Miracle-dz/UniFAR-FTUs huggingface dataset:
Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts

Objective. There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan. Methods. We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse. Results. Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p < 0.01). The proportion of patients with proteinase 3 (PR3)-ANCA was lower in Japan than in the UK (61.0% vs 85.2%, p < 0.01), while the proportion of myeloperoxidase-ANCA was higher in Japan than the UK (34.1% vs 8.6%, p < 0.01). Serum creatinine at diagnosis was lower in Japan than the UK (68.1 μmol/l vs 101.0 μmol/l, p < 0.01). Respiratory involvement was more frequent in Japan than the UK (69.5% vs 40.6%, p < 0.01). In both countries, most patients received both glucocorticoids and cyclophosphamide. At 60 months the cumulative survival rates were 87.6% and 94.3% in Japan and the UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p < 0.01). Conclusion. Japanese patients with GPA were older at disease onset, with less PR3-ANCA positivity, milder renal dysfunction, and more frequent respiratory involvement than UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.
The cognitive profile of behavioural variant FTD and its similarities with ALS: a systematic review and meta-analysis

Approximately 30% of patients with amyotrophic lateral sclerosis (ALS) have cognitive impairment and 8%–14% fulfil the criteria for behavioural variant frontotemporal dementia (bv-FTD). The cognitive profiles of ALS and bv-FTD have been reported to be comparable, but this has never been systematically investigated. We aimed to determine the cognitive profile of bv-FTD and examine its similarities with that of ALS, to provide evidence for the existence of a cognitive disease continuum encompassing bv-FTD and ALS. We therefore systematically reviewed neuropsychological studies on bv-FTD patients and healthy volunteers. Neuropsychological tests were divided in 10 cognitive domains and effect sizes were calculated for all domains and compared with the cognitive profile of ALS by means of a visual comparison and a Pearson’s r correlation coefficient. We included 120 studies, totalling 2425 bv-FTD patients and 2798 healthy controls. All cognitive domains showed substantial effect sizes, indicating cognitive impairment in bv-FTD patients compared to healthy controls. The cognitive domains with the largest effect sizes were social cognition, verbal memory and fluency (1.77–1.53). The cognitive profiles of bv-FTD and ALS (10 cognitive domains, 1287 patients) showed similarities on visual comparison and a moderate correlation 0.58 (p=0.13). When social cognition, verbal memory, fluency, executive functions, language and visuoperception were considered, i.e. the cognitive profile of ALS, Pearson’s r was 0.73 (p=0.09), which raised to 0.92 (p=0.03), when language was excluded in this systematic analysis of patients with a non-language subtype of FTD. The cognitive profile of bv-FTD consists of deficits in social cognition, verbal memory, fluency and executive functions and shows similarities with the cognitive profile of ALS. These findings support a cognitive continuum encompassing ALS and bv-FTD.
Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor

Chronic sinusitis is universal in cystic fibrosis (CF) and our current treatments are ineffective in reversing sinus disease. The objective of this work was to determine if increasing CF transmembrane conductance regulator (CFTR) activity by ivacaftor could treat CF sinus disease and assess its effect on primary sinus epithelial cultures.
An overview of pharmacotherapy for bipolar I disorder

ABSTRACT Introduction: Bipolar I disorder (BD I) is complex with a chronic course that significantly impacts a sufferer’s quality of life. As of right now, there are many available treatments that aim to rapidly treat manic or depressive episodes and stabilize mood. The purpose of this report is to provide an up-to-date comprehensive review of the available evidence-based trials of pharmacotherapy for the treatment of BD I. Areas covered: This paper reviews randomized active comparator-controlled or placebo-controlled trials evaluating the use of current pharmacotherapy in adults with BD I from phase III to clinical practice. Monotherapy and combination therapy for acute and long-term treatment were reviewed for this purpose. Expert opinion: There are many treatments available for BD mania; however, the depressive and stabilization phases of the illness remain a clinical challenge. Unfortunately, randomized controlled trials do not represent ‘real world’ patients, as their strict inclusion and exclusion criteria do not allow for different features sometimes present in patients to be considered. Research efforts must also focus on treating cognitive deficits, which adds to lower functional outcome. The authors believe that there is dire need for new, more targeted treatments in BD I, with a critical view of the side effects.
Formulation for a novel inhaled peptide therapeutic for idiopathic pulmonary fibrosis

