VARIOUS Naxos Disease
Naxos disease is an autosomal recessive disorder that combines palmoplantar keratoderma and other ectodermal features with cardiac disorders suggesting arrhythmogenic right ventricular dysplasia/cardiomyopathy. It was first reported in families on the Greek island of Naxos by Protonotarios (1986). At that time the authors described 4 families and 9 cases. Seven of the 9 affected persons showed signs and symptoms of heart disease, including cardiomegaly, EKG abnormalities, episodes of ventricular tachycardia, and, in 1 patient, sudden death. All patients had echocardiographic evidence of enlargement of the right ventricle and a right ventricular band; in 3, the left ventricle was also affected. Protonotarios et al. (1986) pictured the scalp hair, which was described as dense, rough, and bristly, resembling steel wire.
More on NCBI
share your opinion of it with other travelers. Write
a review! |