Disease

Long-chain 3-hydroxyl-CoA dehydrogenase deficiency1, 2, 3

Gene

HADHA

Inheritance AR
Current Treatments IV glucose during acute episodes, avoid fasting, carnitine, restrict LCFA, bezafibrate, triheptanoin
Evidence for Treatment expert opinion
Non-Molecular Testing Availability no
Disease Frequency (per 100,000) 1.161, 4
Genes Included in Disease Frequency HADHA and HADHB
Experimental / Investigational Therapies https://clinicaltrials.gov/ct2/results?cond=hadha&term=&cntry=&state=&city=&dist=
Last Updated: March 5, 2026
RX Genes
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