Abstract A caveolin-1 scaffolding domain, CSP7, is a newly developed peptide for the treatment of idiopathic pulmonary fibrosis. To develop a CSP7 formulation for further use we have obtained, characterized and compared a number of lyophilized formulations of CSP7 trifluoroacetate with DPBS and in combination with excipients (mannitol and lactose at molar ratios 1:5, 70 and 140). CSP7 trifluoroacetate was stable (>95%) in solution at 5 and 25 °C for up to 48 h and tolerated at least 5 freeze/thaw cycles. Lyophilized cakes of CSP7 trifluoroacetate with excipients were stable (>96%) for up to 4 weeks at room temperature (RT), and retained more than 98% of the CSP7 trifluoroacetate in the solution at 8 h after reconstitution at RT. The lyophilized CSP7 formulations were stable for up to 10 months at 5 °C protected from moisture. Exposure of the lyophilized cakes of CSP7 to 75% relative humidity (RH) resulted in an increase in the absorbed moisture, promoted crystallization of the excipients and induced reversible formation of CSP7 aggregates. Increased molar ratio of mannitol slightly affected formation of the aggregates. In contrast, lactose significantly decreased (up to 20 times) aggregate formation with apparent saturation at the molar ratio of 1:70. The possible mechanisms of stabilization of CSP7 trifluoroacetate in solid state by lactose include physical state of the bulking agent and the interactions between lactose and CSP7 trifluoroacetate (e.g. formation of a Schiff base with the N-terminal amino group of CSP7). Finally, CSP7 trifluoroacetate exhibited excellent stability during nebulization of formulations containing mannitol or lactose.
The Effect of Pathological Narcissism on Interpersonal and Affective Processes in Social Interactions

Narcissism has significant interpersonal costs, yet little research has examined behavioral and affective patterns characteristic of narcissism in naturalistic settings. Here we studied the effect of narcissistic features on the dynamic processes of interpersonal behavior and affect in daily life. We used interpersonal theory to generate transactional models of social interaction (i.e., linkages among perceptions of others’ behavior, affect, and one’s own behavior) predicted to be characteristic of narcissism. Psychiatric outpatients (N = 102) completed clinical interviews and a 21-day ecological momentary assessment protocol using smartphones. After social interactions (N = 5,781), participants reported on perceptions of their interaction partner’s behavior (scored along the dimensions of dominant–submissive and affiliative–quarrelsome), their own affect, and their own behavior. Multilevel structural equation modeling was used to examine dynamic links among behavior and affect across interactions, and the role of narcissism in moderating these links. Results showed that perceptions of others’ dominance did not predict dominant behavior, but did predict quarrelsome behavior, and this link was potentiated by narcissism. Furthermore, the link between others’ dominance and one’s own quarrelsome behavior was mediated by negative affect. Moderated mediation was also found: Narcissism amplified the link between ratings of others’ dominance and one’s own quarrelsomeness and negative affect. Narcissism did not moderate the link between other dominance and own dominance, nor the link between other affiliation and own affiliation. These results suggest that narcissism is associated with specific interpersonal and affective processes, such that sensitivity to others’ dominance triggers antagonistic behavior in daily life.
The Methodological Quality and Effectiveness of Technology-Based Smoking Cessation Interventions for Disadvantaged Groups: A Systematic Review and Meta-analysis

Aims To assess the methodological quality and effectiveness of technology-based smoking cessation interventions in disadvantaged groups. Method Four databases (EMBASE, Cochrane, Medline, and PsycInfo) were searched for studies conducted from 1980 to May 2016. Randomized controlled trials that compared a behavioral smoking cessation intervention delivered primarily through a technology-based platform (eg, mobile phone) with a no-intervention comparison group among disadvantaged smokers were included. Three reviewers assessed all relevant studies for inclusion, and one reviewer extracted study, participant and intervention-level data, with a subset crosschecked by a second reviewer. Results Thirteen studies targeting disadvantaged smokers (n =4820) were included. Only one study scored highly in terms of methodological rigor on EPOC criteria for judging risk of bias. Of the 13 studies using a technology-based platform, most utilized websites (n = 5) or computer programs (n = 5), and seven additionally offered nicotine replacement therapy. Technology-based interventions increased the odds of smoking cessation for disadvantaged groups at 1 month (odds ratio [OR] 1.70, 95% confidence interval [CI] 1.10, 2.63), 3 months (OR 1.30, 95% CI 1.07, 1.59), 6 months (OR 1.29, 95% CI 1.03, 1.62), and 18 months post-intervention (OR 1.83, 95% CI 1.11, 3.01). Conclusion Few methodologically rigorous studies were identified. Mobile phone text-messaging, computer- and website-delivered quit support showed promise at increasing quit rates among Indigenous, psychiatric and inpatient substance use disorder patients. Further research is needed to address the role technology-based interventions have on overcoming health inequalities to meet the needs of disadvantaged groups. Implications This review provides the first quantitative evidence of the effectiveness of a range of technology-based smoking cessation interventions among disadvantaged smokers, with separate estimates on the basis of intervention type, and cessation outcome measure. Providing cost-effective, easily accessible and real-time smoking cessation treatment is needed, and innovative technology-based platforms will help reach this endpoint. These interventions need to be tested in larger scale randomized controlled trial designs and target broader disadvantaged groups. Data collection beyond 6 months is also needed in order to establish the efficacy of these intervention approaches on long-term cessation rates among disadvantaged population groups.
Hydroxychloroquine Blood Levels in Systemic Lupus Erythematosus: Clarifying Dosing Controversies and Improving Adherence

