material for: High Preoperative Platelet to Lymphocyte Ratio is Associated with a Greater Risk of Postoperative Complications and Hematogenous Recurrences in Esophageal Squamous Cell Carcinoma Patients Receiving Neoadjuvant Treatment

description: Background. Neoadjuvant treatment is currently the gold standard for advanced esophageal squamous cell carcinoma (ESCC). Several studies have examined the value of blood count-based indexes for predicting short- and long-term outcomes after esophagectomy for ESCC, but the relative predictive value of pretreatment, preoperative, and postoperative indexes has not yet been examined. Methods. This study included 320 patients with thoracic ESCC who underwent subtotal esophagectomy after neoadjuvant chemotherapy or chemoradiotherapy at our institution. A total of 19 candidate blood parameters were measured before neoadjuvant treatment as well as preoperatively and postoperatively. The ability of the parameters to predict postoperative complications, overall survival (OS), and relapse-free survival (RFS) were assessed using receiver operating characteristic (ROC) curve analysis and Cox regression analysis. Results. ROC curve analysis indicated that the preoperative platelet to lymphocyte ratio (PLR) had the best predictive value with an optimal cutoff value of 166. Patients with high preoperative PLR (≥166) had significantly shorter OS and RFS and significantly higher incidences of hematogenous recurrence and postoperative pneumonia compared with patients with low preoperative PLR (&lt;166). In multivariate analysis, high preoperative PLR and high preoperative serum carcinoembryonic antigen level were independent predictors of poor prognosis. Conclusions. Preoperative PLR is a good predictor of short- and long-term prognosis in patients with advanced ESCC who receive neoadjuvant treatment followed by radical resection.
DOI: 10.6084/m9.figshare.22262104
title: Supplementary Material for: Clinical Guidelines for Diagnosis and Management of Peutz-Jeghers Syndrome in Children and Adults

description: Background: Peutz-Jeghers syndrome (PJS) is a rare disease characterized by the presence of hamartomatous polyposis throughout the gastrointestinal tract, except for the esophagus, along with characteristic mucocutaneous pigmentation. It is caused by germline pathogenic variants of the STK11 gene, which exhibit an autosomal dominant mode of inheritance. Some patients with PJS develop gastrointestinal lesions in childhood and require continuous medical care until adulthood, and sometimes have serious complications that significantly reduce their quality of life (QOL). Hamartomatous polyps in the small bowel may cause bleeding, intestinal obstruction, and intussusception. Novel diagnostic and therapeutic endoscopic procedures such as small-bowel capsule endoscopy (SBCE) and balloon-assisted enteroscopy (BAE) have been developed in recent years. Summary: Under these circumstances, there is growing concern about the management of PJS in Japan, and there are no practice guidelines available. To address this situation, the guideline committee was organized by the Research Group on Rare and Intractable Diseases granted by the Ministry of Health, Labour and Welfare with specialists from multiple academic societies. The present clinical guidelines explain the principles in the diagnosis and management of PJS together with four clinical questions and corresponding recommendations based on a careful review of the evidence and involved incorporating the concept of the Grading of Recommendations Assessment, Development and Evaluation system. Key Messages: Herein, we present the English version of the clinical practice guidelines of PJS to promote seamless implementation of accurate diagnosis and appropriate management of pediatric, adolescent, and adult patients with PJS.
DOI: 10.6084/m9.figshare.22551352
title: Supplementary Material for: Echocardiographic Measures of Left Atrial Structure and Function and the Association with Atrial Fibrillation following Acute Coronary Syndrome

description: Background: Acute coronary syndrome (ACS) is associated with an increased risk of developing atrial fibrillation (AF). This arrhythmia is associated with adverse outcomes making it important to identify high-risk patients. The aim was to evaluate the prognostic value of measures of left atrial (LA) structure and function in AF prediction following ACS. Methods: Three hundred and eighty-one patients who had a percutaneous coronary intervention for ACS were included in the study. Our endpoint was new-onset AF. Results: With a median follow-up time of 5.4 [3.9-6.8] years 56 patients (14.7%) developed AF. Patients developing AF had significantly (P≤0.05) increased maximal and minimal LA volumes (LAVmax and LAVmin, respectively). LAVmax and LAVmin remained significantly increased in AF patients when indexing to either body surface area (LAVmax/BSA and LAVmin/BSA, respectively), left ventricle length in end diastole (LAVmax/LVLd and LAVmin/LVLd, respectively) or late mitral annular diastolic velocity (LAVmax/a’ and LAVmin/a’, respectively) while LA expansion index (LAEi), LA emptying fraction (LAEF) and peak LA longitudinal strain (PALS) were decreased. In univariable cox regressions, all LA measures were found to be predictors of AF. After multivariable adjustment for clinical and echocardiographic parameters all measures reflecting atrial function (LAVmin, LAVmin/BSA, LAVmin/LVLd, LAVmin/a’, LAVmax/a’, LAEF, LAEi and PALS) (P≤0.05) but no structural measures (LAVmax, LAVmax/BSA and LAVmax/LVLd) remained significant independent predictors of AF. Discussion/Conclusion: Echocardiographic measures of LA function are independent predictors of AF following ACS. Evaluation of LA function might improve the prognostic workup, aid in risk stratification for AF and improve selection for further examinations.
DOI: 10.6084/m9.figshare.22567216
title: Supplementary Material for: High incidence of signs of neuropathy and self-reported substance use disorder (SUD) for nitrous oxide (N2O) in patients intoxicated with N2O

