ArticleThe European respiratory journal2019
Development of a prognostic model of respiratory insufficiency or death in amyotrophic lateral sclerosis.
Article in The European respiratory journal, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07071935 (A Pilot Randomized Clinical Trial of Early Ventilation in Amyotrophic Lateral Sclerosis), which is not on this map. Cited by 24 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
A Pilot Randomized Clinical Trial of Early Ventilation in Amyotrophic Lateral Sclerosis (EVENT ALS)
Who cites it
24 citing papers in PubMed, 2 syntheses or guidelines pooled it, 43 citations in OpenAlex.
- Methods and Applications in Respiratory Physiology: Respiratory Mechanics, Drive and Muscle Function in Neuromuscular and Chest Wall Disorders.Frontiers in physiology · 2022Pooled it
- Can patient-reported outcome measures predict mortality in neurological populations? A systematic review.Frontiers in neurologyPooled it
- A nomogram for estimating baseline respiratory insufficiency in patients with amyotrophic lateral sclerosis.Annals of medicine · 2026Article
- The role of adiponectin and cytokines in Amyotrophic lateral sclerosis: assessment of disease progression and survival status.Scientific reports · 2026Article
- Clinical and Paraclinical Predictors of Survival in Amyotrophic Lateral Sclerosis: Results from a Three-Year Longitudinal Cohort Study.Medical sciences (Basel, Switzerland) · 2025Article
- Article
- FVC-DiP correlates with neurofilament light chain levels in serum and cerebrospinal fluid in patients with ALS.BMJ neurology open · 2025Article
- Effects of Respiratory Training on Pulmonary Function, Cough, and Functional Independence in Patients with Amyotrophic Lateral Sclerosis.Neurology international · 2024Article
- Predicting clinical events characterizing the progression of amyotrophic lateral sclerosis via machine learning approaches using routine visits data: a feasibility study.BMC medical informatics and decision making · 2024Article
- Review
- Pulmonary care for ALS: Progress, gaps, and paths forward.Muscle & nerve · 2023Review
- Predicting dysphagia onset in patients with ALS: the ALS dysphagia risk score.Amyotrophic lateral sclerosis & frontotemporal degeneration · 2022Article
- The cumulative incidence of dysphagia and dysphagia-free survival in persons diagnosed with amyotrophic lateral sclerosis.Muscle & nerve · 2021Article
- Noninvasive Ventilation Use Is Associated with Better Survival in Amyotrophic Lateral Sclerosis.Annals of the American Thoracic Society · 2021Article
- Highlights from the Respiratory Failure and Mechanical Ventilation 2020 Conference.ERJ open research · 2021Review
- Modeling and Bioinformatics Identify Responders to G-CSF in Patients With Amyotrophic Lateral Sclerosis.Frontiers in neurology · 2021Article
- The adult multidisciplinary respiratory neuromuscular clinic.Breathe (Sheffield, England) · 2020Review
- Sleep and Sleep Disruption in Amyotrophic Lateral Sclerosis.Current neurology and neuroscience reports · 2020Review
- Patterns of VC Decline in Amyotrophic Lateral Sclerosis. A More Robust Prognostication?American journal of respiratory and critical care medicine · 2019Article
- Classifying Patients with Amyotrophic Lateral Sclerosis by Changes in FVC. A Group-based Trajectory Analysis.American journal of respiratory and critical care medicine · 2019Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
A clinically useful model to prognose onset of respiratory insufficiency in amyotrophic lateral sclerosis (ALS) would inform disease interventions, communication and clinical trial design. We aimed to derive and validate a clinical prognostic model for respiratory insufficiency within 6 months of presentation to an outpatient ALS clinic.We used multivariable logistic regression and internal cross-validation to derive a clinical prognostic model using a single-centre cohort of 765 ALS patients who presented between 2006 and 2015. External validation was performed using the multicentre Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database with 7083 ALS patients. Predictors included baseline characteristics at first outpatient visit. The primary outcome was respiratory insufficiency within 6 months, defined by initiation of noninvasive ventilation, forced vital capacity (FVC) <50% predicted, tracheostomy, or death.Of 765 patients in our centre, 300 (39%) had respiratory insufficiency or death within 6 months. Six baseline characteristics (diagnosis age, delay between symptom onset and diagnosis, FVC, symptom onset site, amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) total score and ALSFRS-R dyspnoea score) were used to prognose the risk of the primary outcome. The derivation cohort c-statistic was 0.86 (95% CI 0.84-0.89) and internal cross-validation produced a c-statistic of 0.86 (95% CI 0.85-0.87). External validation of the model using the PRO-ACT cohort produced a c-statistic of 0.74 (95% CI 0.72-0.75).We derived and externally validated a clinical prognostic rule for respiratory insufficiency in ALS. Future studies should investigate interventions on equivalent high-risk patients.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.