Abstract
A 10 year old girl with advanced chronic renal failure (GFR 11ml/min/1.73m2) and subnormal growth was treated with a protein restricted diet supplemented with oral essential amino acids and the ketoacid analogues (KAA) of valine, leucine, isoleucine, phenylalanine and methionine. Blood urea fell from 20 to 6 mmol/l and nitrogen balance improved from +1.5 to +2.1 GN/day. Growth velocity during 0.8 yrs treatment increased from 4.6 to 10.5cm/year, growth velocity standard deviation score for bone age from -0.79 to +4.36, height standard deviation score for bone age from -1.56 to -0.57. Skinfold thickness increased from the 10th to 75th percentile, weight for height from 84 to 99%. The diet was well tolerated and no complications except mild hypercalcaemia were seen. These results suggest that the anabolic effect of KAA may be beneficial to growth in uraemic children. Eight further children are currently on treatment. The effects of KAA on their growth, body composition, plasma amino and ketoacid levels, and hormonal and metabolic status, will be presented.
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