{"id":11830,"date":"2025-07-25T23:25:27","date_gmt":"2025-07-25T21:25:27","guid":{"rendered":"https:\/\/valintermed.com\/?p=11830"},"modified":"2025-07-25T23:25:27","modified_gmt":"2025-07-25T21:25:27","slug":"defitsit-dlinnotsepochechnoy-3-gidroksiatsil-koa-degidrogenazy","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/deficit-of-long-acting-3-hydroxyethyl-starch-dehydrogenase\/","title":{"rendered":"Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency"},"content":{"rendered":"
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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) is a rare genetic disorder that belongs to a group of diseases associated with impaired oxidative metabolism of fatty acids. This defect causes accumulation of long-chain acyl-CoA derivatives, which leads to various symptoms, including myopathy, heart failure, and metabolic crisis. The basis of the pathology is insufficient activity of the enzyme 3-hydroxyacyl-CoA dehydrogenase, which plays a key role in the oxidative metabolism of long-chain fatty acids necessary for the normal functioning of cells and tissues. LCHAD deficiency can manifest itself early in life and requires early diagnosis and treatment to prevent serious complications and improve the quality of life of patients.<\/p>\n

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