Document Detail

Arrhythmogenic right ventricular cardiomyopathy (Naxos disease): report of a Turkish boy.
MedLine Citation:
PMID:  14675023     Owner:  NLM     Status:  MEDLINE    
Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. This disease is a heart muscle disorder causing life-threatening ventricular arrhythmias, heart failure, and sudden cardiac death. The pathological hallmark of the disease is the progressive replacement of myocardial cells by fat and fibrous tissue. It appears in families descending from the Hellenic island of Naxos. We presented a 13-year-old Turkish boy with Naxos disease associated with ventricular tachycardia because of its rarity, and reviewed the literature.
Nazmi Narin; Mustafa Akcakus; Tamer Gunes; Alpay Celiker; Ali Baykan; Kazim Uzum; Ayten Ferahbas
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Pacing and clinical electrophysiology : PACE     Volume:  26     ISSN:  0147-8389     ISO Abbreviation:  Pacing Clin Electrophysiol     Publication Date:  2003 Dec 
Date Detail:
Created Date:  2003-12-16     Completed Date:  2004-07-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7803944     Medline TA:  Pacing Clin Electrophysiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2326-9     Citation Subset:  IM    
Department of Pediatrics, Erciyes University, School of Medicine, Kayseri, Turkey.
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MeSH Terms
Arrhythmogenic Right Ventricular Dysplasia* / complications,  diagnosis
Hair Diseases* / complications,  diagnosis
Keratoderma, Palmoplantar* / complications,  diagnosis

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