II.5 Where to find the CoRoT data?

This book is dedicated to all the people interested in the CoRoT mission and the beautiful data that were delivered during its six year duration. Either amateurs, professional, young or senior researchers, they will find treasures not only at the time of this publication but also in the future twenty or thirty years. It presents the data in their final version, explains how they have been obtained, how to handle them, describes the tools necessary to understand them, and where to find them. It also highlights the most striking first results obtained up to now. CoRoT has opened several unexpected directions of research and certainly new ones still to be discovered

Generation of three iPSC lines from fibroblasts of a patient with Aicardi Goutières Syndrome mutated in TREX1

Fibroblasts from a patient with Aicardi Gouti\ue8res Syndrome (AGS) carrying a compound heterozygous mutation in TREX1, were reprogrammed into induced pluripotent stem cells (iPSCs) to establish isogenic clonal stem cell lines: UNIBSi006-A, UNIBSi006-B, and UNIBSi006-C. Cells were transduced using the episomal Sendai viral vectors, containing human OCT4, SOX2, c-MYC and KLF4 transcription factors. The transgene-free iPSC lines showed normal karyotype, expressed pluripotent markers and displayed in vitro differentiation potential toward cells of the three embryonic germ layers

Probabilistic models to evaluate effectiveness of steel bridge weld fatigue retrofitting by peening

The purpose of this study was to evaluate, with two probabilistic analytical models, the effectiveness of several alternative fatigue management strategies for steel bridge welds. The investigated strategies employed, in various combinations, magnetic particle inspection, gouging and rewelding, and postweld treatment by peening. The analytical models included a probabilistic strain-based fracture mechanics model and a Markov chain model. For comparing the results obtained with the two models, the fatigue life was divided into a small, fixed number of condition states based on crack depth, similar to those often used by bridge management systems to model deterioration due to other processes, such as corrosion and road surface wear. The probabilistic strain-based fracture mechanics model was verified first by comparison with design S-N curves and test data for untreated welds. Next, the verified model was used to determine the probability that untreated and treated welds would be in each condition state in a given year; the probabilities were then used to calibrate transition probabilities for a much simpler Markov chain fatigue model. Then both models were used to simulate a number of fatigue management strategies. From the results of these simulations, the performance of the different strategies was compared, and the accuracy of the simpler Markov chain fatigue model was evaluated. In general, peening was more effective if preceded by inspection of the weld. The Markov chain fatigue model did a reasonable job of predicting the general trends and relative effectiveness of the different investigated strategies

Transiting exoplanets from the CoRoT space mission VIII. CoRoT-7b: the first Super-Earth with measured radius

We report the discovery of very shallow (DF/F = 3.4 10-4), periodic dips in the light curve of an active V = 11.7 G9V star observed by the CoRoT satellite, which we interpret as due to the presence of a transiting companion. We describe the 3-colour CoRoT data and complementary ground-based observations that support the planetary nature of the companion. Methods. We use CoRoT color information, good angular resolution ground-based photometric observations in- and out- of transit, adaptive optics imaging, near-infrared spectroscopy and preliminary results from Radial Velocity measurements, to test the diluted eclipsing binary scenarios. The parameters of the host star are derived from optical spectra, which were then combined with the CoRoT light curve to derive parameters of the companion. We examine carefully all conceivable cases of false positives, and all tests performed support the planetary hypothesis. Blends with separation larger than 0.40 arcsec or triple systems are almost excluded with a 8 10-4 risk left. We conclude that, as far as we have been exhaustive, we have discovered a planetary companion, named CoRoT-7b, for which we derive a period of 0.853 59 +/- 3 10-5 day and a radius of Rp = 1.68 +/- 0.09 REarth. Analysis of preliminary radial velocity data yields an upper limit of 21 MEarth for the companion mass, supporting the finding. CoRoT-7b is very likely the first Super-Earth with a measured radius.Comment: Accepted in Astronomy and Astrophysics; typos and language corrections; version sent to the printer w few upgrade

Interface Gain-of-Function Mutations in TLR7 Cause Systemic and Neuro-inflammatory Disease

TLR7 recognizes pathogen-derived single-stranded RNA (ssRNA), a function integral to the innate immune response to viral infection. Notably, TLR7 can also recognize self-derived ssRNA, with gain-of-function mutations in human TLR7 recently identified to cause both early-onset systemic lupus erythematosus (SLE) and neuromyelitis optica. Here, we describe two novel mutations in TLR7, F507S and L528I. While the L528I substitution arose de novo, the F507S mutation was present in three individuals from the same family, including a severely affected male, notably given that the TLR7 gene is situated on the X chromosome and that all other cases so far described have been female. The observation of mutations at residues 507 and 528 of TLR7 indicates the importance of the TLR7 dimerization interface in maintaining immune homeostasis, where we predict that altered homo-dimerization enhances TLR7 signaling. Finally, while mutations in TLR7 can result in SLE-like disease, our data suggest a broader phenotypic spectrum associated with TLR7 gain-of-function, including significant neurological involvement

Development and validation of the needs of children questionnaire: An instrument to measure children\u27s self-reported needs in hospital

AIM: To develop and psychometrically test the needs of children questionnaire (NCQ), a new instrument to measure school-aged children\u27s self-reported psychosocial physical and emotional needs in paediatric wards. DESIGN: This is an instrument development study based on recommendations for developing a reliable and valid questionnaire. METHOD: The NCQ was developed over three phases between February 2013-April 2017 and included item generation; content adequacy assessment; questionnaire administration; factor analysis; internal consistency assessment and construct validity. Psychometric properties were assessed after 193 school-aged children completed the needs of children\u27s questionnaire in four paediatric areas in Australia and New Zealand. RESULTS: The development and validation of the NCQ over two countries resulted in a 16-item, four-category tool to measure the self-reported importance and fulfilment of school-aged children\u27s needs in hospital. Cronbach\u27s alpha for the combined samples was 0.93. CONCLUSION: The NCQ bridges a gap to measure the level of importance and fulfilment of school-aged children\u27s self-reported needs in hospital. Future testing and validation is needed in other paediatric areas and countries. IMPACT: The 16-item NCQ is a valid measure to evaluate if the quality of care delivered and received in hospital is in line with what children self-report as important and required and to date indicates good usability and utility. Child self-reports are essential to inform healthcare delivery, policy, research and theory development from a child and family-centred care lens that honours the United Nations Convention on the Rights of the Child and the best interests of the child

Применение препарата пелокс-400 (пефлоксацин) в лечении острого пиелонефрита

Дана оценка эффективности применения антибактериального препарата пелокс−400 (пефлоксацин) в лечении острого пиелонефрита и рекомендовано его применение в комплексной терапии.The authors assess the efficacy of antibacterial medication рelox−400 (pefloxacin) in treatment of acute pyelonephritis. It is recommended to use it in complex therapy

The 2021 Eurpean Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type i interferonopathies: CANDLE/PRAAS, SAVI and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of \u27points to consider\u27 to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, \u27points to consider\u27 to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes

The 2021 EULAR and ACR points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of 'points to consider' to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, 'points to consider' to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes

The 2021 EULAR and ACR points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS

Objective: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of 'points to consider' to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. Methods: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, 'points to consider' to guide patient management were developed. Results: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. Conclusion: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes