Atteintes Oculaires Au Cours Du Syndrome De Wolfram À Propos De Deux Cas Et Revue De La Littérature

Introduction: Wolfram syndrome is an autosomal recessive neurodegenerative disorder. Diabetes mellitus and juvenile bilateral optic atrophy are its major signs. It is recognized that this association, which started in childhood or during adolescence, is sufficient to diagnose Wolfram syndrome. Optic atrophy occurs in 98% to 100% of cases with an average age of onset of 11 years. We reported a study of two confirmed cases referred by the internal medicine department. Observations: Case 1: A 23- year-old woman, deaf and dumb by birth, went through a diabetic ketosis test. Ophthalmologic examination showed reduced visual acuity in the fingers at 5 meters P2 in both eyes. Also, the fundus of the eye showed bilateral atrophic papillary palpation with no signs of retinopathy. She had deafness of deep perception and hypogonadotropic hypogonadism. Deafness, diabetes, optic atrophy, and hypogonadism led to the diagnosis. Case 2: A 21-year-old man born from a first-degree consanguineous marriage serves as a supplement to the management of diabetes. The visual acuity was at counting fingers at 1m to the right eye and sees the hand move to 0.5 m to the left eye. On examination at the slit lamp, it had a bilateral dense cataract. After phacoexeresis, the base revealed bilateral optic atrophy. Ultrasound of the urinary tree showed hypotonia of the renal cavities and a neurogenic bladder. Also, audiometry showed mild sensory deafness. The diagnosis of Wolfram syndrome was made in front of the tetrad: diabetes, optic atrophy, deafness, and urinary signs. Discussion: Wolfram syndrome may be familial or sporadic. The gene however is located on the short arm of chromosome 4. Optic atrophy is secondary to the involvement of pre-genetic fibers, and it is characterized initially by temporal palpation of the papilla. The evolution towards diffuse whitish discoloration occurs in a few months or years with the gradual establishment of a blindness around the age of 17 to 30 years. Conclusion: Wolfram syndrome is a clinical entity characterized by clinical and genetic polymorphism. This diagnosis should be considered in the presence of any type I diabetes associated with optic atrophy in childre

Etude des connaissances, des attitudes, et des pratiques des Chirurgiens-dentistes exerçant au Mali, sur la radioprotection.

The use of ionizing radiation (IR) for medical purposes, the main artificial source of exposure, can be responsible for long-term stochastic effects in practitioners and patients. The objective of this study was to evaluate the knowledge, attitudes, and practices of dental surgeons practicing in Mali, in terms of radiation protection. A self-administered questionnaire designed on Googleforms was sent via Whatsapp to practitioners. The main items explored were: knowledge of radiation protection institutions, use of radiation protection devices, knowledge of the most exposed organs, risks of exposure to ionizing radiation, and application of radiation protection measures during radiographic examinations of children and pregnant women. The data were exported and analyzed using SPSS 20.0 software. The level of significance was set at p≤0.05.Of 123 questionnaires sent, 51 were completely filled out, for a response rate of 41.46%. Nearly 70% (60.8%) of the practitioners were unaware of the existence of any regulatory authority for radiation protection. The thyroid was considered the organ most exposed to ionizing radiation by 33.3% of practitioners. Only 31.4% of practitioners reported having a radiation protection device in their facility. Among practitioners who performed radiographic examinations themselves, 46.15% did not use any radiation protection equipment during examinations of children and pregnant women. There is a real need to strengthen the capacity of dentists practicing in Mali in the area of radiation protection through the introduction of continuing education on the subject.      L’utilisation médicale des rayonnements ionisants (RI) peut être responsable d’effets stochastiques à long terme chez les praticiens et les patients. L’objectif de cette étude était, d’évaluer les connaissances, attitudes, et pratiques des Chirurgiens-dentistes exerçant au Mali, en matière de radioprotection.Un questionnaire auto-admi­nistré conçu sur Google Forms a été envoyé aux praticiens. Les principaux items explorés étaient : la connaissance des institutions de radioprotection, l’usage des dispositifs de radioprotection, la connaissance des organes les plus exposés, des  risques de l’exposition aux rayonnements ionisants et l’application des mesures de radioprotection lors des examens radiographiques chez les enfants et les femmes enceintes. Les données ont été  exportées et analysées à l’aide du lo­giciel SPSS 20.0. Le niveau de significativité est fixé à p≤0,05.Sur 123 questionnaires envoyés, 51 ont été complètement renseignés soit un taux de réponse de 41,46 %. Près de 70% (60,8%) des praticiens ignoraient l’existence de tout organisme de règlementation en matière de radioprotection. La thyroïde était considérée comme l’organe le plus exposé aux rayonnements ionisants par 33,3%  des praticiens. Seuls 31,4% des praticiens déclaraient avoir un dis­positif de radioprotection dans leur structure. Parmi les praticiens réalisant eux-mêmes les examens radiographiques, 46,15% n’utilisaient aucun moyen de radioprotection lors des examens chez l’enfant et les femmes enceintes. Il existe un réel besoin de renforcement des capacités des chirurgiens-dentistes exerçant au Mali en matière de radioprotection à travers l’instauration d’une formation continue sur le sujet

