Anne Daly1, S Evans2, A Gerrard2, S Santra2, S Vijay2, A MacDonald2. 1. Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK. ANNE.DALY@bch.nhs.uk. 2. Departments of Dietetics and Inherited Metabolic Disorders, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
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.
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.
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Authors: A Daly; A Pinto; S Evans; M F Almeida; M Assoun; A Belanger-Quintana; S M Bernabei; S Bollhalder; D Cassiman; H Champion; H Chan; J Dalmau; F de Boer; C de Laet; A de Meyer; A Desloovere; A Dianin; M Dixon; K Dokoupil; S Dubois; F Eyskens; A Faria; I Fasan; E Favre; F Feillet; A Fekete; G Gallo; C Gingell; J Gribben; K Kaalund Hansen; N M Ter Horst; C Jankowski; R Janssen-Regelink; I Jones; C Jouault; G E Kahrs; I L Kok; A Kowalik; C Laguerre; S Le Verge; R Lilje; C Maddalon; D Mayr; U Meyer; A Micciche; U Och; M Robert; J C Rocha; H Rogozinski; C Rohde; K Ross; I Saruggia; A Schlune; K Singleton; E Sjoqvist; R Skeath; L H Stolen; A Terry; C Timmer; L Tomlinson; A Tooke; K Vande Kerckhove; E van Dam; T van den Hurk; L van der Ploeg; M van Driessche; M van Rijn; A van Wegberg; C Vasconcelos; H Vestergaard; I Vitoria; D Webster; F J White; L White; H Zweers; A MacDonald Journal: Mol Genet Metab Rep Date: 2017-10-03