Clinical utility of low branched-chain amino acid modular diets in patients with isovaleric aciduria and maple syrup urine disease

Tim-Aroon Thipwimol, . and Wattanasirichaigoon Duangrurdee, . and Chatvuttinun Suthida, . and Chongviriyaphan Nalinee, . and Chavasit Visith, . and Dumrongwongsiri Oraporn, . and Suthutvoravut Umaporn, . Clinical utility of low branched-chain amino acid modular diets in patients with isovaleric aciduria and maple syrup urine disease. pp. 349-361. ISSN 1394–035X

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.

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Abstract

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.

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Item Type:	Article
AGROVOC Term:	patients
AGROVOC Term:	Clinical examination
AGROVOC Term:	Disease treatment
AGROVOC Term:	Child care
AGROVOC Term:	Clinical foods
AGROVOC Term:	Health diets
AGROVOC Term:	Public health
AGROVOC Term:	Metabolic disorders
AGROVOC Term:	Diet treatment
AGROVOC Term:	Hospital diets
Depositing User:	Mr. AFANDI ABDUL MALEK
Last Modified:	24 Apr 2025 00:55
URI:	http://webagris.upm.edu.my/id/eprint/10110

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