Search

Magnetotellurics (MT) is widely used to image volcanic regions' three-dimensional (3-D) electrical conductivity structures. This requires a robust modeling solver which can deal with models with large conductivity contrast between melts and solid rock as well as topography. To this end, we developed a parallel 3-D MT forward modeling tool using the adaptive finite element method based on the open-source and scalar C++ library called the modular finite element method (MFEM). Tetrahedral grids were adopted to discretize the whole modeling domain, which can deal with complex underground structures with arbitrary topography. The flexible generalized minimum residual solver (FGMRES) with an auxiliary Maxwell solver pre-conditioner was applied to solve the final large-scale system of linear equations. We performed several experiments on synthetic models to verify the accuracy and efficiency of the developed tool. Then, we applied the tool to calculate the MT responses (impedance and tipper) at Mount St. Helens taking into account topography in the modeling to demonstrate the capability of the developed tool for dealing with complex models. The tool will be incorporated into the inversion tool JIF3D and substantially open-access., The 28th IUGG General Assembly (IUGG2023) (Berlin 2023)

Detection of geophysical signatures associated with a geologic event, such as a volcanic eruption, is key to understanding the underlying physical processes and making an accurate hazard assessment. Magma reservoirs are the main repositories for eruptible magma, and understanding them requires the ability to detect and interpret changes in the magmatic system from surface measurements. Traditionally, monitoring for these changes has been done with seismic and geodetic approaches, both of which require dynamic ‘active’ changes within the magmatic system. Neither of these techniques is sensitive to the petrology or temperature of the magma though. Thus, additional monitoring techniques able to detect ‘static’ phase changes in the evolving magma and the thermal structure of the magma reservoir are needed. The magnetotelluric method, measures subsurface electrical properties and is sensitive to both ‘magma on the move’ and petrological changes that occur within the magma reservoir. Using Mount St Helens where a detailed magnetotelluric survey was completed during the most recent dome building eruptive phase 2005-06, and is now in a period of quiescence, we compare the original measurements to repeated measurements in the same locations in 2022 to develop temporal analysis approaches required for monitoring. In addition to the repeat campaign we have deployed 4 long-term continuous monitoring stations with telemetry to local servers. First, qualitative, comparisons of the data from different time periods indicate some significant changes in subsurface conductivity. We present an overview of the newly acquired data and the monitoring setup and discuss where the most significant changes occur., The 28th IUGG General Assembly (IUGG2023) (Berlin 2023)

Rationale Primary ciliary dyskinesia is a genetically heterogeneous inherited condition characterised by progressive lung disease arising from abnormal cilia function. Approximately half of patients have situs inversus. The estimated prevalence of primary ciliary dyskinesia in the UK South Asian population is 1:2265. Early, accurate diagnosis is key to implementing appropriate management but clinical diagnostic tests can be equivocal. Objectives To determine the importance of genetic screening for primary ciliary dyskinesia in a UK South Asian population with a typical clinical phenotype, where standard testing is inconclusive. Methods Next-generation sequencing was used to screen 86 South Asian patients who had a clinical history consistent with primary ciliary dyskinesia. The effect of a CCDC103 p.His154Pro missense variant compared with other dynein arm-associated gene mutations on diagnostic/phenotypic variability was tested. CCDC103 p.His154Pro variant pathogenicity was assessed by oligomerisation assay. Results Sixteen of 86 (19%) patients carried a homozygous CCDC103 p.His154Pro mutation which was found to disrupt protein oligomerisation. Variable diagnostic test results were obtained including normal nasal nitric oxide levels, normal ciliary beat pattern and frequency and a spectrum of partial and normal dynein arm retention. Fifteen (94%) patients or their sibling(s) had situs inversus suggesting CCDC103 p.His154Pro patients without situs inversus are missed. Conclusions The CCDC103 p.His154Pro mutation is more prevalent than previously thought in the South Asian community and causes primary ciliary dyskinesia that can be difficult to diagnose using pathology-based clinical tests. Genetic testing is critical when there is a strong clinical phenotype with inconclusive standard diagnostic tests.

