{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,28]],"date-time":"2023-10-28T11:15:10Z","timestamp":1698491710303},"reference-count":7,"publisher":"Akademiai Kiado Zrt.","issue":"46","license":[{"start":{"date-parts":[[2021,11,14]],"date-time":"2021-11-14T00:00:00Z","timestamp":1636848000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["akjournals.com"],"crossmark-restriction":true},"short-container-title":["OH"],"published-print":{"date-parts":[[2021,11,14]]},"abstract":"<jats:p><jats:bold>\u00d6sszefoglal\u00f3.<\/jats:bold> A dystrophia myotonica (DM) multisziszt\u00e9m\u00e1s,\n                    autoszom\u00e1lis domin\u00e1ns m\u00f3don \u00f6r\u00f6kl\u0151d\u0151, t\u00f6bbs\u00e9g\u00e9ben feln\u0151ttkori izombetegs\u00e9g,\n                    melynek incidenci\u00e1ja 1 : 8000. A betegs\u00e9g kapcs\u00e1n fell\u00e9p\u0151 izomsz\u00f6veti\n                    degener\u00e1ci\u00f3 a har\u00e1ntcs\u00edkolt izomsz\u00f6vet \u00e1t\u00e9p\u00fcl\u00e9se mellett a sz\u00edvizomsz\u00f6vetet is\n                    \u00e9rinti, ami fontos oki szerepet j\u00e1tszik az \u00e9rintett betegek cs\u00f6kkent v\u00e1rhat\u00f3\n                    \u00e9lettartam\u00e1ban. A DM-ben szenved\u0151k hal\u00e1loz\u00e1s\u00e1nak k\u00f6zel egyharmad\u00e1\u00e9rt a\n                    cardiovascularis okok tehet\u0151k felel\u0151ss\u00e9. Esetriportunkban egy 52 \u00e9ves, kor\u00e1bban\n                    kritikus bradycardia \u00e9s I. fok\u00fa atrioventricularis blokk miatt\n                    pacemakerimplant\u00e1ci\u00f3n \u00e1tesett, DM-mel diagnosztiz\u00e1lt n\u0151beteg kardiol\u00f3giai\n                    ut\u00e1nk\u00f6vet\u00e9s\u00e9t mutatjuk be. A hirtelen sz\u00edvhal\u00e1l rizik\u00f3stratifik\u00e1ci\u00f3ja c\u00e9lj\u00e1b\u00f3l\n                    elv\u00e9gzett invaz\u00edv elektrofiziol\u00f3giai vizsg\u00e1lat sor\u00e1n kamrafibrill\u00e1ci\u00f3 l\u00e9pett\n                    fel, \u00edgy a kor\u00e1bban implant\u00e1lt pacemakerelektr\u00f3d\u00e1k mell\u00e9 sokkelektr\u00f3da ker\u00fclt\n                    be\u00fcltet\u00e9sre, a pacemakerk\u00e9sz\u00fcl\u00e9ket implant\u00e1lhat\u00f3 kardioverter-defibrill\u00e1torra\n                    (ICD) cser\u00e9lt\u00fck. Az 1 \u00e9ves ICD-kontrollvizsg\u00e1lat sor\u00e1n azt tal\u00e1ltuk, hogy a\n                    be\u00fcltet\u00e9s \u00f3ta 22, tart\u00f3s kamrai tachycardi\u00e1val j\u00e1r\u00f3 epiz\u00f3d l\u00e9pett fel, melyek\n                    k\u00f6z\u00fcl a k\u00e9sz\u00fcl\u00e9k valamennyit sikeresen termin\u00e1lta. Az eset bemutat\u00e1s\u00e1val\n                    szeretn\u00e9nk r\u00e1mutatni arra, hogy a magas cardiovascularis rizik\u00f3csoportba tartoz\u00f3\n                    DM-betegek azonos\u00edt\u00e1sa kiemelked\u0151 fontoss\u00e1g\u00fa lehet a hirtelen sz\u00edvhal\u00e1l\n                    megel\u0151z\u00e9se \u00e9rdek\u00e9ben. Orv Hetil. 2021; 162(46): 1856\u20131858.<\/jats:p>\n                <jats:p><jats:bold>Summary.<\/jats:bold> Myotonic dystrophy (DM) is one of the most frequent\n                    adulthood diseases of the skeletal muscles, which develops multisystemic\n                    features and shows autosomal dominant trait. In DM, tissue degeneration affects\n                    not only the skeletal, but the cardiac muscle, too. In one third of the\n                    patients, the cause of death is of cardiac origin. We report on our patient\u2019s\n                    case, who was diagnosed with DM at the age of 52, in whom episodes of critical\n                    bradycardia with first-degree atrioventricular block was detected, resulting in\n                    a pacemaker implantation. Invasive cardiac electrophysiological study was\n                    performed, during which ventricular fibrillation was registered. A shock\n                    electrode was added to the previously implanted pacemaker, enabling\n                    defibrillation in case of detection of a sustained ventricular arrhythmia.