Chapter title |
The Nutritional Intake of Patients with Organic Acidaemias on Enteral Tube Feeding: Can We Do Better?
|
---|---|
Chapter number | 443 |
Book title |
JIMD Reports, Volume 28
|
Published in |
JIMD Reports, January 2015
|
DOI | 10.1007/8904_2015_443 |
Pubmed ID | |
Book ISBNs |
978-3-66-252846-4, 978-3-66-252847-1
|
Authors |
Anne Daly, S. Evans, A. Gerrard, S. Santra, S. Vijay, A. MacDonald, Daly, Anne, Evans, S., Gerrard, A., Santra, S., Vijay, S., MacDonald, A. |
Abstract |
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. Nutritional intake (energy, protein, precursor-free L-amino acids, vitamins and minerals), anthropometry and nutritional biochemistry data were collated from diagnosis to current age. 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. 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. |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 28 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Bachelor | 6 | 21% |
Student > Postgraduate | 5 | 18% |
Other | 4 | 14% |
Student > Ph. D. Student | 3 | 11% |
Student > Master | 2 | 7% |
Other | 3 | 11% |
Unknown | 5 | 18% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 9 | 32% |
Nursing and Health Professions | 4 | 14% |
Arts and Humanities | 2 | 7% |
Biochemistry, Genetics and Molecular Biology | 2 | 7% |
Sports and Recreations | 2 | 7% |
Other | 2 | 7% |
Unknown | 7 | 25% |