Etude des connaissances, des attitudes, et des pratiques des Chirurgiens-dentistes exerçant au Mali, sur la radioprotection.

The use of ionizing radiation (IR) for medical purposes, the main artificial source of exposure, can be responsible for long-term stochastic effects in practitioners and patients. The objective of this study was to evaluate the knowledge, attitudes, and practices of dental surgeons practicing in Mali, in terms of radiation protection. A self-administered questionnaire designed on Googleforms was sent via Whatsapp to practitioners. The main items explored were: knowledge of radiation protection institutions, use of radiation protection devices, knowledge of the most exposed organs, risks of exposure to ionizing radiation, and application of radiation protection measures during radiographic examinations of children and pregnant women. The data were exported and analyzed using SPSS 20.0 software. The level of significance was set at p≤0.05.Of 123 questionnaires sent, 51 were completely filled out, for a response rate of 41.46%. Nearly 70% (60.8%) of the practitioners were unaware of the existence of any regulatory authority for radiation protection. The thyroid was considered the organ most exposed to ionizing radiation by 33.3% of practitioners. Only 31.4% of practitioners reported having a radiation protection device in their facility. Among practitioners who performed radiographic examinations themselves, 46.15% did not use any radiation protection equipment during examinations of children and pregnant women. There is a real need to strengthen the capacity of dentists practicing in Mali in the area of radiation protection through the introduction of continuing education on the subject.      L’utilisation médicale des rayonnements ionisants (RI) peut être responsable d’effets stochastiques à long terme chez les praticiens et les patients. L’objectif de cette étude était, d’évaluer les connaissances, attitudes, et pratiques des Chirurgiens-dentistes exerçant au Mali, en matière de radioprotection.Un questionnaire auto-admi­nistré conçu sur Google Forms a été envoyé aux praticiens. Les principaux items explorés étaient : la connaissance des institutions de radioprotection, l’usage des dispositifs de radioprotection, la connaissance des organes les plus exposés, des  risques de l’exposition aux rayonnements ionisants et l’application des mesures de radioprotection lors des examens radiographiques chez les enfants et les femmes enceintes. Les données ont été  exportées et analysées à l’aide du lo­giciel SPSS 20.0. Le niveau de significativité est fixé à p≤0,05.Sur 123 questionnaires envoyés, 51 ont été complètement renseignés soit un taux de réponse de 41,46 %. Près de 70% (60,8%) des praticiens ignoraient l’existence de tout organisme de règlementation en matière de radioprotection. La thyroïde était considérée comme l’organe le plus exposé aux rayonnements ionisants par 33,3%  des praticiens. Seuls 31,4% des praticiens déclaraient avoir un dis­positif de radioprotection dans leur structure. Parmi les praticiens réalisant eux-mêmes les examens radiographiques, 46,15% n’utilisaient aucun moyen de radioprotection lors des examens chez l’enfant et les femmes enceintes. Il existe un réel besoin de renforcement des capacités des chirurgiens-dentistes exerçant au Mali en matière de radioprotection à travers l’instauration d’une formation continue sur le sujet

