EPS/Metop-SG Scatterometer Mission Science Plan

89 pages, figures, tablesThis Science Plan describes the heritage, background, processing and control of C-band scatterometer data and its remaining exploitation challenges in view of SCA on EPS/MetOp-SGPeer reviewe

A longitudinal follow-up of autoimmune polyendocrine syndrome type 1

Source:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4971337/Context: Autoimmune polyendocrine syndrome type 1 (APS1) is a childhood-onset monogenic disease defined by the presence of two of the three major components: hypoparathyroidism, primary adrenocortical insuffi- ciency, and chronic mucocutaneous candidiasis (CMC). Information on longitudinal follow-up of APS1 is sparse. Objective: To describe the phenotypes of APS1 and correlate the clinical features with autoantibody profiles and autoimmune regulator ( AIRE) mutations during extended follow-up (1996–2016). Patients: All known Norwegian patients with APS1. Results: Fifty-two patients from 34 families were identified. The majority presented with one of the major disease components during childhood. Enamel hypoplasia, hypoparathyroidism, and CMC were the most frequent compo- nents.Withage,mostpatientspresentedthreetofivediseasemanifestations,althoughsomehadmilderphenotypes diagnosed in adulthood. Fifteen of the patients died during follow-up (median age at death, 34 years) or were deceasedsiblingswithahighprobabilityofundisclosedAPS1.Allexceptthreehadinterferon- )autoantibodies,and allhadorgan-specificautoantibodies.Themostcommon AIRE mutationwasc.967_979del13,foundinhomozygosity in 15 patients. A mild phenotype was associated with the splice mutation c.879 1G A. Primary adrenocortical insufficiency and type 1 diabetes were associated with protective human leucocyte antigen genotypes. Conclusions: Multiple presumable autoimmune manifestations, in particular hypoparathyroidism, CMC, and enamel hypoplasia, should prompt further diagnostic workup using autoantibody analyses (eg, interferon- ) and AIRE sequencing to reveal APS1, even in adults. Treatment is complicated, and mortality is high. Structured follow-up should be performed in a specialized center

The Copernicus Marine Environment Monitoring Service Ocean State Report

The Copernicus Marine Environment Monitoring Service (CMEMS) Ocean State Report (OSR) provides an annual report of the state of the global ocean and European regional seas for policy and decision-makers with the additional aim of increasing general public awareness about the status of, and changes in, the marine environment. The CMEMS OSR draws on expert analysis and provides a 3-D view (through reanalysis systems), a view from above (through remote-sensing data) and a direct view of the interior (through in situ measurements) of the global ocean and the European regional seas. The report is based on the unique CMEMS monitoring capabilities of the blue (hydrography, currents), white (sea ice) and green (e.g. Chlorophyll) marine environment. This first issue of the CMEMS OSR provides guidance on Essential Variables, large-scale changes and specific events related to the physical ocean state over the period 1993–2015. Principal findings of this first CMEMS OSR show a significant increase in global and regional sea levels, thermosteric expansion, ocean heat content, sea surface temperature and Antarctic sea ice extent and conversely a decrease in Arctic sea ice extent during the 1993–2015 period. During the year 2015 exceptionally strong large-scale changes were monitored such as, for example, a strong El Niño Southern Oscillation, a high frequency of extreme storms and sea level events in specific regions in addition to areas of high sea level and harmful algae blooms. At the same time, some areas in the Arctic Ocean experienced exceptionally low sea ice extent and temperatures below average were observed in the North Atlantic Ocean

Electric Light; Light Fair 2012

Lighting devices -- Lamps -- Electric lamp

Data Assembly and Processing for Operational Oceanography: Ten Years of Achievements

Data assembly and processing centers are essential elements of the operational oceanography infrastructure. They provide data and products needed by modeling and data assimilation systems; they also provide products directly useable for applications. This paper discusses the role and functions of the data centers for operational oceanography. It describes some of the main data assembly centers (Argo and in situ data, altimetry, sea surface temperature) developed during the Global Ocean Data Assimilation Experiment. An overview of other data centers (wind and fluxes, ocean color, sea ice) is also given. Much progress has been achieved over the past ten years to validate, intercalibrate, and merge altimeter data from multiple satellites. Accuracy and timeliness of products have been improved, and new products have been developed. The same is true for sea surface temperature data through the Global High-Resolution Sea Surface Temperature Pilot Project. A breakthrough in processing, quality control, and assembly for in situ data has also been achieved through the development of the real-time and delayed-mode Argo data system. In situ and remote-sensing data are now systematically and jointly used to calibrate, validate, and monitor over the long term the quality and consistency of the global ocean observing system. Main results are illustrated. There is also a review of the development and use of products that merge in situ and remote-sensing data. Future issues and main prospects are discussed in the conclusion

