Does timing of diagnosis effect outcome in primary ciliary dyskinesia?
Bibliographic record
Abstract
Introduction: Early diagnosis in cystic fibrosis has been shown to improve long term outcomes; similar evidence is lacking in children with primary ciliary dyskinesia. The aim of this study was to compare long term clinical outcomes according to age of diagnosis. Methods: This is a retrospective study of children with confirmed diagnosis of PCD followed at The Hospital for Sick Children (Toronto, Canada) between 2000-2022 . The cohort was divided into three different age groups: 1) early diagnosis group, when diagnosed<1 year of age, 2) diagnosis at ages 5-9 years, 3) diagnosis at ages 10-14 years. Results: 93 children (40/28/25 children in groups 1/2/3 respectively) with PCD were included in the study. Laterality defect (LD) and unexplained neonatal respiratory distress (NRD) were significantly more common in group 1 compared to both group 2 (LD 63% vs 36%, NRD 80% vs 36%) and group 3 (LD 63% vs 32%, NRD 80% vs 52%). At age 8, there was no significant differences in FEV1 precent predicted or body mass index between group 1 and 2 (92.4% [9.5] vs 95.8% [17.1]). At age 14, patients in group 2 had significantly higher FEV1 values compared to group 3 (92.3% (19.6) vs 81.4% (15), p=0.048). For the following three consecutive years, (ages 15-17), although not statistically significant, a similar trend was seen with annual higher FEV1 values in group 2. At age 17, mean FEV1 values were 93% (22.7) vs 80.7% in groups 2 and 3, respectively. Conclusion: Late diagnosis in PCD was associated with lower lung function during the adolescent years, emphasizing the importance of earlier diagnosis in children with PCD.
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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 | 0.003 | 0.010 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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".