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What are fatty acid oxidation disorders?
Long-chain Fatty Acid Oxidation Disorders include a group of rare inborn errors of metabolism characterized by a deficiency of enzymes or transporters required for the breakdown of long-chain fatty acids. (1).
Types of fatty acid oxidation disorders:
- CPT I deficiency
- CACT deficiency
- CPT II deficiency
- VLCAD deficiency
- TFP deficiency
- LCHAD deficiency
Enzym or transporter defect:
The disorder disrupts the proper metabolism of dietary fats, preventing them from being efficiently converted into energy. Instead, long-chain fatty acids build up in the body, leading to potential harmful effects (2).
![[Translate to English:] Enzyme energy](/fileadmin/user_upload/Battery.svg)
Therapy:
Therapy includes a low-fat diet, avoidance of fasting, and supplementation of MCT fats. (2).
Things to know:
How are fatty acid oxidation disorders treated?
Avoidance of long fasting periods
To avoid an energy deficiency, regular meals are an important part of nutritional therapy. During fasting periods, the body relies on its own carbohydrate and fat stores. However, carbohydrate stores are quickly depleted. After that, the body begins to break down long-chain fatty acids from body fat. People with fatty acid oxidation disorders, due to enzyme or transporter defects, cannot derive energy from these broken-down fatty acids. Therefore, avoiding prolonged fasting periods, both during the day and at night, is especially important to continuously supply the body with energy. (2, 3).
Low fat diet
Naturally occurring fats are primarily LCT fats, which contain long-chain fatty acids. Due to the enzyme or transporter defect, these fatty acids cannot be broken down and used for energy production. Depending on the severity of the defect, a moderate or strict reduction in the intake of conventional LCT fats should therefore be observed. (3).
Supplementation with MCT fats
Due to the limitation of LCT fat intake, the body relies on other energy sources. MCT fats can bypass the enzyme or transporter defect and provide an important alternative energy source. (3).
Types of diets for fatty acid oxidation disorders
Moderate fat reduction
Many foods are naturally low in fat. These include, with a few exceptions, fruits, vegetables, legumes, potatoes, and cereal products. As long as the fat content of these foods is not increased through processing, they can be consumed without restriction. High-fat foods, such as butter, cream, bacon, and fatty fish, should be avoided. However, many of these foods can be replaced with lower-fat alternatives (up to 1.5% fat). (3).
Strict fat reduction
Many foods are naturally low in fat. These include, with a few exceptions, fruits, vegetables, legumes, potatoes, and cereal products. Just like in a diet with moderate fat reduction, these foods can be consumed without restriction as long as their fat content is not increased through processing. High-fat foods, such as butter, cream, bacon, and fatty fish, should be avoided. Many of these foods can be replaced with very low-fat alternatives (up to 0.1% fat). (3).
MCT fats in fatty acid oxidation disorders
What are MCT fats?
Medium-chain triglycerides are a type of fatty acid that has between six and 12 carbon atoms.
MCTs are found naturally in foods like coconut and palm kernel oils and full-fat dairy products; they're also found in human breast milk.
Benefits of MCT fats
Medium-chain fatty acids are an important component of the diet for the treatment of Fatty Acid Oxidation Disorders (FAOD).
Different enzymes and transporters are required for the breakdown of MCT fats compared to other types of fats.
Consequently, in Acid Oxidation Disorders (FAOD) the defect can be bypassed using MCT fats which provide an important source of energy. MCT fats can also be used as transporters of important fat-soluble vitamins.
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The most important information summarized briefly:
- Half a teaspoon of MCT oil is approximately 1 g/ml.
- One teaspoon of MCT oil is approximately 3 g/ml.
- One tablespoon of MCT oil is approximately 6 g/ml.
- MCT margarine is ideal for spreading on bread as well as for sautéing and baking.
- MCT oil can be used for salad preparation and for gentle frying at low temperatures (approximately 150°C).
- Do not keep MCT fats warm for too long, heat them too hot, or reheat them (in the microwave) as this could result in a bitter taste.
The term "metabolism" encompasses all processes necessary for building the body, maintaining bodily functions, and obtaining energy. To fulfill these tasks, the food ingested or reserves stored in the body are processed in many carefully coordinated steps. These steps are facilitated by enzymes and transporters.
Low fat recipes with MCT fats
