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Cardiac arrest in kearns-sayre syndrome.
van Beynum I, Morava E, Taher M, Rodenburg RJ, Karteszi J, Toth K, Szabados E. van Beynum I, et al. JIMD Rep. 2012;2:7-10. doi: 10.1007/8904_2011_32. Epub 2011 Sep 6. JIMD Rep. 2012. PMID: 23430846 Free PMC article.
The prognosis of progressive ophthalmoplegia in patients with large-scale mitochondrial DNA deletions is highly variable and almost unpredictable. The risk to develop cardiac involvement and sudden cardiac death is strikingly high, especially in patien …
The prognosis of progressive ophthalmoplegia in patients with large-scale mitochondrial DNA deletions is highly variable and almost unpredic …
Progressive involvement of cardiac conduction system in paediatric patients with Kearns-Sayre syndrome: how to predict occurrence of complete heart block and sudden cardiac death?
Di Mambro C, Tamborrino PP, Silvetti MS, Yammine ML, Marcolin C, Righi D, Baban A, Martinelli D, Dionisi Vici C, Drago F. Di Mambro C, et al. Europace. 2021 Jun 7;23(6):948-957. doi: 10.1093/europace/euaa335. Europace. 2021. PMID: 33336258
AIMS: The aims of this study are to evaluate the progressive involvement of the cardiac conduction system in the Kearn-Sayre syndrome (KSS) and to establish criteria for the prevention of episodes of syncope or sudden cardiac death. METHO …
AIMS: The aims of this study are to evaluate the progressive involvement of the cardiac conduction system in the Kearn-Sayre …
2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders.
Groh WJ, Bhakta D, Tomaselli GF, Aleong RG, Teixeira RA, Amato A, Asirvatham SJ, Cha YM, Corrado D, Duboc D, Goldberger ZD, Horie M, Hornyak JE, Jefferies JL, Kääb S, Kalman JM, Kertesz NJ, Lakdawala NK, Lambiase PD, Lubitz SA, McMillan HJ, McNally EM, Milone M, Namboodiri N, Nazarian S, Patton KK, Russo V, Sacher F, Santangeli P, Shen WK, Sobral Filho DC, Stambler BS, Stöllberger C, Wahbi K, Wehrens XHT, Weiner MM, Wheeler MT, Zeppenfeld K. Groh WJ, et al. Heart Rhythm. 2022 Oct;19(10):e61-e120. doi: 10.1016/j.hrthm.2022.04.022. Epub 2022 Apr 29. Heart Rhythm. 2022. PMID: 35500790
The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy …
The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, B …
Kearns Sayre Syndrome: Looking beyond A-V conduction.
Krishna MR. Krishna MR. Indian Pacing Electrophysiol J. 2017 May-Jun;17(3):78-80. doi: 10.1016/j.ipej.2017.03.003. Epub 2017 Mar 19. Indian Pacing Electrophysiol J. 2017. PMID: 29073001 Free PMC article.
A 15-year-old boy was diagnosed with Kayne Sayre Syndrome. He presented with pigmentary retinopathy, progressive ophthalmoplegia and complete heart block. ...This case highlights the importance of recognizing mechanisms other than heart block as a cause of sudden …
A 15-year-old boy was diagnosed with Kayne Sayre Syndrome. He presented with pigmentary retinopathy, progressive ophthalmopleg …
Ventricular arrhythmias in Kearns-Sayre syndrome: A cohort study using the National Inpatient Sample database 2016-2019.
Wiseman K, Gor D, Udongwo N, Alshami A, Upadhaya V, Daniels SJ, Chung WK, Koo CH. Wiseman K, et al. Pacing Clin Electrophysiol. 2022 Dec;45(12):1357-1363. doi: 10.1111/pace.14607. Epub 2022 Oct 27. Pacing Clin Electrophysiol. 2022. PMID: 36208035
BACKGROUND: Degeneration of the cardiac conduction system resulting in complete heart block (CHB), ventricular arrhythmias (VA), and sudden cardiac death (SCD) is recognized in patients with Kearns-Sayre syndrome (KSS) and is poten …
BACKGROUND: Degeneration of the cardiac conduction system resulting in complete heart block (CHB), ventricular arrhythmias (VA), and …
The heart in m.3243A>G carriers.
Finsterer J, Zarrouk-Mahjoub S. Finsterer J, et al. Herz. 2020 Jun;45(4):356-361. doi: 10.1007/s00059-018-4739-6. Epub 2018 Aug 20. Herz. 2020. PMID: 30128910 Review. English.
RESULTS: The m.3243A>G variant may manifest phenotypically as mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), maternally inherited diabetes and deafness (MIDD), myoclonic epilepsy with ragged red fiber (MERRF), Leigh syndrome, or MELAS/K …
RESULTS: The m.3243A>G variant may manifest phenotypically as mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (ME …
Systematic review of cardiac electrical disease in Kearns-Sayre syndrome and mitochondrial cytopathy.
Kabunga P, Lau AK, Phan K, Puranik R, Liang C, Davis RL, Sue CM, Sy RW. Kabunga P, et al. Int J Cardiol. 2015 Feb 15;181:303-10. doi: 10.1016/j.ijcard.2014.12.038. Epub 2014 Dec 13. Int J Cardiol. 2015. PMID: 25540845 Review.
Kearns-Sayre syndrome (KSS) is a mitochondrial disorder characterised by onset before the age of 20years, progressive external ophthalmoplegia, and pigmentary retinopathy, accompanied by either cardiac conduction defects, elevated cerebrospinal fluid p …
Kearns-Sayre syndrome (KSS) is a mitochondrial disorder characterised by onset before the age of 20years, progressive e …
Kearns-Sayre syndrome: a case series of 35 adults and children.
Khambatta S, Nguyen DL, Beckman TJ, Wittich CM. Khambatta S, et al. Int J Gen Med. 2014 Jul 3;7:325-32. doi: 10.2147/IJGM.S65560. eCollection 2014. Int J Gen Med. 2014. PMID: 25061332 Free PMC article.
BACKGROUND: Kearns-Sayre syndrome (KSS) is a rare mitochondrial cytopathy, first described at Mayo Clinic in 1958. ...In addition to the conduction system abnormalities identified in previous series, our cohort included patients with syncope and sudden …
BACKGROUND: Kearns-Sayre syndrome (KSS) is a rare mitochondrial cytopathy, first described at Mayo Clinic in 1958. ...I …
Kearns-Sayre Syndrome With Persistent Ventricular Tachycardia Refractory to Shocks and Medications.
Udongwo N, Gor D, Wiseman K, Alshami A, Daniels S. Udongwo N, et al. Cureus. 2021 Aug 14;13(8):e17175. doi: 10.7759/cureus.17175. eCollection 2021 Aug. Cureus. 2021. PMID: 34548978 Free PMC article.
Cardiovascular conduction delay makes up part of the triad associated with Kearns-Sayre syndrome (KSS). Although there have been a few reported cases of prolonged Qtc and polymorphic ventricular tachycardia associated with this disease, despite the use of aut …
Cardiovascular conduction delay makes up part of the triad associated with Kearns-Sayre syndrome (KSS). Although there …
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