cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.5123737
title: Supplementary Material for: Questionnaire-Based Survey Conducted in 2011 concerning Endoscopic Management of Barrett’s Esophagus in East Asian Countries

description: <b><i>Background/Aims:</i></b> Endoscopic definitions and management of Barrett’s esophagus vary widely among countries. To examine the current situation regarding diagnosis, epidemiology, management and treatment of Barrett’s esophagus in East Asian countries using a questionnaire-based survey. <b><i>Methods:</i></b> Representative members of the Committee of the International Gastrointestinal Consensus Symposium developed and sent a questionnaire to major institutions in China, South Korea, Japan, Thailand, Indonesia, and the Philippines. <b><i>Results:</i></b> A total of 56 institutions in the 6 countries participated in the survey. We found that the presence of specialized columnar metaplasia is considered to be important for diagnosing Barrett’s esophagus in East Asian countries except for Japan. C&M criteria have not been well accepted in East Asia. The palisade vessels are mainly used as a landmark for the esophagogastric junction in Japan. The prevalence of long segment Barrett’s esophagus is extremely low in East Asia, while the prevalence of short segment Barrett’s esophagus is very high only in Japan, likely due to different diagnostic criteria. <b><i>Conclusion:</i></b> Among East Asian countries, we found both similarities and differences regarding diagnosis and management of Barrett’s esophagus. The findings in the present survey are helpful to understand the current situation of Barrett’s esophagus in East Asian countries.
DOI: 10.6084/m9.figshare.5123740.v1
title: Supplementary Material for: Natural History of Postoperative Nonfunctioning Pituitary Adenomas: A Systematic Review and Meta-Analysis

description: <b><i>Objective:</i></b> Previous studies attempting to define the natural history of postoperative nonfunctioning pituitary adenomas (pNFPAs) were somewhat limited by selection bias and/or small numbers and/or lack of consistency among the study findings. The aim of this study was to scrutinize the literature in order to analyze the natural history of pNFPAs. <b><i>Methods:</i></b> Electronic database including MEDLINE, PubMed and Cochrane CENTRAL were searched. The literature relating to the patients with pNFPAs without postoperative radiotherapy and pharmacotherapy was collected. Eligible studies reported on the rate of tumor recurrence, the tumor growth-free survival rate (TGFSR) at 5 and 10 years, and/or the residual tumor volume doubling time (TVDT). <b><i>Results:</i></b> 19 studies met the criteria. The pNFPAs were divided into two groups: the pooled recurrence rate of group I without detectable residual tumor (371 patients) was 12% (95% CI 6–19%), the TGFSR at 5 and 10 years were 96% (95% CI 89–99%) and 82% (95% CI 65–94%), respectively. The pooled recurrence rate of group II with residual tumor (600 patients) was 46% (95% CI 36–56%), the TGFSR at 5 and 10 years were 56% (95% CI 41–71%) and 40% (95% CI 27–53%), respectively. The mean TVDT was 3.4 years (95% CI 2.4–4.5 years). <b><i>Conclusions:</i></b> pNFPAs, with or without detectable residual tumor, need stratification of treatment and radiological/endocrinological follow-up strategy. According to the TVDT, residual tumor regrowth is very slow, which permits an extensive and safe follow-up program for most patients.
DOI: 10.6084/m9.figshare.5123725
title: Supplementary Material for: Secreted Factors from Human Mast Cells Trigger Inflammatory Cytokine Production by Human Airway Smooth Muscle Cells

