ANALYSIS OF AN ADULT DUCHENNE MUSCULAR DYSTROPHY POPULATION: TEN YEARS ON
Bibliographic record
Abstract
Background Duchenne muscular dystrophy (DMD) is a progressive, multi-systemic condition, management of which has benefitted from improvements in research and healthcare. Aim 1. To review the multidisciplinary management of a late adolescent and adult DMD population attending the Lane Fox Respiratory Unit. 2. To compare the findings to those of an audit conducted 10 years previously (Parker et al, QJM. 2005.), so to identify areas of improvement and issues that require further input. Method We studied 30 patients with DMD referred to an adult neuromusclular clinic over a ten-year period. Clinical details were obtained from case notes or direct observations. Results The age for our cohort ranged from 19 to 49 years, all of whom became symptomatic before ten years old. Nine patients died during the study period. 25 patients were wheelchair bound in their mid-teens. Over the last ten years, areas of improvement include provision of non-invasive ventilation, provision of aids, and access to education (9/30 of our cohort attended university or college, and three patients are in active employment). Areas of care that need further input are management of mood, bowel hygiene, fatigue and pain. Discussion Despite improvements in several areas, there is still a need for a consistent and multi-faceted approach to management of DMD.
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.001 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| 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".