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Disease Information for Naxos syndrome

Clinical Manifestations

Signs & Symptoms

Cardiac Symptoms/Signs

Curly Hair

Palmar keratosis

Demographics & Risk Factors

Ethnic or Racial Factors

Mediterranean racial stock/population

Population Group

Child

Population/Pediatrics population

Sex & Age Groups

Population/Child

Population/Child-Infant Only

Population/Children/all

Diagnostic Test Results

EKG (ECG)

EKG/Wide QRS pattern tachycardia (ECG)

EKG/Widened QRS pattern (ECG)

EKG/Changes/abnormalities (ECG)

Angiography

Heart cath/Right side abnormal

Associated Diseases & Rule outs

Associated Disease & Complications

Death/Unanticipated

Venticular tach/non sustained/recurrent

Wooley hair disease

Cardiac death, sudden

Disease Mechanism & Classification

Class

CLASS/Cardiovascular (category)

CLASS/Heart disorder (ex)

Pathophysiology

Pathophysiology/Gene locus 14q23-q24

Pathophysiology/Gene locus Chromosome 1

Pathophysiology/Gene locus chromosome 14

Pathophysiology/Gene Locus Identified/OMIM database

Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)

Process

PROCESS/Autosomal dominant hereditary disease (ex).

PROCESS/Dysplasia/aplasia (ex)

PROCESS/Hereditofamilial (category)

PROCESS/Vegetative-Autonomic/Endocrine (category)

Synonyms

Synonym

Naxos syndrome, Synonym/Naxos Familial variant RV dysplasia, Synonym/Naxos Wooley hair-RVDysplasia var

Definition

Arrhythmic right ventricular dysplasia is characterized by focal fibrous-fatty replacement of the right ventricle; The right ventricular free wall and the atria are primarily involved, giving rise to ventricular and supraventricular arrhythmias, which are often the presenting symptom; Proposed pathology includes congenital hypoplasia of myocardial tissue and focal injury with fibrous replacement; Some patients present with left ventricular dysfunction, without initial recognition of the right ventricular abnormalities, which are often unappreciated on routine echocardiography; Currently, about one third of patients have evidence of familial involvement in an autosomal dominant pattern, with at least four genes currently identified on chromosomes 1 and 14; A well-known variant is Naxos syndrome originating from the Mediterranean area, in which the affected family members share strikingly curly hair and palmar hyperkeratosis; -------------[Cecil 2004]---------------------

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External Links Related to Naxos syndrome

PubMed (National Library of Medicine)

NGC (National Guideline Clearinghouse)

Medscape (eMedicine)

Harrison's Online (accessmedicine)

NEJM (The New England Journal of Medicine)

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