description: <b><i>Background:</i></b> A notable feature of allergic asthma is the infiltration of mast cells into smooth muscle in the human airway. Thus, mast cells and human airway smooth muscle (hASM) cells are likely to exhibit mutual functional modulation via direct cell-cell contact or through released factors. This study examined mast cell modulation of hASM cell cytokine release. <b><i>Methods:</i></b> The mast cell line HMCα was used to model mast cell function. hASM cells were either co-cultured directly with resting or IgE/antigen-stimulated HMCα cells or treated with HMCα-conditioned media to examine the impact on cytokine release. The activation pathways triggered in hASM cells by the mast cell-derived factors were examined through the use of selective inhibitors and by Western blotting. <b><i>Results:</i></b> HMCα cells, or their conditioned media, induced the expression of cytokines (IL-8 and IL-6) by hASM cells at both the mRNA and the protein level. Cytokine expression in hASM cells was greatly amplified when HMCα cells were IgE/antigen-activated. The effects of the conditioned media were not mediated by the chemokines MCP-1 and MIP-1α or by exosomes. While the mast cell-derived factor(s) increased p38<sup>MAPK</sup> phosphorylation in hASM cells, cytokine production was not inhibited by the p38<sup>MAPK</sup> inhibitor SB203580. hASM cell production of IL-8 induced by HMCα condition media but not IL-6 was, however, attenuated by the Src tyrosine kinase inhibitor PP2. <b><i>Conclusions:</i></b> Our study shows that the release of soluble mediators by activated mast cells can stimulate hASM cells to elicit production of proinflammatory cytokines that may then exacerbate airway inflammation in asthma.
DOI: 10.6084/m9.figshare.5123731.v1
title: Supplementary Material for: Less Cognitive and Neurological Deficits in Schizophrenia Patients Carrying Risk Variant in <b><i>ZNF804A</i></b>

description: <b><i>Background:</i></b> The rs1344706 single nucleotide polymorphism in the <i>ZNF804A</i> gene is a common variant with strong evidence for association with schizophrenia. Recent studies show an association of rs1344706 with cognitive functioning, and there is some evidence suggesting that the risk allele may increase susceptibility for a subtype of schizophrenia with relatively spared cognition. <b><i>Methods:</i></b> We tested the effect of rs1344706 genotype in 89 schizophrenia patients on 3 basic cognitive domains (working memory, processing speed and attention) shown to be severely impaired in schizophrenia. Also we investigated the effect of rs1344706 on the severity of neurological soft signs, subtle impairments in motor and sensory functions highly frequent in schizophrenia patients. Neurological soft signs and cognitive deficits are central features of schizophrenia and are tightly linked with clinical, social and functional outcome. <b><i>Results:</i></b> Our results show an association of higher rs1344706 risk allele load with improved performance on processing speed and with fewer neurological soft signs. <b><i>Conclusions:</i></b> Together with other studies, our findings suggest that <i>ZNF804A</i> is associated with a subtype of schizophrenia with better cognitive and neurological functioning. Discovery of the specific pathways through which <i>ZNF804A</i> is exerting this effect may lead to better prevention, diagnosis and treatment for a specific group of schizophrenia patients.
DOI: 10.6084/m9.figshare.5123734.v1
title: Supplementary Material for: Novel Uromodulin Mutation in Familial Juvenile Hyperuricemic Nephropathy

description: <b><i>Background:</i></b> Familial juvenile hyperuricemic nephropathy (FJHN) is an autosomal dominant disorder characterized by early onset of hyperuricemia, decreased fractional renal urate excretion and progressive interstitial nephropathy. Mutations in the uromodulin<i> (UMOD)</i> gene encoding uromodulin/Tamm-Horsfall, a glycosylphosphatidylinositol (GPI)-anchored protein, cause this disease. <b><i>Methods:</i></b> One Chinese family with 13 FJHN-affected individuals is described. Clinical data, blood and urine samples of 7 affected members (all alive patients in this family) and 15 unaffected members were collected. Mutation analysis of the <i>UMOD</i> gene was performed by polymerase chain reaction and direct sequencing. Urinary uromodulin from affected or unaffected members of this family and healthy controls was examined by enzyme-linked immunosorbent assay kit. Expression of uromodulin in renal tissue was shown with immunofluorescence. <b><i>Results:</i></b> A novel mutation (p.T605G) within the uromodulin GPI anchor signal segment was identified in the affected individuals of this FJHN family. There was a markedly increased expression of uromodulin in renal tissue and significantly decreased urinary excretion of uromodulin in affected patients with an estimated glomerular filtration rate &lt;60 ml/min/1.73 m<sup>2</sup>. <b><i>Conclusions:</i></b> The present study reported a novel mutation in exon 9 of <i>UMOD</i> in the Chinese Han population, within the GPI anchor signal segment of uromodulin. Since the GPI anchor is linked with the release or secretion of proteins, our finding may provide further evidence for the underlying mechanism of decreased urinary excretion of uromodulin in FJHN.
DOI: 10.6084/m9.figshare.5123719
title: Supplementary Material for: Computed Tomography Findings for Intracerebral Hemorrhage Have Little Incremental Impact on Post-Stroke Mortality Prediction Model Performance

