ArticleMuscle & nerve2017
Cardiopulmonary dysfunction in patients with limb-girdle muscular dystrophy 2A.
Article in Muscle & nerve, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed.
- French National Protocol for Diagnosis and Care of Calpainopathy (LGMD R1/LGMD D4): consensus guidelines for clinical practice.Orphanet journal of rare diseases · 2026Review
- Calpainopathy (limb-girdle muscular dystrophy type R1): clinical features, diagnostic approaches, and biotechnological treatment methods.Journal of neuromuscular diseases · 2025Review
- Limb-girdle muscular dystrophies: A scoping review and overview of currently available rehabilitation strategies.Muscle & nerve · 2025Article
- Family and literature analysis demonstrates phenotypic effect of two variants in the calpain-3 gene.Neurogenetics · 2023Article
- Defining and identifying satellite cell-opathies within muscular dystrophies and myopathies.Experimental cell research · 2022Article
- Review
- Combined high flow nasal cannula and negative pressure ventilation as a novel respiratory approach in a patient with acute respiratory failure and limb-girdle muscular dystrophy.Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2021Article
- Variants of CAPN3 cause limb-girdle muscular dystrophy type 2A in two Chinese families.Experimental and therapeutic medicine · 2021Article
- Clinical, pathological, imaging, and genetic characterization in a Taiwanese cohort with limb-girdle muscular dystrophy.Orphanet journal of rare diseases · 2020Article
- Physiological aspects of muscular adaptations to training translated to neuromuscular diseases.Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2019Article
- Limb-Girdle Muscular Dystrophies (LGMDs): The Clinical Application of NGS Analysis, a Family Case Report.Frontiers in neurology · 2019Article
- Electrocardiographic and echocardiographic findings in muscular dystrophy patients with heart failure.Heart and vessels · 2018Article
- A novel CAPN3 mutation in late-onset limb-girdle muscular dystrophy with early respiratory insufficiency.Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia · 2018Article
- Entresto therapy effectively protects heart and lung against transverse aortic constriction induced cardiopulmonary syndrome injury in rat.American journal of translational research · 2018Article
Corrections and comments
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionLittle is known about the frequency of cardiopulmonary failure in limb-girdle muscular dystrophy type 2A (calpainopathy) patients, although some studies have reported severe cardiomyopathy or respiratory failure.
methodsTo clarify the frequency of cardiopulmonary dysfunction in this patient population, we retrospectively reviewed the respiratory and cardiac function of 43 patients with calpainopathy.
resultsNine of the 43 patients had forced vital capacity (FVC) < 80%, and 3 used noninvasive positive pressure ventilation. Mean FVC was significantly lower in patients who were nonambulant and had normal creatine kinase levels. Only 1 patient had a prolonged QRS complex duration. Echocardiography revealed that 1 patient had very mild left ventricular dysfunction.
conclusionsThese findings suggest that patients with calpainopathy may develop severe respiratory failure, but cardiac dysfunction is infrequent. Muscle Nerve 55: 465-469, 2017.
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