Ketogenic Diet in Refractory Childhood Epilepsy:Starting With a Liquid Formulation in an Outpatient Setting

Background: Ketogenic diet in children with epilepsy has a considerable impact on daily life and is usually adopted for at least 3 months. Our aim was to evaluate whether the introduction of an all-liquid ketogenic diet in an outpatient setting is feasible, and if an earlier assessment of its efficacy can be achieved. Methods: The authors conducted a prospective, observational study in a consecutive group of children with refractory epilepsy aged 2 to 14 years indicated for ketogenic diet. Ketogenic diet was started as an all-liquid formulation of the classical ketogenic diet, KetoCal 4:1 LQ, taken orally or by tube. After 6 weeks, the liquid diet was converted into solid meals. The primary outcome parameter was time-to-response (>50% seizure reduction). Secondary outcome parameters were time to achieve stable ketosis, the number of children showing a positive response, and the retention rate at 26 weeks. Results: Sixteen children were included. Four of them responded well with respect to seizure frequency, the median time-to-response was 14 days (range 7-28 days). The mean time to achieve stable ketosis was 7 days. The retention rate at 26 weeks was 50%. Of the 8 children who started this protocol orally fed, 6 completed it without requiring a nasogastric tube. Conclusions: Introduction of ketogenic diet with a liquid formulation can be accomplished in orally fed children without major complications. It allowed for fast and stable ketosis

Prospective blinded evaluation of a novel sensing methodology designed to reduce inappropriate shocks by the subcutaneous implantable cardioverter-defibrillator

Background: Most inappropriate shocks from the subcutaneous implantable cardioverter-defibrillator (S-ICD) are caused by cardiac oversensing. A novel sensing methodology, SMART Pass (SP; Boston Scientific Corporation, Natick, MA), aims to reduce cardiac oversensing. Objective: The purpose of this study was to evaluate the effect of SP on shocks in ambulatory patients with S-ICD. Methods: Patients implanted in 2015–2016 and enrolled in a remote patient monitoring system were included and followed for 1 year. Shocks were adjudicated by 3 independent blinded reviewers as appropriate or inappropriate. Shock incidence was calculated for patients with SP programmed enabled or disabled at implantation, censoring patients when SP programming changed or at the last transmission. The SP setting (enabled vs disabled) was modeled as a time-dependent Cox regression variable. Results: The cohort consisted of 1984 patients, and a total of 880 shocks were adjudicated. At implantation, SP was enabled in 655 patients (33%) and disabled in 1329 patients (67%). SP reduced the risk for the first inappropriate shock by 50% (P <.001) and the risk for all inappropriate shocks by 68% (P <.001) in multivariate analysis adjusted for age and device programming. The incidence of inappropriate shocks was 4.3% in the SP enabled arm vs 9.7% in the SP disabled arm. The incidence of appropriate shocks was similar (5.2

a descriptive study on familiarity with, adherence to and trust in the WHO preventive measures

Background Risk communication is a key component of public health interventions during an outbreak. As the coronavirus pandemic unfolded in late 2019, the World Health Organization (WHO) was at the forefront in the development of risk communication strategies. The WHO introduced a range of activities with the purpose of enabling the public to avail verified and timely information on COVID-19 prevention behaviors. Given the various WHO activities to protect the public health during COVID-19, it is important to investigate the extent of familiarity and uptake of the WHO recommendations among the public during the first wave of the pandemic. Methods To do this, we conducted a large-scale Pan-European survey covering around 7500 individuals that are representative of populations from seven European countries, collected online during April 2-April 15, 2020. We use descriptive statistics including proportions and correlations and graphical representations such as bar charts to analyze and display the data. Results Our findings suggest that information from the WHO in the context of COVID-19 is well trusted and acted upon by the public. Overall familiarity and adherence were quite high in most countries. Adherence was higher for social distancing recommendations compared to hygiene measures. Familiarity and adherence were higher among older, female, and highly educated respondents. However, country level heterogeneities were observed in the level of trust in information from the WHO, with countries severely affected by the pandemic reporting lower levels of trust. Conclusion Our findings call for efforts from health authorities to get regular feedback from the public on their familiarity and compliance with recommendations for preventive measures at all stages of the pandemic, to further develop and adapt risk communication as the pandemic evolves.publishersversionpublishe

