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efficacy/effectiveness and convenient administration/dosing of canakinumab were the most common reasons for canakinumab initiation among children, whereas lack-of-response to previous treatment and ability to discontinue/spare steroids being the most frequent reasons among adults.

In US clinical practice, physician perceived/experienced efficacy/effectiveness of canakinumab and lack-of-response to previous treatment were the primary reasons for canakinumab initiation among patients with SJIA/AOSD. Physician perceived/experienced efficacy/effectiveness and convenient administration/dosing of canakinumab were the most common reasons for canakinumab initiation among children, whereas lack-of-response to previous treatment and ability to discontinue/spare steroids being the most frequent reasons among adults.

Many studies have been conducted worldwide to estimate herpes zoster (HZ) incidence rates. We synthesized studies of HZ incidence rates in the general population using meta-analysis models.

A random effects meta-analysis was conducted to estimate HZ incidence from a published worldwide systematic literature review (SLR) including only individuals aged 50years and older. Meta-regression was used to explore whether variability in incidence rates could be explained by a combination of study-specific characteristics including age, gender, continent and year of study data. The impact of adding additional covariates-case detection method (general practitioner surveillance, healthcare database, sentinel network, etc.), case definition (medical record-based, self-reported), study design (retrospective passive surveillance, retrospective active surveillance, etc.), incidence type (cumulative incidence/1000 persons or incidence rate/1000 person-years), patient type (outpatients or in- and out-patients) and latitudef study data.

Prevalence and burden of headache disorders in real-world settings is relatively unstudied. We explored the associations between passively collected activity data, headache burden, and quality of life in headache sufferers.

Data from wearable activity tracking devices and daily short questionnaires were collected over 12weeks to assess occurrence of headache, activity, quality of life and self-rated health. Variables were analyzed using a series of mixed-effects models and stratified based on headache type. Multiple linear and logistic regressions were used to analyze treatment preferences.

Behaviors inferred from activity tracker data suggested that individuals slept more, had reduced physical activity, and had lower maximum heart rate on days with headache. As headache-specific impact on quality of life increased, activity and maximum heart rate decreased and sleep increased. Headache days with higher self-rated health were associated with less napping, higher step count and maximum heart rate, correlating with increased activity. Migraineurs experienced greater burden in everyday life compared with tension-type headache sufferers.

This study adds to existing evidence that activity trackers can be used to quantify headache burden in real-world settings and aid in understanding symptom management.

This study adds to existing evidence that activity trackers can be used to quantify headache burden in real-world settings and aid in understanding symptom management.

Hemophilia A (HA) is a rare disease that is characterized by congenital underproduction or dysfunction of the endogenous coagulation factor VIII (FVIII). The aim of the present study was to determine the value of prophylaxis versus on-demand treatment strategies for moderate to severe HA (MtSHA) patients and the value of emicizumab in the prophylaxis of MtSHA in Greece, compared with short half-life (SHL) FVIII and extended half-life (EHL) FVIII through multicriteria decision analysis (MCDA).

A literature review was performed to identify a set of criteria relevant to the therapeutic approaches and therapies under investigation. A performance matrix was populated by two literature reviews and meta-analyses. The criteria selected were hierarchically classified by allocating weights on a 0-100 scale. The performances of therapies were scored at the 100-point scale. The value judgments utilized for weighing and scoring were sourced via a survey among independent multidisciplinary system stakeholders. A linearizumab adds higher value and improves patient QoL compared with replacement therapy for MtSHA in Greece. Emicizumab addresses important unmet needs due to its improved efficacy and mode of administration.

This MCDA has shown that the prophylaxis strategy was perceived as a more valuable option for managing MtSHA patients when compared with the on-demand strategy. Moreover, emicizumab adds higher value and improves patient QoL compared with replacement therapy for MtSHA in Greece. Emicizumab addresses important unmet needs due to its improved efficacy and mode of administration.

To describe the clinical course of COVID-19 in patients with cystic fibrosis (CF) and to identify risk factors for severe COVID-19.

We conducted a prospective study within the Italian CF Society. CF centers collected baseline and follow-up data of patients with virologically confirmed SARS-CoV-2 infection between March 2020 and June 2021. Odds ratios (ORs) for severe SARS-CoV-2 (as defined by hospital admission) were estimated by logistic regression models.

The study included 236 patients with positive molecular test for SARS-CoV-2. Six patients died, 43 patients were admitted to hospital, 4 admitted to intensive care unit. Pancreatic insufficiency was associated with increased risk of severe COVID-19 (OR 4.04, 95% CI 1.52; 10.8). After adjusting for age and pancreatic insufficiency, forced expiratory volume in one second (FEVp) < 40% (OR 4.54, 95% CI 1.56; 13.2), oxygen therapy (OR 12.3, 95% CI 2.91-51.7), underweight (OR 2.92, 95% CI 1.12; 7.57), organ transplantation (OR 7.31, 95% CI 2.59; 20.7), diabetes (OR 2.67, 95% CI 1.23; 5.80) and liver disease (OR 3.67, 95% CI 1.77; 7.59) were associated with increased risk of severe COVID-19, while use of dornase alfa was associated with a reduced risk (OR 0.34, 95% CI 0.13-0.88). No significant changes were observed in FEVp from baseline to a median follow-up of 2months (median difference 0, interquartile range -4; 5, P = 0.62).

