Observation Impact over the Antarctic During the Concordiasi Field Campaign

The impact of observations on analysis uncertainty and forecast performance was investigated for Austral Spring 2010 over the Southern polar area for four different systems (NRL, GMAO, ECMWF and Meteo-France), at the time of the Concordiasi field experiment. The largest multi model variance in 500 hPa height analyses is found in the southern sub-Antarctic oceanic region, where there are strong atmospheric dynamics, rapid forecast error growth, and fewer upper air wind observation data to constrain the analyses. In terms of data impact the most important observation components are shown to be AMSU, IASI, AIRS, GPS-RO, radiosonde, surface and atmospheric motion vector observations. For sounding data, radiosondes and dropsondes, one can note a large impact of temperature at low levels and a large impact of wind at high levels. Observing system experiments using the Concordiasi dropsondes show a large impact of the observations over the Antarctic plateau extending to lower latitudes with the forecast range, with a large impact around 50 to 70deg South. These experiments indicate there is a potential benefit of better using radiance data over land and sea-ice and innovative atmospheric motion vectors obtained from a combination of various satellites to fill the current data gaps and improve NWP in this region

systemic contact dermatitis from herbal and homeopathic preparations used for herpes virus treatment

Systemic contact dermatitis may occur in contactsensitized individuals when they are exposed to haptens orally, transcutaneously, intravenously or by inhalation. We report the case of a woman developing a diffuse skin eruption after the topical use of Rhus toxicodendron alcoholic extract and the oral introduction of a homeopathic preparation of the same substance for herpes treatment. An open test, performed with the Rhus toxicodendron tincture, showed an erythematooedematous response at 48 h and vesicular reaction at 96 h that was still present after 7 days. Patch test with 65% ethyl alcohol gave negative results. The open test performed, as control, in eight healthy informed subjects revealed negative responses to Rhus tincture application. The result is interesting because in Italy, allergic contact dermatitis to Rhus is uncommon and this case increases the understanding of the pathogenetic mechanism leading to systemic contact dermatitis development. Key words: homeopathy; plant extracts; mother tincture; Rhus toxicodendron; herpes virus infection

Chronic Idiopathic and Chronic Autoimmune Urticaria: Clinical and Immunopathological Features of 68 Subjects

Skin tests with autologous serum elicit an immediate wheal-and-flare response in about 30 ‐ 50% of chronic idiopathic urticaria subjects, suggesting that an autoimmune mechanism might be involved in the pathogenesis of this disease. The aim of the present work, involving 68 subjects with chronic idiopathic urticaria, was to distinguish between the serum-positive and serum-negative cases and highlight the clinical differences between the two groups on the basis of the Breneman scale score. We also tried to correlate the finding of a positive response to the autologous serum skin test with other autoimmune diatheses or fully developed autoimmune disorders. Our results did not demonstrate any significant differences between the two groups with regard to mean age, sex distribution, angioedema and mucosal/cutaneous atopy. However, all subjects with positive autologous serum skin test presented more severe clinical features than serumnegative subjects. We found no differences between the two groups in the incidence of autoimmune disease. Key words: chronic urticaria; autologous serum skin test; autoimmunity

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p

Impact of airborne Doppler lidar observations on ECMWF forecasts

During the Atlantic THORPEX Regional Campaign (A-TReC) in autumn 2003, the airborne Doppler lidar of the Deutsches Zentrum f¨ur Luft- und Raumfahrt (DLR) was used to observe wind in predicted sensitive regions. In eight flights, the system measured a total of 1600 wind profiles that were experimentally assimilated in the global assimilation system at the European Centre for Medium-Range Weather Forecasts (ECMWF). The present study assesses the impact of these Doppler lidar measurements in Numerical Weather Prediction (NWP). It is shown that lidar observations have a significant impact on the analyses as well as on forecasts owing to high accuracy and spatial resolution. The measurements reduce the errors of the 1–4 day forecasts of geopotential height, wind, and humidity over Europe throughout the troposphere. On average, Doppler lidar measurements reduce the 2–4 day forecast error of geopotential height over Europe by 3%. This is a promising result, considering that observations have been gathered from only 28.5 flight hours. Dropsondes released in the same area as the Doppler lidar was operating show good agreement for measured winds, but smaller analysis impact and less reduction of the forecast error

Impact of Targeted Winter Storm Reconnaissance Dropwindsonde Data on Midlatitude Numerical Weather Predictions

Abstract The impact of assimilating data from the 2011 Winter Storm Reconnaissance (WSR) program on numerical weather forecasts was assessed. Parallel sets of analyses and deterministic 120-h numerical forecasts were generated using the ECMWF four-dimensional variational data assimilation (4D-Var) and Integrated Forecast System. One set of analyses was generated with all of the normally assimilated data plus WSR targeted dropwindsonde data, the other with only the normally assimilated data. Forecasts were then generated from the two analyses. The comparison covered the period from 10 January to 28 March 2011, during which 98 flights and 776 total dropwindsondes were deployed from four different air bases in the Pacific basin and the United States. The dropwindsondes were deployed in situations where guidance indicated the potential for high-impact weather and/or the potential for large subsequent forecast errors. Downstream target verification regions where the high-impact weather was expected were identified for each case. Forecast errors around the target verification regions were evaluated using an approximation to the total-energy norm. Precipitation forecasts were also evaluated over the contiguous United States using the equitable threat score and bias. Forecast impacts were generally neutral and thus smaller than reported in previous studies, most from over a decade ago, perhaps because of the improved forecast and assimilation system and the somewhat denser observation network. Target areas may also have been undersampled in this study. The neutral results from 2011 suggest that it may be more beneficial to explore other targeted observation concepts for the midlatitudes, such as assimilation of a denser set of cloud-drift winds and radiance data in dynamically sensitive regions

The impact of airborne wind and water vapour lidar measurements on ECMWF analyses and forecasts

Recent impact studies show that the ECMWF forecast error could be reduced significantly by assimilating airborne Doppler lidar observations taken during the Atlantic THORPEX Regional Campaign (A-TReC). This is a very promising result, considering that observations have been gathered from only 28.5 flight hours in a 2-week period. Due to low instrumental and representativeness errors of the observations, lidar winds have a higher analysis influence than dropsondes and conventional wind observations. These encouraging results motivated similar impact studies using airborne Differential Absorption Lidar (DIAL) water vapour observations from several research campaigns. In addition to low observational errors, the high vertical resolution of DIAL lidars of up to a few hundred meters is expected to be crucial for a proper representation of the water vapour field in NWP models. In the current global observing system water vapour is primarily observed through passive satellite instruments, which have a very low vertical resolution and much higher observational errors than lidars. This paper summarizes the impact of airborne wind and water vapour lidar data on ECMWF analysis and forecast fields. Furthermore, an outlook on planned future field campaigns using airborne lidars for targeted observations is presented (COPS/ETReC, T-PARC, TNADEX)