Abstract
Background: Detailed nutritional intake data on children with organic acidaemias (OA) (propionic acidaemia (PA), vitamin B12 nonresponsive methylmalonic acidaemia (MMA) and isovaleric acidaemia (IVA)) remains unreported.
Aim and subjects: A review of the longitudinal nutritional intake of 14 children with organic acidaemias (PA n = 8; MMA n = 5; IVA n = 1) dependent on enteral tube feeding (≥90% of energy requirements) from a single treatment centre.
Methods: Nutritional intake (energy, protein, precursor-free l-amino acids, vitamins and minerals), anthropometry and nutritional biochemistry data were collated from diagnosis to current age.
Results: The median energy intake was only 72% (63–137) of the estimated average DH (1991) requirement (EAR), decreasing significantly by 40% between 6 months and 5 years (p < 0.05). Total protein intake met WHO/FAO/UNU (2007) safe intake levels with median (range) precursor-free l-amino acids providing 21% (14–28) of total protein intake. Median mineral intake for sodium was 57% (20–97%), potassium 64% (27–125%) and magnesium 72% (22–116%) and was consistently < RNI for all age points. Fibre median intake was 4 g/day (0–11 g), and fluid intake provided 80% (60–100%) of the requirements for age. Linear growth was poor, and children were overweight for their height (1–10 years: z score median weight +0.6, height −1.2). Nutritional markers consistently indicated that plasma valine concentrations were < target reference ranges in PA and MMA. Iron deficiency anaemia was common in MMA/PA, and in PA, 50% of plasma zinc concentrations were < reference range.
Conclusion: In MMA/PA, energy intake decreases over time, weight gain accelerates, but linear height is poor. There are many nutrient deficiencies which may affect short- and long-term outcome of patients with organic acidaemias. The quality of long-term diet in these conditions deserves more attention.
Competing interests: None declared
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Communicated by: Ivo Barić, M.D., PhD, Professor of Pediatrics
Appendices
Appendix: Detailed Nutrient Intake Analysis for Subjects on Enteral Tube Feeding with Organic Acidaemia
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A. Daly – research funding from Vitaflo; financial support from Nutricia and Vitaflo to attend study days and conferences.
S. Evans – a research dietitian funded by Nutricia; financial support from Nutricia and Vitaflo to attend study days and conferences
A. MacDonald – research funding and honoraria from Nutricia, Vitaflo International and Merck Serono, Member of European Nutrition Expert Panel (Merck Serono International), Member of Sapropterin Advisory Board (Merck Serono International), Member of the Advisory Board Element (Danone Nutricia)
S Vijay – has no conflict of interest with nutritional-based companies
S Santra – has no conflict of interest with nutritional-based companies
A Gerrard – has no conflict of interest with nutritional-based companies
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All authors were involved in the analysis and interpretation of data, critical revision of the paper for important intellectual content and final approval of the version to be published. Anita MacDonald and Anne Daly were additionally involved in the initial study conception and design; Anne Daly was involved in the collection of data; and Anita MacDonald and Anne Daly in the collation of data and drafting of the initial article
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Daly, A., Evans, S., Gerrard, A., Santra, S., Vijay, S., MacDonald, A. (2015). The Nutritional Intake of Patients with Organic Acidaemias on Enteral Tube Feeding: Can We Do Better?. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 28. JIMD Reports, vol 28. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2015_443
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DOI: https://doi.org/10.1007/8904_2015_443
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