description: Introduction: The number of patients with excessive nitrous oxide (N2O) use and neurological disorders has been rising, indicating an addictive potential of N2O. We studied the incidence of self-reported substance use disorder (SUD)-related symptoms, signs of neuropathy and the patterns of use in N2O intoxicated patients. Methods: The Dutch Poisons Information Center (DPIC) provides information by telephone on the management of intoxications to healthcare professionals. Retrospective data on signs of neuropathy and patterns of use were collected for all N2O intoxications reported to the DPIC in 2021 and 2022. Frequent and heavy use were self-reported as “often/frequent/weekly use” and as “use of tanks or &gt;50 balloons/session”, respectively. From this cohort, we included patients with excessive N2O use or signs of neuropathy in a prospective observational cohort study. Online surveys were sent 1 week, 1 month and 3 months after DPIC consultation. The survey included the DUD questionnaire (validated to measure self-reported substance abuse (SA) and dependence (SD) based on DSM-IV-TR criteria), and questions on patterns of use and signs of neuropathy. DSM-IV-TR criteria were translated to DSM-V criteria to score for mild, moderate or severe substance use disorder (SUD), with 2-3, 4-5 or ≥6 symptoms respectively. Results: We included 101 N2O intoxicated patients in the retrospective study. Of these, 41% showed signs of neuropathy (N=41), 53% used N2O tanks to fill balloons (N=53), 71% used frequently (N=72) and 76% used heavily (N=77). We included 75 patients in the prospective study and 10 (13%) completed the first survey. All 10 patients fulfilled the criteria for SA and SD (DSM-IV-TR, median number of questions answered “yes”= 10/12), all used N2O tanks to fill balloons, and 90% (N=9) experienced signs of neuropathy. After 1 and 3 months, 6/7 and 1/1 patients respectively, continued to fulfill SA and SD criteria. Translating to DSM-V criteria, 1/10 patients fulfilled the critera for (self-reported) mild SUD, 1/10 patients for moderate SUD and 8/10 patients for severe SUD, 1 week after consultation. Discussion/Conclusion: The high proportion of N2O intoxicated patients reporting frequent and heavy use of N2O indicates an addictive potential of N2O. Although follow-up rate was low, all patients fulfilled self-reported SA, SD (DSM-IV-TR), and SUD (DSM-V) criteria for N2O. Somatic healthcare professionals treating patients with N2O intoxications, should be aware of possible addictive behaviour in patients. The screening, brief intervention, and referral to treatment (SBIRT) approach should be considered to treat patients with self-reported SUD symptoms.
DOI: 10.6084/m9.figshare.22954532
title: Supplementary Material for: Long-term Safety and Efficacy of Eltrombopag for Advanced Myelodysplastic Syndromes or Acute Myeloid Leukemia and Severe Thrombocytopenia: Results of the ASPIRE Extension Study

description: ASPIRE, a three-part, international, phase II trial (ClinicalTrials.gov identifier: NCT01440374), investigated eltrombopag efficacy and safety in patients with advanced myelodysplastic syndrome or acute myeloid leukemia, and grade 4 thrombocytopenia (&lt;25 × 109 platelets/L). Approximately 30–65% of patients in this open-label extension phase experienced clinically relevant thrombocytopenic events; no conclusions could be made regarding long-term efficacy (non-randomized design, no placebo control), and survival rates may simply reflect advanced disease. Long-term safety was consistent with the double-blind phase and contrasted with earlier SUPPORT study findings in higher-risk patients, suggesting that eltrombopag may have a role in treating thrombocytopenia in patients with low-/intermediate-risk myelodysplastic syndrome.
DOI: 10.6084/m9.figshare.23041751
title: Hibiscus syriacus cv. Baekdansim Draft Genome

description: Draft genome assembly of Hibiscus syriacus var. Baekdansim