Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) – A Polish family with novel SACS mutations

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare hereditary ataxia, characterized by the triad of early-onset cerebellar ataxia, peripheral sensorimotor neuropathy and lower limb spasticity. Although ARSACS is increasingly reported worldwide, we present the first Polish family with a comprehensive clinical and neuropsychological assessment, harboring two novel mutations in the SACS gene. Our results demonstrate the variability in cognitive and behavioral profiles in ARSACS, which is in line with other heredodegenerative ataxias. One should be aware of ARSACS in cases of autosomally recessive inherited ataxias without common mutations

Design status of ASPIICS, an externally occulted coronagraph for PROBA-3

The "sonic region" of the Sun corona remains extremely difficult to observe with spatial resolution and sensitivity sufficient to understand the fine scale phenomena that govern the quiescent solar corona, as well as phenomena that lead to coronal mass ejections (CMEs), which influence space weather. Improvement on this front requires eclipse-like conditions over long observation times. The space-borne coronagraphs flown so far provided a continuous coverage of the external parts of the corona but their over-occulting system did not permit to analyse the part of the white-light corona where the main coronal mass is concentrated. The proposed PROBA-3 Coronagraph System, also known as ASPIICS (Association of Spacecraft for Polarimetric and Imaging Investigation of the Corona of the Sun), with its novel design, will be the first space coronagraph to cover the range of radial distances between ~1.08 and 3 solar radii where the magnetic field plays a crucial role in the coronal dynamics, thus providing continuous observational conditions very close to those during a total solar eclipse. PROBA-3 is first a mission devoted to the in-orbit demonstration of precise formation flying techniques and technologies for future European missions, which will fly ASPIICS as primary payload. The instrument is distributed over two satellites flying in formation (approx. 150m apart) to form a giant coronagraph capable of producing a nearly perfect eclipse allowing observing the sun corona closer to the rim than ever before. The coronagraph instrument is developed by a large European consortium including about 20 partners from 7 countries under the auspices of the European Space Agency. This paper is reviewing the recent improvements and design updates of the ASPIICS instrument as it is stepping into the detailed design phase

The psychological science accelerator’s COVID-19 rapid-response dataset

In response to the COVID-19 pandemic, the Psychological Science Accelerator coordinated three large-scale psychological studies to examine the effects of loss-gain framing, cognitive reappraisals, and autonomy framing manipulations on behavioral intentions and affective measures. The data collected (April to October 2020) included specific measures for each experimental study, a general questionnaire examining health prevention behaviors and COVID-19 experience, geographical and cultural context characterization, and demographic information for each participant. Each participant started the study with the same general questions and then was randomized to complete either one longer experiment or two shorter experiments. Data were provided by 73,223 participants with varying completion rates. Participants completed the survey from 111 geopolitical regions in 44 unique languages/dialects. The anonymized dataset described here is provided in both raw and processed formats to facilitate re-use and further analyses. The dataset offers secondary analytic opportunities to explore coping, framing, and self-determination across a diverse, global sample obtained at the onset of the COVID-19 pandemic, which can be merged with other time-sampled or geographic data

Maintenance of a real estate as a cost of usage of reverse mortgage

Obtaining a reverse mortgage is related to the obligation of maintaining a proper technical condition of real estate which is a security for the repayment of the credit. The debtor has to bear the costs of necessary repairs and renovations throughout the duration of the contract, which in fact reduce the economic value of credit amount. The article is an attempt to analyse legal regulations applicable in Poland if it comes to detailed specification of the debtor’s responsibilities connected with the maintenance of real estate, as well as guaranteeing the potential debtor comprehensive information on this issue. It will also be analysed whether the legislator provided sufficient measures to protect the debtor in case of inability to meet the obligation in question, resulting from health or financial reasons. The considerations are conducted on the assumption that the debtor is an elderly person considering a reverse mortgage mostly as a measure required to fill in the deficits in the household budget. As can be seen from the analysis, Polish legislator in the Act on reverse mortgage did not properly take into account the importance of the debtor’s obligation to maintain the property in a non-deteriorated condition, which, as opposed to the assumptions underlying the Act on reverse mortgage, may result in the service that is a high risk transaction for elderly people.Z zaciągnięciem odwróconego kredytu hipotecznego związany jest obowiązek utrzymania w należytym stanie technicznym nieruchomości stanowiącej zabezpieczenie jego spłaty. Kredytobiorca przez cały okres trwania umowy musi ponosić koszty niezbędnych napraw i remontów, które faktycznie pomniejszają wartość ekonomiczną uzyskanej kwoty kredytu. Artykuł stanowi próbę analizy obowiązujących w Polsce regulacji prawnych pod kątem szczegółowości określenia zakresu obowiązków kredytobiorcy związanych z utrzymaniem nieruchomości, jak również zagwarantowania potencjalnemu kredytobiorcy wyczerpującej informacji w tym przedmiocie. Przeanalizowane zostanie także, czy ustawodawca przewidział wystarczające środki ochrony kredytobiorcy w przypadku niemożności zadośćuczynienia omawianemu zobowiązaniu, wynikającej z przyczyn zdrowotnych lub finansowych. Rozważania prowadzone są przy założeniu, że kredytobiorcą będzie osoba starsza, upatrująca w odwróconym kredycie hipotecznym przede wszystkim uzupełnienia braków w budżecie domowym. Z przeprowadzonej analizy wynika, że polski ustawodawca w ustawie o odwróconym kredycie hipotecznym nie uwzględnił w należyty sposób wagi obowiązku kredytobiorcy do utrzymania nieruchomości w stanie niepogorszonym, co wbrew założeniom leżącym u podstaw uchwalenia ustawy o odwróconym kredycie hipotecznym może spowodować, że usługa ta będzie dla seniora transakcją wysokiego ryzyka

