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.

This review summarises the officially published recommended energy and nutrient intake values in five Southeast Asia (SEA) countries namely Indonesia, Malaysia, Philippines, Thailand and Vietnam. The background information, general approaches and references used for setting up recommendations and the recommended intakes levels for energy, protein, fat and carbohydrate, dietary fibre, sugars,14 vitamins and 15 minerals of these countries were tabulated and compared. The recommended intake values show remarkable similarities in terms of approaches and principles taken, as well as references used as the basis for the recommendations development and the application of the recommendations in respective country. There are nevertheless some differences in age groupings, reference height and weight used, as well as the final recommendations of the intake levels for some nutrients, after adjustment to suit local situations. All five countries had provided recommendations in terms of recommended nutrient intakes (RNI) or recommended dietary allowance (RDA) for almost all the nutrients. Due to the limited availability of local data and resources, countries in the region have referred to several references, including those from Food and Agriculture Organization/World Health Organization (FAO/WHO) consultation report and recommendations from research organisations in United States and Europe and adapted the values for local uses. Opportunities should be created to enable closer dialogue and collaboration regarding future developments in nutrient recommendations for populations in the region. These could include consideration of establishing more appropriate nutrient recommendations and the call for setting up harmonised approaches to establishing recommended nutrient intake values for the region.

Introduction: Modular diets (MDs) with low amount of offending amino acids have been developed using locally available food ingredients as alternatives to commercial formulas for the treatment of branched-chain organic acidurias (BCOAs). Herein, we conducted a clinical investigation of MDs in patients with BCOAs. Methods: Modular diet A (MDA), with low leucine was produced for maple syrup urine disease (MSUD), and modular diet B (MDB) products, MDB-1, -2, -3, and -4, with low leucine, valine, methionine and threonine were made for isovaleric aciduria (IVA)/methylmalonic aciduria (MMA)/propionic aciduria (PA). Children aged 4-18 years, with MSUD, IVA, PA or MMA were invited to participate in the study. The research subjects switched from metabolic formula protocol to modular diet protocol. They were followed-up at 0, 1, 2, 4, and 6 months. Clinical efficacies of MDs were determined by completion of study, compliance to MDs, clinical outcomes and complications, and parental satisfaction. Results: Six children (2 MSUD and 4 IVA) participated and completed the study. Compliance to MDA was 100% in MSUD subjects with G-tube feeding, while compliance to MDB varied among self-fed individuals with IVA. One subject with MSUD was clinically stable throughout the study, while the other experienced metabolic instability. All IVA individuals showed clinical and laboratory stability during the study. One MSUD and three IVA families preferred the metabolic formula, whereas the other IVA family reported no preference and the other MSUD subject preferred MDs. Conclusion: We provided a proof of concept in developing modular diets for BCOAs, and showed favourable outcomes when using MDs in IVA and varying clinical benefits in MSUD.