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• One year after the first ON episode, the global risk of MS is 42 % (higher than in the ONTT). The distinction between 'possible MS' and 'strictly isolated' ON is not predictive, with a 20 % risk of conversion in both cases. As a delay in the MS diagnosis can lead to functional impairment, every patient with a first episode of isolated ON must be regularly screened for MS, even if their ON is classified as strictly isolated (i.e., without brain MRI abnormalities and oligoclonal bands at cerebral spinal fluid analysis). The last updates to diagnostic criteria for multiple sclerosis (MS) included a diagnostic category of 'possible MS'. However, no recent data is available to assess how much this distinction helps predict MS after isolated optic neuritis (ON). This study aimed to assess the global risk of developing MS one year after a first ON episode, and the specific risk according to the initial diagnosis of isolated ON or ON with possible MS. One-year follow-up of a multicentric prospective cohort of adult patients with acute ON. This study included 55 patients with acute ON of no known etiological diagnosis. Overall, the final diagnosis at one year was MS (23, 42 %), MOGAD (7, 13 %), NMOSD (1, 2 %), CRION (3, 5 %), possible MS (6, 11 %), secondary ON (3, 5 %), and strictly isolated ON (12, 22 %). Three of the 17 (18 %) patients with strictly isolated ON and 2/8 (25 %) with possible MS at baseline progressed to MS. All secondary MS diagnosis were made through radiological monitoring. One year after the first ON episode, we observed a similar conversion rate to MS for patients with strictly isolated ON and possible MS, with a higher prevalence of MS than found by the ONTT. [ABSTRACT FROM AUTHOR]

• A second-line treatment with plasmapheresis or intravenous immunoglobulin allows better visual recovery than corticosteroids alone. • It is essential to propose rapidly a second line treatment, be it plasmapheresis or intravenous immunoglobulin, in the case of steroid resistant optic neuritis. • In our network meta-analysis, a second line treatment with intravenous immunoglobulin seems more effective than plasmapheresis. A prospective randomised multicenter study is nevertheless needed to confirm it. To compare the efficacy of treatment of optic neuritis (ON) with corticosteroids (CTC) alone, CTC+plasmapheresis (PLP), and CTC+intravenous immunoglobulin (IVIG). After an episode of ON, although visual recovery is usually good, some patients may have significant visual sequelae. While the efficacy of first-line CTC is now indisputable, there is no consensus on the nature of second-line treatment. To date, no systematic review has compared the efficacy of treatment of ON with CTC alone, CTC+plasmapheresis (PLP), and CTC+intravenous immunoglobulin (IVIG). A meta-analysis is needed to compare the efficacy of PLP and IVIG in steroid-resistant ON. This systematic review included all studies comparing at least two of the three treatments for steroid-resistant ON (CTC alone, CTC+PLP, and CTC+IVIG). From all articles published on PubMed between January 2000 and June 2022, two independent ophthalmologists selected studies of interest using the PRISMA method. Methodology, patient characteristics, and outcomes were identified. A network metaanalysis was then performed to compare the efficacy of the three treatments. Six comparative studies were included, representing 209 patients. The percentage of significant visual recovery after CTC alone, CTC+PLP, and CTC+IVIG in the acute treatment of steroid-resistant ON was 30 %, 45 %, and 77 %, respectively. Comparison of CTC+IVIG vs CTC alone, CTC+PLP vs CTC only, and CTC+PLP vs CTC+IVIG yielded odds ratios of 12.81, 2.47, and 0.19 respectively. Treatment of steroid-resistant ON with CTC+PLP or CTC+IVIG is more effective than treatment with CTC alone. Although no study has directly compared the two treatments, IVIG may be more effective than PLP. [ABSTRACT FROM AUTHOR]

Abstract The monitoring of wind turbines using SCADA data has received lately a growing interest from the fault diagnosis community because of the very low cost of these data, which are available in number without the need for any additional sensor. Yet, these data are highly variable due to the turbine constantly changing its operating conditions and to the rapid fluctuations of the environmental conditions (wind speed and direction, air density, turbulence, ...). This makes the occurrence of a fault difficult to detect. To address this problem, we propose a multi-level (turbine and farm level) strategy combining a mono- and a multi-turbine approach to create fault indicators insensitive to both operating and environmental conditions. At the turbine level, mono-turbine residuals (i.e. a difference between an actual monitored value and the predicted one) obtained with a normal behavior model expressing the causal relations between variables from the same single turbine and learnt during a normal condition period are calculated for each turbine, so as to get rid of the influence of the operating conditions. At the farm level, the residuals are then compared to a wind farm reference in a multi-turbine approach to obtain fault indicators insensitive to environmental conditions. Indicators for the objective performance evaluation are also proposed to compare wind turbine fault detection methods, which aim at evaluating the cost/benefit of the methods from a production manager's point of view. The performance of the proposed combined mono- and multi-turbine method is evaluated and compared to more classical methods proposed in the literature on a large real data set made of SCADA data recorded on a French wind farm during four years : it is shown than it can improve the fault detection performance when compared to a residual analysis limited at the turbine level only. Highlights • A mono- and a multi-turbine indicator is proposed. • It is insensitive to operation and environmental conditions. • Mono turbine residuals are built at the turbine level. • Mono turbine residuals are compared to a wind farm reference. • Objective performance metrics are proposed to evaluate the fault indicators. [ABSTRACT FROM AUTHOR]

