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

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.

Diet therapy in patients with rare diseases: a scoping review

“…Of these, 193 duplicate articles were removed, and 811 articles were excluded because they did not meet the selection criteria for the title and abstract screening. After full‐text screening of the remaining 135 articles, 34 articles were selected for this scoping review: 11 primary studies, 23–33 11 literature reviews, 34–44 7 systematic reviews 45–51 and 5 articles of other research types (conference proceedings and case report) 52–56 . A total of 11 studies (32%) were primary studies, including three RDs (phenylketonuria [PKU], propionic acidemia [PA] and glycogen storage disease type I [GSD‐I]) and two groups of diseases (urea cycle disorders [UCDs] and organic acidurias [OAD]).…”

“…Of these, 193 duplicate articles were removed, and 811 articles were excluded because they did not meet the selection criteria for the title and abstract screening. After full-text screening of the remaining 135 articles, 34 articles were selected for this scoping review: 11 primary studies, [23][24][25][26][27][28][29][30][31][32][33] 11 literature reviews, [34][35][36][37][38][39][40][41][42][43][44] 7 systematic reviews [45][46][47][48][49][50][51] and 5 articles of other research types (conference proceedings and case report). [52][53][54][55][56] A total of 11 studies (32%) were primary studies, including three RDs…”

“…29 The studies with the largest sample size were based on information from European multicentre registries. Prader-Willi syndrome 3 [28,42,56] Glycogen storage disease type I 3 [27,47,52] Propionic acidemia 2 [30,41] Hepatolenticular degeneration (Wilson's disease) 2 [38,43] Galactosaemia 2 [49,54] Urea cycle disorders 2 [25,29] Very-long chain acyl-CoA dehydrogenase deficiency 1 [40] Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency 1 [37] Maple syrup urine disease 1 [48] Multiple sclerosis 1 [46] Organic acidurias 1 [29] Inborn errors of metabolism 1 [53] Diet therapy types…”

“…27,47,52 GSD-I diet therapy is similar to LCHADD, but the optimal LCHADD diet is a normal-calorie, normal-protein and high-carbohydrate diet. 37 Treatment of patients with VLCAD aims to provide adequate energy to prevent catabolic episodes and avoid excessive fasting. 40 The PKU diet therapy studies included three primary studies and two literature reviews.…”

“…30 In addition, a conference paper reported that diet therapy management in patients with GSD-I was a dynamic process that should adapt to patient changes, and a limited literature review studied a new approach to diet therapy management of LCHADD. 37,52 Guidelines for diet therapy A total of five studies (15%) reported diet therapy guidelines for RDs. Five RDs were included: PKU…”