Evaluation of a Coping Kit of Items to Support Children with Developmental Disorders in the Hospital Setting

This study attempted to answer the question, Do nurses perceive coping kits to be effective at meeting the needs of hospitalized children with developmental disabilities who are at increased risk for challenging behaviors? A cross-sectional post-test survey study design was used, with a convenience sample of 24 registered nurses at a Midwestern free-standing children\u27s hospital. A coping kit with simple communication cards, social script book, and distraction items (toys) was developed to enhance communication and distract children with developmental disabilities (including autism spectrum disorder) undergoing procedures in the hospital. A modified version of Hudson\u27s (2006) intervention effectiveness survey was used to measure the nurse\u27s perception of the effectiveness of the coping kit. Nurses perceived the coping kits to be effective for decreasing their patient\u27s anxiety, calming the child\u27s behavior, and increasing cooperation during procedures. The nurse can develop a plan of care that includes a coping kit to help gain cooperation with the hospitalized child with challenging behaviors

Spitzer Telemetry Processing System

The Spitzer Telemetry Processing System (SirtfTlmProc) was designed to address objectives of JPL's Multi-mission Image Processing Lab (MIPL) in processing spacecraft telemetry and distributing the resulting data to the science community. To minimize costs and maximize operability, the software design focused on automated error recovery, performance, and information management. The system processes telemetry from the Spitzer spacecraft and delivers Level 0 products to the Spitzer Science Center. SirtfTlmProc is a unique system with automated error notification and recovery, with a real-time continuous service that can go quiescent after periods of inactivity. The software can process 2 GB of telemetry and deliver Level 0 science products to the end user in four hours. It provides analysis tools so the operator can manage the system and troubleshoot problems. It automates telemetry processing in order to reduce staffing costs

Physics of the Muon Spectrometer of the ALICE Experiment

The main goal of the Muon spectrometer of the ALICE experiment at LHC is the measurement of heavy quark production in p+p, p+A and A+A collisions at LHC energies, via the muonic channel. Physics motivations and expected performances have been presented in this talk.Comment: 10 pages and 4 figures. Talk presented in the ICPAQGP Conference, February 8-12, 2005, Salt Lake City, Kolkata, India. Web page of the conference : http://www.veccal.ernet.in/~icpaqgp

Superoxide dismutating molecules rescue the toxic effects of PINK1 and parkin loss.

Reactive oxygen species exert important functions in regulating several cellular signalling pathways. However, an excessive accumulation of reactive oxygen species can perturb the redox homeostasis leading to oxidative stress, a condition which has been associated to many neurodegenerative disorders. Accordingly, alterations in the redox state of cells and mitochondrial homeostasis are established hallmarks in both familial and sporadic Parkinson's disease cases. PINK1 and Parkin are two genes which account for a large fraction of autosomal recessive early-onset forms of Parkinson's disease and are now firmly associated to both mitochondria and redox homeostasis. In this study we explored the hypothesis that superoxide anions participate in the generation of the Parkin and PINK1 associated phenotypic effect by testing the capacity of endogenous and exogenous superoxide dismutating molecules to rescue the toxic effects induced by loss of PINK1 or Parkin, in both cellular and fly models. Our results demonstrate the positive effect of an increased level of superoxide dismutase proteins on the pathological phenotypes, both in vitro and in vivo. A more pronounced effectiveness for mitochondrial SOD2 activity points to the superoxide radicals generated in the mitochondrial matrix as the prime suspect in the definition of the observed phenotypes. Moreover, we also demonstrate the efficacy of a SOD-mimetic compound, M40403, to partially ameliorate PINK1/Parkin phenotypes in vitro and in vivo. These results support the further exploration of SOD-mimetic compounds as a therapeutic strategy against Parkinson's disease

Heritability of sleep quality in a middle-aged twin sample from Spain

Study objectives: Sleep quality is associated with health throughout the life span, which is particularly salient in middle-age and older adulthood. Sleep quality appears to be influenced by both genetic and environmental factors. However, there is still limited information about genetic influences on sleep quality in middle-aged adults, and particularly in those from certain geographical locations. We estimated the magnitude of genetic and environmental influences on sleep quality in a representative sample of middle-aged Spanish twins. Methods: The sample comprised 2150 individuals born between 1939 and 1966, who participate in the Murcia Twin Registry. In order to estimate the heritability of sleep quality variables we performed univariate analyses for the global score on the Pittsburgh sleep quality index and for each of its components. Results: We found moderate but significant heritability (34%) for sleep quality. The genetic variance of the components of the Pittsburgh index ranged from 30% to 45%, except for sleep efficiency for which no genetic influence could be detected. In summary, there was a moderate genetic influence on most dimensions of sleep quality in a sample of adult male and female twins. Shared environment influences were not found. Conclusions: This study adds new information regarding the underlying determinants of sleep quality by providing heritability estimates in a middle-aged population-based representative sample from a geographical location that has not been included in studies of this type previously. This could provide a reference point for future research regarding sleep research in middle-age

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

Expansion of the polyglutamine repeat within the protein Huntingtin (Htt) causes Huntington's disease, a neurodegenerative disease associated with aging and the accumulation of mutant Htt in diseased neurons. Understanding the mechanisms that influence Htt cellular degradation may target treatments designed to activate mutant Htt clearance pathways. We find that Htt is phosphorylated by the inflammatory kinase IKK, enhancing its normal clearance by the proteasome and lysosome. Phosphorylation of Htt regulates additional post-translational modifications, including Htt ubiquitination, SUMOylation, and acetylation, and increases Htt nuclear localization, cleavage, and clearance mediated by lysosomal-associated membrane protein 2A and Hsc70. We propose that IKK activates mutant Htt clearance until an age-related loss of proteasome/lysosome function promotes accumulation of toxic post-translationally modified mutant Htt. Thus, IKK activation may modulate mutant Htt neurotoxicity depending on the cell's ability to degrade the modified species

Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey.

Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function. Patients with PIRD develop clinical manifestations associated with diminished and exaggerated immune responses. Management of these patients is complicated; oftentimes immunosuppressive therapies are insufficient, and patients may require hematopoietic cell transplant (HCT) for treatment. Analysis of HCT data in PIRD patients have previously focused on a single gene defect. This study surveyed transplanted patients with a phenotypic clinical picture consistent with PIRD treated in 33 Primary Immune Deficiency Treatment Consortium centers and European centers. Our data showed that PIRD patients often had immunodeficient and autoimmune features affecting multiple organ systems. Transplantation resulted in resolution of disease manifestations in more than half of the patients with an overall 5-years survival of 67%. This study, the first to encompass disorders across the PIRD spectrum, highlights the need for further research in PIRD management

Measurement of χ c1 and χ c2 production with s√ = 7 TeV pp collisions at ATLAS

The prompt and non-prompt production cross-sections for the χ c1 and χ c2 charmonium states are measured in pp collisions at s√ = 7 TeV with the ATLAS detector at the LHC using 4.5 fb−1 of integrated luminosity. The χ c states are reconstructed through the radiative decay χ c → J/ψγ (with J/ψ → μ + μ −) where photons are reconstructed from γ → e + e − conversions. The production rate of the χ c2 state relative to the χ c1 state is measured for prompt and non-prompt χ c as a function of J/ψ transverse momentum. The prompt χ c cross-sections are combined with existing measurements of prompt J/ψ production to derive the fraction of prompt J/ψ produced in feed-down from χ c decays. The fractions of χ c1 and χ c2 produced in b-hadron decays are also measured

Distinct cardiovascular phenotypes are associated with prognosis in systemic sclerosis: a cardiovascular magnetic resonance study

AIMS: Cardiovascular involvement in systemic sclerosis (SSc) is heterogeneous and ill-defined. This study aimed to: (i) discover cardiac phenotypes in SSc by cardiovascular magnetic resonance (CMR); (ii) provide a CMR-based algorithm for phenotypic classification; and (iii) examine for associations between phenotypes and mortality. METHODS AND RESULTS: A retrospective, single-centre, observational study of 260 SSc patients who underwent clinically indicated CMR including native myocardial T1 and T2 mapping from 2016 to 2019 was performed. Agglomerative hierarchical clustering using only CMR variables revealed five clusters of SSc patients with shared CMR characteristics: dilated right hearts with right ventricular failure (RVF); biventricular failure dilatation and dysfunction (BVF); and normal function with average cavity (NF-AC), normal function with small cavity (NF-SC), and normal function with large cavity (NF-LC) sizes. Phenotypes did not co-segregate with clinical or antibody classifications. A CMR-based decision tree for phenotype classification was created. Sixty-three (24%) patients died during a median follow-up period of 3.4 years. After adjustment for age and presence of pulmonary hypertension (PH), independent CMR predictors of all-cause mortality were native T1 (P  0.14). Hazard ratios (HR) were statistically significant for RVF (HR = 8.9, P < 0.001), BVF (HR = 5.2, P = 0.006), and NF-LC (HR = 4.9, P = 0.002) groups. The NF-LC group remained significantly predictive of mortality after adjusting for RVEF, native T1, and PH diagnosis (P = 0.0046). CONCLUSION: We identified five CMR-defined cardiac SSc phenotypes that did not co-segregate with clinical data and had distinct outcomes, offering opportunities for a more precision-medicine based management approach