Research Article
Clinical Course and Nutritional Management of Propionic and Methylmalonic Acidemias
Table 7
Comparison between PA and MMA groups.
| Variable | Total | PA | MMA | value | Mean ± SD |
| Dietary intake (gm/kg/day) | | | | | (i) Total protein | 2.097 ± 0.24 | 2.075 ± 0.28 | 2.12 ± 0.2 | 0.69 | (ii) Natural protein | 0.73 ± 0.99 | 0.71 ± 0.09 | 0.75 ± 0.1 | 0.32 | (iii) Synthetic amino acid formula | 1.37 ± 0.24 | 1.38 ± 0.26 | 1.36 ± 0.23 | 0.86 | (iv) Natural protein: synthetic protein | 0.55 ± 0.13 | 0.53 ± 0.13 | 0.55 ± 0.13 | 0.54 | Anthropometric measures (percentiles) | | | | | (i) Length/height | 10.85 ± 6.71 | 8.6 ± 5.56 | 13.1 ± 7.27 | 0.13 | (ii) Head circumference | 26.25 ± 14.13 | 25 ± 15.09 | 27.5 ± 13.79 | 0.7 | (iii) Weight | 21.25 ± 14.03 | 17.5 ± 13.59 | 25 ± 14.14 | 0.24 | Clinical course | | | | | (i) Length of hospital stay | 5.55 ± 4.35 | 8.4 ± 4.4.59 | 0.33 ± 0.5 | 0.003 | (ii) Length of hospital stay | 6.1 ± 0.65 | 7.9 ± 3.1 | 4.3 ± 1.15 | 0.005 | (iii) Decompensation episodes per year | 1.9 ± 1.15 | 2.6 ± 1.07 | 1 ± 0.43 | 0.003 | (iv) PICU admission frequency | 0.9 ± 1.02 | 1.3 ± 1.15 | 0.33 ± 0.5 | 0.04 | (v) Attacks in the first year | 2.32 ± 1.34 | 2.8 ± 1.47 | 1.6 ± 0.8 | 0.14 | (vi) IQ/DQ | 83. ± 0.24 | 70 ± 23.09 | 97.1 ± 6.22 | 0.004 |
|
|
During the initial decompensation episode and after the initial episode. |