methylmalonic acidemia

ICD-10: E71.1ORPHA: 289504

Overview

organic acidemia that involves an accumulation of methylmalonic acid in the blood

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with methylmalonic acidemia, sourced from HPO and Orphanet clinical annotations.

Methylmalonic acidemiaDicarboxylic aciduriaMethylmalonic aciduriaDicarboxylic acidemiaSeizureMicrocephalyAtypical behaviorDelayed speech and language developmentGlobal developmental delayEncephalopathyDystoniaFailure to thriveAcidosisHypoglycemiaDehydrationKetoacidosisVomitingMigraineIntermittent diarrheaMemory impairmentFocal impaired awareness seizureElevated circulating hepatic transaminase concentrationAxial hypotoniaGeneralized clonic seizureImpaired continenceNasogastric tube feeding

Classification & Codes

ICD-10 Code

E71.1

Orphanet Code

ORPHA:289504
methylmalonic acidemia
ICD-10E71.1
OrphanetORPHA:289504
Treatments0 drug(s)
Symptoms on record26 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO