aangeboren defect van stofwisseling	acidemie	autosomaal recessieve hereditaire aandoening	enzymopathie	stoornis van metabolisme van vertakte-keten-aminozuur
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deficiëntie van isovaleryl-co-enzym A-dehydrogenase (aandoening)
	deficiëntie van isovaleryl-co-enzym A-dehydrogenase
	isovaleryl-CoA-dehydrogenasedeficiëntie isovaleriaanacidurie
	Isovaleryl-CoA dehydrogenase deficiency
	Isovaleric acid CoA dehydrogenase deficiency Isovaleric acidemia IVD-gene related isovaleryl-coenzyme A dehydrogenase deficiency Isovaleric acid-CoA dehydrogenase deficiency Isovaleryl-coenzyme A dehydrogenase deficiency

Id	87827003
Status	Primitive

Occurrence

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	E71.1
Term	Overige gespecificeerde stofwisselingsstoornissen van aminozuren met vertakte keten

SNOMED CT to Orphanet simple map

33

SNOMED CT to ICD-10 extended map

Target	E71.1
Rule	TRUE
Advice	ALWAYS E71.1
Correlation	SNOMED CT source code to target map code correlation not specified