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Tuberous sclerosis complex is a rare genetic disorder leading to the growth of hamartomas in multiple organs, including cardiac rhabdomyomas. Children with symptomatic cardiac rhabdomyoma require frequent admissions to intensive care units, have major complications, namely, arrhythmias, cardiac outflow tract obstruction and heart failure, affecting the quality of life and taking on high healthcare cost. Currently, there is no standard pharmacological treatment for this condition, and the management includes a conservative approach and supportive care. Everolimus has shown positive effects on subependymal giant cell astrocytomas, renal angiomyolipoma and refractory seizures associated with tuberous sclerosis complex. However, evidence supporting efficacy in symptomatic cardiac rhabdomyoma is limited to case reports. The ORACLE trial is the first randomised clinical trial assessing the efficacy of everolimus as a specific therapy for symptomatic cardiac rhabdomyoma.
ORACLE is a phase II, prospective, randomised, placebo-controlled, double-blind, multicentre protocol trial. A total of 40 children with symptomatic cardiac rhabdomyoma secondary to tuberous sclerosis complex will be randomised to receive oral everolimus or placebo for 3 months. The primary outcome is 50% or more reduction in the tumour size related to baseline. As secondary outcomes we include the presence of arrhythmias, pericardial effusion, intracardiac obstruction, adverse events, progression of tumour reduction and effect on heart failure.
ORACLE protocol addresses a relevant unmet need in children with tuberous sclerosis complex and cardiac rhabdomyoma. The results of the trial will potentially support the first evidence-based therapy for this condition.
The rocky shores of the north-east Atlantic have been long studied. Our focus is from Gibraltar to Norway plus the Azores and Iceland. Phylogeographic processes shape biogeographic patterns of biodiversity. Long-term and broadscale studies have shown the responses of biota to past climate fluctuations and more recent anthropogenic climate change. Inter- and intra-specific species interactions along sharp local environmental gradients shape distributions and community structure and hence ecosystem functioning. Shifts in domination by fucoids in shelter to barnacles/mussels in exposure are mediated by grazing by patellid limpets. Further south fucoids become increasingly rare, with species disappearing or restricted to estuarine refuges, caused by greater desiccation and grazing pressure. Mesoscale processes influence bottom-up nutrient forcing and larval supply, hence affecting species abundance and distribution, and can be proximate factors setting range edges (e.g., the English Channel, the Iberian Peninsula). Impacts of invasive non-native species are reviewed. Knowledge gaps such as the work on rockpools and host–parasite dynamics are also outlined.
Children with CHD and acquired heart disease have unique, high-risk physiology. They may have a higher risk of adverse tracheal-intubation-associated events, as compared with children with non-cardiac disease.
Materials and methods
We sought to evaluate the occurrence of adverse tracheal-intubation-associated events in children with cardiac disease compared to children with non-cardiac disease. A retrospective analysis of tracheal intubations from 38 international paediatric ICUs was performed using the National Emergency Airway Registry for Children (NEAR4KIDS) quality improvement registry. The primary outcome was the occurrence of any tracheal-intubation-associated event. Secondary outcomes included the occurrence of severe tracheal-intubation-associated events, multiple intubation attempts, and oxygen desaturation.
A total of 8851 intubations were reported between July, 2012 and March, 2016. Cardiac patients were younger, more likely to have haemodynamic instability, and less likely to have respiratory failure as an indication. The overall frequency of tracheal-intubation-associated events was not different (cardiac: 17% versus non-cardiac: 16%, p=0.13), nor was the rate of severe tracheal-intubation-associated events (cardiac: 7% versus non-cardiac: 6%, p=0.11). Tracheal-intubation-associated cardiac arrest occurred more often in cardiac patients (2.80 versus 1.28%; p<0.001), even after adjusting for patient and provider differences (adjusted odds ratio 1.79; p=0.03). Multiple intubation attempts occurred less often in cardiac patients (p=0.04), and oxygen desaturations occurred more often, even after excluding patients with cyanotic heart disease.
