Suppl. Table 6. International dietary recommendations

A limitation of the use of standard international dietary recommendations as a basis for the dietary treatment in glutaric aciduria type I is their utilization of different reference proteins. In previous recommendations for age groups >6 months (ie when introducing weaning food), calculations of protein intake were based on natural protein from a mixed diet. In contrast, more recent recommendations use a reference protein with a high biological valueand a high degree of digestibility – continuously in all age groups. Therefore, values for protein intake are lower in the current recommendations. Dietary treatment protocols using restriction of natural protein in general and animal products in particular – like in glutaric aciduria type I – have to consider these differences carefully. Since the biological value of natural protein used in these diets is much lower than in the reference diets, there is a risk of protein and essential amino acids deficiency if the the reduced biological value of the dietary protein is not considered in the calculation of dietary treatment.

Protein requirements are given as g/kg body weight per day, except for *g/day. AI, adequate intake.

Based on a reference protein with a high biological value / Based on a mixed diet
FAO/WHO/
UNU
(1985)1 / FAO/WHO/
UNU
(2007)2 / Dewey et al (1996)3 / DRI
(2002)4 / D-A-CH (2000)5 / DRV (1991)6 / DGE
(1985)7 / RDA
(1980)8
Months
0-1 / - / 1.77 / 2.69 / 1.52 (AI) / 2.7 / 2.1 / 2.3 / 2.2
1-2 / - / 1.50 / 2.04 / 1.52 (AI) / 2.0 / 2.1 / 2.3–2.1 / 2.2
2-3 / - / 1.36 / 1.53 / 1.52 (AI) / 1.5 / 2.1 / 2.3–2.1 / 2.2
3-4 / 1.86 / 1.24 / 1.37 / 1.52 (AI) / 1.5 / 2.1 / 2.3–2.1 / 2.2
4-5 / 1.86 / 1.14 / 1.25 / 1.52 (AI) / 1.3 / 1.6 / 2.1–2.0 / 2.0
5-6 / 1.86 / 1.31 / 1.19 / 1.52 (AI) / 1.3 / 1.6 / 2.1–2.0 / 2.0
6-9 / 1.65 / 1.31 / 1.09 / 1.5 / 1.1 / 1.5 / 2.1–2.0 / 2.0
9-12 / 1.48 / 1.31 / 1.02 / 1.5 / 1.1 / 1.5 / 2.1–2.0 / 2.0
Years
1-1.5 / 1.26 / 1.14 / 1.0 / 1.1 / 1.0 / 1.1 / 1.7 / 1.8
1.5-2 / 1.17 / 1.03 / 0.94 / 1.1 / 1.0 / 1.1 / 1.7 / 1.8
2-3 / 1.13 / 0.97 / 0.92 / 1.1 / 1.0 / 1.1 / 1.7 / 1.8
3-4 / 1.09 / 0.9 / 0.9 / 1.1 / 1.0 / 1.1 / 1.7 / 1.8
4-5 / 1.06 / 0.86 / 0.88 / 0.95 / 0.9 / 1.1 / 1.6 / 1.5
5-6 / 1.02 / 0.85 / 0.86 / 0.95 / 0.9 / 1.1 / 1.6 / 1.5
6-7 / 1.01 / 0.89 / 0.86 / 0.95 / 0.9 / 1.1 / 1.6 / 1.5
7-8 / 1.01 / 0.91 / 0.86 / 0.95 / 0.9 / 28.3* / 1.4 / 1.5
8-9 / 1.01 / 0.92 / 0.86 / 0.95 / 0.9 / 28.3* / 1.4 / 1.2
9-10 / 0.99 / 0.92 / 0.86 / 0.95 / 0.9 / 28.3* / 1.4 / 1.2
10-11 / 1.0 / 0.91 / 0.87 / 0.95 / 0.9 / 28.3* / 1.1 / 1.0
11-12 / 0.98 / 0.90-0.91 / 0.86 / 0.95 / 0.9 / 42.1* / 1.1 / 1.0
12-13 / 0.96–1.0 / 0.89-0.9 / 0.85 / 0.95 / 0.9 / 42.1* / 1.1 / 1.0
13-14 / 0.94-0.97 / 0.88-0.9 / 0.84 / 0.95 / 0.9 / 42.1* / 1.0 / 1.0
14-15 / 0.90–0.96 / 0.87-0.89 / 0.81 / 0.85 / 0.9 / 42.1* / 1.0 / 1.0
15-16 / 0.87–0.92 / 0.85-0.88 / 0.81 / 0.85 / 0.8 / 45.4-55.2* / 0.8–0.9 / 0.9
16-17 / 0.83–0.90 / 0.84-0.87 / 0.78 / 0.85 / 0.8 / 45.4-55.2* / 0.8–0.9 / 0.9
17-18 / 0.80–0.86 / 0.83-0.86 / 0.77 / 0.85 / 0.8 / 45.4-55.2* / 0.8–0.9 / 0.9
>18 / - / 0.82-0.85 / - / 0.8 / 0.8 / - / 0.8–0.9 / 0.9

Dietary treatment for glutaric aciduria type I should also include a well-balanced and sufficient supply of essential amino acids, minerals and micronutrients. Recommendations for essential amino acids are listed in Suppl. Table 7.

References

  1. World Health Organization (1985) Energy and protein requirements: Report of an FAO/WHO/UNU Expert Consultation. WHO Technical Report Series 724. Geneva: World Health Organization.
  2. World Health Organization (2007) Protein and amino acid requirements in human nutrition. Report of a Joint FAO/WHO/UNU Expert Consultation. WHO Technical Report Series 935. Geneva: World Health Organization
  3. Dewey KG, Beaton G, Fjeld C, Lonnerdal B, Reeds P (1996) Protein requirements of infants and children. Eur J Clin Nutr50: 119-147.
  4. Institute of Medicine of the National Academies (2002) Dietary Reference Intakes for Energy, Carbohydrate, Fiber, Fat, Fatty Acids, Cholesterol, Protein and Amino Acids. WashingtonDC: The National Academies Press.
  5. Deutsche Gesellschaft für Ernährung, Österreichische Gesellschaft für Ernährung, Schweizerische Gesellschaft für Ernährungsforschung, Schweizerische Vereinigung für Ernährung (2000)Referenzwerte für die Nährstoffzufuhr. Frankfurt/Main: Umschau.
  6. Department of Health (1991) Report on Health and Social subjects No. 41. Dietary Reference Values for Food, Energy and Nutrientsfor the United Kingdom. London: The Stationary Office.
  7. Deutsche Gesellschaft für Ernährung (1985) Empfehlungen für die Nährstoffzufuhr, 4th edition. Frankfurt/Main: Umschau.
  8. National Research Council (1980) Recommended Dietary Allowances, 9th edition.WashingtonDC: NationalAcademy Press.