ArticleJournal of neurology2017
Respiratory involvement in ambulant and non-ambulant patients with facioscapulohumeral muscular dystrophy.
Article in Journal of neurology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis 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.
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.
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Who cites it
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 44 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
- Association of Diaphragm Involvement Assessed by Ultrasound With Disease Severity in Facioscapulohumeral Muscular Dystrophy.Journal of cachexia, sarcopenia and muscle · 2025Observational
- Describing phenotypes in FSHD: an update of the comprehensive clinical evaluation form.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Respiratory function and evaluation in individuals with facioscapulohumeral muscular dystrophy in the Muscular Dystrophy Surveillance, Tracking and Research Network.Neuromuscular disorders : NMD · 2025Article
- Current landscape for the management of facioscapulohumeral muscular dystrophy and emerging treatment modalities: A literature review.AIMS neuroscience · 2025Review
- French National Protocol for diagnosis and care of facioscapulohumeral muscular dystrophy (FSHD).Journal of neurology · 2024Review
- Self-reported reduced sleep quality and excessive daytime sleepiness in facioscapulohumeral muscular dystrophy.Muscle & nerve · 2022Article
- Long-Term Systemic Treatment of a Mouse Model Displaying Chronic FSHD-like Pathology with Antisense Therapeutics That InhibitBiomedicines · 2022Article
- Long-term follow-up of respiratory function in facioscapulohumeral muscular dystrophy.Journal of neurology · 2022Article
- Review
- Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences.International journal of molecular sciences · 2021Review
- Facioscapulohumeral muscular dystrophy: genetics, gene activation and downstream signalling with regard to recent therapeutic approaches: an update.Orphanet journal of rare diseases · 2021Review
- Management of respiratory complications and rehabilitation in individuals with muscular dystrophies: 1st Consensus Conference report from UILDM - Italian Muscular Dystrophy Association (Milan, January 25-26, 2019).Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2021Article
- A case of type 1 facioscapulohumeral muscular dystrophy (FSHD) with restrictive ventilatory defect and congestive heart failure.eNeurologicalSci · 2020Article
- Early-Onset Infantile Facioscapulohumeral Muscular Dystrophy: A Timely Review.International journal of molecular sciences · 2020Review
- Response to: Respiratory muscle dysfunction in facioscapulohumeral muscular dystrophy. Letter to the editor-reference article: sleep-related breathing disorders in facioscapulohumeral dystrophy (https://doi.org/10.1007/s11325-019-01843-1) by Santos DB et al.Sleep & breathing = Schlaf & Atmung · 2020Article
- Practical approach to respiratory emergencies in neurological diseases.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020Review
- Sleep-related breathing disorders in facioscapulohumeral dystrophy.Sleep & breathing = Schlaf & Atmung · 2019Article
- Assessment of diaphragm motion using ultrasonography in a patient with facio-scapulo-humeral dystrophy: A case report.Medicine · 2019Article
- The French National Registry of patients with Facioscapulohumeral muscular dystrophy.Orphanet journal of rare diseases · 2018Article
Corrections and comments
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Authors and funding
14 authors at 4 institutions in 2 countries.
Funding
Abstract
Understand the occurrence and predictors of respiratory impairment in FSHD. Data from 100 FSHD patients was collected regarding demographics, genetics, respiratory status and pulmonary function tests, clinical manifestations and Clinical Severity Scale (CSS) scores. Patients were assigned to two severity groups using CSS: mild (scores <3.5) and moderate/severely affected (scores ≥3.5). Forced Vital Capacity (FVC) was classified as severely impaired if less than 50% of the predicted. Statistical analysis was performed using IBM SPSS Statistics 23, tests were two-tailed and the level of significance set at 5%. Spirometry was available for 94 patients; 41.5% had abnormal results with a restrictive pattern in 38.3% patients. There was a correlation between FVC; CSS score and D4Z4 fragment length with a higher probability of severe respiratory involvement in the early onset group, moderate/severe disease and D4Z4 fragments <18 kb. Patients with severe respiratory involvement showed a high prevalence of sleep-disordered breathing. FVC decline over time was indicative of three progression groups. Respiratory involvement for both ambulant and non-ambulant patients with FSHD is more frequent and severe than previously suggested. Sleep-disordered breathing is frequent and negatively influences the respiratory status. Annual screening of the respiratory status with spirometry and clinical assessment is thus warranted in FSHD patients, even while ambulant.
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Registered trials
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