Literature DB >> 17763890

Kearns-Sayre syndrome presenting as complete heart block.

Sanjay Chawla1, Jorida Coku, Thomas Forbes, Sujatha Kannan.   

Abstract

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disorder characterized by large-scale deletions of mitochondrial DNA. Neuromuscular and cardiac conduction systems are most commonly involved in these patients. Here, we discuss a 10-year-old patient with diabetes mellitus who presented in complete heart block leading to the diagnosis of KSS. The cardiovascular complications of this syndrome are reviewed and discussed.

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Year:  2007        PMID： 17763890     DOI： 10.1007/s00246-007-9040-z

Source DB:  PubMed          Journal:  Pediatr Cardiol        ISSN： 0172-0643            Impact factor:   1.655


  11 in total

1.  ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines).

Authors:  Gabriel Gregoratos; Jonathan Abrams; Andrew E Epstein; Roger A Freedman; David L Hayes; Mark A Hlatky; Richard E Kerber; Gerald V Naccarelli; Mark H Schoenfeld; Michael J Silka; Stephen L Winters; Raymond J Gibbons; Elliott M Antman; Joseph S Alpert; Gabriel Gregoratos; Loren F Hiratzka; David P Faxon; Alice K Jacobs; Valentin Fuster; Sidney C Smith
Journal:  Circulation       Date:  2002-10-15       Impact factor: 29.690

2.  Retinitis pigmentosa, external ophthalmophegia, and complete heart block: unusual syndrome with histologic study in one of two cases.

Authors:  T P KEARNS; G P SAYRE
Journal:  AMA Arch Ophthalmol       Date:  1958-08

3.  Widespread tissue distribution of mitochondrial DNA deletions in Kearns-Sayre syndrome.

Authors:  S Shanske; C T Moraes; A Lombes; A F Miranda; E Bonilla; P Lewis; M A Whelan; C A Ellsworth; S DiMauro
Journal:  Neurology       Date:  1990-01       Impact factor: 9.910

4.  Cardiac conduction in the Kearns-Sayre syndrome (a neuromuscular disorder associated with progressive external ophthalmoplegia and pigmentary retinopathy). Report of 2 cases and review of 17 published cases.

Authors:  N K Roberts; J K Perloff; R A Kark
Journal:  Am J Cardiol       Date:  1979-12       Impact factor: 2.778

Review 5.  Kearns-Sayre syndrome: a case report and review of cardiovascular complications.

Authors:  Timothy J Young; Amit K Shah; Mun Heng Lee; David L Hayes
Journal:  Pacing Clin Electrophysiol       Date:  2005-05       Impact factor: 1.976

6.  Kearns-Sayre syndrome associated with trifascicular block and QT prolongation.

Authors:  Pavlos Karanikis; Panagiotis Korantzopoulos; Evaggelos Kountouris; Vasiliki Dimitroula; Dimitrios Patsouras; Eugenia Pappa; Konstantinos Siogas
Journal:  Int J Cardiol       Date:  2005-05-11       Impact factor: 4.164

7.  Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome.

Authors:  C T Moraes; S DiMauro; M Zeviani; A Lombes; S Shanske; A F Miranda; H Nakase; E Bonilla; L C Werneck; S Servidei
Journal:  N Engl J Med       Date:  1989-05-18       Impact factor: 91.245

8.  Cardiac involvement in mitochondrial diseases. A study on 17 patients with documented mitochondrial DNA defects.

Authors:  R Anan; M Nakagawa; M Miyata; I Higuchi; S Nakao; M Suehara; M Osame; H Tanaka
Journal:  Circulation       Date:  1995-02-15       Impact factor: 29.690

Review 9.  Endocrine dysfunction in Kearns-Sayre syndrome.

Authors:  J N Harvey; D Barnett
Journal:  Clin Endocrinol (Oxf)       Date:  1992-07       Impact factor: 3.478

10.  Molecular analysis of the muscle pathology associated with mitochondrial DNA deletions.

Authors:  C T Moraes; E Ricci; V Petruzzella; S Shanske; S DiMauro; E A Schon; E Bonilla
Journal:  Nat Genet       Date:  1992-08       Impact factor: 38.330
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  9 in total

1.  Cardiac arrest in kearns-sayre syndrome.

Authors:  Ingrid van Beynum; Eva Morava; Marjan Taher; Richard J Rodenburg; Judit Karteszi; Kalman Toth; Eszter Szabados
Journal:  JIMD Rep       Date:  2011-09-06

2.  Pearson marrow-pancreas syndrome with cardiac conduction abnormality necessitating prophylactic pacemaker implantation.

Authors:  Mats Steffi Jennifer; Daniel Cortez
Journal:  Ann Noninvasive Electrocardiol       Date:  2019-09-01       Impact factor: 1.468

3.  Case Report: Kearns Sayre Syndrome Complicated With Postpartum Cardiac Failure.

Authors:  Caixia Han; Zongyang Jia; Guangcai Zhao; Wenhui Chen; Yue Hu; Haiying Liu
Journal:  Front Med (Lausanne)       Date:  2022-06-20

4.  Causes of Death in Adults with Mitochondrial Disease.

Authors:  Marlieke Barends; Lotte Verschuren; Eva Morava; Victoria Nesbitt; Doug Turnbull; Robert McFarland
Journal:  JIMD Rep       Date:  2015-09-10

5.  A novel mitochondrial DNA deletion in a patient with Kearns-Sayre syndrome: a late-onset of the fatal cardiac conduction deficit and cardiomyopathy accompanying long-term rGH treatment.

Authors:  Monika Obara-Moszynska; Jaroslaw Maceluch; Waldemar Bobkowski; Artur Baszko; Oskar Jaremba; Maciej R Krawczynski; Marek Niedziela
Journal:  BMC Pediatr       Date:  2013-02-20       Impact factor: 2.125

6.  Hypoparathyroidism as the first manifestation of kearns-sayre syndrome: a case report.

Authors:  Farah Ashrafzadeh; Nosrat Ghaemi; Javad Akhondian; Mehran Beiraghi Toosi; Saghi Elmi
Journal:  Iran J Child Neurol       Date:  2013

Review 7.  Myocardial and Arrhythmic Spectrum of Neuromuscular Disorders in Children.

Authors:  Anwar Baban; Valentina Lodato; Giovanni Parlapiano; Corrado di Mambro; Rachele Adorisio; Enrico Silvio Bertini; Carlo Dionisi-Vici; Fabrizio Drago; Diego Martinelli
Journal:  Biomolecules       Date:  2021-10-25

Review 8.  Cardiomyopathies in Children and Systemic Disorders When Is It Useful to Look beyond the Heart?

Authors:  Valentina Lodato; Giovanni Parlapiano; Federica Calì; Massimo Stefano Silvetti; Rachele Adorisio; Michela Armando; May El Hachem; Antonino Romanzo; Carlo Dionisi-Vici; Maria Cristina Digilio; Antonio Novelli; Fabrizio Drago; Massimiliano Raponi; Anwar Baban
Journal:  J Cardiovasc Dev Dis       Date:  2022-01-31

9.  Ophthalmoplegia in Mitochondrial Disease.

Authors:  Sang Jun Lee; Ji Hoon Na; Jinu Han; Young Mock Lee
Journal:  Yonsei Med J       Date:  2018-12       Impact factor: 2.759
  9 in total

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