The overall incidence of adverse tracheal-intubation-associated events in cardiac patients was not different from that in non-cardiac patients. However, the presence of a cardiac diagnosis was associated with a higher occurrence of both tracheal-intubation-associated cardiac arrest and oxygen desaturation.
Our understanding of the complex relationship between schizophrenia symptomatology and etiological factors can be improved by studying brain-based correlates of schizophrenia. Research showed that impairments in value processing and executive functioning, which have been associated with prefrontal brain areas [particularly the medial orbitofrontal cortex (MOFC)], are linked to negative symptoms. Here we tested the hypothesis that MOFC thickness is associated with negative symptom severity.
This study included 1985 individuals with schizophrenia from 17 research groups around the world contributing to the ENIGMA Schizophrenia Working Group. Cortical thickness values were obtained from T1-weighted structural brain scans using FreeSurfer. A meta-analysis across sites was conducted over effect sizes from a model predicting cortical thickness by negative symptom score (harmonized Scale for the Assessment of Negative Symptoms or Positive and Negative Syndrome Scale scores).
Meta-analytical results showed that left, but not right, MOFC thickness was significantly associated with negative symptom severity (βstd = −0.075; p = 0.019) after accounting for age, gender, and site. This effect remained significant (p = 0.036) in a model including overall illness severity. Covarying for duration of illness, age of onset, antipsychotic medication or handedness weakened the association of negative symptoms with left MOFC thickness. As part of a secondary analysis including 10 other prefrontal regions further associations in the left lateral orbitofrontal gyrus and pars opercularis emerged.
Using an unusually large cohort and a meta-analytical approach, our findings point towards a link between prefrontal thinning and negative symptom severity in schizophrenia. This finding provides further insight into the relationship between structural brain abnormalities and negative symptoms in schizophrenia.
To determine parental experiences and preferences regarding the conduct of pediatric research in an emergency department (ED) setting.
We conducted a cross-sectional study of parents of children ages 0 – 14 years who visited the ED of a tertiary care children’s hospital. Parents completed a Web-based survey designed to assess perceptions regarding: 1) background/training of research personnel, 2) location and timing of research discussions, and 3) factors influencing their consent/refusal decision.
Parents totalling 339 were approached, and 227 (67%) surveys were completed. Overall, 87% (197/227; 95% confidence interval [CI] 83, 92) reported they would be comfortable being approached by a university student to discuss research. This proportion did not change when stratified by the child’s gender, illness severity, or season of visit. Whereas only 37% (84/227; 95% CI 31, 43) of respondents would be comfortable being approached in the waiting room, 68% (154/227; 95% CI 62, 75) would be comfortable if approached in a separate area of the main waiting room. The majority reported comfort with follow-up via email (83%; 188/227; 95% CI 78, 88) or telephone (80%; 182/227; 95% CI 75, 85); only 51% (116/227; 95% CI 44, 57) would be comfortable with a scheduled follow-up visit in the hospital. Participants identified potential complications or side effects as the most common reason for declining consent (69%; 157/227; 95% CI 63, 75).
The majority of parents are comfortable being approached by trained university students, preferably in a separate area of an ED waiting room, and email and telephone follow-ups are preferred over a scheduled re-visit.
We present an overview of the survey for radio emission from active stars that has been in progress for the last six years using the observatories at Fleurs, Molonglo, Parkes and Tidbinbilla. The role of complementary optical observations at the Anglo-Australian Observatory, Mount Burnett, Mount Stromlo and Siding Spring Observatories and Mount Tamborine are also outlined. We describe the different types of star that have been included in our survey and discuss some of the problems in making the radio observations.