Remote sensing of sea ice

NERSC contribution to OceanObs09, 21 – 25 September 2009, Venice, Ital

Autoimmune Thyroid Disorders in Autoimmune Addison Disease

Context: Autoimmune thyroid disease is the most common endocrine co-morbidity in autoimmune Addison's disease (AAD), but detailed investigations of prevalence and clinical course is lacking. Objective: Provide comprehensive epidemiological and clinical data on autoimmune thyroid disorders in AAD. Design and patients: A nationwide registry-based study including 442 patients with AAD and autoimmune thyroid disease, identified through the Norwegian National Registry of Autoimmune Diseases. Results: Of 912 registered AAD patients, 442 (48%) were diagnosed with autoimmune thyroid disease. Three hundred and eighty (42%) had autoimmune hypothyroidism. Of the 302 with available thyroid function tests at time for diagnosis, 20% had overt hypothyroidism, 73% had subclinical hypothyroidism and 7% had thyroid levels in the normal range. Negative thyroid peroxidase antibodies was found in 32%. Ninety-eight percent were treated with levothyroxine, 5% with combination therapy with liothyronine or thyroid extracts, and 1% were observed without treatment. Seventy-eight patients (9%) were diagnosed with Graves' disease (GD), of whom 16 (21%) were diagnosed with autoimmune hypothyroidism either before onset or after remission of GD. At the end of follow-up 33% had normal thyroid hormone levels without antithyroid-drugs or levothyroxine treatment. The remaining had either active disease (5%), had undergone ablative treatment (41%), or had developed autoimmune hypothyroidism (21%). Conclusion: The true prevalence of hypothyroidism in AAD is lower than reported in current literature. Careful consideration of the indication to start thyroxin therapy is warranted. Long-term remission rates in GD patients with AAD are comparable to recent reports on long-term follow-up of patients without AAD.publishedVersio

Vaccination prevents severe COVID-19 outcome in patients with neutralizing type 1 interferon autoantibodies

Summary: A hallmark of patients with autoimmune polyendocrine syndrome type 1 (APS-1) is serological neutralizing autoantibodies against type 1 interferons (IFN-I). The presence of these antibodies has been associated with severe course of COVID-19. The aims of this study were to investigate SARS-CoV-2 vaccine tolerability and immune responses in a large cohort of patients with APS-1 (N = 33) and how these vaccinated patients coped with subsequent infections. We report that adult patients with APS-1 were able to mount adequate SARS-CoV-2 spike-specific antibody responses after vaccination and observed no signs of decreased tolerability. Compared with age- and gender-matched healthy controls, patients with APS-1 had considerably lower peak antibody responses resembling elderly persons, but antibody decline was more rapid in the elderly. We demonstrate that vaccination protected patients with APS-1 from severe illness when infected with SARS-CoV-2 virus, overriding the systemic danger of IFN-I autoantibodies observed in previous studies

Autoimmune Thyroid Disorders in Autoimmune Addison Disease

Context: Autoimmune thyroid disease is the most common endocrine co-morbidity in autoimmune Addison's disease (AAD), but detailed investigations of prevalence and clinical course is lacking. Objective: Provide comprehensive epidemiological and clinical data on autoimmune thyroid disorders in AAD. Design and patients: A nationwide registry-based study including 442 patients with AAD and autoimmune thyroid disease, identified through the Norwegian National Registry of Autoimmune Diseases. Results: Of 912 registered AAD patients, 442 (48%) were diagnosed with autoimmune thyroid disease. Three hundred and eighty (42%) had autoimmune hypothyroidism. Of the 302 with available thyroid function tests at time for diagnosis, 20% had overt hypothyroidism, 73% had subclinical hypothyroidism and 7% had thyroid levels in the normal range. Negative thyroid peroxidase antibodies was found in 32%. Ninety-eight percent were treated with levothyroxine, 5% with combination therapy with liothyronine or thyroid extracts, and 1% were observed without treatment. Seventy-eight patients (9%) were diagnosed with Graves' disease (GD), of whom 16 (21%) were diagnosed with autoimmune hypothyroidism either before onset or after remission of GD. At the end of follow-up 33% had normal thyroid hormone levels without antithyroid-drugs or levothyroxine treatment. The remaining had either active disease (5%), had undergone ablative treatment (41%), or had developed autoimmune hypothyroidism (21%). Conclusion: The true prevalence of hypothyroidism in AAD is lower than reported in current literature. Careful consideration of the indication to start thyroxin therapy is warranted. Long-term remission rates in GD patients with AAD are comparable to recent reports on long-term follow-up of patients without AAD