Table 1.
Characteristics of Subjects and Mean Feeding Scores
| Orally fed | sex | Anatomical Severity Score* |
plasma sterols§
|
visits # |
age first visit† yr |
age last visit yr |
MEAN
|
||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| subject | cholesterol mmol/L |
7DHC mmol/L |
TOTAL FEEDING SCORE |
ORAL STRUCTUE SCORE |
ORAL FUNCTION SCORE |
OTHER SCORE |
|||||
| 1 | M | 20 | 0.64 | 0.187 | 2 | 0.9 | 1.7 | 33.5 | 6.0 | 27.0 | 0.5 |
| 2 | F | 5 | 2.20 | 0.242 | 5 | 1.0 | 5.9 | 35.2 | 7.0 | 25.4 | 2.8 |
| 3 | M | 20 | 0.62 | 0.283 | 2 | 1.1 | 1.6 | 40.0 | 7.5 | 29.0 | 3.5 |
| 4 | M | 15 | 3.42 | 0.052 | 2 | 2.5 | 5.9 | 34.5 | 2.5 | 31.0 | 1.0 |
| 5 | M | 20 | 2.87 | 0.003 | 1 | 2.6 | 4.3 | 10.5 | 3.5 | 6.0 | 1.0 |
| 6 | M | 5 | 3.39 | 0.242 | 1 | 3.9 | 3.9 | 33.0 | 8.0 | 23.0 | 2.0 |
| 7 | M | 5 | 3.67 | 0.005 | 2 | 4.4 | 7.2 | 24.0 | 4.5 | 18.0 | 1.5 |
| 8 | F | 17 | 3.13 | 0.003 | 1 | 5.8 | 5.8 | 2.0 | 0.0 | 2.0 | 0.0 |
| 9 | M | 6 | 2.46 | 0.125 | 1 | 5.9 | 5.9 | 17.0 | 1.0 | 15.0 | 1.0 |
| 10 | M | 17 | 1.97 | 0.244 | 2 | 7.6 | 12.0 | 3.5 | 2.0 | 1.5 | 0.0 |
| 11 | F | 10 | 2.64 | 0.060 | 3 | 9.1 | 12.5 | 5.7 | 1.3 | 3.7 | 0.7 |
| 12 | F | 10 | 2.46 | 0.122 | 4 | 9.3 | 12.4 | 8.0 | 3.7 | 4.3 | 0.0 |
| 13 | F | 10 | 2.17 | 0.130 | 4 | 10.2 | 13.2 | 9.3 | 4.3 | 4.3 | 0.7 |
| 14 | F | 17 | 2.82 | 0.208 | 3 | 11.3 | 14.4 | 6.7 | 2.7 | 3.3 | 0.7 |
| 15 | M | 20 | 0.80 | 0.328 | 1 | 11.6 | 11.6 | 33.0 | 3.0 | 29.0 | 1.0 |
| 16 | M | 6 | 2.72 | 0.354 | 1 | 19.4 | 19.4 | 10.0 | 1.0 | 5.0 | 4.0 |
|
|
|||||||||||
| mean | 12.69 | 2.37 | 0.162 | 6.66 | 19.12 | 3.63 | 14.23 | 1.27 | |||
| sd | 6.13 | 0.96 | 0.116 | 5.02 | 13.67 | 2.45 | 11.51 | 1.21 | |||
| G-Tube fed | |||||||||||
| 17 | F | 40 | 0.16 | 0.270 | 2 | 0.4 | 1.1 | 52.0 | 12.0 | 36.0 | 4.0 |
| 18 | M | 38 | 0.51 | 0.356 | 3 | 0.6 | 1.7 | 50.7 | 9.0 | 35.3 | 6.3 |
| 19 | M | 40 | 0.21 | 0.270 | 2 | 0.6 | 1.2 | 58.5 | 10.5 | 41.0 | 7.0 |
| 20 | M | 35 | 1.22 | 0.359 | 8 | 0.7 | 5.3 | 62.7 | 8.8 | 47.2 | 6.7 |
| 21 | F | 25 | 1.91 | 0.473 | 3 | 1.1 | 3.1 | 67.3 | 8.7 | 52.0 | 6.7 |
| 22 | F | 11 | 2.41 | 0.174 | 1 | 2.1 | 2.3 | 35.0 | 7.0 | 26.0 | 2.0 |
| 23 | M | 30 | 1.45 | 0.244 | 3 | 5.4 | 7.4 | 60.0 | 11.3 | 42.3 | 6.3 |
| 24 | M | 11 | 2.28 | 0.343 | 8 | 5.8 | 10.8 | 61.0 | 5.8 | 47.0 | 8.2 |
| 25 | F | 17 | 1.22 | 0.247 | 2 | 8.2 | 12.2 | 42.0 | 5.5 | 33.0 | 3.5 |
| 26 | F | 33 | 2.28 | 0.343 | 2 | 13.2 | 15.7 | 52.5 | 9.0 | 37.5 | 6.0 |
|
| |||||||||||
| mean | 28.00 | 1.36 | 0.31 | 3.80 | 54.17 | 8.77 | 39.73 | 5.67 | |||
| SD | 11.42 | 0.86 | 0.08 | 4.30 | 9.89 | 2.17 | 7.74 | 1.88 | |||
| t-test | p=0.002 | p=0.011 | p=0.001 | p<0.0001 | p<0.0001 | p<0.0001 | p<0.0001 | ||||
| 95%CI | (−44.62,−25.4) | (−7.05,−3.22)(−33.32,−17.71) | (−5.84,−2.95) | ||||||||
| Wilcoxon Rank Sum test | p<0.0001 | p<0.0003 | p<0.0001 | p<0.0001 | |||||||
| 95%CI | (−48.70,−25.00) | (−7.50,−3.00) | (−35.00,−16.00) | (−35.00,−16.00) | |||||||
*
(Kelley, R. I., Hennekam, R. C. The Smith-Lemli-Opitz syndrome 2000. 37:321.)
§
Plasma sterols as marker of biochemical severity
†
age at first feeding evaluation visit