description: <b><i>Background:</i></b> Stroke outcome studies often combine cases of intracerebral hemorrhage (ICH) and ischemic stroke (IS). These studies of mixed stroke typically ignore computed tomography (CT) findings for ICH cases, though the impact of omitting these traditional predictors of ICH mortality is unknown. We investigated the incremental impact of ICH CT findings on mortality prediction model performance. <b><i>Methods:</i></b> Cases of ICH and IS (2000–2003) were identified from the Brain Attack Surveillance in Corpus Christi (BASIC) project. Base models predicting 30-day mortality included demographics, stroke type, and clinical findings (National Institutes of Health Stroke Scale (NIHSS) +/– Glasgow Coma Scale (GCS)). The impact of adding CT data (volume, intraventricular hemorrhage, infratentorial location) was assessed with the area under the curve (AUC), unweighted sum of squared residuals (Ŝ), and integrated discrimination improvement (IDI). The model assessment was performed first for the mixed case of IS and ICH, and then repeated for ICH cases alone to determine whether any lack of improvement in model performance with CT data for mixed stroke type was due to IS cases naturally forming a larger proportion of the total sample than ICH. <b><i>Results:</i></b> A total of 1,256 cases were included (86% IS, 14% ICH). Thirty-day mortality was 16% overall (11% for IS; 43% for ICH). When both clinical scales (NIHSS and GCS) were included, none of the model performance measures showed improvement with the addition of CT findings whether considering IS and ICH together (ΔAUC: 0.002, 95% CI –0.01, 0.02; ΔŜ: –3.0, 95% CI –9.1, 2.6; IDI: 0.017, 95% CI –0.004, 0.05) or considering ICH cases alone (ΔAUC: 0.02, 95% CI –0.02, 0.08; ΔŜ: –2.0, 95% CI –9.7, 3.4; IDI 0.065, 95% CI –0.03, 0.21). If NIHSS was the only clinical scale included, there was still no improvement in AUC or Ŝ when CT findings were added for the sample with IS/ICH combined (ΔAUC: 0.005, 95% CI –0.01, 0.02; ΔŜ: –5.0, 95% CI –11.6, 1.0) or for ICH cases alone (ΔAUC: 0.05, 95% CI –0.002, 0.11; ΔŜ: –4.2, 95% CI –11.5, 2.3). However, IDI was improved when NIHSS was the only clinical scale for IS/ICH combined (IDI: 0.029, 95% CI 0.002, 0.065) and ICH alone (IDI: 0.12, 95% CI 0.005, 0.26). <b><i>Conclusions:</i></b> Excluding ICH CT findings had only minimal impact on mortality prediction model performance whether examining ICH and IS together or ICH alone. These findings have important implications for the design of clinical studies involving ICH patients.
DOI: 10.6084/m9.figshare.5123716
title: Supplementary Material for: Decompression through Tracheobronchial Endoscopy of Bronchial Atresia Presenting as Massive Pulmonary Tumor: A New Indication for Fetoscopic Surgery

