The Nutritional Intake of Patients with Organic Acidaemias on Enteral Tube Feeding: Can We Do Better?
BACKGROUND
Detailed nutritional intake data on children with organic acidaemias (OA) (propionic acidaemia (PA), vitamin B12nonresponsive 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.


CONCLUSIONS
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.
Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK. ANNE.DALY@bch.nhs.uk.
Anne
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Daly
Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
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S
Evans
Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
A
A
Gerrard
Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
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S
Santra
Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
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S
Vijay
Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
A
A
MacDonald
Amino acids organic acidaemias; Energy; Isovaleric acidaemia; Methylmalonic acidaemia; Propionic acidaemia; Protein