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Combined malonic and methylmalonic acidemia

ORPHA:289504· ICD-10 E71.1

Definition

A rare inborn error of metabolism characterized by elevation of malonic acid (MA) and methylmalonic acid (MMA) in body fluids, with higher levels of MMA than MA. CMAMMA presents in childhood with metabolic acidosis, developmental delay, dystonia and failure to thrive or in adulthood with seizures, memory loss and cognitive decline.

Prevalence
Unknown
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
All ages