Mozrzymas Renata, Konikowska Klaudia, Regulska-Ilow Bożena
Regional Specialist Hospital in Wrocław, Research and Development Center, Poland.
Department of Dietetics, Wroclaw Medical University, Poland.
Adv Clin Exp Med. 2017 May-Jun;26(3):515-525. doi: 10.17219/acem/62132.
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is a rare genetic disease. The LCHADD treatment is mainly based on special diet. In this diet, energy from long-chain triglycerides (LCT) cannot exceed 10%, however energy intake from the consumption of medium-chain triglycerides (MCTs) should increase. The daily intake of energy should be compatible with energy requirements and treatment should involve frequent meals including during the night to avoid periods of fasting. In fact, there are no recommendations for total content of LCT in all of the allowed food in the LCHADD diet. The aim of the study was to present a new method of diet composition in LCHADD with the use of blocks based on energy exchangers with calculated LCT content. In the study, the diet schema was shown for calculating the energy requirements and LCT content in the LCHADD diet. How to create the diet was also shown, based on a food pyramid developed for patients with LCHADD. The blocks will make it possible, in a quick and simple way, to create a balanced diet which provides adequate energy value, essential nutrients and LCT content. This method can be used by doctors and dietitians who specialize in treating rare metabolic diseases. It can also be used by patients and their families for accurate menu planning with limited LCT content.
长链 3-羟酰基辅酶 A 脱氢酶缺乏症（LCHADD）是一种罕见的遗传疾病。LCHADD 的治疗主要基于特殊饮食。在这种饮食中，长链甘油三酯（LCT）提供的能量不能超过 10%，而中链甘油三酯（MCT）的能量摄入量应增加。每日能量摄入量应与能量需求相匹配，治疗应包括少食多餐，包括夜间进食，以避免禁食期。事实上，对于 LCHADD 饮食中所有允许食物的 LCT 总含量并无相关建议。本研究的目的是提出一种新的 LCHADD 饮食组成方法，即使用基于能量交换器且计算出 LCT 含量的模块。在该研究中，展示了用于计算 LCHADD 饮食中能量需求和 LCT 含量的饮食方案。还展示了如何基于为 LCHADD 患者制定的食物金字塔来制定饮食。这些模块将使人们能够以快速简便的方式制定出一种均衡饮食，该饮食能提供足够的能量值、必需营养素和 LCT 含量。这种方法可供专门治疗罕见代谢疾病的医生和营养师使用。患者及其家属也可使用它来进行 LCT 含量有限的精确食谱规划。