Copyright of Canadian Journal of Ophthalmology is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Autosomal dominant cerebellar ataxia is a rare heterogeneous group of diseases characterized by cerebellar symptoms, often associated with other multisystemic signs. Mild optic neuropathy has been associated with spinocerebellar ataxia type 1 (SCA1), but macular dysfunction has been reported in only 2 cases. We report the first family with SCA1 with several members affected by visual loss related to maculopathy.Cross-sectional clinical and electrophysiological study of a family with genetically confirmed SCA1. Patients with unexplained visual loss were included.Four patients from the same family, carrying the same genetic mutation, were examined. Testing revealed an increased CAG trinucleotide repeat number within the SCA1 gene. Genetic testing results for SCA7 were negative. Visual acuities ranged between 20/20 and 20/200. Visual fields revealed central scotomas in most of the eyes, and funduscopy was unremarkable in most patients. Central retinal thinning of the retina or disorganized photoreceptor layers were found with optical coherence tomography (OCT). In one patient, multifocal electroretinography (mfERG) revealed central retinal dysfunction.A clinically subtle or even occult maculopathy can occur in association with SCA1. Macular OCT and mfERG can be abnormal even in asymptomatic patients. Unexplained visual loss in SCA1 patients should prompt evaluation of macular function, even if clinical signs of maculopathy are absent. [ABSTRACT FROM AUTHOR]

The article discusses a study which combined whole-body magnetic resonance angiography (WB-MRA) with multislice computed tomography (MSCT) of the coronary arteries and ultrasound-based imaging of the carotid arteries to examine the prevalence and type of vascular abnormalities in adult patients with STAT3 deficiency. Twenty one adult patients were enrolled in the study. It suggests that adult patients with STAT3 deficiency were found to have a high prevalence of vascular abnormalities. INSET: CLINICAL PERSPECTIVE.

Copyright of Néphrologie & Thérapeutique is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Néphrologie & Thérapeutique is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Commensal gut bacteria are essential for the development and maintenance of the gut''s immune system. Some bacteria strains, such as Lactobacillus and Bifidobacterium species, have been reported to provide protection from allergic and inflammatory bowel diseases. However, the interactions between these commensal bacteria and the immune system are largely unknown. Objective: We studied the effects of a supernatant from the culture of B breve C50 (BbC50) on the maturation, activation, and survival of human dendritic cells (DCs). Methods: DCs were differentiated from human monocytes with IL-4 and GM-CSF for 5 days and cultured with BbC50 supernatant (BbC50SN) or LPS for 2 days. Results: BbC50SN induced DC maturation, with increase in CD83, CD86, and HLA-DR expression. We also showed, for the first time, that BbC50SN prolonged DC survival, with high IL-10 and low IL-12 production compared with that seen in LPS-DCs. Moreover, BbC50SN inhibited the effects of LPS on DCs, both in terms of IL-12 production and in terms of survival. The prolonged DC survival was independent of IL-10 production and nuclear factor κB pathway but was associated with an upregulation of Bcl-xL and Phospho-Bad. Finally, BbC50SN induced activation of Toll-like receptor 2 (TLR2)–transfected cells in contrast to TLR4-, TLR7-, and TLR9-transfected cells. Conclusion: The supernatant of B breve C50 can induce DC maturation and prolonged DC survival through TLR2, with high IL-10 production. These properties might correspond to a regulatory DC profile, which could limit the excessive TH1 response and control the excessive TH2 polarization observed in atopic newborns. [Copyright &y& Elsevier]

Introduction L’étude Spiesser a montré qu’une immunosuppression associant de la thymoglobuline, du sirolimus (SRL), du MMF et des corticoïdes sevrés dans les premiers mois permettait l’obtention d’une meilleure fonction rénale qu’une immunosuppression basée sur la cyclosporine (CsA) jusqu’à 5 ans après la transplantation rénale. Nous rapportons ici les résultats à 8 ans. Patients et méthodes Un questionnaire a été adressé aux 119 patients avec un greffon fonctionnel à 5 ans. Résultats Les données de suivi étaient disponibles pour 112 patients (55 SRL et 57 CsA). Le SRL a été arrêté chez 6 patients et la CsA chez 2 et finalement 26 (37 %) et 36 (49 %) patients sont restés dans leur bras respectif. En intention de traiter, la fonction rénale était meilleure dans le bras SRL (eDFG-MDRD = 62,5 ± 27,3 vs 47,8 ± 17,1 mL/min, p = 0,004) alors que plus de patients avaient une diminution de plus de 20 % de leur DFG après 1 an dans le bras CsA (34 vs 16 %, p = 0,037). La survie des patients et des greffons était similaires dans les deux groupes alors que 4 décès (3 SRL et 1 CsA) et 8 pertes de greffons (2 SRL et 6 CsA) ont été rapportés après 5 ans. Des DSA sont apparus chez 17 patients et le risque d’immunisation de novo était similaire dans chaque bras (log rank p = 0,52). La majorité des cancers apparus entre 5 et 8 ans sont apparus dans le bras sirolimus (5/6) mais 3 d’entre eux sont survenus alors que le sirolimus avait préalablement été remplacé par de la cyclosporine. Finalement le risque de cancer n’était pas différent entre chaque groupe (log rank p = 0,76). Enfin, aucune différence concernant le risque de diabète, d’anémie et le contrôle de la PA n’a été mise en évidence. Discussion et conclusion L’utilisation du sirolimus de novo en transplantation rénale chez les receveurs à faible risque immunologique recevant un rein d’un donneur décédé à cœur battant permet d’obtenir une meilleure fonction rénale à 8 ans sans que le risque d’apparition de DSA n’augmente. [ABSTRACT FROM AUTHOR]