Réalisation d'un capteur d'images matriciel avec sortie numérique

Cet article présente un prototype de capteur d'images conçu au laboratoire LE2I du Creusot. Il est réalisé sous la forme d'un ASIC en technologie Cmos 1.0mm. Il est destiné à être utilisé en contrôle qualité par vision artificielle pour des scènes présentant de forts contrastes lumineux. Un procédé original de numérisation du niveau de gris permet de contourner la notion classique de temps d'exposition variable et de traiter aisément les problèmes d'éblouissement local. Il permet également d'ouvrir de larges perspectives dans le domaine des capteurs intelligents, en effet la numérisation de l'information au niveau même du photosite permet, à terme d'envisager l'intégration d'un classifieur dans le voisinage immédiat du pixel, et de fournir une décision binaire en sortie du capteur

The Clinical Variability of Maternally Inherited Diabetes and Deafness Is Associated with the Degree of Heteroplasmy in Blood Leukocytes

Context: Maternally inherited diabetes and deafness (MIDD) is a rare form of diabetes with a matrilineal transmission, sensorineural hearing loss, and macular pattern dystrophy due to an A to G transition at position 3243 of mitochondrial DNA (mtDNA) (m.3243A>G). The phenotypic heterogeneity of MIDD may be the consequence of different levels of mutated mtDNA among mitochondria in a given tissue. Objective: The aim of the present study was thus to ascertain the correlation between the severity of the phenotype in patients with MIDD and the level of heteroplasmy in the blood leukocytes. Participants: The GEDIAM prospective multicenter register was initiated in 1995. Eighty-nine Europid patients from this register, with MIDD and the mtDNA 3243A>G mutation, were included. Patients with MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) or with mitochondrial diabetes related to other mutations or to deletions of mtDNA were excluded. Results: A significant negative correlation was found between levels of heteroplasmy and age of the patients at the time of sampling for molecular analysis, age at the diagnosis of diabetes, and body mass index. After adjustment for age at sampling for molecular study and gender, the correlation between heteroplasmy levels and age at the diagnosis of diabetes was no more significant. The two other correlations remained significant. A significant positive correlation between levels of heteroplasmy and HbA1c was also found and remained significant after adjustment for age at molecular sampling and gender. Conclusions: These results support the hypothesis that heteroplasmy levels are at least one of the determinants of the severity of the phenotype in MIDD. Heteroplasmy levels are at least one of the determinants of the severity of the phenotype of maternally inherited diabetes and deafness

Orphanet J Rare Dis

BACKGROUND: The Registry of Adult and Paediatric Patients Treated with Cystadane(R) - Homocystinuria (RoCH) is a non-interventional, observational, multi-centre, post-authorization safety study that aimed to identify safety of betaine anhydrous (Cystadane(R)) in the treatment of patients with inborn errors of homocysteine metabolism (homocystinuria) in order to minimise the treatment associated risks and establish better knowledge on its clinical use. The registry included patients of all ages with homocystinuria who were treated with betaine anhydrous in conjunction with other therapies. Clinical data were collected retrospectively from 2007 to 2013, then prospectively up to February 2014. All adverse events (AEs) reported during the study were recorded. The clinical and biological status of patients was monitored at least once a year. RESULTS: A total of 125 patients with homocystinuria (adults [> 18 years]: 50; paediatric [</=18 years]: 75) were enrolled at 29 centres in France and Spain. Patients were treated with betaine anhydrous for a mean duration of 7.4 +/- 4.3 years. The median total daily dose of betaine anhydrous at the first and last study visits was 6 g/day for cystathionine beta-synthase (CBS)-deficient vitamin B6 responders and 9 g/day for methylenetetrahydrofolate reductase-deficient patients, while the median daily dose increased in CBS-deficient B6 non-responders (from 6 to 9 g/day) and cobalamin metabolism-defective patients (from 3 to 6 g/day) between the first and last visits. Treatment caused a mean overall reduction of 29% in plasma homocysteine levels in the study population. A total of 277 AEs were reported during the study, of which two non-serious AEs (bad taste and headache) and one serious AE (interstitial lung disease) were considered to be drug related. Overall, betaine anhydrous was well tolerated with no major safety concerns. CONCLUSIONS: Data from the RoCH registry provided real-world evidence on the clinical safety and efficacy of betaine anhydrous in the management of homocystinuria in paediatric and adult patients

Benralizumab for eosinophilic granulomatosis with polyangiitis: a retrospective, multicentre, cohort study

Background: Interleukin-5 (IL-5) inhibitors represent novel therapies for eosinophilic granulomatosis with polyangiitis (EGPA). This study assessed the effectiveness and safety of the IL-5 receptor inhibitor benralizumab in a European cohort of patients with EGPA. Methods: This retrospective cohort study included patients with EGPA from 28 European referral centres of the European EGPA Study Group across six countries (Italy, France, UK, Russia, Spain, and Switzerland) who received benralizumab as any line of treatment between Jan 1, 2019, and Sep 30, 2022. We assessed the rates of complete response, defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] of 0) and a prednisone dose of up to 4 mg/day, in contrast to partial response, defined as a BVAS of 0 and a prednisone dose greater than 4 mg/day. Active disease manifestations, pulmonary function, variation in glucocorticoid dose, and safety outcomes were also assessed over a 12-month follow-up. Findings: 121 patients with relapsing–refractory EGPA treated with benralizumab at the dose approved for eosinophilic asthma were included (64 [53%] women and 57 [47%] men; median age at the time of beginning benralizumab treatment 54·1 years [IQR 44·2–62·2]). Complete response was reported in 15 (12·4%, 95% CI 7·1–19·6) of 121 patients at month 3, 25 (28·7%, 19·5–39·4) of 87 patients at month 6, and 32 (46·4%, 34·3–58·8) of 69 patients at month 12; partial response was observed in an additional 43 (35·5%, 27·0–44·8) patients at month 3, 23 (26·4%, 17·6–37·0) at month 6, and 13 (18·8%, 10·4–30·1) at month 12. BVAS dropped from 3·0 (IQR 2·0–8·0) at baseline to 0·0 (0·0–2·0) at months 3 and 6, and to 0·0 (0·0–1·0) at month 12. The proportion of patients with systemic manifestations, active peripheral neurological disease, ear, nose, and throat involvement, and pulmonary involvement decreased, with an improvement in lung function tests. Six patients relapsed after having a complete response. The oral prednisone (or equivalent) dose decreased from 10·0 mg/day (5·0–12·5) at baseline to 5·0 mg/day (3·6–8·5) at month 3 (p&lt;0·01), to 5·0 mg/day (2·5–6·3) at month 6, and to 2·5 mg/day (0·0–5·0) at month 12 (p&lt;0·0001). 19 (16%) of 121 patients had adverse events and 16 (13%) discontinued benralizumab. Interpretation: These data suggest that benralizumab could be an effective treatment for EGPA in real-life clinical practice. Further clinical trials are required to confirm the efficacy of benralizumab in patients with a higher baseline disease activity. Funding: None