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Summary: Recessive dystrophic epidermolysis bullosa (RDEB), Kindler syndrome (KS) and Xeroderma pigmentosum C (XPC) are three genetic skin diseases (i.e. genodermatoses) in which patients are at increased risk of developing cancer. The events leading to cancer in these diseases, including those taking place at the level of the skin, are still not well understood even though they are under intense investigation. To deepen the knowledge of these rare diseases with the ultimate goal of finding new treatments, the Spanish research group investigated the gene expression profile (signature) of a type of skin cell called dermal fibroblasts from patients with RDEB, KS and XPC. The researchers found that the fibroblasts from three genodermatoses share a large number of genes aberrantly (wrongly) expressed in a similar fashion. That in turn translates into what is defined as an activated phenotype and leads to changes in dermal (a skin layer) stiffness and an imbalance of the redox (a type of chemical reaction) status. This overlapping signature seems not to depend on the primary disease‐causing deficiency defect (i.e. loss of type VII collagen, kindlin‐1 or XPC) but rather the consequence of a common injury‐responsive event not yet fully understood. In conclusion, this study adds to the current knowledge about the role played by dermal fibroblasts in RDEB, XPC and KS and highlights new targets for possible treatments. Linked Article: Chacón‐Solano et al. Br J Dermatol 2019; 181:512–522 [ABSTRACT FROM AUTHOR]

Presents research which studied the carbon monoxide (CO) emission of the molecular clouds of M31, the Andromeda galaxy. Star formation in clouds of cold molecular hydrogen (H2); Problems studying H2 in the cold interstellar medium; Use of CO emissions to trace H2; Tie between CO emission and cosmic dust; CO useful to trace molecular gas.

Objective To present a novel surgical concept using preoperative imaging to estimate length needed for ileal segment. This enables robotic ileal interposition to be completed with only 1 position change during surgery. Materials and Methods The index patient is a 69-year-old woman with a history of stage IIIB cervical cancer treated with chemotherapy and radiation 5 years prior to presentation. The patient subsequently developed a long segment stricture of the left ureter, which had been managed with ureteral stents. The patient decided to proceed with robotic ileal ureter for management of her ureteral stricture disease. We used preoperative computed tomography scan measurements to estimate length needed for ileal segment. This eliminated the need for multiple position changes during surgery. Results The patient tolerated the procedure well. Estimated blood loss was 50 cc. Kidney ultrasound at 1 month post-op revealed no hydronephrosis. Renal scan at 12 months post-op revealed stable function at 32% and no evidence of obstruction. The patient reports she is doing well and is pain free at this time. Conclusion Robotic ileal interposition is a technically feasible procedure with good functional outcomes. By using preoperative imaging to estimate length needed for ileal segment, only 1 position change is needed during surgery. [ABSTRACT FROM AUTHOR]

Measuring vision in rodents is a critical step for understanding vision, improving models of human disease, and developing therapies. Established behavioural tests for perceptual vision, such as the visual water task, rely on learning. The learning process, while effective for sighted animals, can be laborious and stressful in animals with impaired vision, requiring long periods of training. Current tests that that do not require training are based on sub-conscious, reflex responses (e.g. optokinetic nystagmus) that don't require involvement of visual cortex and higher order thalamic nuclei. A potential alternative for measuring vision relies on using visually guided innate defensive responses, such as escape or freeze, that involve cortical and thalamic circuits. In this study we address this possibility in mice with intact and degenerate retinas. We first develop automatic methods to detect behavioural responses based on high dimensional tracking and changepoint detection of behavioural time series. Using those methods, we show that visually guided innate responses can be elicited using parametisable stimuli, and applied to describing the limits of visual acuity in healthy animals and discriminating degrees of visual dysfunction in mouse models of retinal degeneration. [ABSTRACT FROM AUTHOR]

