One of the most vital elements of management for patients with inborn errors of intermediary metabolism is the promotion of anabolism, the state in which the body builds new components, and avoidance of catabolism, the state in which the body breaks down its own stores for energy. Anabolism is maintained through the provision of a sufficient supply of substrates for energy, as well as critical building blocks of essential amino acids, essential fatty acids, and vitamins for synthetic function and growth. Patients with metabolic diseases are at risk for decompensation during prolonged fasting, which often occurs during illnesses in which enteral intake is compromised. During these times, intravenous nutrition must be supplied to fully meet the specific nutritional needs of the patient. We detail our approach to intravenous management for metabolic patients and its underlying rationale. This generally entails a combination of intravenous glucose and lipid as well as early introduction of protein and essential vitamins. We exemplify the utility of our approach in case studies, as well as scenarios and specific disorders which require a more careful administration of nutritional substrates or a modification of macronutrient ratios.

对于先天性中间代谢错误患者，最重要的管理要素之一是促进合成代谢，即身体构建新成分的状态，以及避免分解代谢，即身体分解自身能量储存的状态. 合成代谢是通过提供充足的能量底物供应以及必需氨基酸、必需脂肪酸和维生素的关键组成部分来维持合成功能和生长的。患有代谢疾病的患者在长期禁食期间有失代偿的风险，这通常发生在肠内摄入受到损害的疾病期间。在此期间，必须提供静脉营养以充分满足患者的特定营养需求。我们详细介绍了我们对代谢患者进行静脉内管理的方法及其基本原理。这通常需要静脉注射葡萄糖和脂质以及早期引入蛋白质和必需维生素。我们举例说明了我们的方法在案例研究中的效用，以及需要更仔细地管理营养底物或修改常量营养素比例的场景和特定疾病。