{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T20:42:07Z","timestamp":1789764127995,"version":"4.0.1"},"reference-count":34,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,9,10]],"date-time":"2020-09-10T00:00:00Z","timestamp":1599696000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Echocardiograms from 56 subjects (157 echocardiograms) with LGMDR9 were retrospectively reviewed. The cumulative probability of having an abnormal echocardiogram as a function of age was assessed by survival analysis for interval\u2010censored data by genotype. Correlations between cardiac and clinical function were evaluated.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Twenty\u2010five (45%) participants had cardiomyopathy. The median age at first abnormal echocardiogram for subjects homozygous for the c.826C&gt;A variant was 54.2 y compared to 18.1 y for all other fukutin\u2010related protein (\n                      <jats:italic>FKRP<\/jats:italic>\n                      ) genotypes (\n                      <jats:italic>P<\/jats:italic>\n                      &lt;\u2009.0001). There was a weak correlation between ejection fraction and 10\u2010Meter Walk Test speed (r = 0.25), but no correlation with forced vital capacity (r = 0.08).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>Cardiomyopathy is prevalent among those with LGMDR9 and occurs later in subjects homozygous for the c.826C&gt;A mutation. These data will help to guide surveillance and management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mus.27052","type":"journal-article","created":{"date-parts":[[2020,9,24]],"date-time":"2020-09-24T14:25:06Z","timestamp":1600957506000},"page":"626-632","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Cardiomyopathy in limb girdle muscular dystrophy\n                    <scp>R9<\/scp>\n                    ,\n                    <scp>\n                      <i>FKRP<\/i>\n                    <\/scp>\n                    related"],"prefix":"10.1002","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5557-3429","authenticated-orcid":false,"given":"Eric M.","family":"Libell","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia A.","family":"Richardson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie L.","family":"Lutz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benton Y.","family":"Ng","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shelley R. H.","family":"Mockler","sequence":"additional","affiliation":[{"name":"Center for Disabilities and Development University of Iowa Stead Family Children's Hospital  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie M.","family":"Laubscher","sequence":"additional","affiliation":[{"name":"Center for Disabilities and Development University of Iowa Stead Family Children's Hospital  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carrie M.","family":"Stephan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bridget M.","family":"Zimmerman","sequence":"additional","affiliation":[{"name":"Department of Biostatistics College of Public Health, University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erik R.","family":"Edens","sequence":"additional","affiliation":[{"name":"Children's Heart Center Children's Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin E.","family":"Reinking","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine D.","family":"Mathews","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Iowa Carver College of Medicine  Iowa City Iowa USA"},{"name":"Department of Neurology University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,9,10]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/88865"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr240"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gde.2011.02.001"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1086\/324412"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10559"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63160-4"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.jnen.0000235854.77716.6c"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2474-8-115"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-019-1291-2"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.649"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2008.06.365"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.109.199489"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1081465"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20283"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2014.12.090"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-006-0213-0"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/1532-429X-13-39"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2013.05.010"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25192"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2003.013953"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2007.141804"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20824"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.12561"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1080\/10473289.2003.10466174"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2012.05.006"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.21432"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.09.013"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.3390\/genes10110856"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2014.12.008"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2010.08.008"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2017.02.015"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.62.12.1894"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1530\/ERP-16-0037"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1093\/ehjci\/jev014"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27052","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.27052","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27052","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,4]],"date-time":"2023-09-04T15:59:35Z","timestamp":1693843175000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.27052"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,10]]},"references-count":34,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,11]]}},"alternative-id":["10.1002\/mus.27052"],"URL":"https:\/\/doi.org\/10.1002\/mus.27052","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,10]]},"assertion":[{"value":"2020-03-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-08-22","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-09-10","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}