Change detection methods are often incapable of accurately detecting changes within time series that are heavily influenced by seasonal variations. Techniques for de-seasoning time series or methods that apply the spatial context have been used to improve the results of change detection. However, few studies have explored Landsat's shortwave infrared channel (SWIR 2) to discriminate between seasonal changes and land use/land cover changes (LULCC). Here, we explored the effectiveness of Operational Land Imager (OLI) spectral bands and vegetation indices for detecting deforestation in highly seasonal areas of Brazilian savannas. We adopted object-based image analysis (OBIA), applying a multidate segmentation to an OLI time series to generate input data for discrimination of deforestation from seasonal changes using the Random Forest (RF) algorithm. We found adequate separability between deforested objects and seasonal changes using SWIR 2. Using spectral indices computed from SWIR 2, the RF algorithm generated a change map with an overall accuracy of 88.3%. For deforestation, the producer's accuracy was 88.0% and the user's accuracy was 84.6%. The SWIR 2 channel as well as the mid-infrared burn index presented the highest importance among spectral variables computed by the RF average impurity decrease measure. Our results give support to further change detection studies regarding to suitable spectral channels and provided a useful foundation for savanna change detection using an object-based method applied to Landsat time series. [ABSTRACT FROM AUTHOR]

BAs was predicted to have an unusually high thermal conductivity with a room temperature value of 2000 W m-1 K-1, comparable to that of diamond. However, the experimentally measured thermal conductivity of BAs single crystals is still lower than this value. To identify the origin of this large inconsistency, we investigate the lattice structure and potential defects in BAs single crystals at the atomic scale using aberration-corrected scanning transmission electron microscopy (STEM). Rather than finding a large concentration of As vacancies (VAs), as widely thought to dominate the thermal resistance in BAs, our STEM results show an enhanced intensity of some B columns and a reduced intensity of some As columns, suggesting the presence of antisite defects with AsB (As atom on a B site) and BAs (B atom on an As site). Additional calculations show that the antisite pair with AsB next to BAs is preferred energetically among the different types of point defects investigated and confirm that such defects lower the thermal conductivity for BAs. Using a concentration of 1.8(8)% (6.6±3.0×1020 cm-3 in density) for the antisite pairs estimated from STEM images, the thermal conductivity is estimated to be 65-100 W m-1 K-1, in reasonable agreement with our measured value. Our study suggests that AsB-BAs antisite pairs are the primary lattice defects suppressing thermal conductivity of BAs. Possible approaches are proposed for the growth of high-quality crystals or films with high thermal conductivity. Employing a combination of state-of-the-art synthesis, STEM characterization, theory, and physical insight, this work models a path toward identifying and understanding defect-limited material functionality. [ABSTRACT FROM AUTHOR]

Objective: To present a novel surgical concept using preoperative imaging to estimate length needed for ileal segment. This enables robotic ileal interposition to be completed with only 1 position change during surgery.Materials and Methods: The index patient is a 69-year-old woman with a history of stage IIIB cervical cancer treated with chemotherapy and radiation 5 years prior to presentation. The patient subsequently developed a long segment stricture of the left ureter, which had been managed with ureteral stents. The patient decided to proceed with robotic ileal ureter for management of her ureteral stricture disease. We used preoperative computed tomography scan measurements to estimate length needed for ileal segment. This eliminated the need for multiple position changes during surgery.Results: The patient tolerated the procedure well. Estimated blood loss was 50 cc. Kidney ultrasound at 1 month post-op revealed no hydronephrosis. Renal scan at 12 months post-op revealed stable function at 32% and no evidence of obstruction. The patient reports she is doing well and is pain free at this time.Conclusion: Robotic ileal interposition is a technically feasible procedure with good functional outcomes. By using preoperative imaging to estimate length needed for ileal segment, only 1 position change is needed during surgery. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Pulmonary exacerbations lead to significant morbidity and mortality in patients with cystic fibrosis (CF). National consensus guidelines exist, but few studies report current practice in the treatment and monitoring of pulmonary exacerbations. The goal of this study was to characterize consistency and variability in the inpatient management of CF-related pulmonary exacerbations. We focused on the use of guideline-recommended maintenance therapies, antibiotic selection and treatment regimens, use of systemic corticosteroids, and frequency of lung function testing. We hypothesized that significant variability in these treatment practices exists nationally. METHODS: This trial was a retrospective cross-sectional study. It included patients with CF aged ≤18 years hospitalized for pulmonary exacerbations between July 1, 2010, and June 30, 2015, at hospitals within the US Pediatric Health Information System database that are also Cystic Fibrosis Foundation-accredited care centers. One exacerbation per patient was randomly selected over the 5-year study period. RESULTS: From 38 hospitals, 4827 individual pulmonary exacerbations were examined. Median length of stay was 10.0 days (interquartile range, 6-14.0 days). Significant variation was seen among centers in the use of hypertonic saline (11%-100%), azithromycin (5%- 83%), and systemic corticosteroids (3%-61%) and in the frequency of lung function testing. Four different admission antibiotic regimens were used >10% of the time, and the most commonly used admission antibiotic regimen comprised 2 intravenous antibiotics with no additional oral or inhaled antibiotics (29%). CONCLUSIONS: Significant variation exists in the treatment and monitoring of pulmonary exacerbations across Pediatric Health Information System-participating, Cystic Fibrosis Foundation-accredited care centers. Results from this study can inform future research working toward standardized inpatient pulmonary exacerbation management to improve CF care for children and adolescents. [ABSTRACT FROM AUTHOR]

