Inborn defects affecting the MTP can translate in isolated long-chain-3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency (generally associated with HADHA gene mutations), or in decreased levels of the three MTP-borne enzymes activities (generally associated with HADHB gene mutations) [14,18,41]. Here, HADHA is linked to long chain 3-hydroxyacyl-CoA dehydrogenase deficiency.