Psychometric evaluation of the Mental Health Quality of Life (MHQoL) instrument in seven European countries

Introduction: To make efficient use of available resources, decision-makers in healthcare may assess the costs and (health) benefits of health interventions. For interventions aimed at improving mental health capturing the full health benefits is an important challenge. The Mental Health Quality of Life (MHQoL) instrument was recently developed to meet this challenge. Evaluating the pyschometric properties of this instrument in different contexts remains important. Methods: A psychometric evaluation of the MHQoL was performed using existing international, cross-sectional data with 7155 respondents from seven European countries (Denmark, France, Germany, Italy, Portugal, The Netherlands, Portugal and the United Kingdom). Reliability was examined by calculating Cronbach’s alpha, a measure of internal consistency of the seven MHQoL dimensions, and by examining the association of the MHQoL sum scores with the MHQoL-VAS scores. Construct validity was examined by calculating Spearman’s rank correlation coefficients between the MHQoL sum scores and EQ-5D index scores, EQ-VAS scores, EQ-5D anxiety/depression dimension scores, ICECAP-A index scores and PHQ-4 sum scores. Results: The MHQoL was found to have good internal consistency for all seven countries. The MHQoL sum score and the MHQoL-VAS had a high correlation. Spearman’s rank correlation coefficients were moderate to very high for all outcomes. Conclusion: Our results, based on data gathered in seven European countries, suggest that the MHQoL shows favourable psychometrical characteristics. While further validation remains important, the MHQoL may be a useful instrument in measuring mental health-related quality of life in the Western European context

The efficacy of the modified Atkins diet in North Sea Progressive Myoclonus Epilepsy:an observational prospective open-label study

Background: North Sea Progressive Myoclonus Epilepsy is a rare and severe disorder caused by mutations in the GOSR2 gene. It is clinically characterized by progressive myoclonus, seizures, early-onset ataxia and areflexia. As in other progressive myoclonus epilepsies, the efficacy of antiepileptic drugs is disappointingly limited in North Sea Progressive Myoclonus Epilepsy. The ketogenic diet and the less restrictive modified Atkins diet have been proven to be effective in other drug-resistant epilepsy syndromes, including those with myoclonic seizures. Our aim was to evaluate the efficacy of the modified Atkins diet in patients with North Sea Progressive Myoclonus Epilepsy. Results: Four North Sea Progressive Myoclonus Epilepsy patients (aged 7-20 years) participated in an observational, prospective, open-label study on the efficacy of the modified Atkins diet. Several clinical parameters were assessed at baseline and again after participants had been on the diet for 3 months. The primary outcome measure was healthrelated quality of life, with seizure frequency and blinded rated myoclonus severity as secondary outcome measures. Ketosis was achieved within 2 weeks and all patients completed the 3 months on the modified Atkins diet. The diet was well tolerated by all four patients. Health-related quality of life improved considerably in one patient and showed sustained improvement during long-term follow-up, despite the progressive nature of the disorder. Health-related quality of life remained broadly unchanged in the other three patients and they did not continue the diet. Seizure frequency remained stable and blinded rating of their myoclonus showed improvement, albeit modest, in all patients. Conclusions: This observational, prospective study shows that some North Sea Progressive Myoclonus Epilepsy patients may benefit from the modified Atkins diet with sustained health-related quality of life improvement. Not all our patients continued on the diet, but nonetheless we show that the modified Atkins diet might be considered as a possible treatment in this devastating disorder

Femtosecond formation dynamics of the spin Seebeck effect revealed by terahertz spectroscopy.