Clinical features indicative of severe form of CF are associated with increased risk of COVID-19 hospitalization. SARS-CoV-2 infected patients do not experience a deterioration of respiratory function.

Clinical features indicative of severe form of CF are associated with increased risk of COVID-19 hospitalization. SARS-CoV-2 infected patients do not experience a deterioration of respiratory function.Cerebrospinal fluid (CSF) neurofilament light protein (NfL) is a marker of central nervous system neuro-axonal injury. A novel, ultra-sensitive assay can determine plasma NfL. In untreated people-with-HIV (PWH), CSF and plasma NfL are strongly correlated. We aimed to assess this correlation in PWH on suppressive antiretroviral treatment (ART) and lifestyle-similar HIV-negative individuals enrolled into the COmorBidity in Relation to AIDS (COBRA) study. Differences in paired CSF (sandwich ELISA, UmanDiagnostics) and plasma (Simoa digital immunoassay, Quanterix™) NfL between PWH and HIV-negative participants were tested using Wilcoxon's test; associations were assessed using Pearson's correlation. CSF and plasma NfL, standardised to Z-scores, were included as dependent variables in linear regression models to identify factors independently associated with values in PWH and HIV-negative participants. Overall, 132 PWH (all with plasma HIV RNA  less then  50 copies/mL) and 79 HIV-negative participants were included. Neither CSF (median 570 vs 568 pg/mL, p = 0.37) nor plasma (median 10.7 vs 9.9 pg/mL, p = 0.15) NfL differed significantly between PWH and HIV-negative participants, respectively. CSF and plasma NfL correlated moderately, with no significant difference by HIV status (PWH rho = 0.52; HIV-negative participants rho = 0.47, p (interaction) = 0.63). In multivariable regression analysis, higher CSF NfL Z-score was statistically significantly associated with older age and higher CSF protein, and higher plasma NfL Z-score with older age, higher serum creatinine and lower bodyweight. In conclusion, in PWH on ART, the correlation between CSF and plasma NfL is moderate and similar to that observed in lifestyle-similar HIV-negative individuals. Consideration of renal function and bodyweight may be required when utilising plasma NfL.Progressive multifocal leukoencephalopathy (PML) is a rare but devastating neurological disease caused by reactivation of the JC virus in susceptible individuals. The illness has classically been associated with the human immunodeficiency virus (HIV) and multiple sclerosis (MS) patients who are treated with natalizumab. It is also associated with haematological malignancies, organ transplantation, autoimmune disease and immunodeficiency. Aside from natalizumab, a range of other immunomodulators including obinutuzumab and rituximab have been associated with PML. The nature of these associations is unclear due to the overall low incidence of PML associated with these drugs and the fact that most patients will have other confounding risk factors for developing the disease. There is no known effective treatment available for PML in the non-HIV, non-MS cohort. Recent case studies and series have proposed that pembrolizumab, an anti-PD-1 immune checkpoint inhibitor, may be a potentially efficacious option for these patients. We present two cases of non-HIV, non-MS patients with PML who were treated with pembrolizumab with little clinical benefit. The literature surrounding pembrolizumab use in PML is discussed, with a focus on potential indicators of successful outcomes for patients who receive this therapy.We present PuPl (Pu pillometry P ipel iner), an Octave-compatible library of Matlab functions for processing pupillometry data with an easy-to-use graphical user interface (GUI). PuPl's preprocessing tools include blink correction, data smoothing, and gaze correction. PuPl can also define and sort trials, and segment data to isolate event-related pupil dilation responses. selleck chemicals PuPl's flexible tabular export tools enable a wide variety of statistical analyses. Furthermore, PuPl can translate GUI interactions into a Matlab script, enabling easy creation and reconfiguration of reusable data processing pipelines. Finally, PuPl is designed to be extensible, and users can easily contribute functionality as best practices for pupillometry evolve. Here we demonstrate PuPl by replicating published results using publicly available data. PuPl can be downloaded from github.com/kinleyid/pupl .

Engaging influential stakeholders in meaningful exchange is essential for pharmaceutical companies aiming to improve care. At a time where opportunities for face-to-face engagement are limited, the ability to interact,learnand generate actionable insights through digital channels such as Twitter, is of considerable value.

The aim of this study was to evaluate digital engagement among global diabetes mellitus researchers.

We identified every global tweet (20,614,515) and scientific publication (44,135) regarding diabetes mellitus from 1 August 2018 to 1 August 2020. Through author matching we combined datasets, resulting in a list of digitally active scientific authors. Generalised linear modelling identified factors predicting their digital engagement.

Globally, 2686 diabetes researchers used Twitter to discuss the management of diabetes mellitus, posting 110,346 diabetes-related tweets. As Twitter followers increased, so did tweet frequency (p<0.001), retweets (p<0.001) and replies (p<0.001) to their content.