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Titolo:
Does cytosine-thymine-guanine (CTG) expansion size predict cardiac events and electrocardiographic progression in myotonic dystrophy?
Autore:
Clarke, NRA; Kelion, AD; Nixon, J; Hilton-Jones, D; Forfar, JC;
Indirizzi:
John Radcliffe Hosp, Oxford Radcliffe NHS Trust, Dept Cardiol, Oxford OX3 9DU, England John Radcliffe Hosp Oxford England OX3 9DU diol, Oxford OX3 9DU, England Oxford Radcliffe NHS Trust, Radcliffe Infirm, Dept Clin Neurol, Oxford, England Oxford Radcliffe NHS Trust Oxford England Clin Neurol, Oxford, England
Titolo Testata:
HEART
fascicolo: 4, volume: 86, anno: 2001,
pagine: 411 - 416
SICI:
1355-6037(200110)86:4<411:DC(ESP>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
TRINUCLEOTIDE REPEAT LENGTH; VENTRICULAR-TACHYCARDIA; INVOLVEMENT; ABNORMALITIES; MORTALITY; MUTATION; DISEASE; REGION;
Keywords:
myotonic dystrophy; atrioventricular block; delayed intraventricular conduction; cytosine-thymine-guanine expansion;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
31
Recensione:
Indirizzi per estratti:
Indirizzo: Clarke, NRA Northampton Gen NHS Trust, Northampton NN1 5BD, England Northampton Gen NHS Trust Northampton England NN1 5BD ngland
Citazione:
N.R.A. Clarke et al., "Does cytosine-thymine-guanine (CTG) expansion size predict cardiac events and electrocardiographic progression in myotonic dystrophy?", HEART, 86(4), 2001, pp. 411-416

Abstract

Objective-To assess whether the size of the cytosine-thymine-guanine(CTG) expansion mutation in myotonic dystrophy predicts progression of conductionsystem disease and cardiac events. Design-Longitudinal study involving ECG and clinical follow up over (mean (SD)) 4.8 (1.8) and 6.2 (1.9) years, respectively, of patients stratified by CTG expansion size (EO to E4). Patients-73 adult patients under annual review in a regional myotonic dystrophy clinic. Patients were grouped into E0/E1 (n = 25), E2 (n = 34), and E3/E4 (n = 14). Results-The proportion of patients with a QRS complex > 100 ms at baselineincreased with the size of the CTG expansion (EO/E1, 4%; E2, 12%; E3/E4, 36%; p = 0.02). This trend was more pronounced at follow up (E0/E1, 4%; E2, 21%; E3/E4, 57%; p = 0.0004). The rate of widening of the QRS complex (ms/year) was similarly related to the size of the mutation (EO/E1, 0.4 (1.3); E2, 1.4 (2.5); E3/E4, 1.5 (1.6); p = 0.04). First degree atrioventricular block was present in 23% of patients at baseline and 34% at follow up, with no significant relation to expansion size. Seven patients suffered a cardiacevent during follow up (sudden death in two, permanent pacemaker insertionin three, chronic atrial arrhythmia in two), of whom six were in CTG expansion group E2 or greater. Patients who experienced a cardiac event during follow up had more rapid rates of PR interval increase (9.9 (11.1) v 1.6 (2.9) ms/year; p = 0.008) and a trend to greater QRS complex widening (3.6 (4.5) v 0.9 (1.5) ms/year; p = 0.06) than those who did not. Conclusions-Larger CTG expansions are associated with a higher rate of conduction disease progression and a trend to increased risk of cardiac eventsin myotonic dystrophy.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/04/20 alle ore 18:42:44