HSD10 disease

Orpha code: 391417OMIM code: 300438

Definition

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

Disease data
Classification

Disease

Synonyms
2-methyl-3-hydroxybutyric aciduria
Acyduria 2-metylo-3-hydroksymasłowa
Niedobór dehydrogenazy 2-metylo-3-hydroksymasłowego-CoA
Niedobór HSD10
Niedobór MHBD
2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency
HSD10 deficiency
MHBD deficiency
ORPHA code
391417
OMIM code
300438
ICD10 code
E72.8
ICD11 code
-

No additional description.

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