Unknown

Dataset Information

0

Intestinal lesions are associated with altered intestinal microbiome and are more frequent in children and young adults with cystic fibrosis and cirrhosis.


ABSTRACT:

Background and aims

Cirrhosis (CIR) occurs in 5-7% of cystic fibrosis (CF) patients. We hypothesized that alterations in intestinal function in CF contribute to the development of CIR.

Aims

Determine the frequency of macroscopic intestinal lesions, intestinal inflammation, intestinal permeability and characterize fecal microbiome in CF CIR subjects and CF subjects with no liver disease (CFnoLIV).

Methods

11 subjects with CFCIR (6 M, 12.8 yrs ± 3.8) and 19 matched with CFnoLIV (10 M, 12.6 yrs ± 3.4) underwent small bowel capsule endoscopy, intestinal permeability testing by urinary lactulose: mannitol excretion ratio, fecal calprotectin determination and fecal microbiome characterization.

Results

CFCIR and CFnoLIV did not differ in key demographics or CF complications. CFCIR had higher GGT (59±51 U/L vs 17±4 p = 0.02) and lower platelet count (187±126 vs 283±60 p = 0.04) and weight (-0.86 ± 1.0 vs 0.30 ± 0.9 p = 0.002) z scores. CFCIR had more severe intestinal mucosal lesions on capsule endoscopy (score ?4, 4/11 vs 0/19 p = 0.01). Fecal calprotectin was similar between CFCIR and CFnoLIV (166 ?g/g ±175 vs 136 ± 193 p = 0.58, nl <120). Lactulose:mannitol ratio was elevated in 27/28 subjects and was slightly lower in CFCIR vs CFnoLIV (0.08±0.02 vs 0.11±0.05, p = 0.04, nl ?0.03). Small bowel transit time was longer in CFCIR vs CFnoLIV (195±42 min vs 167±68 p<0.001, nl 274 ± 41). Bacteroides were decreased in relative abundance in CFCIR and were associated with lower capsule endoscopy score whereas Clostridium were more abundant in CFCIR and associated with higher capsule endoscopy score.

Conclusions

CFCIR is associated with increased intestinal mucosal lesions, slower small bowel transit time and alterations in fecal microbiome. Abnormal intestinal permeability and elevated fecal calprotectin are common in all CF subjects. Disturbances in intestinal function in CF combined with changes in the microbiome may contribute to the development of hepatic fibrosis and intestinal lesions.

SUBMITTER: Flass T 

PROVIDER: S-EPMC4319904 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature

altmetric image

Publications

Intestinal lesions are associated with altered intestinal microbiome and are more frequent in children and young adults with cystic fibrosis and cirrhosis.

Flass Thomas T   Tong Suhong S   Frank Daniel N DN   Wagner Brandie D BD   Robertson Charles E CE   Kotter Cassandra Vogel CV   Sokol Ronald J RJ   Zemanick Edith E   Accurso Frank F   Hoffenberg Edward J EJ   Narkewicz Michael R MR  

PloS one 20150206 2


<h4>Background and aims</h4>Cirrhosis (CIR) occurs in 5-7% of cystic fibrosis (CF) patients. We hypothesized that alterations in intestinal function in CF contribute to the development of CIR.<h4>Aims</h4>Determine the frequency of macroscopic intestinal lesions, intestinal inflammation, intestinal permeability and characterize fecal microbiome in CF CIR subjects and CF subjects with no liver disease (CFnoLIV).<h4>Methods</h4>11 subjects with CFCIR (6 M, 12.8 yrs ± 3.8) and 19 matched with CFnoL  ...[more]

Similar Datasets

| S-EPMC7920415 | biostudies-literature
| S-EPMC4629800 | biostudies-literature
| S-EPMC10449510 | biostudies-literature
| S-EPMC9675243 | biostudies-literature
| S-EPMC6014676 | biostudies-literature
| S-EPMC7996874 | biostudies-literature
| S-EPMC7686462 | biostudies-literature
| S-EPMC8048771 | biostudies-literature
| S-EPMC6314917 | biostudies-literature
| S-EPMC10462202 | biostudies-literature