Metabolic

Isovaleric Acidemia

Also known as IVA, isovaleryl-CoA dehydrogenase deficiency, IVD deficiency, isovaleric acid CoA dehydrogenase deficiency

Isovaleric acidemia is an organic acidemia caused by deficiency of isovaleryl-CoA dehydrogenase, an enzyme involved in leucine catabolism. Accumulation of isovaleric acid and its metabolites causes a distinctive 'sweaty feet' body odor and

ORPHA:33 ↗Gene IVDPrevalence 1-9 per 100,000 (Orphanet)Onset Neonatal, InfantileAutosomal recessive genetic

0

studies recruiting now

as of 7 Sept 2026

7

studies registered in total

as of 7 Sept 2026

0

countries with a recruiting site

as of 7 Sept 2026

None

recruiting study posted to date

among recruiting studies

Recruiting trials

No recruiting trial found right now.

7 studies are registered for Isovaleric Acidemia, but none was recruiting as of 7 Sept 2026. Here is what is still worth doing.

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About Isovaleric Acidemia

Isovaleric acidemia is an organic acidemia caused by deficiency of isovaleryl-CoA dehydrogenase, an enzyme involved in leucine catabolism. Accumulation of isovaleric acid and its metabolites causes a distinctive 'sweaty feet' body odor and can lead to acute metabolic crises with vomiting, lethargy, and coma. The clinical spectrum ranges from severe neonatal-onset disease to a chronic intermittent form identified through newborn screening.

Common clinical features

Sweaty feet odorMetabolic crisisVomiting and lethargyHyperammonemiaNeutropeniaThrombocytopeniaIntellectual disability

From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.

Before you apply

Things trial teams commonly ask about for Isovaleric Acidemia. Not eligibility rules; those are set by each study.

  • Newborn screening identification versus symptomatic diagnosis affects disease severity and trial stratification
  • Plasma isovalerylcarnitine (C5) level is the primary biomarker — document newborn screening results if available
  • Glycine and carnitine supplementation history should be disclosed as it affects metabolite levels
  • Mild/asymptomatic newborn-screened patients may qualify for natural history studies rather than interventional trials

Related conditions

Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).