Sweet's Syndrome and Relapsing Polychondritis Signal Myelodysplastic Syndrome

Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica. Palavras-chave: Policondrite recidivante; Síndrome de Sweet; Síndromes paraneoplásica

How to clutch skills from higher education curricula: analysis of Portuguese learning outcomes

In Portugal, the Agency for Assessment and Accreditation of Higher Education - A3ES - promotes and ensures the quality of higher education, following the development of quality management systems in the European space. Thus, their mission is to contribute to improving the quality of Portuguese higher education, through the assessment and accreditation of higher education institutions and their study programmes, and to ensure the integration of Portugal in the European quality assurance system of higher education. This agency performs the assessment and accreditation of higher education study programmes in 1st, 2nd and 3rd cycles. At an initial stage of the accreditation process, HEI submit an accreditation proposal to A3ES. In the description of each study programme for evaluation, HEI must describe what are the “intended learning outcomes” they expected students will achieve at the end of a specific period of learning. Data analysis focused on information included in this question, and which is limited to 1000 characters. All proposal of study programs submitted to accreditation in A3ES, from 2009 to 2014, were examined (n = 2961). A content analysis was carried out using the MAXQDA software for qualitative data analysis (version 12). Adopting a theoretical thematic analysis approach, instead of a data-driven option (Braun & Clarke, 2006), a competence matrix of technical and generic skills were identified. Based on these qualitative results, a set of 30 learning competencies cited in all the Portuguese study cycle curricula official documentation was analysed. The relative importance of a given competency, in each curriculum, was roughly estimated by counting the number of corresponding mentions in the document submitted to A3ES for approval. A Principal Component Analysis, with varimax rotation, allowed the association of the 30 competencies with nine components. Each new component was, then, characterized based on the initial variables’ loadings. The nine components could also be associated with several distinct features of the higher education study programmes (study cycle and scientific area) and institutes (namely geographical location, university/polytechnic subsystem, and private/public sector). This study aims to contribute to the identification of areas for improvement of study programmes in Portugal.info:eu-repo/semantics/acceptedVersio

Restyling the higher education landscape: regional (a)symmetries across Portugal

This work studies the disparities found in higher education institutions across Portugal - in terms of geographical location, being polytechnics institutes or universities, and operating in the private or public sector - in relation to their offering of 1st, 2nd and 3rd cycle study programmes and scientific areas. Bearing in mind that, on the one hand, any education institution must adapt to the surrounding population, and that, on the other hand, the population also ends up adapting to the existing educational offer, there is always some synergy between the characteristics of higher education institutions and local population/social context. Therefore, it is of surmount importance to characterize the educational system and verify the presence of asymmetries, by evaluation of the way it relates and is related to the physical and social setting. At an initial stage of the accreditation process, HEI submit an accreditation proposal to national accreditation agency. In the description of each study programme for evaluation, HEI must describe what are the “intended learning outcomes” they expected students will achieve at the end of a specific period of learning. Data analysis focused on information included in this question, and which is limited to 1000 characters. All proposal of study programs submitted to accreditation, from 2009 to 2014, were examined (n = 2961). A content analysis was carried out using the MAXQDA software for qualitative data analysis (version 12). Adopting a theoretical thematic analysis approach, instead of a data-driven option (Braun & Clarke, 2006), a competence matrix of technical and generic skills were identified. Based on a descriptive analysis of the data, it was possible to cross-tabulate several variables and to do a thorough discussion, highlighting the influence of different internal aspects of the Portuguese Higher Education System. This data-based reflection is a contribute for future works aiming to understand the underlying dynamics behind such asymmetries. Knowing the regional asymmetries may provide opportunities to find innovative solutions that foster education on disadvantaged regions.info:eu-repo/semantics/acceptedVersio

Role of advanced technology in the detection of sight-threatening eye disease in a UK community setting.