Understanding the architecture of the West African craton at depth is essential to be able to reconstruct its evolution. Our study focuses on the crustal imaging of structures and geometries characterizing the crust of the Leo-Man shield with broadband and long period magnetotelluric data collected in southern Burkina Faso and covering a 220 km long profile. The resulting 3D resistivity crustal model highlights the distribution of the granite-greenstone assemblages with depth showing excellent correlation with mapped surficial lithologies. The whole crust of southern Burkina Faso is resistive, with lateral as opposed to vertical major resistivity contrasts, reflecting the location of major-scale shear zones characterizing this part of the Baoule-Mossi domain. Ground gravity data acquired along the same line as the MT data were also modeled and show relatively good correspondence with the resistivity model. The new resistivity and gravity models compared with results from joint inversion of gravity and aeromagnetic data highlight significant changes between the greenstone belts and granitoid domains along the profile. The comparison of the geophysics with the geology enables us to define new depth constraints of the main tectonic features in the area. The observed large scale dipping shear zones favour the model of crustal building through major parallel thrust faults.

The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "gold standard" reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments. The guideline is based on pre-defined questions relevant for clinical care, a systematic review of the literature, and assessment of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach. It focuses on clinical presentation, nasal nitric oxide, analysis of ciliary beat frequency and pattern by high-speed video-microscopy analysis, transmission electron microscopy, genotyping and immunofluorescence. It then used a modified Delphi survey to develop an algorithm for the use of diagnostic tests to definitively confirm and exclude the diagnosis of primary ciliary dyskinesia; and to provide advice when the diagnosis was not conclusive. Finally, this guideline proposes a set of quality criteria for future research on the validity of diagnostic methods for primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is an inherited autosomal-recessive disorder of motile cilia characterised by chronic lung disease, rhinosinusitis, hearing impairment and subfertility. Nasal symptoms and respiratory distress usually start soon after birth, and by adulthood bronchiectasis is invariable. Organ laterality defects, usually situs inversus, occur in ∼50% of cases. The estimated prevalence of PCD is up to ∼1 per 10 000 births, but it is more common in populations where consanguinity is common. This review examines who to refer for diagnostic testing. It describes the limitations surrounding diagnosis using currently available techniques and considers whether recent advances to genotype patients with PCD will lead to genetic testing and screening to aid diagnosis in the near future. It discusses the challenges of monitoring and treating respiratory and ENT disease in children with PCD.

RATIONALE: Spirometry in children with cystic fibrosis (CF) frequently fails to return to baseline after treatment for a pulmonary exacerbation. It is unclear whether the same is true for children with primary ciliary dyskinesia (PCD). OBJECTIVES: To determine in children with PCD treated with intravenous antibiotics for a pulmonary exacerbation: (1) the proportion who recover to baseline forced expiratory volume at 1 sec (FEV1 ) within 3 months after treatment and (2) to try to identify factors which are associated with failure to regain pre-exacerbation FEV1 . METHODS: Cohort study using the PCD database for children at the Royal Brompton Hospital, 2003-2013. We selected the first pulmonary exacerbation treated with intravenous antibiotics. The best FEV1 within 3 months after treatment was compared to the best FEV1 in the 12 months before treatment (baseline). Recovery to baseline was defined as any FEV1 after treatment that was greater than or equal to 90% of the baseline FEV1 . RESULTS: 32/150 children (21%) had at least one pulmonary exacerbation. 23/30 (77%) regained baseline spirometry within 3 months of treatment. There was no difference between responders and non-responders in any baseline characteristics. CONCLUSIONS: Around 25% of children with PCD fail to recover to baseline lung function within 3 months following treatment for a pulmonary exacerbation, similar to CF. Better treatment strategies are needed, and the results also suggest that prevention of exacerbations would be a useful end-point in clinical trials. Pediatr Pulmonol. 2016;9999:XX-XX. © 2016 Wiley Periodicals, Inc.

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course. The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course. To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22). Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care. © ERS 2016.

