RESUMO
@#Introduction: Branched-chain organic acidurias include maple syrup urine disease (MSUD), isovaleric acidemia (IVA), propionic acidemia (PA), and methylmalonic acidemia (MMA). Long term management requires diets of adequate energy and protein with restriction of the offending amino acids. Standard commercial formulas are expensive and unaffordable to patients of low socio-economic status. Methods: This study aimed to develop food products for branched-chain organic acidurias children aged 4-15 years using locally available raw materials in Thailand. Fish maw (Pangasius hypophthalmus) and roasted sunflower kernel (Helianthus annuus) were selected as protein sources due to their low leucine contents. Five formulations were developed, namely (i) powder (low leucine, isoleucine, and valine for MSUD) for tube feeding preparation, (ii) – (v) rice sprinkle powder, bouillon cube, instant cocoa drink, and snack bar, respectively with low leucine for IVA; low valine, isoleucine, methionine and threonine for PA and MMA. Results: All five formulated products provide 500-600 kcal/100 g, adequate protein in which the offending amino acids were controlled at non-harmful levels. These products were shelf stable at room temperature (Aw = 0.3-0.5). Conclusion: The products that were formulated from fish maw and roasted sunflower kernel provide proteins of appropriate quality and quantity for long-term management of branched-chain organic acidurias. The developed products should be further tested for efficacy among patients in accordance with an adequately powered study design.