Maintenance of a real estate as a cost of usage of reverse mortgage

Obtaining a reverse mortgage is related to the obligation of maintaining a proper technical condition of real estate which is a security for the repayment of the credit. The debtor has to bear the costs of necessary repairs and renovations throughout the duration of the contract, which in fact reduce the economic value of credit amount. The article is an attempt to analyse legal regulations applicable in Poland if it comes to detailed specification of the debtor’s responsibilities connected with the maintenance of real estate, as well as guaranteeing the potential debtor comprehensive information on this issue. It will also be analysed whether the legislator provided sufficient measures to protect the debtor in case of inability to meet the obligation in question, resulting from health or financial reasons. The considerations are conducted on the assumption that the debtor is an elderly person considering a reverse mortgage mostly as a measure required to fill in the deficits in the household budget. As can be seen from the analysis, Polish legislator in the Act on reverse mortgage did not properly take into account the importance of the debtor’s obligation to maintain the property in a non-deteriorated condition, which, as opposed to the assumptions underlying the Act on reverse mortgage, may result in the service that is a high risk transaction for elderly people.Z zaciągnięciem odwróconego kredytu hipotecznego związany jest obowiązek utrzymania w należytym stanie technicznym nieruchomości stanowiącej zabezpieczenie jego spłaty. Kredytobiorca przez cały okres trwania umowy musi ponosić koszty niezbędnych napraw i remontów, które faktycznie pomniejszają wartość ekonomiczną uzyskanej kwoty kredytu. Artykuł stanowi próbę analizy obowiązujących w Polsce regulacji prawnych pod kątem szczegółowości określenia zakresu obowiązków kredytobiorcy związanych z utrzymaniem nieruchomości, jak również zagwarantowania potencjalnemu kredytobiorcy wyczerpującej informacji w tym przedmiocie. Przeanalizowane zostanie także, czy ustawodawca przewidział wystarczające środki ochrony kredytobiorcy w przypadku niemożności zadośćuczynienia omawianemu zobowiązaniu, wynikającej z przyczyn zdrowotnych lub finansowych. Rozważania prowadzone są przy założeniu, że kredytobiorcą będzie osoba starsza, upatrująca w odwróconym kredycie hipotecznym przede wszystkim uzupełnienia braków w budżecie domowym. Z przeprowadzonej analizy wynika, że polski ustawodawca w ustawie o odwróconym kredycie hipotecznym nie uwzględnił w należyty sposób wagi obowiązku kredytobiorcy do utrzymania nieruchomości w stanie niepogorszonym, co wbrew założeniom leżącym u podstaw uchwalenia ustawy o odwróconym kredycie hipotecznym może spowodować, że usługa ta będzie dla seniora transakcją wysokiego ryzyka

Invasive electrophysiologyThe vein of Marshall as a source of various types of atrial arrhythmias, including adrenergic paroxysmal atrial fibrillation – successful catheter ablation

We describe a case of a 44-year old man with recurrent atrial arrhythmias (atrial ectopy, atrial tachycardia and fibrillation) originating from the vein of Marshall (VoM). Stress, exertion and isoproterenol increased frequency and duration of highly symptomatic arrhythmia paroxysms. In 2002 he underwent two sessions of RF ablation. In the area of the left inferior pulmonary vein ostium (LIPV) and along the course the VoM, spiky, firing potentials were recorded. Ablation of the VoM region induced high frequency focal firing (CL~200 ms) with 1: 1 VoM ® LA conduction. Before sinus rhythm return, a gradual increase of conduction of VoM ® LA block was observed. During the second session residual breakthroughs were recorded. During 5 years of follow-up the patient remained free from arrhythmia without antiarrhythmic drugs

Microfluidic Method of Pig Oocyte Quality Assessment in relation to Different Follicular Size Based on Lab-on-Chip Technology

Since microfollicular environment and the size of the follicle are important markers influencing oocyte quality, the aim of this study is to present the spectral characterization of oocytes isolated from follicles of various sizes using lab-on-chip (LOC) technology and to demonstrate how follicle size may affect oocyte quality. Porcine oocytes (each, n=100) recovered from follicles of different sizes, for example, from large (>5 mm), medium (3–5 mm), and small (<3 mm), were analyzed after preceding in vitro maturation (IVM). The LOC analysis was performed using a silicon-glass sandwich with two glass optical fibers positioned “face-to-face.” Oocytes collected from follicles of different size classes revealed specific and distinguishable spectral characteristics. The absorbance spectra (microspectrometric specificity) for oocytes isolated from large, medium, and small follicles differ significantly (P<0.05) and the absorbance wavelengths were between 626 and 628 nm, between 618 and 620 nm, and less than 618 nm, respectively. The present study offers a parametric and objective method of porcine oocyte assessment. However, up to now this study has been used to evidence spectral markers associated with follicular size in pigs, only. Further investigations with functional-biological assays and comparing LOC analyses with fertilization and pregnancy success and the outcome of healthy offspring must be performed