Very-long-chain acyl-CoA dehydrogenase deficiency (VLCADD) is a disorder of fatty acid oxidation. Symptoms are managed by dietary supplementation with medium-chain fatty acids that bypass the metabolic block. However, patients remain vulnerable to hospitalisations because of rhabdomyolysis, suggesting pathologic processes other than energy deficit. Since rhabdomyolysis is a self-destructive process that can signal inflammatory/immune cascades, we tested the hypothesis that inflammation is a physiologic dimension of VLCADD.

中文翻译：

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极长链酰基辅酶 A 脱氢酶 (VLCADD) 缺乏症患者的普遍炎症激活

超长链酰基辅酶A脱氢酶缺乏症（VLCADD）是一种脂肪酸氧化障碍。通过膳食补充绕过代谢阻滞的中链脂肪酸来控制症状。然而，由于横纹肌溶解症，患者仍然容易住院，这表明存在能量不足以外的病理过程。由于横纹肌溶解是一种可以发出炎症/免疫级联信号的自我破坏过程，我们测试了炎症是 VLCADD 的生理维度的假设。