Global pollination is threatened by declining insect pollinator populations that may be linked to neonicotinoid pesticide use. Neonicotinoids over stimulate neurons and depolarize their mitochondria, producing immobility and death. However, mitochondrial function can be improved by near infrared light absorbed by cytochrome c oxidase in mitochondrial respiration. In flies, daily exposure to 670nm light throughout life increases average lifespan and aged mobility, and reduces systemic inflammation. Here we treat bumble bees with Imidacloprid a common neonicotinoid. This undermined ATP and rapidly induced immobility and reduced visual function and survival. Bees exposed to insecticide and daily to 670nm light showed corrected ATP levels and significantly improved mobility allowing them to feed. Physiological recordings from eyes revealed that light exposure corrected deficits induced by the pesticide. Overall, death rates in bees exposed to insecticide but also given 670nm light were indistinguishable from controls. When Imidacloprid and light exposure were withdrawn, survival was maintained. Bees and insects generally cannot see deep red light so it does not disturb their behaviour. Hence, we show that deep red light exposure that improves mitochondrial function, reverses the sensory and motor deficits induced by Imidacloprid. These results may have important implications as light delivery is economic and can be placed in hives/colonies.

Introduction and objectives Mutation type may affect clinical phenotype in PCD, as shown by differences in forced expiratory volume in 1 s (FEV1) (AJRCCM 2015;191:316–324). LCI, measured using multi-breath washout (MBW) is raised in PCD (AJRCCM 2013;188:545–549) but the relative sensitivities of the two physiological measurements is disputed (Thorax 2015;70:339–345, and 305–306). We hypothesised that LCI would be more sensitive to genotype-phenotype differences in PCD. Methods MBW (using sulphur hexafluoride MBW with a photoacoustic gas analyser) and spirometry were performed in 77 PCD patients (mean age 16.4 years (range 4–62.2), 33 males, mean FEV1 z score -2.09 (range -5.33–1.59)). 44 had outer dynein arms (ODA) defects, or both inner (IDA) and ODA, 18 had microtubular defects (either transposition or microtubule disorganisation with absent IDA), 15 had normal ultrastructure (diagnosis made on either genetics (n = 10), low nasal NO, clinical phenotype and consistent dyskinesia on light microscopy (n = 1), or low nasal NO, clinical phenotype and an affected sibling (n = 3)). There was no significant difference in age or gender composition between the 3 groups. Results Patients with normal ultrastructure had significantly higher FEV1 and lower LCI, indicating milder disease. Those with ODA +/- IDA had a more normal LCI than those with microtubular defects (Figure 1), but similar FEV1.

The mammalian visual range is approximately 400-700nm, although recent evidence suggests varying ultraviolet (UV) extensions in diverse terrestrial species. UV sensitivity may have advantages in the dim, blue light shifted environment experienced by submerged marine mammals. It may also be advantageous when seals are hauled out as UV is reflected by snow and ice but absorbed by fur, enhancing visual contrast. Here we show that the pelagic hooded seal (Cystophora cristata) has a highly UV permissive cornea and lens. Seals like other carnivores have a tapetum lucidum (TL) reflecting light back through the retina increasing sensitivity. The TL in this seal is unusual being white and covering almost the entire retina unlike that in other carnivores. Spectral reflectance from its surface selectively increases the relative UV/blue components >10 times than other wavelengths. Retinal architecture is consistent with a high degree of convergence. Enhanced UV from a large TL surface with a high degree of retinal convergence will increase sensitivity at a cost to acuity. UV electrophysiological retina responses were only obtained to dim, rod mediated stimuli, with no evidence of cone input. As physiological measurements of threshold sensitivity are log units higher than those for psychophysical detection, these seals are likely to be more UV sensitive than our results imply. Hence, UV reflections from the TL will afford increased sensitivity in dim oceanic environments.

Primary ciliary dyskinesia (PCD) is an inherited chronic respiratory obstructive disease with randomized body laterality and infertility, resulting from cilia and sperm dysmotility. PCD is characterized by clinical variability and extensive genetic heterogeneity, associated with different cilia ultrastructural defects and mutations identified in >20 genes. Next generation sequencing (NGS) technologies therefore present a promising approach for genetic diagnosis which is not yet in routine use. We developed a targeted panel-based NGS pipeline to identify mutations by sequencing of selected candidate genes in 70 genetically undefined PCD patients. This detected loss-of-function RSPH1 mutations in four individuals with isolated central pair (CP) agenesis and normal body laterality, from two unrelated families. Ultrastructural analysis in RSPH1-mutated cilia revealed transposition of peripheral outer microtubules into the ‘empty’ CP space, accompanied by a distinctive intermittent loss of the central pair microtubules. We find that mutations in RSPH1, RSPH4A and RSPH9, which all encode homologs of components of the ‘head’ structure of ciliary radial spoke complexes identified in Chlamydomonas, cause clinical phenotypes that appear to be indistinguishable except at the gene level. By high-resolution immunofluorescence we identified a loss of RSPH4A and RSPH9 along with RSPH1 from RSPH1-mutated cilia, suggesting RSPH1 mutations may result in loss of the entire spoke head structure. CP loss is seen in up to 28% of PCD cases, in whom laterality determination specified by CP-less embryonic node cilia remains undisturbed. We propose this defect could arise from instability or agenesis of the ciliary central microtubules due to loss of their normal radial spoke head tethering.

