Diabète de la mucoviscidose à l’ère des modulateurs de CFTR
Bibliographic record
Abstract
La mucoviscidose (MV) est une maladie génétique caractérisée par une détérioration des fonctions respiratoires et digestives. Chez environ 30 % des adultes vivant avec la MV, la maladie peut par ailleurs se compliquer d’un diabète spécifique appelé diabète de la mucoviscidose (DMV), longtemps associé à un pronostic défavorable. La systématisation du dépistage et du traitement précoce du DMV a heureusement permis une augmentation notable de l’âge médian de survie. En outre, de nouveaux traitements appelés modulateurs de CFTR ( cystic fibrosis transmembrane regulator ), dont l’elexacaftor/tezacaftor/ivacaftor (E/T/I), améliorent encore davantage le parcours de vie des personnes vivant avec la MV (pvMV). Ces modulateurs corrigent les défauts moléculaires à l’origine des symptômes et améliorent ainsi drastiquement l’état respiratoire et nutritionnel, ainsi que la qualité de vie des pvMV. Cependant, l’effet des modulateurs sur le DMV reste équivoque. Quelques études suggèrent que l’E/T/I pourrait préserver la sécrétion d’insuline et améliorer les variables glycémiques, tandis que d’autres ne confirment pas ces résultats. De plus, les prises de poids parfois importantes sous E/T/I pourraient entraîner des conséquences métaboliques, en majorant la résistance à l’insuline et potentiellement le risque cardiovasculaire. Cette revue résume les connaissances actuelles sur le DMV, et particulièrement sur son évolution à l’ère des modulateurs. Cystic fibrosis (CF) is a genetic disease characterized by impaired respiratory and digestive functions. Approximately 30% of adults living with CF develop a complication called CF-related diabetes (CFRD). Until recently, CFRD was associated with worse health outcomes, but the systematization of early detection and treatment of CFRD has greatly increased the median age of survival. Furthermore, recently developed modulator treatments, such as elexacaftor/tezacaftor/ivacaftor (E/T/I), are further improving the life course of people with CF (pwCF). These modulators correct the molecular causes of CF, and have demonstrated drastic improvements in respiratory and nutritional outcomes, as well as quality of life among pwCF. The metabolic effects of these modulators, however, remain equivocal. While some studies suggest that E/T/I may preserve insulin secretion and improve glycemic variables, others fail to replicate these findings. Additionally, weight gain following E/T/I treatment could result in metabolic consequences, with increased insulin resistance and potentially elevated cardiovascular risk. This review aims to provide a comprehensive summary of the current understanding of CFRD, with a specific focus on its evolution in the era of modulators.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.005 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".