\n                    During the 1-year follow-up, 22 episodes of sustained ventricular tachycardia\n                    were identified, with the device successfully terminating the malignant\n                    arrhythmias. Our case shows that electrophysiological study and the succeeding\n                    implantation of an implantable cardiac defibrillator is highly important in\n                    identifying and terminating ventricular arrhythmias in high-risk DM patients.\n                    Orv Hetil. 2021; 162(46): 1856\u20131858.<\/jats:p>","DOI":"10.1556\/650.2021.32279","type":"journal-article","created":{"date-parts":[[2021,12,13]],"date-time":"2021-12-13T13:24:37Z","timestamp":1639401877000},"page":"1856-1858","update-policy":"http:\/\/dx.doi.org\/10.1556\/policypage.crossmark.crossref.1","source":"Crossref","is-referenced-by-count":1,"title":["Implant\u00e1lhat\u00f3 kardioverter-defibrill\u00e1tor \u00e1ltal sikeresen megsz\u00fcntetett malignus kamrai ritmuszavar dystrophia myotonic\u00e1ban szenved\u0151 betegben"],"prefix":"10.1556","volume":"162","author":[{"given":"P\u00e9ter","family":"Kup\u00f3","sequence":"first","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Sz\u00edvgy\u00f3gy\u00e1szati Klinika, P\u00e9cs, Ifj\u00fas\u00e1g \u00fat 13., 7624"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eszter","family":"F\u0151di","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Sz\u00edvgy\u00f3gy\u00e1szati Klinika, P\u00e9cs, Ifj\u00fas\u00e1g \u00fat 13., 7624"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dorottya","family":"Debreceni","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Sz\u00edvgy\u00f3gy\u00e1szati Klinika, P\u00e9cs, Ifj\u00fas\u00e1g \u00fat 13., 7624"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Endre","family":"P\u00e1l","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Neurol\u00f3giai Klinika, P\u00e9cs"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R\u00e9ka","family":"Faludi","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Sz\u00edvgy\u00f3gy\u00e1szati Klinika, P\u00e9cs, Ifj\u00fas\u00e1g \u00fat 13., 7624"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dalma","family":"T\u00e9nyi","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Neurol\u00f3giai Klinika, P\u00e9cs"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fanni","family":"Moln\u00e1r","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Sz\u00edvgy\u00f3gy\u00e1szati Klinika, P\u00e9cs, Ifj\u00fas\u00e1g \u00fat 13., 7624"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emese","family":"Lovadi","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Neurol\u00f3giai Klinika, P\u00e9cs"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D\u00e1vid","family":"Varga","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Neurol\u00f3giai Klinika, P\u00e9cs"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tam\u00e1s","family":"Simor","sequence":"additional","affiliation":[{"name":"P\u00e9csi Tudom\u00e1nyegyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Klinikai K\u00f6zpont, Sz\u00edvgy\u00f3gy\u00e1szati Klinika, P\u00e9cs, Ifj\u00fas\u00e1g \u00fat 13., 7624"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"425","reference":[{"key":"ref0","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1159\/000369343","article-title":"Prevalence of muscular dystrophies: a systematic literature review","volume":"43","author":"1 Theadom","year":"2014","unstructured":"1 Theadom A, Rodrigues M, Roxburgh R, et al. 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