creators_name: Protonotarios, Nikos creators_name: Tsatsopoulou, Adalena editors_name: Singh, Balbir editors_name: Lokhandwala, Yash editors_name: Francis, Johnson editors_name: Gupta, Anup type: journalp datestamp: 2005-04-16 lastmod: 2011-03-11 08:55:56 metadata_visibility: show title: Naxos Disease ispublished: pub subjects: ipej full_text_status: public keywords: Arrhythmogenic right ventricular dysplasia/cardiomyopathy; Naxos disease; Cell adhesions; Sudden death abstract: Since 1995, according to the World Health Organisation’s classification of cardiomyopathies, Naxos disease has been considered as the recessive form of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).1 It is a stereotype association of ARVD/C with a cutaneous phenotype, characterised by woolly hair and palmoplantar keratoderma.2 Epidemiology Naxos disease was first reported in 1986 by Protonotarios et al in patients originating from the Hellenic island of Naxos.2,3 Apart from Naxos, cases have also been reported from other Hellenic islands, as well as from Turkey, Israel and Saudi Arabia.4 The prevalence of the disease in Hellenic islands reaches 1:1000. A variety of Naxos disease presenting at a younger age with more pronounced left ventricular involvement has been described in families from India and Ecuador (Carvajal syndrome). date: 2005-04 date_type: published publication: Indian Pacing and Electrophysiology Journal volume: 5 number: 2 publisher: Indian Pacing and Electrophysiology Group pagerange: 76-80 refereed: TRUE referencetext: 1. Richardson P, McKenna W, Bristow M, et al: Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation 1996; 93: 841-842. 2. Protonotarios N, Tsatsopoulou A, Patsourakos P, Alexopoulos D, Gezerlis P, Simitsis S, Scampardonis G. Cardiac abnormalities in familial palmoplantar keratosis. Br Heart J 1986;56:321-326. 3. Luderitz B. Naxos disease. J Interv Card Electrophysiol 2003;9:405-406. 4. 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J Cell Sci 2000;113:3127-3139. 21. .Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y. Right ventricular dysplasia: a report of 24 adult cases. Circulation 982;65:384-398. citation: Protonotarios, Nikos and Tsatsopoulou, Adalena (2005) Naxos Disease. [Journal (Paginated)] document_url: http://cogprints.org/4215/1/tsatsopoulou.htm document_url: http://cogprints.org/4215/2/tsatsopoulou.pdf