Evidence for a Cystic Fibrosis Enteropathy.

Marlou P M Adriaanse,Edward Dompeling,Anita M Van Den Neucker,Linda J T M Van Der Sande,Wim A Buurman,Paul P C A Menheere,Anita C E Vreugdenhil,Nades Palaniyar

doi:10.1371/journal.pone.0138062

Marlou P M Adriaanse, Edward Dompeling + Show 6 more

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https://doi.org/10.1371/journal.pone.0138062

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Journal: PloS one	Publication Date: Oct 20, 2015
Citations: 33	License type: CC BY 4.0

Affiliation: Maastricht University Medical Centre

Abstract

BackgroundPrevious studies have suggested the existence of enteropathy in cystic fibrosis (CF), which may contribute to intestinal function impairment, a poor nutritional status and decline in lung function. This study evaluated enterocyte damage and intestinal inflammation in CF and studied its associations with nutritional status, CF-related morbidities such as impaired lung function and diabetes, and medication use.MethodsSixty-eight CF patients and 107 controls were studied. Levels of serum intestinal-fatty acid binding protein (I-FABP), a specific marker for enterocyte damage, were retrospectively determined. The faecal intestinal inflammation marker calprotectin was prospectively studied. Nutritional status, lung function (FEV1), exocrine pancreatic insufficiency (EPI), CF-related diabetes (CFRD) and use of proton pump inhibitors (PPI) were obtained from the medical charts.ResultsSerum I-FABP levels were elevated in CF patients as compared with controls (p<0.001), and correlated negatively with FEV1 predicted value in children (r-.734, p<0.05). Faecal calprotectin level was elevated in 93% of CF patients, and correlated negatively with FEV1 predicted value in adults (r-.484, p<0.05). No correlation was found between calprotectin levels in faeces and sputum. Faecal calprotectin level was significantly associated with the presence of CFRD, EPI, and PPI use.ConclusionThis study demonstrated enterocyte damage and intestinal inflammation in CF patients, and provides evidence for an inverse correlation between enteropathy and lung function. The presented associations of enteropathy with important CF-related morbidities further emphasize the clinical relevance.

Highlights

Cystic fibrosis (CF) is a complex multisystem disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, leading to dehydrated luminal secretions and impaired secretion clearance, affecting mainly the respiratory and gastrointestinal tract
Serum intestinal-fatty acid binding protein (I-FABP) levels were elevated in CF patients as compared with controls (p
Faecal calprotectin level was significantly associated with the presence of CFrelated diabetes (CFRD), exocrine pancreatic insufficiency (EPI), and pump inhibitors (PPI) use

Summary

Introduction

Cystic fibrosis (CF) is a complex multisystem disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, leading to dehydrated luminal secretions and impaired secretion clearance, affecting mainly the respiratory and gastrointestinal tract. The contribution of intestinal involvement in CF to the disease progress and development of complications is largely unknown. A compromised gut, with inflammation and enterocyte damage, both associated with malabsorption, may contribute to a poor nutritional status in CF patients [4, 5]. Poor nutritional status results in impaired growth and affects lung function and survival [6, 7]. Intestinal damage and inflammation, with consequent loss of barrier function [8], might potentially adversely affect lung function by translocation of bacteria and their toxins, further aggravating lung inflammation and worse clinical outcome [9, 10]

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