ArticlePediatric pulmonology2017
Comparison of sitting and supine forced vital capacity in collagen VI-related dystrophy and laminin α2-related dystrophy.
Article in Pediatric pulmonology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 2 of them syntheses that pooled it.
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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.
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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.
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Who cites it
9 citing papers in PubMed, 2 syntheses or guidelines pooled it, 22 citations in OpenAlex.
- A systematic review of the frequency of sleep evaluations, sleep disorders, and treatments among individuals with muscular dystrophy.Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine · 2026Pooled it
- Reliability and validity of ultrasonography in evaluating the thickness, excursion, stiffness, and strain rate of respiratory muscles in non-hospitalized individuals: a systematic review.BMC oral health · 2023Pooled it
- Comparing home polysomnography with transcutaneous COJournal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine · 2025Article
- Investigation of respiratory muscle strength and its influence on exercise capacity and quality of life in patients with idiopathic pulmonary fibrosis.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
- Article
- Ultrasonic detection of vagus, accessory, and phrenic nerve atrophy in amyotrophic lateral sclerosis: Relation to impairment and mortality.European journal of neurology · 2024Observational
- Interpretation of pulmonary function tests in children.BJA education · 2023Review
- LAMA2-related muscular dystrophy: Natural history of a large pediatric cohort.Annals of clinical and translational neurology · 2020Article
- Longitudinal changes in clinical outcome measures in COL6-related dystrophies and LAMA2-related dystrophies.Neurology · 2019Article
Corrections and comments
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Authors and funding
17 authors at 6 institutions in 1 country.
Funding
Abstract
backgroundProgressive, restrictive, respiratory insufficiency is the major cause of morbidity and mortality in Congenital Muscular Dystrophy (CMD). Nocturnal hypoventilation precedes daytime alveolar hypoventilation, and if untreated, may lead to respiratory failure and cor pulmonale. CMD consensus care guidelines recommend screening for respiratory insufficiency by conventional and dynamic (sitting to supine) pulmonary function testing (PFT) and evaluating for sleep disordered breathing if there is more than 20% relative reduction from sitting to supine FVC(L) (ΔFVC).
objectiveThe objective of this retrospective study was to explore and characterize dynamic FVC measures in 51 individuals with two common subtypes of CMD, COL6-RD, and LAMA2-RD.
methodsWe compared sitting and supine FVC in patients with confirmed mutation(s) in either COL6 or LAMA2. We investigated influences of age, CMD subtype, gender, race, ambulatory status, and non-invasive positive pressure ventilation (NIPPV) status on FVC percent predicted (FVCpp) and ΔFVC.
resultsCOL6-RD participants exhibited a significant difference between sitting and supine mean FVCpp (sitting 66.1, supine 55.1; P < 0.0001) and were 5.4 times more likely to have -ΔFVC >20% than those with LAMA2-RD when controlling for ambulant status. FVCpp sitting correlated inversely with age in individuals ≤18 years.
conclusionFVCpp sitting decreases progressively in childhood in both CMD subtypes. However, our results point to a difference in diaphragmatic involvement, with COL6-RD individuals having more disproportionate diaphragmatic weakness than LAMA2-RD. A ΔFVC of greater than -20% should continue to be used to prompt evaluation of sleep-disordered breathing. Timely initiation of NIPPV may be indicated to treat nocturnal hypoventilation. Pediatr Pulmonol. 2017;52:524-532. © 2017 Wiley Periodicals, Inc.
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