Liver Disease in Cystic Fibrosis May Be Caused by Increased Intestinal Lesions, Microbiome Alterations

Researchers at the University of Colorado School of Medicine at the Anschutz Medical Campus discovered a potential cause of liver disease in young people with cystic fibrosis. The study titled “Intestinal Lesions Are Associated with Altered Intestinal Microbiome and Are More Frequent in Children and Young Adults with Cystic Fibrosis and Cirrhosis” is published in the journal PLoS One.

Cystic fibrosis is a genetic condition that affects the lungs and the digestive system. The condition usually manifests itself in children, with estimations showing that in the United States approximately 30,000 children and adults suffer from CF. Cirrhosis has been found to occur in 5–7% of cystic fibrosis patients. Based on these estimates, the research team led by Michael Narkewicz, aimed to determine if alterations in intestinal function in cystic fibrosis contribute to the development of cirrhosis.

A total of 11 subjects with liver disease were compared to 19 subjects without the condition. The results revealed that young people with liver disease had a microbiome (gut bacteria) differentiation compared to young people without the condition. Compared to young people without liver disease, young people with liver disease were found to have lower small bowel motility and more small bowel inflammation signs compared to those without liver disease.

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Based on these findings, the researchers concluded that liver disease is associated with increased intestinal mucosal lesions, slower small bowel transit time and alterations in fecal microbiome.

The researchers also suggest that abnormal intestinal permeability and elevated fecal calprotectin are common in subjects with cystic fibrosis. This discovery highlights that disturbances in intestinal function in patients with cystic fibrosis combined with changes in the microbiome may contribute to the development of hepatic fibrosis and intestinal lesions.

“While some intestinal symptoms are common in all cystic fibrosis patients,” Narkewicz said, “we found there are some who have disturbances in intestinal function combined with changes in the gut microbiome that may contribute to liver disease. We hope this finding will point toward a better understanding of why only 5 to 7 percent of cystic fibrosis patients develop severe liver disease and will suggest potential therapies to help those patients.”