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.