Common outcome scales in acute stroke trials are ordered categorical or pseudocontinuous in structure but most have been analyzed as binary measures. The use of fixed dichotomous analysis of ordered categorical outcomes after stroke (such as the modified Rankin Scale) is rarely the most statistically efficient approach and usually requires a larger sample size to demonstrate efficacy than other approaches. Preferred statistical approaches include sliding dichotomous, ordinal, or continuous analyses. Because there is no best approach that will work for all acute stroke trials, it is vital that studies are designed with a full understanding of the type of patients to be enrolled (in particular their case mix, which will be critically dependent on their age and severity), the potential mechanism by which the intervention works (ie, will it tend to move all patients somewhat, or some patients a lot, and is a common hazard present), a realistic assessment of the likely effect size, and therefore the necessary sample size, and an understanding of what the intervention will cost if implemented in clinical practice. If these approaches are followed, then the risk of missing useful treatment effects for acute stroke will diminish.

No abstract available.

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare hereditary recessive disease with symptoms of recurrent pneumonia, chronic bronchitis, bronchiectasis, and chronic sinusitis. Chronic rhinitis is often the presenting symptom in newborns and infants. Approximately half of the patients show visceral mirror image arrangements (situs inversus). In this study, we aimed 1) to determine the number of paediatric PCD patients in Austria, 2) to show the diagnostic and therapeutic modalities used in the clinical centres and 3) to describe symptoms of children with PCD. PATIENTS, MATERIAL AND METHODS: For the first two aims, we analysed data from a questionnaire survey of the European Respiratory Society (ERS) task force on Primary Ciliary Dyskinesia in children. All paediatric respiratory units in Austria received a questionnaire. Symptoms of PCD patients from Vienna Children's University Hospital (aim 3) were extracted from case histories. RESULTS: In 13 Austrian clinics 48 patients with PCD (36 aged from 0–19 years) were identified. The prevalence of reported cases (aged 0–19 yrs) in Austria was 1:48000. Median age at diagnosis was 4.8 years (IQR 0.3–8.2), lower in children with situs inversus compared to those without (3.1 vs. 8.1 yrs, p = 0.067). In 2005–2006, the saccharine test was still the most commonly used screening test for PCD in Austria (45%). Confirmation of the diagnosis was usually by electron microscopy (73%). All clinics treated exacerbations immediately with antibiotics, 73% prescribed airway clearance therapy routinely to all patients. Other therapies and diagnostic tests were applied very inconsistently across Austrian hospitals. All PCD patients from Vienna (n = 13) had increased upper and lower respiratory secretions, most had recurring airway infections (n = 12), bronchiectasis (n = 7) and bronchitis (n = 7). CONCLUSION: Diagnosis and therapy of PCD in Austria are inhomogeneous. Prospective studies are needed to learn more about the course of the disease and to evaluate benefits and harms of different treatment strategies.

The feasibility is investigated of a dedicated trigger stream in the CMS trigger menu, with an output rate of cal O (1)~kHz on the First Level Trigger and cal O (1)~Hz on the High Level Trigger. By combining jet trigger information from the CMS calorimeter with information from the TOTEM Roman Pot detectors at 220 m distance from the interaction point, the default dijet trigger thresholds foreseen in the CMS trigger tables can be lowered substantially while respecting the CMS trigger bandwidth limits. The efficacy of the dedicated diffractive trigger stream is demonstrated for hard single-diffractive and double-Pomeron exchange events.