Observational Study
Copyright ©The Author(s) 2015.
World J Gastroenterol. Jan 7, 2015; 21(1): 318-325
Published online Jan 7, 2015. doi: 10.3748/wjg.v21.i1.318
Table 1 Long-term follow-up of the cohort n (%)
Follow-up length in yearsStudy groupControl group
1023 (88.4)29 (93.5)
1520 (76.9)26 (83.8)
2017 (65.3)19 (61.3)
2513 (50.0)9 (35.4)
305 (19.2)1 (3.2)
Total number of patients per group2631
Table 2 Characteristics of the cohort n (%)
Study groupControl groupP-value
Patients2631NS
Sex
Male19 (73.1)20 (64.5)NS
Female7 (26.9)9 (35.5)NS
Age, mean ± SD (years-old)9.7 ± 22.56.7 ± 20.9NS
Genotype
Homozygote13 (50.0)12 (38.7)NS
Heterozygote8 (30.8)9 (35.5)NS
Unknown5 (19.2)8 (25.8)NS
DeltaF50812 (46.2)18 (58.1)NS
BMI, weight/height18.419.3NS
PI18 (69.2)27 (87.1)NS
MI in the past17 (65.4)0 (0.0)< 0.021
Lung colonization
Pseudomonas aeruginosa22 (84.6)28 (90.3)NS
Staphylococcus aureus14 (53.8)13 (41.9)NS
Burkholderia cepacia2 (7.7)0 (0.0)NS
MOTT2 (7.7)5 (16.1)NS
Aspergillus spp.9 (34.6)1 (3.2)< 0.021
Table 3 Forced expired volume in 1 s (% predicted) comparing study patients and controls in the 5-year period prior to the study
FEV1Study group
Control group
P-value
nMean % predicted ± SDnMean % predicted ± SD
1st yr1768.8 ± 19.52270.0 ± 16.90.837
2nd yr2166.6 ± 19.12971.0 ± 16.30.386
3rd yr2169.7 ± 21.02569.9 ± 18.20.978
4th yr2167.7 ± 20.42968.5 ± 18.60.876
5th yr2270.7 ± 20.11768.5 ± 19.80.691
Table 4 Management strategies for the intestinal obstruction in the study group
Type of treatmentn (%)
Conservative45 (75.0)
Gastrografin PO15 (33.3)
Enema12 (26.6)
Combination of Gastrografin PO + enema and/or Gastrografin enema and/or N-acetylcysteine PO11 (24.4)
Gastrografin enema4 (8.8)
N-acetylcysteine enema3 (6.6)
Surgical11 (18.3)
Laparotomy with milking3 (27.2)
Other8 (72.7)
No intervention4 (6.6)
Total60 (100)
Supportive37 (61.6)
Antibiotics10 (27.0)
IV fluids13 (35.1)
Feeding tube6 (16.2)
Peglax6 (16.2)
Laxatives2 (5.4)