Does health literacy mediate the relationship between socioeconomic status and health related outcomes in the Belgian adult population?

peer reviewed[en] BACKGROUND: Health literacy (HL) has been put forward as a potential mediator through which socioeconomic status (SES) affects health. This study explores whether HL mediates the relation between SES and a selection of health or health-related outcomes. METHODS: Data from the participants of the Belgian health interview survey 2018 aged 18 years or older were individually linked with data from the Belgian compulsory health insurance (n = 8080). HL was assessed with the HLS-EU-Q6. Mediation analyses were performed with health behaviour (physical activity, diet, alcohol and tobacco consumption), health status (perceived health status, mental health status), use of medicine (purchase of antibiotics), and use of preventive care (preventive dental care, influenza vaccination, breast cancer screening) as dependent outcome variables, educational attainment and income as independent variables of interest, age and sex as potential confounders and HL as mediating variable. RESULTS: The study showed that unhealthy behaviours (except alcohol consumption), poorer health status, higher use of medicine and lower use of preventive care (except flu vaccination) were associated with low SES (i.e., low education and low income) and with insufficient HL. HL partially mediated the relationship between education and health behaviour, perceived health status and mental health status, accounting for 3.8-16.0% of the total effect. HL also constituted a pathway by which income influences health behaviour, perceived health status, mental health status and preventive dental care, with the mediation effects accounting for 2.1-10.8% of the total effect. CONCLUSIONS: Although the influence of HL in the pathway is limited, our findings suggest that strategies for improving various health-related outcomes among low SES groups should include initiatives to enhance HL in these population groups. Further research is needed to confirm our results and to better explore the mediating effects of HL

Multimorbidity healthcare expenditure in Belgium: a 4-year analysis (COMORB study)

Background: The complex management of health needs in multimorbid patients, alongside limited cost data, presents challenges in developing cost-effective patient-care pathways. We estimated the costs of managing 171 dyads and 969 triads in Belgium, taking into account the influence of morbidity interactions on costs. Methods: We followed a retrospective longitudinal study design, using the linked Belgian Health Interview Survey 2018 and the administrative claim database 2017–2020 hosted by the Intermutualistic Agency. We included people aged 15 and older, who had complete profiles (N = 9753). Applying a system costing perspective, the average annual direct cost per person per dyad/triad was presented in 2022 Euro and comprised mainly direct medical costs. We developed mixed models to analyse the impact of single chronic conditions, dyads and triads on healthcare costs, considering two-/three-way interactions within dyads/triads, key cost determinants and clustering at the household level. Results: People with multimorbidity constituted nearly half of the study population and their total healthcare cost constituted around three quarters of the healthcare cost of the study population. The most common dyad, arthropathies + dorsopathies, with a 14% prevalence rate, accounted for 11% of the total national health expenditure. The most frequent triad, arthropathies + dorsopathies + hypertension, with a 5% prevalence rate, contributed 5%. The average annual direct costs per person with dyad and triad were €3515 (95% CI 3093–3937) and €4592 (95% CI 3920–5264), respectively. Dyads and triads associated with cancer, diabetes, chronic fatigue, and genitourinary problems incurred the highest costs. In most cases, the cost associated with multimorbidity was lower or not substantially different from the combined cost of the same conditions observed in separate patients. Conclusion: Prevalent morbidity combinations, rather than high-cost ones, made a greater contribution to total national health expenditure. Our study contributes to the sparse evidence on this topic globally and in Europe, with the aim of improving cost-effective care for patients with diverse needs

Travel burden and clinical presentation of retinoblastoma: analysis of 1024 patients from 43 African countries and 518 patients from 40 European countries

BACKGROUND: The travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe. METHODS: A cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries. RESULTS: Capture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI -12.4 to -5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease. CONCLUSIONS: Fewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral