Trial reportPloS one2016
Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI.
Trial report in PloS one, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 1 of them a synthesis 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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 39 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
- Fully automated diaphragm segmentation on routine 2D-cine and 3D thoracic MRI: multireader validation.European radiology experimental · 2026Article
- Real-Time MRI With Deep Learning for Efficient Evaluation of Neuromuscular Breathing Impairment.MedComm · 2026Article
- Advancements in imaging techniques for monitoring the respiratory muscles.Critical care (London, England) · 2025Review
- Quantifying Normal Diaphragmatic Motion and Shape and Their Developmental Changes via Dynamic MRI.CHEST pulmonary · 2025Article
- Characteristics of Diaphragmatic and Chest Wall Motion in People with Normal Pulmonary Function: A Study with Free-Breathing Dynamic MRI.Journal of clinical medicine · 2022Article
- Non-Invasive Respiratory Assessment in Duchenne Muscular Dystrophy: From Clinical Research to Outcome Measures.Life (Basel, Switzerland) · 2021Review
- Chest MRI to diagnose early diaphragmatic weakness in Pompe disease.Orphanet journal of rare diseases · 2021Article
- Dynamic respiratory muscle function in late-onset Pompe disease.Scientific reports · 2019Article
- Long-term follow-up of 17 patients with childhood Pompe disease treated with enzyme replacement therapy.Journal of inherited metabolic disease · 2018Article
- Paediatric lung imaging: the times they are a-changin'.European respiratory review : an official journal of the European Respiratory Society · 2018Review
- Holistic segmentation of the lung in cine MRI.Journal of medical imaging (Bellingham, Wash.) · 2017Article
- Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy.Annals of clinical and translational neurology · 2017Article
- Quantification of Pediatric Abdominal Organ Motion With a 4-Dimensional Magnetic Resonance Imaging Method.International journal of radiation oncology, biology, physics · 2017Article
- Practical Recommendations for Diagnosis and Management of Respiratory Muscle Weakness in Late-Onset Pompe Disease.International journal of molecular sciences · 2016Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundDiaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respiratory muscle involvement is necessary to initiate treatment in time and possibly prevent irreversible damage. In this paper we investigate the suitability of dynamic MR imaging in combination with state-of-the-art image analysis methods to assess respiratory muscle weakness.
methodsThe proposed methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle.
resultsResults in 16 3D+t MRI scans (10 Pompe patients and 6 controls) of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function tests. Pompe patients with severely reduced pulmonary function showed severe diaphragm weakness presented by minimal motion of the diaphragm. In patients with moderately reduced pulmonary function, cranial displacement of posterior diaphragm parts was reduced and the diaphragm dome was oriented more horizontally at full inspiration compared to healthy controls.
conclusionDynamic 3D MRI provides data for analyzing the contribution of both diaphragm and thoracic muscles independently. The proposed image analysis method has the potential to detect less severe diaphragm weakness and could thus be used to determine the optimal start of treatment in adult patients with Pompe disease in prospect of increased treatment response.
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