Disease Directory MCAD Deficiency
Metabolic

MCAD Deficiency

Also known as: Medium-chain acyl-CoA dehydrogenase deficiency, ACADM deficiency, medium-chain fatty acid oxidation defect

Prevalence

1-5 per 10,000 (Orphanet)

Onset

Infantile, Childhood

Type

Autosomal recessive genetic

Gene

ACADM

About MCAD Deficiency

MCAD deficiency is the most common fatty acid oxidation disorder, caused by mutations in the ACADM gene encoding medium-chain acyl-CoA dehydrogenase, which is required for mitochondrial oxidation of medium-chain fatty acids. During fasting or metabolic stress, patients cannot produce ketones from fatty acids, leading to hypoketotic hypoglycemia, encephalopathy, and potentially fatal metabolic crises. Newborn screening has dramatically reduced mortality; most patients identified by screening do well with fasting avoidance.

Common Clinical Features

Hypoketotic hypoglycemia Encephalopathy during fasting or illness Lethargy and vomiting Hepatomegaly Elevated liver transaminases Sudden death (pre-newborn-screening era) Fatty infiltration of liver

Clinical Trial Eligibility Tips

What to know before applying to MCAD Deficiency trials.

Newborn screening detection is now near-universal in developed countries — document screening card results and ACADM variant classification

C8 (octanoylcarnitine) level on acylcarnitine profile is the primary diagnostic biomarker

Most trials focus on natural history, outcomes research, and emergency protocol optimization rather than pharmacological intervention

Patients with documented metabolic crises despite fasting avoidance are the primary population for interventional trials

Patient Resources

Patient Organization

Fatty Oxidation Disorders (FOD) Family Support Group

Visit website ↗

Natural History Registry

FOD Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:42)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting MCAD Deficiency trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for MCAD Deficiency. Updated daily.

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