Objective. Hydroxychloroquine (HCQ) is used for its effect on systemic lupus erythematosus (SLE) disease activity and longterm benefits. This can be limited by adherence. One way to assess adherence is to measure blood levels. Conflicting data exist regarding blood levels and disease activity. There is disagreement about dosing; rheumatologists recommend weight-based dosing while some other specialists advocate height-based “ideal body weight” dosing. Methods. Patients were prescribed HCQ not exceeding 6.5 mg/kg (max 400 mg/day). In hemodialysis, the dose was 200 mg after each session, and in renal insufficiency it was 200 mg/day. Levels were measured at each visit with a therapeutic range of 500–2000 ng/ml. Patients were divided according to baseline blood level. To assess the effect of measurement and counseling on adherence, we compared the proportion of patients with a level of 500 ng/ml or higher based on the number of prior assessments. Results. The proportion of patients with HCQ levels in the therapeutic range differed significantly by age, sex, and Vitamin D level. There was a trend toward lower levels with renal failure. Blood levels were similar regardless of height and ideal body weight. Comparing those with undetectable, subtherapeutic, and therapeutic levels, disease activity decreased (SLE Disease Activity Index 2.92, 2.36, and 2.20, p = 0.04 for trend). At first, 56% were therapeutic, and by the third measurement this increased to 80% (p ≤ 0.0001). Conclusion. There was a trend toward higher disease activity with lower HCQ levels. Renal failure dosing led to suboptimum levels. We show that weight-based dosing (max 400 mg daily) is appropriate and that height does not appear to influence levels. Measurement, counseling, and repeated testing can increase adherence rates.
The general results of the RN4CAST survey in Italy.

The issue of health workforce shortage and in particular of nurses, has been debated globally for almost three decades (Aiken & Mullinix 1987, Aiken et al. 1996, 2001, 2010), and has been exacerbated by the recent global financial crisis. The European RN4CAST project has shifted focus from considering only nursing workforce planning and workforce volumes to considering the impact of adequate nurse-patient ratios and work environment on patient safety and the quality of care (Sermeus et al. 2011).
LAKE 2.0: a model for temperature, methane, carbon dioxide and oxygen dynamics in lakes

Abstract. A one-dimensional (1-D) model for an enclosed basin (lake) is presented, which reproduces temperature, horizontal velocities, oxygen, carbon dioxide and methane in the basin. All prognostic variables are treated in a unified manner via a generic 1-D transport equation for horizontally averaged property. A water body interacts with underlying sediments. These sediments are represented by a set of vertical columns with heat, moisture and CH4 transport inside. The model is validated vs. a comprehensive observational data set gathered at Kuivajarvi Lake (southern Finland), demonstrating a fair agreement. The value of a key calibration constant, regulating the magnitude of methane production in sediments, corresponded well to that obtained from another two lakes. We demonstrated via surface seiche parameterization that the near-bottom turbulence induced by surface seiches is likely to significantly affect CH4 accumulation there. Furthermore, our results suggest that a gas transfer through thermocline under intense internal seiche motions is a bottleneck in quantifying greenhouse gas dynamics in dimictic lakes, which calls for further research.
Increased recognition of Chryseobacterium species as an emerging cause of nosocomial urinary tract infection following introduction of matrix-assisted laser desorption/ionisation-time of flight for bacterial identification

Chryseobacterium species are rarely reported as aetiological agents of nosocomial urinary tract infection. Here, we evaluated the clinical significance of 19 isolates of Chryseobacterium species (15 Chryseobacterium indologenes and 4 Chryseobacterium gleum; identified by matrix-assisted laser desorption/ionisation-time of flight [MALDI-TOF]) obtained from urine or percutaneous nephrostomy drainage of 16 patients with urological complaints. The strains possessed drug resistance to multiple antibiotics. 14 isolates showed the presence of carbapenemases. Both MALDI-TOF and repetitive sequence-based-polymerase chain reaction grouped them into three clusters (Kappa 1.000). They may colonise the urinary tract acting as a reservoir for dissemination of drug resistance within hospital environment.
Ventilatory responses of aged male and female rats to hypercapnia and to hypoxia.

