Pyruvate dehydrogenase E1-alpha deficiency

Orpha code: 79243OMIM code: 312170

Definition

A disorder that is the most frequent form of pyruvate dehydrogenase deficiency (PDHD) characterized by variable lactic acidosis, impaired psychomotor development, hypotonia and neurological dysfunction.

Disease data
Classification

Clinical subtype

Synonyms
PDHAD
Niedobór dehydrogenazy pirogronianowej
Niedobór dehydrogenazy pirogronianowej kompleksu E1 komponentu podjednostki alfa
PDHAD
Pyruvate decarboxylase deficiency
Pyruvate dehydrogenase complex E1 component subunit alpha deficiency
ORPHA code
79243
OMIM code
312170
ICD10 code
E74.4
ICD11 code
-

No additional description.

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