description: This is the first report of fetoscopy to successfully treat a case of bilobar bronchial atresia presenting in the form of a massive right lung tumor causing severe thoracic compression with mediastinal shift, pulmonary hypoplasia and eventually fetal hydrops. The presence of an echolucent tubular structure in the pulmonary hilum was identified as the intrapulmonary continuation of the interrupted main bronchus, and led to the diagnosis of main bronchial atresia. After extensive discussion and counseling, a fetoscopic operation was designed and indicated. The procedure consisted of a fetal tracheoscopy and access to the right main bronchus, which was perforated with a diode laser until a communication with the dead end of the intrapulmonary main bronchus was achieved. After the operation a significant reduction in size was observed, hydrops and mediastinal shift disappeared and O/E LHR increased to 85%. The fetus was born at term and lobectomy of two dysplastic pulmonary lobes was performed. The infant is developing well at 14 months of age. This report demonstrates that fetoscopic airway decompression can achieve fetal survival in the rare event of main bronchus atresia.
DOI: 10.6084/m9.figshare.5123719.v1
title: Supplementary Material for: Computed Tomography Findings for Intracerebral Hemorrhage Have Little Incremental Impact on Post-Stroke Mortality Prediction Model Performance

description: <b><i>Background:</i></b> Stroke outcome studies often combine cases of intracerebral hemorrhage (ICH) and ischemic stroke (IS). These studies of mixed stroke typically ignore computed tomography (CT) findings for ICH cases, though the impact of omitting these traditional predictors of ICH mortality is unknown. We investigated the incremental impact of ICH CT findings on mortality prediction model performance. <b><i>Methods:</i></b> Cases of ICH and IS (2000–2003) were identified from the Brain Attack Surveillance in Corpus Christi (BASIC) project. Base models predicting 30-day mortality included demographics, stroke type, and clinical findings (National Institutes of Health Stroke Scale (NIHSS) +/– Glasgow Coma Scale (GCS)). The impact of adding CT data (volume, intraventricular hemorrhage, infratentorial location) was assessed with the area under the curve (AUC), unweighted sum of squared residuals (Ŝ), and integrated discrimination improvement (IDI). The model assessment was performed first for the mixed case of IS and ICH, and then repeated for ICH cases alone to determine whether any lack of improvement in model performance with CT data for mixed stroke type was due to IS cases naturally forming a larger proportion of the total sample than ICH. <b><i>Results:</i></b> A total of 1,256 cases were included (86% IS, 14% ICH). Thirty-day mortality was 16% overall (11% for IS; 43% for ICH). When both clinical scales (NIHSS and GCS) were included, none of the model performance measures showed improvement with the addition of CT findings whether considering IS and ICH together (ΔAUC: 0.002, 95% CI –0.01, 0.02; ΔŜ: –3.0, 95% CI –9.1, 2.6; IDI: 0.017, 95% CI –0.004, 0.05) or considering ICH cases alone (ΔAUC: 0.02, 95% CI –0.02, 0.08; ΔŜ: –2.0, 95% CI –9.7, 3.4; IDI 0.065, 95% CI –0.03, 0.21). If NIHSS was the only clinical scale included, there was still no improvement in AUC or Ŝ when CT findings were added for the sample with IS/ICH combined (ΔAUC: 0.005, 95% CI –0.01, 0.02; ΔŜ: –5.0, 95% CI –11.6, 1.0) or for ICH cases alone (ΔAUC: 0.05, 95% CI –0.002, 0.11; ΔŜ: –4.2, 95% CI –11.5, 2.3). However, IDI was improved when NIHSS was the only clinical scale for IS/ICH combined (IDI: 0.029, 95% CI 0.002, 0.065) and ICH alone (IDI: 0.12, 95% CI 0.005, 0.26). <b><i>Conclusions:</i></b> Excluding ICH CT findings had only minimal impact on mortality prediction model performance whether examining ICH and IS together or ICH alone. These findings have important implications for the design of clinical studies involving ICH patients.
DOI: 10.6084/m9.figshare.5123722
title: Supplementary Material for: Atraumatic Nonaneurysmal Sulcal Subarachnoid Hemorrhages: A Diagnostic Workup Based on a Case Series