Ventilatory parameters such as tidal volume, minute ventilation (VE), and inspiratory flow rate decrease in 24- vs. 12-month-old male and female rats. Differences between male and female values are maintained with age. Ventilatory response male and female rats exhibit to hypercapnia is altered by a decrease of the intercept, but not the slope value. The pattern of breathing exhibited by young females and males in response to hypercapnia (i.e. an increase of VT and f) is different than that noted in old males and females (i.e. an increase in VT only). In contrast, the ventilatory response both of slope and intercept male rats show by 24 months is decreased compared to the 12-month value; but the slope value is actually increased in the older vs. younger female rats in response to hypoxia.
Acceptability of Computer Assessments among Ethnically Diverse, Low-Income Smokers

Purpose. To examine the acceptability of computer-based assessments among an ethnically diverse, low-income population of primary care patients. Although computers have been used to provide assessments and interventions in health care settings, members of ethnic minority and low-income households have less access to computers than other groups, and therefore the acceptability of computers as a health care assessment and delivery tool needs to be examined. Design. We examined the acceptability of computers for providing assessments of smoking history, nicotine dependence, and other related variables among an ethnically diverse, low-income primary care population. No intervention was used in this study. Setting. Three inner-city primary care clinics located in hospitals were used as sites for this study. These hospitals were located in areas of the city where low-income and ethnic minority households are overrepresented relative to the total population. Subjects. Adult male and female smokers (n = 522) were recruited while awaiting appointments in each primary care clinic. Measures. A questionnaire assessing smoking rate, patterns, history, motivation to quit smoking and other smoking-related variables was administered using either a paper-and-pencil format or a laptop computer. Results. Frequency counts, analysis of variance, and χ2 tests were used where appropriate. Most subjects (78.5 %) used the computer to complete the baseline survey. Almost all subjects (92%) rated the computer “very easy” or “easy” to use. Subjects who were Spanish-speaking, were born outside the United States, or were Hispanic tended to rate the program as slightly less easy to use than other subjects. Conclusions. Computer-based assessments appear highly acceptable to individuals in low-income populations.
Effects of Octreotide Pretreatment in Experimental Acute Pancreatitis

Background: Severity of systemic lesions and mortality of experimental acute pancreatitis (AP) are reduced after pancreatic enzyme content reduction induced by cerulein administration. Octreotide has been used both prophylatically and therapeutically in AP. The possible effects of octreotide on pancreatic enzyme content and its influence on pulmonary lesions of experimental AP were assessed in this study. Methods: Wistar male rats were divided in two branches: Branch I – Animals divided into three groups: Group Sa (n = 10) intravenous saline infusion; Group Ce (n = 10) intravenous cerulein infusion, (0.133 µg/kg–1·h–1) and Group Oc (n = 10) SC octreotide (10 µg/kg–1). Trypsin, elastase and amylase pancreatic contents as well as serum amylase were determined thereafter in all three groups; Branch II – Rats treated as in branch I, were submitted to sodium taurocholate AP (Groups Sa+AP, Ce+AP and Oc+AP). Two hours thereafter amylase and TAP assays were performed in serum, ascites and pancreatic tissue in eight animals of each group. Pulmonary histology was studied by morphometry 24 h after AP in the remaining animals. Results: Increased serum amylase and pancreatic enzyme contents were observed in octreotide-treated animals when compared to animals receiving saline or cerulein. After AP increases of serum and ascitic fluid amylase and of pancreatic TAP were observed in octreotide pre-treated animals when compared to saline and cerulein groups. Pulmonary interstitial and alveolar edema after AP was significantly increased in rats receiving octreotide as compared to the cerulein group. Conclusion: Octreotide administration acutely increases the enzymatic content of the pancreas and thus may have a potential deleterious influence in the evolution of AP.
Completeness and Legibility of Handwritten Prescriptions in Sana’a, Yemen

Objective: The aim of this study was to investigate the completeness and legibility of prescriptions dispensed in community pharmacies located in Sana’a, Yemen. Materials and Methods: A cross-sectional study was conducted at 23 randomly selected community pharmacies throughout the capital city of Sana’a, Yemen, from May 2015 to January 2016. A total of 2,178 prescriptions were analyzed for the essential elements of a complete prescription using a validated checklist. Results: Of the 2,178 prescriptions, 19 (0.87%) were considered to be of good quality. The remaining 2,159 (99.12%) were considered as being of very poor quality. Writing errors relating to patients and prescribed medications were the most common errors. Conclusion: In this study, the quality of prescription writing was found to be very poor. Hence, continuous professional development programs are recommended to improve the quality of prescription writing among physicians. Future studies in other cities and investigation of the impact of continuous educational programs on the quality of prescription writing are strongly recommended.
Non‐invasive diagnosis and treatment strategies for traumatic brain injury: an update

Traumatic Brain Injury (TBI) remains the leading cause of morbidity and mortality in U.S. Since the last decade, there have been several advances in the understanding and management of TBI that have shown the potential to improve outcomes. The aim of this review is to provide a useful overview of these potential diagnostic and treatment strategies that have yet to be proven, along with an assessment of their impact on outcomes after a TBI.