Congenital Myotonic Dystrophy: Assisted Ventilation Duration and Outcome
Bibliographic record
Abstract
OBJECTIVE: To clarify the relationship between initial assisted ventilation duration and outcome for patients with congenital myotonic dystrophy (CDM). METHODS: A retrospective chart review was conducted of cases of CDM that presented to the Children's Hospital of Eastern Ontario (Ottawa, Ontario, Canada) between 1980 and 2000. Inclusion criteria were conclusive testing for CDM and clinical presentation in the first 30 days of life. Duration of assisted ventilation, morbidity, mortality, and developmental outcome were measured. RESULTS: A total of 23 children met the inclusion criteria. One child died at 5 days of age, and 2 others had withdrawal of ventilation. The remaining 20 children were divided into 2 groups on the basis of whether they needed > or <30 days of ventilation. In the first year of life, 25% mortality was noted in the children with prolonged ventilation, whereas no child in the short ventilation duration group died. After 1 year of age, 1 child in each group died with follow-up of 2 to 16 years. The children with prolonged ventilation needed more hospitalizations. Delays were noted in development in both groups of children at ages 1, 3, and 6 years; however, there was an improvement in motor and language scores over time in all children. Children who required ventilation for <30 days had better motor, language, and activities of daily living scores at all ages. CONCLUSIONS: Children with CDM with prolonged ventilation experienced 25% mortality in the first year. The use of a specific time period of ventilation to decide on withdrawal of therapy must be reconsidered given these findings. Prolonged ventilation was followed by greater morbidity and developmental delay than children with shorter ventilation duration.
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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 | 0.004 |
| 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".