We present some preliminary results of an optical and radio study of the very active RS CVn binary HD 127535. Photometric measurements show the presence of a large amplitude wave which exhibits marked changes in shape and range on time scales as short as a few months. This photometric variation is almost certainly due to large cool starspots on the cooler, more luminous component. As part of a survey of southern active-chromosphere stars with the Parkes radio telescope, HD 127535 has been observed at 5, 8.4 and 22 GHz. No detection was made at 5 GHz, possibly because of confusion due to the angular proximity of the star to the galatic plane. However, it is one of the strongest sources detected in the 8.4 GHz survey, and is one of only two stars detected at 22 GHz. Photometry obtained two cycles before the 8.4 GHz observations suggest a possible correlation between the radio emission and the photometric wave, i.e. spot visibility, but more data are needed.
We aimed to quantitatively gauge local public health workers’ perceptions toward disaster recovery role expectations among jurisdictions in New Jersey and Maryland affected by Hurricane Sandy.
An online survey was made available in 2014 to all employees in 8 Maryland and New Jersey local health departments whose jurisdictions had been impacted by Hurricane Sandy in October 2012. The survey included perceptions of their actual disaster recovery involvement across 3 phases: days to weeks, weeks to months, and months to years. The survey also queried about their perceptions about future involvement and future available support.
Sixty-four percent of the 1047 potential staff responded to the survey (n=669). Across the 3 phases, 72% to 74% of the pre-Hurricane Sandy hires knew their roles in disaster recovery, 73% to 75% indicated confidence in their assigned roles (self-efficacy), and 58% to 63% indicated that their participation made a difference (response efficacy). Of the respondents who did not think it likely that they would be asked to participate in future disaster recovery efforts (n=70), 39% indicated a willingness to participate.
The marked gaps identified in local public health workers’ awareness of, sense of efficacy toward, and willingness to participate in disaster recovery efforts after Hurricane Sandy represent a significant infrastructural concern of policy and programmatic relevance. (Disaster Med Public Health Preparedness. 2016;10:371–377)
We are developing a purely commensal survey experiment for fast (<5 s) transient radio sources. Short-timescale transients are associated with the most energetic and brightest single events in the Universe. Our objective is to cover the enormous volume of transients parameter space made available by ASKAP, with an unprecedented combination of sensitivity and field of view. Fast timescale transients open new vistas on the physics of high brightness temperature emission, extreme states of matter and the physics of strong gravitational fields. In addition, the detection of extragalactic objects affords us an entirely new and extremely sensitive probe on the huge reservoir of baryons present in the IGM. We outline here our approach to the considerable challenge involved in detecting fast transients, particularly the development of hardware fast enough to dedisperse and search the ASKAP data stream at or near real-time rates. Through CRAFT, ASKAP will provide the testbed of many of the key technologies and survey modes proposed for high time resolution science with the SKA.
EMU is a wide-field radio continuum survey planned for the new Australian Square Kilometre Array Pathfinder (ASKAP) telescope. The primary goal of EMU is to make a deep (rms ∼ 10 μJy/beam) radio continuum survey of the entire Southern sky at 1.3 GHz, extending as far North as +30° declination, with a resolution of 10 arcsec. EMU is expected to detect and catalogue about 70 million galaxies, including typical star-forming galaxies up to z ∼ 1, powerful starbursts to even greater redshifts, and active galactic nuclei to the edge of the visible Universe. It will undoubtedly discover new classes of object. This paper defines the science goals and parameters of the survey, and describes the development of techniques necessary to maximise the science return from EMU.
We present distance determinations to the LMC and SMC based on long period eclipsing binary systems composed of giants. Our LMC distance provides the best zero point for the whole extragalactic distance scale, while the precise relative distance between the LMC and SMC offers unique opportunity to study populational effects on the stellar distance indicators. Apart from distances, analysis of these systems allowed us to measure with a very good accuracy basic stellar parameters (1–2% radii, masses, effective temperature, etc.) of relatively large sample of giants. This catalog provides unique opportunity to calibrate evolutionary models of giant stars.