{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,26]],"date-time":"2026-03-26T14:24:34Z","timestamp":1774535074587,"version":"3.50.1"},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2016,1,27]],"date-time":"2016-01-27T00:00:00Z","timestamp":1453852800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>We retrospectively reviewed respiratory and cardiac function in patients with dysferlinopathy, including 2 autopsy cases with respiratory dysfunction.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Subjects included 48 patients who underwent respiratory evaluation (<jats:italic>n<\/jats:italic> = 47), electrocardiography (<jats:italic>n<\/jats:italic> = 46), and echocardiography (<jats:italic>n<\/jats:italic> = 23).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 47 patients, 10 had reduced percent forced vital capacity (%FVC), and 4 required non\u2010invasive positive pressure ventilation. %FVC was significantly correlated with disease duration, and mean %FVC was significantly lower in non\u2010ambulatory patients, as well as in those aged \u226565 years with normal creatine kinase levels. On electrocardiography, QRS complex duration was prolonged in 19 patients, although no significant association with age, disease duration, or respiratory function was found. Echocardiography indicated no left ventricular dysfunction in any patient. Histopathology of autopsied cases revealed mild cardiomyopathy and moderate diaphragm involvement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients with dysferlinopathy may develop severe respiratory failure and latent cardiac dysfunction. Both respiratory and cardiac function should be monitored diligently. <jats:italic>Muscle Nerve<\/jats:italic> 53: 394\u2013401, 2016<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.24741","type":"journal-article","created":{"date-parts":[[2015,6,18]],"date-time":"2015-06-18T23:21:17Z","timestamp":1434669677000},"page":"394-401","source":"Crossref","is-referenced-by-count":26,"title":["Respiratory and cardiac function in japanese patients with dysferlinopathy"],"prefix":"10.1002","volume":"53","author":[{"given":"Atsuko","family":"Nishikawa","sequence":"first","affiliation":[{"name":"Department of Neurology, National Center Hospital National Center of Neurology and Psychiatry Tokyo Japan"},{"name":"Department of Education Interdisciplinary Graduate School of Medicine and Engineering University of Yamanashi Yamanashi Japan"},{"name":"Department of Neuromuscular Research, National Institute of Neuroscience National Center of Neurology and Psychiatry Tokyo 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