description: <b><i>Introduction:</i></b> Atraumatic and nonaneurysmal sulcal subarachnoid hemorrhage (sSAH) is a rare type of cerebrovascular disease with various etiologies previously reported in small case reports. In this study, we propose to analyze clinical presentations, imaging patterns and etiologies in a large case series of such patients in order to propose a diagnostic workup. <b><i>Methods:</i></b> We retrospectively analyzed clinical and radiological data of consecutive patients with a diagnosis of atraumatic and nonaneurysmal sSAH, admitted to our institution between 2008 and 2011. All patients had both computed tomography (CT) and magnetic resonance imaging (MRI) as a part of their initial evaluation. <b><i>Results:</i></b> 30 patients (18 women and 12 men, mean age: 60 years) were identified. The main clinical symptoms at presentation were focal and transient neurological deficit (n = 22) and thunderclap headache (n = 10). Four patients had progressive headache and 4 other had partial or generalized epileptic seizures. MRI abnormalities associated with sSAH were prior hemorrhages, microbleeds, severe leukoencephalopathy and hemosiderosis suggesting cerebral amyloid angiopathy (CAA; n = 9), vasogenic edema in parieto-occipital areas compatible with a posterior reversible encephalopathy syndrome (PRES; n = 3), cortical venous thrombosis (n = 2) and concomitant acute cortical stroke (n = 3). Other underlying causes of sSAH, not diagnosed on MRI, were reversible cerebral vasoconstriction syndrome (RCVS) based on clinical criteria and conventional angiography (n = 4), angiitis diagnosed by skin biopsy (n = 1), vascular malformation diagnosed on CT and digital subtraction angiographies (n = 3), and overanticoagulation (n = 1). Four cases remained unresolved. <b><i>Conclusion:</i></b> This study confirmed that sSAH is a rare condition related to a wide spectrum of etiologies. Combination of brain MRI and magnetic resonance angiography and eventually digital subtraction angiography allowed the identification of an underlying etiology for 87% of patients. CAA, RCVS and PRES represented more than 50% of the etiological mechanisms. Among older patients, sSAH was mainly related to CAA while in younger patients, RCVS represented the most frequent etiology.
DOI: 10.6084/m9.figshare.5123725.v1
title: Supplementary Material for: Secreted Factors from Human Mast Cells Trigger Inflammatory Cytokine Production by Human Airway Smooth Muscle Cells

description: <b><i>Background:</i></b> A notable feature of allergic asthma is the infiltration of mast cells into smooth muscle in the human airway. Thus, mast cells and human airway smooth muscle (hASM) cells are likely to exhibit mutual functional modulation via direct cell-cell contact or through released factors. This study examined mast cell modulation of hASM cell cytokine release. <b><i>Methods:</i></b> The mast cell line HMCα was used to model mast cell function. hASM cells were either co-cultured directly with resting or IgE/antigen-stimulated HMCα cells or treated with HMCα-conditioned media to examine the impact on cytokine release. The activation pathways triggered in hASM cells by the mast cell-derived factors were examined through the use of selective inhibitors and by Western blotting. <b><i>Results:</i></b> HMCα cells, or their conditioned media, induced the expression of cytokines (IL-8 and IL-6) by hASM cells at both the mRNA and the protein level. Cytokine expression in hASM cells was greatly amplified when HMCα cells were IgE/antigen-activated. The effects of the conditioned media were not mediated by the chemokines MCP-1 and MIP-1α or by exosomes. While the mast cell-derived factor(s) increased p38<sup>MAPK</sup> phosphorylation in hASM cells, cytokine production was not inhibited by the p38<sup>MAPK</sup> inhibitor SB203580. hASM cell production of IL-8 induced by HMCα condition media but not IL-6 was, however, attenuated by the Src tyrosine kinase inhibitor PP2. <b><i>Conclusions:</i></b> Our study shows that the release of soluble mediators by activated mast cells can stimulate hASM cells to elicit production of proinflammatory cytokines that may then exacerbate airway inflammation in asthma.
DOI: 10.6084/m9.figshare.5123716.v1
title: Supplementary Material for: Decompression through Tracheobronchial Endoscopy of Bronchial Atresia Presenting as Massive Pulmonary Tumor: A New Indication for Fetoscopic Surgery

