Systemic sclerosis (SSc) is a chronic orphan autoimmune disease with the highest mortality rate among rheumatic diseases. SSc-related interstitial-lung disease (ILD) remains among the leading causes ...of SSc-related mortality with still few therapeutic effective strategies. In patients with crystallin silica exposure, SSc is recognized as an occupational disease according to the French social security system (Table 25A of the general insurance regimen). Lympho-ablative or myeloablative immunosuppression followed by autologous hematopoietic stem-cell transplantation (aHSCT) is the only therapeutic approach with demonstrated efficacy, improved survival with disease modifying effects on SSc-fibrotic manifestations (skin disease and ILD) and quality of life. A documented past and/or present occupational silica exposure, with extensive exposure and/or silica-related ILD and/or with persistent silica content in the broncho-alveolar lavage fluid are contra-indications to aHSCT in SSc patients, due to the risk of silica-related malignancy or of SSc relapse. This article aims to discuss alternative options in SSc patients with a history of silica exposure, and how innovative cellular therapies (mesenchymal stromal cells, CAR cells) could represent new therapeutic options for these patients.
La sclérodermie systémique (SSc) est une maladie auto-immune orpheline présentant le taux de mortalité le plus élevé parmi les rhumatismales inflammatoires chroniques. La maladie pulmonaire interstitielle (ILD) liée à la SSc demeure l’une des principales causes de mortalité liée à la SSc, avec encore peu de stratégies thérapeutiques efficaces. Chez les patients exposés à la silice cristalline, la SSc est reconnue comme une maladie professionnelle selon le système de sécurité sociale français (Tableau 25A du régime général d’assurance). La transplantation de cellules souches hématopoïétiques autologues (aHSCT) suivie d’une immunosuppression lympho-ablative ou myéloablative est la seule approche thérapeutique ayant démontré son efficacité, améliorant la survie, les manifestations fibrosantes de la SSc (maladie cutanée et ILD) et la qualité de vie. Une exposition professionnelle documentée passée et/ou présente à la silice, avec un taux d’exposition important et/ou une ILD liée à la silice et/ou la présence de silice dans le lavage bronchoalvéolaire constituent des contre-indications à l’aHSCT chez les patients atteints de SSc, en raison du risque de malignité liée à la silice ou de rechute de la SSc. Cet article vise à discuter des options alternatives chez les patients atteints de SSc ayant des antécédents d’exposition à la silice, et de la manière dont les thérapies cellulaires innovantes (cellules stromales mésenchymateuses, cellules CAR) pourraient représenter de nouvelles options thérapeutiques pour ces patients.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Uncertainty is a fundamental aspect of medical practice, necessitating incorporation into undergraduate medical training. The integrative model of uncertainty tolerance (UT) developed by Hillen and ...Han serves as a comprehensive framework for exploring clinical uncertainty. While studies have extensively examined UT dimensions, including sources, responses, and moderators, the factors influencing the perception of uncertainty stimuli remain underexplored. However, students' ability to perceive uncertainty and their approach to uncertain stimuli play a crucial role in enabling them to develop adaptive responses to uncertainty, necessary for their comfort in these situations. Defining uncertainty as a metacognitive state suggests significant variability in its perception among individuals and within an individual over time. Moreover, several studies have demonstrated the substantial influence of various individual and contextual factors on how individuals perceive and respond to uncertainty. In this paper, the authors present multiple hypotheses to address the question of whether students genuinely perceive uncertainty stimuli when they should. The authors argue that students' personal relationship with their knowledge is essential in their ability to identify clinical uncertainty, particularly concerning the limits of medical knowledge. Therefore, they propose that an academic culture fostering doubt, through exposing students to a variety of perspectives, would enhance their ability to identify uncertainty zones in a clinical situation at an early stage. Drawing on Dewey's situational theory, the authors emphasize the importance of better understanding, in a work setting, the influence of contextual and situational characteristics on individual perceptions of uncertainty. In line with this idea, ethnographic studies would offer valuable insights into identifying the relationship between the students, their work environment, and their perception of clinical uncertainty.
Splenic infarction is a rare event in clinical practice, diagnosed by CT scan. There are many causes. They often determine the treatment given. However, there is no consensus on etiological ...investigations.
We present here an almost systematic review of the literature, based on data available on Pubmed from 1991 to 2022. Using the keywords "splenic infarct", from 1893 references, 11 cohort studies and 867 clinical cases were included in this review. Articles written in languages using alphabets other than Latin were excluded.
Analysis of these various studies has enabled us to draw up a list that is intended to be as exhaustive as possible of the causes of splenic infarction. The most frequent are emboligenic heart disease, hematological malignancies, solid neoplasia and certain infections. The descriptions available in the literature were mainly based on isolated clinical cases, not always making it possible to establish a causal link with the disease described, especially as around 20% of reported cases of splenic infarction were asymptomatic and potentially of incidental discovery. Based on the findings of this literature review, we propose a protocol for the etiological assessment of splenic infarcts.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Uncertainty in inherent to every aspects of medical practice. As the concept of uncertainty in healthcare is still to explore, deciphering the determinants and the roots of this uncertainty would ...benefit from the insights of various disciplines, such as epistemology, sociology, mathematics, or philosophy. The urgent need to improve physician's ability to cope with uncertainty, has been recently highlighted by the COVID-19 pandemic. Besides, the concept of uncertainty tolerance has been proposed, and could serve as a relevant basis for approaching uncertainty, in medical education. Thus, we propose at first to discuss the uncertainty tolerance framework from Hillen et al. Then, from an educational perspective, we outline some avenues regarding how uncertainty tolerance could be thought, in a competence-based approach, and discuss several educational activities, which have proven efficient in promoting uncertainty tolerance among medical practitioners abroad.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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