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Semin Respir Crit Care Med 2019; 40(06): 825-841
DOI: 10.1055/s-0039-1697591
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis

Megan E. Gabel‡   *
1   Division of Pediatric Gastroenterology, Golisano Children's Hospital at Strong, University of Rochester, Rochester, New York
,
Gary J. Galante‡   *
2   Section of Pediatric Gastroenterology, Hepatology and Nutrition, Alberta Children's Hospital, University of Calgary, Calgary, Alberta
,
Steven D. Freedman
3   Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts
› Author Affiliations
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Publication History

Publication Date:
28 October 2019 (online)

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Abstract

Cystic fibrosis (CF) is a multiorgan disease, and gastrointestinal (GI) manifestations can contribute to significant morbidity and mortality for individuals with CF. Up to 85% of patients with CF experience GI symptoms, thus addressing the GI aspects of this disease is paramount. With the advent of highly effective CF transmembrane conductance regulator modulators that are increasingly available, many individuals with CF now have significantly improved life expectancy. With these advances, GI manifestations that can be a detriment to quality of life such as gastroesophageal reflux disease, dysbiosis, and chronic abdominal pain have become a priority for patients and caregivers. In addition, as individuals have increased longevity, it has become essential for care providers to be aware of topics such as hepatobiliary disease and colorectal cancer screening. An understanding of the wide scope of GI manifestations in CF can enable providers to optimize the overall health and well-being of their patients. In this review, we aim to provide an up-to-date overview of key aspects of GI and hepatic disease in CF.

Keywords

meconium ileus - gastroesophageal reflux disease - distal intestinal obstruction syndrome - dysbiosis - hepatobiliary - pancreatic function

* Both the author contributed equally to the article.