Table 2.
Nutritional status and CFQ scores according to phenotype*.
| Meconium Ileus | Pancreatic Insufficient | Pancreatic Sufficient | Overall p-value | |
|---|---|---|---|---|
| No. subjects (No. observations) | 21 (59) | 59 (163) | 15 (43) | |
| Height percentile, mean ± SD | 27 ± 27a | 43 ± 30b | 60 ± 19c | 0.002† |
| BMI percentile, mean ± SD | 42 ± 20 | 48 ± 26 | 60 ± 31 | 0.21† |
| Eating disturbances, mean ± SD | 89 ± 15 | 90 ± 13 | 92 ± 10 | 0.68† |
| Score distribution [n (%)] | 0.71‡ | |||
| Mostly Low (<66) | 5 ( 9%) | 15 ( 9%) | 3 ( 7%) | |
| Mostly Good (66–99) | 20 (34%) | 48 (29%) | 12 (28%) | |
| Perfect (100) | 33 (57%) | 100 (61%) | 28 (65%) | |
| Physical functioning, mean ± SD | 78 ± 18 | 87 ± 15 | 88 ± 14 | 0.08† |
| Score distribution [n (%)] | 0.04‡ | |||
| Mostly Low (<66) | 15 (25%) | 15( 9%) | 3 ( 7%) | |
| Mostly Good (66–99) | 25 (42%) | 82 (50%) | 24 (56%) | |
| Perfect (100) | 19 (32%) | 66 (40%) | 16 (37%) | |
| Emotional functioning, mean ± SD | 81 ± 14 | 83 ± 13 | 84 ± 10 | 0.42† |
| Score distribution [n (%)] | 0.70‡ | |||
| Mostly Low (<66) | 11 (19%) | 21 (13%) | 2 ( 5%) | |
| Mostly Good (66–99) | 34 (58%) | 107 (66%) | 36 (84%) | |
| Perfect (100) | 14 (24%) | 35 (21%) | 5 (11%) | |
| Body image, mean ± SD | 80 ± 27 | 83 ± 19 | 82 ± 20 | 0.87† |
| Score distribution [n (%)] | 0.99‡ | |||
| Mostly Low (<66) | 13 (22%) | 27 (17%) | 9 (21%) | |
| Mostly Good (66–99) | 18 (31%) | 66 (40%) | 15 (35%) | |
| Perfect (100) | 27 (47%) | 70 (43%) | 19 (44%) | |
| Social functioning, mean ± SD | 80 ± 14 | 80 ± 13 | 87 ± 9 | 0.20 † |
| Score distribution [n (%)] | 0.46‡ | |||
| Mostly Low (<66) | 13 (22%) | 30 (18%) | 2 ( 5%) | |
| Mostly Good (66–99) | 33 (57%) | 107 (66%) | 34 (79%) | |
| Perfect (100) | 12 (21%) | 26 (16%) | 7 (16%) | |
| Respiratory symptoms, mean ± SD | 71 ± 18 | 78 ± 12 | 82 ± 12 | 0.13† |
| Score distribution [n (%)] | 0.03‡ | |||
| Mostly Low (<66) | 19 (32%) | 21 (13%) | 5 (12%) | |
| Mostly Good (66–99) | 35 (59%) | 132 (81%) | 33 (77%) | |
| Perfect (100) | 5 ( 8%) | 10 ( 6%) | 5 (12%) | |
| Treatment burden, mean ± SD | 57 ± 16a | 65 ± 14b | 78 ± 14c | 0.002† |
| Score distribution [n (%)] | <0.0001‡ | |||
| Mostly Low (<66) | 34 (59%) | 69 (42%) | 4 (10%) | |
| Mostly Good (66–99) | 21 (36%) | 86 (53%) | 26 (63%) | |
| Perfect (100) | 3 ( 5%) | 8 ( 5%) | 11 (27%) |
*
mean values represent average of mean scores from each subject
a, b, c
values with different superscripts within the same row were statistically different
†
Continuous outcomes: for WtZ, HtZ and BMIZ, GEE models also included age, sex and mode of diagnosis; for domain scores, the GEE models included CFQ version (child or adolescent), age, sex and mode of diagnosis
‡
Categorical outcomes: CMH models utilized all observations and accounted for CFQ version and age.