Background/aims: To determine the performance of combinations of structural and functional screening tests in detecting sight-threatening eye disease in a cohort of elderly subjects recruited from primary care. Methods: 505 subjects aged ≥60 years underwent frequency doubling technology (FDT) perimetry, iVue optical coherence tomography (iWellness and peripapillary retinal nerve fibre layer (RNFL) scans) and intraocular pressure with the Ocular Response Analyzer, all performed by an ophthalmic technician. The reference standard was a full ophthalmic examination by an experienced clinician who was masked to the index test results. Subjects were classified as presence or absence of sight-threatening eye disease (clinically significant cataract, primary open-angle glaucoma, intermediate or advanced age-related macular degeneration and significant diabetic retinopathy). Univariate and multivariate logistic regression analyses were used to determine the association between abnormal screening test results and the presence of sight-threatening eye disease. Results: 171 subjects (33.8%) had one or more sight-threatening eye diseases. The multivariate analysis found significant associations with any of the target conditions for visual acuity of <6/12, an abnormal FDT and peripapillary RNFL thickness outside the 99% normal limit. The sensitivity of this optimised screening panel was 61.3% (95% CI 53.5 to 68.7), with a specificity of 78.8% (95% CI 74.0 to 83.1), a positive predictive value of 59.5% (95% CI 53.7 to 65.2) and an overall diagnostic accuracy of 72.9% (95% CI 68.8 to 76.8). Conclusions: A subset of screening tests may provide an accurate and efficient means of population screening for significant eye disease in the elderly. This study provides useful preliminary data to inform the development of further larger, multicentre screening studies to validate this screening panel

Limitations and perceived delays for diagnosis and staging of lung cancer in Portugal: A nationwide survey analysis

Background We aimed to identify the perception of physicians on the limitations and delays for diagnosing, staging and treatment of lung cancer in Portugal. Methods Portuguese physicians were invited to participate an electronic survey (Feb-Apr-2020). Descriptive statistical analyses were performed, with categorical variables reported as absolute and relative frequencies, and continuous variables with non-normal distribution as median and interquartile range (IQR). The association between categorical variables was assessed through Pearson’s chi-square test. Mann-Whitney test was used to compare categorical and continuous variables (Stata v.15.0). Results Sixty-one physicians participated in the study (45 pulmonologists, 16 oncologists), with n = 26 exclusively assisting lung cancer patients. Most experts work in public hospitals (90.16%) in Lisbon (36.07%). During the last semester of 2019, responders performed a median of 85 (IQR 55–140) diagnoses of lung cancer. Factors preventing faster referral to the specialty included poor articulation between services (60.0%) and patients low economic/cultural level (44.26%). Obtaining National Drugs Authority authorization was one of the main reasons (75.41%) for delaying the begin of treatment. The cumulative lag-time from patients’ admission until treatment ranged from 42–61 days. Experts believe that the time to diagnosis could be optimized in around 11.05 days [IQR 9.61–12.50]. Most physicians (88.52%) started treatment before biomarkers results motivated by performance status deterioration (65.57%) or high tumor burden (52.46%). Clinicians exclusively assisting lung cancer cases reported fewer delays for obtaining authorization for biomarkers analysis (p = 0.023). Higher waiting times for surgery (p = 0.001), radiotherapy (p = 0.004), immunotherapy (p = 0.003) were reported by professionals from public hospitals. Conclusions Physicians believe that is possible to reduce delays in all stages of lung cancer diagnosis with further efforts from multidisciplinary teams and hospital administration.This work was supported by AstraZeneca. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript

Phototherapy in Childhood: a 17-Year Retrospective Study Regarding Effectiveness and Safety

Introduction: Skin diseases in paediatric age are often distressing conditions with significant impact in children’s psychosocial development. Additionally, systemic therapeutic options are often limited in childhood, due to its potential toxicity in this vulnerable group. Phototherapy is therefore an endorsed option for photo-responsive dermatological conditions. Objective and Methods:This observational retrospective study aims to access efficacy and safety of Phototherapy in our paediatric population. Relevant clinical data from 1996 to present concerning patients aged 18 years or less was collected. Results: 78 patients were included, of which64,1%was female. Mean age was 12,9 years (range 2-18). Distribution according to diagnosis was:47,4%psoriasis, 34,6% alopecia areata, 9,0% vitiligo, 9,0% other diagnosis. Mean number of cycles was 1,5 (range 1-7), with an average of 16,3 treatments per cycle and mean cumulative dose 134 J/cm2. 70,5% was treated with one single cycle. Topic and systemic PUVA were the first choice in 37,2% and 39,7%, respectively, while UVB TL01 and broadband UVB were used in 11,5% each. On the first cycle 67,5% improved, 14,3% showed no sustained clinical response and 19,5% were lost to follow-up. Psoriasis patients had the best response rates (81,8%), followed by alopecia areata (59,3%). Side effects occurred in 21%, being erythema the most common (12%). None led to therapeutic interruption. Discussion: Phototherapy is a safe and effective option in childhood, yet the withdraw rate might be an important limitation