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Development of Modular Diet for Children with Branched‐chain Organic Acidurias Using Locally Available Raw Materials
Author(s) -
Chatvuttinun Suthida,
Wattanasirichaigoon Duangrurdee,
Suthutvoravut Umaporn,
Chongviriyaphan Nalinee,
Chavasit Visith
Publication year - 2016
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.30.1_supplement.lb310
Subject(s) - maple syrup urine disease , leucine , chemistry , food science , valine , isoleucine , amino acid , methylmalonic acidemia , carbohydrate , biochemistry , medicine
Branched‐chain organic acidurias resulted from abnormal specific enzymes involving catabolism of branched‐chain amino acids (BCAAs). The common organic acidurias are maple syrup urine disease (MSUD), isovaleric acidemia (IVA), propionic acidemia (PA), and methylmalonic acidemia (MMA). Long‐term management includes certain amino acid restriction and metabolic formulas to provide adequate energy and protein. Due to high cost and shortage of metabolic formula, we aimed to formulate modular diets (MDs) as an alternative treatment product for children with BCAAs disorders aged 4–15 years. Raw materials locally available in Thailand were analyzed and selected. Fish maw and roasted sunflower kernel were used as protein sources due to their proper leucine contents. Carbohydrate source includes maltodextrin and sucrose. Soy bean oil and/or non‐dairy creamer were fat sources. The diets were developed for 2 applications including (i) MD‐A, low in leucine for MSUD, was prepared in powder form, and (ii) MD‐B, low in leucine for IVA, and low in valine for PA and MMA, was prepared in powder form, seasoning powder, instant powder drink, and chocolate flavored snack. The diets were analyzed for macronutrient composition and amino acid profile. Per 100 g, the MD‐A diet provided 512 kcal with 9.9 g protein (P), 59.1 g carbohydrate (CHO), and 26.2 g fat (F). Energy, protein, carbohydrate, and fat contents per 100 g of MD‐B ranged 546–601 kcal, 8.2–12.3 g, 36.7–51 g, and 33.6–51 g, respectively. Energy and macronutrient contents were comparable to the commercial formula. Costs of raw materials in the MDs were US$ 0.42–0.51 per 100 g. Locally available raw materials could be used for developing MDs for children with BCAAs disorders at low cost. The products are useful for low‐income patients in developing countries. The efficacy of these MDs used as an alternative treatment in children with branched‐chain organic acidurias should be further evaluated. Support or Funding Information This research work is partially supported by RA scholarship from the Faculty of Graduate Studies, Mahidol University Academic Year 2012