Pyruvate dehydrogenase E1-beta deficiency

Orpha code: 255138OMIM code: 614111

Definition

Pyruvate dehydrogenase E1-beta deficiency is an extremely rare form of pyruvate dehydrogenase deficiency (PDHD, see this term) characterized by severe lactic acidosis, developmental delay and hypotonia.

Disease data
Classification

Clinical subtype

Synonyms
PDHBD
Niedobór składnika podjednostki beta kompleksu E1 dehydrogenzay pirogronianowej
PDHBD
Pyruvate dehydrogenase complex E1 component subunit beta deficiency
ORPHA code
255138
OMIM code
614111
ICD10 code
E74.4
ICD11 code
5C53.02

No additional description.

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