Given the strong coupling between the carbon (C) and nitrogen (N) cycles, there is substantial interest in understanding how N availability affects C cycling in terrestrial ecosystems, especially in ecosystems limited by N. However, most studies in temperate and boreal forests have focused on the effects of N addition on tree growth. By comparison, less is known about the effects of N availability on the cycling of C in understory vegetation despite some evidence that dwarf shrubs, mosses, and lichens play an important role in the forest C balance. In this study, we used an in situ 13CO2 pulse‐labelling technique to examine the short‐term dynamics of C partitioning in understory vegetation in three boreal Pinus sylvestris forest stands exposed to different rates of N addition: a low and high N addition that receive annual additions of NH4NO3 of 20 and 100 kg N ha−1, respectively, and an unfertilized control. Labelling was conducted at two distinct periods (early vs. late growing season), which provided a seasonal picture of how N addition affects C dynamics in understory vegetation. In contrast to what has been found in trees, there was no obvious trend in belowground C partitioning in ericaceous plants in response to N additions or seasonality. Increasing N addition led to a greater percentage of 13C being incorporated into ericaceous leaves with a high turnover, whereas high rates of N addition strongly reduced the incorporation of 13C into less degradable moss tissues. Addition of N also resulted in a greater percentage of the 13C label being respired back to the atmosphere and an overall reduction in total understory carbon‐use‐efficiency. Taken together, our results suggest a faster cycling of C in understory vegetation with increasing N additions; yet the magnitude of this general response was strongly dependent on the amount of N added and varied seasonally. These results provide some of the first in situ C and N partitioning estimates for plants growing under the complex web of resource limitations in the boreal understory.This article is protected by copyright. All rights reserved. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor on the possible relationship between linear lichen planus and lichen striatus.

7-11 November 2010, Tenth International Congress on Drug Therapy in HIV Infection, Glasgow, UK [ABSTRACT FROM AUTHOR]

An abstract of the article "Immunological response among patients with undetectable viral load followed for 5 years," by several authors including R Lucas, A Ferreira, and M Tavares is presented.

The article presents an abstract of a research paper on the upregulation of inflammatory genes and downregulation of sclerostin, submitted at the 5th European Workshop on Immune-Mediated Inflammatory Diseases in Sitges-Barcelona, Spain from December 1-3, 2010.

Human and animal sporotrichosis is a relatively common zoonotic disease in the southwest region of Brazil. In a recent outbreak (1999–2000) involving 66 humans in Rio de Janeiro, 79% of patients reported previous contact with infected cats. Over a 17-year period (1986–2002), 25 cases of feline sporotrichosis were diagnosed at our hospital (1.5 cases/year). Among these cases, 80% were male and had the cutaneous form of the disease, and 21 cases (84%) were in mixed-breed animals. The diagnosis was established based on histopathology (H&E and PAS stain) as well as fungal culture (95%) and cytologic examination (82.5%) of the cases. In the 25 homes of these animals, 15 (60%) were sporotrichosis cases of in-contact animals. Humans were infected in seven (28.5%) of the homes. Eighteen of the 49 dogs and cats (36.7%) in homes with infected humans were also affected. Itraconazole at 10 mg/kg once daily was successful even in FIV and FeLV positive cats. Funding: Self-funded. [ABSTRACT FROM AUTHOR]