Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
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ISSN 1899–5276 (print)
ISSN 2451-2680 (online)
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Advances in Clinical and Experimental Medicine

2017, vol. 26, nr 3, May-June, p. 515–525

doi: 10.17219/acem/62132

Publication type: review

Language: English

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Energy exchangers with LCT as a precision method for diet control in LCHADD

Renata Mozrzymas1,A,B,C,D, Klaudia Konikowska2,A,B,C,D, Bożena Regulska-Ilow2,E,F

1 Regional Specialist Hospital in Wrocław, Research and Development Center, Poland

2 Department of Dietetics, Wroclaw Medical University, Poland

Abstract

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.

Key words

diet, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD), long-chain triglycerides (LCT), intake of energy

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