Understanding the transfer of spin angular momentum is essential in modern magnetism research. A model case is the generation of magnons in magnetic insulators by heating an adjacent metal film. Here, we reveal the initial steps of this spin Seebeck effect with <27 fs time resolution using terahertz spectroscopy on bilayers of ferrimagnetic yttrium iron garnet and platinum. Upon exciting the metal with an infrared laser pulse, a spin Seebeck current js arises on the same ~100 fs time scale on which the metal electrons thermalize. This observation highlights that efficient spin transfer critically relies on carrier multiplication and is driven by conduction electrons scattering off the metal-insulator interface. Analytical modeling shows that the electrons' dynamics are almost instantaneously imprinted onto js because their spins have a correlation time of only ~4 fs and deflect the ferrimagnetic moments without inertia. Applications in material characterization, interface probing, spin-noise spectroscopy and terahertz spin pumping emerge

The learning curve associated with the introduction of the subcutaneous implantable defibrillator

Aims: The subcutaneous implantable cardioverter defibrillator (S-ICD) was introduced to overcome complications related to transvenous leads. Adoption of the S-ICD requires implanters to learn a new implantation technique. The aim of this study was to assess the learning curve for S-ICD implanters with respect to implant-related complications, procedure time, and inappropriate shocks (IASs). Methods and results: In a pooled cohort from two clinical S-ICD databases, the IDE Trial and the EFFORTLESS Registry, complications, IASs at 180 days follow-up and implant procedure duration were assessed. Patients were grouped in quartiles based on experience of the implanter and Kaplan-Meier estimates of complication and IAS rates were calculated. A total of 882 patients implanted in 61 centres by 107 implanters with a median of 4 implants (IQR 1,8) were analysed. There were a total of 59 patients with complications and 48 patients with IAS. The complication rate decreased significantly from 9.8% in Quartile 1 (least experience) to 5.4% in Quartile 4 (most experience) (P = 0.02) and non-significantly for IAS from 7.9 to 4.8% (P = 0.10). Multivariable analysis demonstrated a hazard ratio of 0.78 (P = 0.045) for complications and 1.01 (P = 0.958) for IAS. Dual-zone programming increased with experience of the individual implanter (P 13 implants). Conclusion: There is a short and significant learning curve associated with physicians adopting the S-ICD. Performance stab

Совершенствование механизмов формирования доходов бюджета АРК

Целью нашей работы является исследование механизмов формирования доходов бюджета Автономной Республики Крым и разработка мероприятий по их совершенствованию

Effect of genetically low 25-hydroxyvitamin D on mortality risk: Mendelian randomization analysis in 3 large European cohorts

Source at https://doi.org/10.3390/nu11010074.The aim of this study was to determine if increased mortality associated with low levels of serum 25-hydroxyvitamin D (25(OH)D) reflects a causal relationship by using a Mendelian randomisation (MR) approach with genetic variants in the vitamin D synthesis pathway. Individual participant data from three European cohorts were harmonized with standardization of 25(OH)D according to the Vitamin D Standardization Program. Most relevant single nucleotide polymorphisms of the genes CYP2R1 (rs12794714, rs10741657) and DHCR7/NADSYN1 (rs12785878, rs11234027), were combined in two allelic scores. Cox proportional hazards regression models were used with the ratio estimator and the delta method for calculating the hazards ratio (HR) and standard error of genetically determined 25(OH)D effect on all-cause mortality. We included 10,501 participants (50.1% females, 67.1±10.1 years) of whom 4003 died during a median follow-up of 10.4 years. The observed adjusted HR for all-cause mortality per decrease in 25(OH)D by 20 nmol/L was 1.20 (95% CI: 1.15–1.25). The HR per 20 nmol/L decrease in genetically determined 25(OH)D was 1.32 (95% CI: 0.80–2.24) and 1.35 (95% CI of 0.81 to 2.37) based on the two scores. In conclusion, the results of this MR study in a combined sample from three European cohort studies provide further support for a causal relationship between vitamin D deficiency and increased all-cause mortality. However, as the current study, even with ~10,000 participants, was underpowered for the study of the effect of the allele score on mortality, larger studies on genetics and mortality are needed to improve the precision