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Naxos Disease

Naxos Disease Causes

Being a variant of ARVD, the real cause of Naxos Disease is still unknown; however there are two pathological patterns seen in Arrhythmogenic Right Ventricular Dysplasia (ARVD), fatty infiltration involving a partial or near completion of myocardium with fatty tissue without wall thinning and fibro-fatty infiltration, which involves replacement of myocytes with fibrofatty tissue.

Naxos Disease Definition

Naxos Disease is an autosomal recessive modification of Arrhythmogenic right ventricular dysplasia (ARVD, also known as Arrhythmogenic right ventricular cardiomyopathy or ARVC). It is a very rare syndrome characterized primarily by the heart disease and wooly hair.

Naxos Disease Diagnosis

In order to make the diagnosis of Naxos Disease being a variant of ARVD includes the following: * Right Ventricular Angiography, which is considered to be the gold standard in the diagnosis of all ARVD. * Electrocardiogram (EKG or ECG), which has the ability to record the electrical activity of the heart overtime. * Echocardiography is also called as the ultrasound of the heart. * Cardiac MRI

Naxos Disease Symptoms and Signs

The symptoms of Naxos Disease are more critical and severe compared to autosomal dominant Arrhythmogenic right ventricular dysplasia (ARVD). The following are some of the symptoms of this disorder: * Heart disease * Wooly hair * Thickened skin on soles * Thickened skin on palms * Fainting * Ventricular tachycardia * Rough hair * Dry hair * Bristly hair

Naxos Disease Treatment

Treatment of Naxos Disease is achieved through antiarrhythmic drugs and heart transplants.

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