Table 1.
BONUS CF infant Cohort N = 231 |
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---|---|---|---|
| |||
PI + initiated PERT N = 205 |
PI + no PERT use N = 6 |
PS N = 20 |
|
| |||
Fecal Elastase (μg/g) | |||
≤50 | 144 (70.2%) | 1 (16.7%) | 0 (0%) |
50–≤ 100 | 25 (12.2%) | 0 (0%) | 0 (0%) |
100–≤ 200 | 13 (6.3%) | 1 (16.7%) | 0 (0%) |
>200 | 1 (0.5%) | 2 (33.3%) | 16 (80%) |
Unavailable | 22 (10.7%) | 2 (33.3%) | 4 (20%) |
| |||
Genotype Class | |||
I–III/I–III | 186 (90.7%) | 6 (100%) | 3 (15%) |
IV–V/any | 10 (4.9%) | 0 (0%) | 9 (45%) |
I–III/unknown | 8 (3.9%) | 0 (0%) | 8 (40%) |
Unknown/unknown | 1 (0.5%) | 0 (0%) | 0 (0%) |
| |||
Meconium Ileus | |||
Yes | 25 (12.2%) | 1 (16.7%) | 1 (5%) |
No | 180 (87.8%) | 5 (83.3%) | 19 (95%) |
| |||
Birth weight z-score (mean, SD) | −0.19 (0.88) | 0.26 (1.17) | 0.08 (1.09) |
| |||
Breastfeeding Status | |||
Excl BF at 3 mos | 54 (26.3%) | 2 (33.3%) | 1 (5%) |
BF and FML @ 3 mos | 38 (18.5%) | 0 (0%) | 6 (30%) |
Excl FML @ 3 mos | 112 (54.6%) | 3 (50.0%) | 12 (60%) |
| |||
Acid Blocker Usage | |||
None | 61 (29.8%) | 5 (83.3%) | 12 (60%) |
PPI only | 34 (16.6%) | 0 (0%) | 3 (15%) |
H2 Blocker only | 63 (30.7%) | 0 (0%) | 5 (25%) |
PPI + H2 | 47 (22.9%) | 1 (16.7%) | 0 (0%) |
| |||
Participant specific max PERT dose 0–6 mos | |||
Lipase Units/Kg/Meal | |||
N | 205 | ||
mean (sd) | 1882 (621) | ||
min, max | 492, 3727 | ||
<1000 | 18 (8.8%) | ||
1000–2000 | 99 (48.3%) | ||
>2000 | 88 (42.9%) | ||
| |||
Participant specific max PERT dose 6–12 mos | |||
Lipase Units/Kg/Meal | |||
N | 196 | ||
mean (sd) | 1842 (617) | ||
min, max | 313, 3612 | ||
<1000 | 20 (9.8%) | ||
1000–2000 | 90 (43.9%) | ||
>2000 | 86 (42.0%) |
PI=pancreatic insufficient; PS= pancreatic sufficient; SD=standard deviation; Excl= exclusive; BF=breast fed; FML=formula fed; PPI=proton pump inhibitor