Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis

Table 1 Long-term follow-up of the cohort n (%)

Follow-up length in years	Study group	Control group
10	23 (88.4)	29 (93.5)
15	20 (76.9)	26 (83.8)
20	17 (65.3)	19 (61.3)
25	13 (50.0)	9 (35.4)
30	5 (19.2)	1 (3.2)
Total number of patients per group	26	31

Study group: Cystic fibrosis patients diagnosed with distal intestinal obstruction syndrome; Control group: Cystic fibrosis patients.

Table 2 Characteristics of the cohort n (%)

	Study group	Control group	P-value
Patients	26	31	NS
Sex
Male	19 (73.1)	20 (64.5)	NS
Female	7 (26.9)	9 (35.5)	NS
Age, mean ± SD (years-old)	9.7 ± 22.5	6.7 ± 20.9	NS
Genotype
Homozygote	13 (50.0)	12 (38.7)	NS
Heterozygote	8 (30.8)	9 (35.5)	NS
Unknown	5 (19.2)	8 (25.8)	NS
DeltaF508	12 (46.2)	18 (58.1)	NS
BMI, weight/height	18.4	19.3	NS
PI	18 (69.2)	27 (87.1)	NS
MI in the past	17 (65.4)	0 (0.0)	< 0.021
Lung colonization
Pseudomonas aeruginosa	22 (84.6)	28 (90.3)	NS
Staphylococcus aureus	14 (53.8)	13 (41.9)	NS
Burkholderia cepacia	2 (7.7)	0 (0.0)	NS
MOTT	2 (7.7)	5 (16.1)	NS
Aspergillus spp.	9 (34.6)	1 (3.2)	< 0.021

¹Statistically significant. Study group: Cystic fibrosis patients diagnosed with distal intestinal obstruction syndrome; Control group: Cystic fibrosis patients; n: Number of patients; SD: Standard deviation; BMI: Body mass index; MI: Meconium ileus; MOTT: Mycobacteria other than tuberculosis; PI: Pancreatic insufficiency; NS: Not significant.

Table 3 Forced expired volume in 1 s (% predicted) comparing study patients and controls in the 5-year period prior to the study

FEV1	Study group		Control group		P-value
	n	Mean % predicted ± SD	n	Mean % predicted ± SD
1st yr	17	68.8 ± 19.5	22	70.0 ± 16.9	0.837
2nd yr	21	66.6 ± 19.1	29	71.0 ± 16.3	0.386
3rd yr	21	69.7 ± 21.0	25	69.9 ± 18.2	0.978
4th yr	21	67.7 ± 20.4	29	68.5 ± 18.6	0.876
5th yr	22	70.7 ± 20.1	17	68.5 ± 19.8	0.691

Study group: Cystic fibrosis patients diagnosed with distal intestinal obstruction syndrome; Control group: Cystic fibrosis patients; n: Number of patients; SD: Standard deviation; FEV1: Forced expired volume after 1 s of blowing out; SD: Standard deviation. A P < 0.05 is considered statistically significant.

Table 4 Management strategies for the intestinal obstruction in the study group

Type of treatment	n (%)
Conservative	45 (75.0)
Gastrografin PO	15 (33.3)
Enema	12 (26.6)
Combination of Gastrografin PO + enema and/or Gastrografin enema and/or N-acetylcysteine PO	11 (24.4)
Gastrografin enema	4 (8.8)
N-acetylcysteine enema	3 (6.6)
Surgical	11 (18.3)
Laparotomy with milking	3 (27.2)
Other	8 (72.7)
No intervention	4 (6.6)
Total	60 (100)
Supportive	37 (61.6)
Antibiotics	10 (27.0)
IV fluids	13 (35.1)
Feeding tube	6 (16.2)
Peglax	6 (16.2)
Laxatives	2 (5.4)

n: Number of treatments; PO: Per os; IV: Intravenous.