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HSD10 disease

ORPHA:391417· ICD-10 E72.8

Definition

HSD10 disease is a rare, life-threatening neurometabolic disease characterized by a progressive neurodegenerative course, epilepsy, retinopathy and progressive cardiomyopathy.

Prevalence
<1 / 1 000 000
Inheritance
X-linked dominant
Age of onset
Childhood, Infancy, Neonatal