Respondence of steroid predicts the long-term clinical and endoscopic prognosis in Cronkhite–Canada syndrome
Bibliographic record
Abstract
BACKGROUND: Cronkhite-Canada syndrome (CCS) is a rare gastrointestinal polyposis syndrome characterized by hamartoma polyps and ectodermal abnormalities, with an increased risk of gastrointestinal cancers. Our previous study revealed its relapsing disease behavior and the improved long-term prognosis following the medications of steroids. However, the response of steroids varies among CCS patients, and there was no consensus about how to deal with steroid-dependent patients. AIM: This study aims to update the knowledge of long-term prognosis of CCS patients and explore the efficacy and safety of low-dose steroid as maintenance therapy. DESIGN: Observational cohort study comprising CCS patients hospitalized in Peking Union Medical College Hospital from 1999 until December 2023. METHODS: All CCS patients were followed up through telephone interviews and outpatient visits, with clinical data and endoscopic findings sequentially collected. The Cox regression model was used to identify factors associated with clinical outcomes. RESULTS: A total of 64 eligible participants were included with a median (interquartile range) follow-up duration of 49.5 (22.75, 101.25) months. The cumulative probability of overall survival (OS) was 90.2% at 3 years, 87.7% at 5 years, 78.6% at 10 years and relapse-free survival (RFS) after steroid treatment was 76.3% at 1 year, 62.9% at 3 years, 54.4% at 5 years, respectively. The poor response to steroids was identified as independent variable related to worse OS in multivariate model. In the subgroup analysis of relapsed patients, low-dose steroid treatment (prednisone ≤10 mg/day) for at least one year as maintenance therapy was in association with reduced risk of re-relapse (RFS: 72 (46.3, 78) vs. 12 (9, 15.5) months; P = 0.02). CONCLUSIONS: The long-term clinical outcomes of CCS are relatively favorable in patients who responded well to steroid treatment. The low-dose steroid maintenance therapy may prevent re-relapse of disease among CCS patients.
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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 | 0.000 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
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".