ArticlePediatric pulmonology2019
Relationship between respiratory function and need for NIV in childhood SMA.
Article in Pediatric pulmonology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 27 citations in OpenAlex.
- A Systematic Literature Review of the Natural History of Respiratory, Swallowing, Feeding, and Speech Functions in Spinal Muscular Atrophy (SMA).Journal of neuromuscular diseases · 2024Pooled it
- Machine learning-based integration of respiratory oscillometry and spirometry for predicting clinical outcomes in spinal muscular atrophy.European journal of pediatrics · 2026Article
- The role of sleep-disordered breathing in adolescents with spinal muscular atrophy.Sleep & breathing = Schlaf & Atmung · 2026Review
- Multiple respiratory assessment and thresholds for noninvasive ventilation in adult patients with spinal muscular atrophy.Scientific reports · 2025Article
- Sleep and sleep-related breathing disorders in patients with spinal muscular atrophy: a changing perspective from novel treatments?Frontiers in neurology · 2024Review
- Structured Light Plethysmography for Non-Invasive Assessment of Respiratory Pattern in Spinal Muscular Atrophy Type 1.Journal of clinical medicine · 2023Article
- Oscillometry: A substitute of spirometry in children with neuromuscular diseases?Pediatric pulmonology · 2022Article
- Natural history of respiratory muscle strength in spinal muscular atrophy: a prospective national cohort study.Orphanet journal of rare diseases · 2022Article
- Spinal muscular atrophy: Broad disease spectrum and sex-specific phenotypes.Biochimica et biophysica acta. Molecular basis of disease · 2021Review
- Respiratory Trajectories in Type 2 and 3 Spinal Muscular Atrophy in the iSMAC Cohort Study.Neurology · 2021Observational
- Reply to ChackoAmerican journal of respiratory and critical care medicine · 2020Article
- Limited Assessment of Respiratory Muscle Response to Nusinersen Treatment in Infants with Spinal Muscular Atrophy.American journal of respiratory and critical care medicine · 2020Article
- Relationship between respiratory function and need for NIV in childhood SMA.Pediatric pulmonology · 2019Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSpinal muscular atrophy (SMA) causes progressive respiratory muscle weakness but respiratory function (RF) in those using noninvasive ventilation (NIV) is not well described.
objectiveTo describe RF in childhood SMA and assess differences between those using and not using NIV.
methodsA cross-sectional study of childhood SMA assessed polysomnography (PSG), spirometry, forced oscillation technique (FOT), lung clearance index (LCI), sniff nasal inspiratory pressures, peak cough flow, maximal inspiratory and expiratory pressure, and NIV use and indication.
resultsTwenty-five children (median age [interquartile range], 8.96 [5.63] years; 10 F) with SMA 1 (n = 3), 2 (n = 15), and 3 (n = 7) were recruited. Spirometry and FOT testing was feasible in children as young as 3 years. Ten (40%) required NIV, 5 for sleep-disordered breathing (SDB), and 5 initiated during lower respiratory tract infection (LRTI). Children requiring NIV were older (median, 10.52 vs 5.67 years; P < .02) with more abnormal forced vital capacity (FVC) z-score (-5.70 vs -1.39, P < .02), Rsr8 z-score (1.97 vs 0.50, P = .04), and LCI (8.84 vs 7.34, P = .01). Two had normal RF and SDB. For FVC z-score less than -2.5 and LCI greater than 7.5, the odds ratio for NIV was 10.70 (95% confidence interval [CI], 1.39-82.03) and 2 (95% CI, 0.40-10.31), respectively. All children with LCI greater than 8 used NIV. FVC z-score and LCI are associated with maximum transcutaneous carbon dioxide on PSG (r = 0.43, P < .001).
conclusionNIV is common in SMA. Normal RF does not exclude SDB. Children with more abnormal FVC and LCI should be considered at risk of starting NIV during/following an LRTI.
Indexed as
Identifiers
What OpenQuestion holds
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.