[Compliance of the diet restricted with leucine, isoleucine and valine in maple syrup urine disease (MSUD) children].

Abstract

The aim of this study was to evaluate the compliance of the diet with limited branched-chain amino acids (BCAA) content in long-term observation of patients with maple syrup urine disease (MSUD). The study group consisted of 7 children at age of 1.5-18 years. Nutrition evaluation was based on current diet records from 3-4 days, every 3-4 months. Energy and nutrition values of proposed daily products lists and diet records was compared with adequate references and recommendations. Energy and content of most of the nutrients in proposed daily products lists were in agreement with RDI except calcium. Diet analysis at MSUD children revealed insufficient contents of: iron, zinc, copper, vitamin B1, B2, niacin and vitamin C (often below 90% RDI).

• Authors: Agnieszka Kowalik, Lucyna Narojek, Jolanta Sykut-Cegielska
• Journal: Roczniki Panstwowego Zakladu Higieny (Rocz Panstw Zakl Hig) Vol. 58 Issue 1 Pg. 95-101 ( 2007) ISSN: 0035-7715 [Print] Poland
• Vernacular Title: Realizacja diety z ograniczeniem leucyny, izoleucyny i waliny u dzieci z choroba syropu klonowego (MSUD).
• PMID: 17711097 (Publication Type: Journal Article)

Chemical References

• Dietary Proteins
• Vitamins
• Isoleucine
• Copper
• Leucine
• Valine
• Zinc
• Calcium

Topics

• Adolescent
• Calcium (deficiency)
• Child
• Child, Preschool
• Copper (deficiency)
• Diet Records
• Dietary Proteins (metabolism)
• Energy Intake
• Female
• Humans
• Infant
• Iron Deficiencies
• Isoleucine (administration & dosage, metabolism)
• Leucine (administration & dosage, metabolism)
• Longitudinal Studies
• Male
• Maple Syrup Urine Disease (diet therapy, metabolism)
• Nutritional Requirements
• Patient Compliance (statistics & numerical data)
• Valine (administration & dosage, metabolism)
• Vitamins (administration & dosage)
• Zinc (deficiency)