description: This is the first report of fetoscopy to successfully treat a case of bilobar bronchial atresia presenting in the form of a massive right lung tumor causing severe thoracic compression with mediastinal shift, pulmonary hypoplasia and eventually fetal hydrops. The presence of an echolucent tubular structure in the pulmonary hilum was identified as the intrapulmonary continuation of the interrupted main bronchus, and led to the diagnosis of main bronchial atresia. After extensive discussion and counseling, a fetoscopic operation was designed and indicated. The procedure consisted of a fetal tracheoscopy and access to the right main bronchus, which was perforated with a diode laser until a communication with the dead end of the intrapulmonary main bronchus was achieved. After the operation a significant reduction in size was observed, hydrops and mediastinal shift disappeared and O/E LHR increased to 85%. The fetus was born at term and lobectomy of two dysplastic pulmonary lobes was performed. The infant is developing well at 14 months of age. This report demonstrates that fetoscopic airway decompression can achieve fetal survival in the rare event of main bronchus atresia.
DOI: 10.6084/m9.figshare.5123722.v1
title: Supplementary Material for: Atraumatic Nonaneurysmal Sulcal Subarachnoid Hemorrhages: A Diagnostic Workup Based on a Case Series

description: <b><i>Introduction:</i></b> Atraumatic and nonaneurysmal sulcal subarachnoid hemorrhage (sSAH) is a rare type of cerebrovascular disease with various etiologies previously reported in small case reports. In this study, we propose to analyze clinical presentations, imaging patterns and etiologies in a large case series of such patients in order to propose a diagnostic workup. <b><i>Methods:</i></b> We retrospectively analyzed clinical and radiological data of consecutive patients with a diagnosis of atraumatic and nonaneurysmal sSAH, admitted to our institution between 2008 and 2011. All patients had both computed tomography (CT) and magnetic resonance imaging (MRI) as a part of their initial evaluation. <b><i>Results:</i></b> 30 patients (18 women and 12 men, mean age: 60 years) were identified. The main clinical symptoms at presentation were focal and transient neurological deficit (n = 22) and thunderclap headache (n = 10). Four patients had progressive headache and 4 other had partial or generalized epileptic seizures. MRI abnormalities associated with sSAH were prior hemorrhages, microbleeds, severe leukoencephalopathy and hemosiderosis suggesting cerebral amyloid angiopathy (CAA; n = 9), vasogenic edema in parieto-occipital areas compatible with a posterior reversible encephalopathy syndrome (PRES; n = 3), cortical venous thrombosis (n = 2) and concomitant acute cortical stroke (n = 3). Other underlying causes of sSAH, not diagnosed on MRI, were reversible cerebral vasoconstriction syndrome (RCVS) based on clinical criteria and conventional angiography (n = 4), angiitis diagnosed by skin biopsy (n = 1), vascular malformation diagnosed on CT and digital subtraction angiographies (n = 3), and overanticoagulation (n = 1). Four cases remained unresolved. <b><i>Conclusion:</i></b> This study confirmed that sSAH is a rare condition related to a wide spectrum of etiologies. Combination of brain MRI and magnetic resonance angiography and eventually digital subtraction angiography allowed the identification of an underlying etiology for 87% of patients. CAA, RCVS and PRES represented more than 50% of the etiological mechanisms. Among older patients, sSAH was mainly related to CAA while in younger patients, RCVS represented the most frequent etiology.