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Beyond pancreatic insufficiency and liver disease in cystic fibrosis

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Abstract

Cystic fibrosis is a life shortening hereditary disease, primarily leading to progressive pulmonary infection and exocrine pancreatic dysfunction. Several gastrointestinal complications other than malabsorption can arise during the disease course and with the progressively increasing life span of patients with CF; new and more rare complications are being recognized. We review the literature on gastrointestinal manifestations in CF, excluding the liver and pancreas.

Conclusion: We describe the clinical presentation and treatment of more common conditions like gastroesophageal reflux, small intestinal bacterial overgrowth, intussusception, meconium ileus, distal intestinal obstruction syndrome, and constipation, and we also discuss what is known on celiac disease, appendicitis, fibrosing colonopathy, inflammation and inflammatory bowel disease and gastrointestinal cancer.

What is Known:

• Gastrointestinal complications arise early in the course of the disease and have a severe impact on the quality of life of the patients.

What is New:

• This review is a concise summary of the current literature on gastrointestinal complications of cystic fibrosis.

• We focused on clinical presentation and diagnostic investigations and provide a comprehensive resume of the current treatment options.

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Abbreviations

CF:

Cystic fibrosis

CFTR:

Cystic fibrosis transmembrane conductance regulator

DIOS:

Distal intestinal obstruction syndrome

ESPGHAN:

European Society for Paediatric Gastroenterology Hepatology and Nutrition

FEV1:

Forced expiratory volume at one second

GER:

Gastroesophageal reflux

GERD:

Gastroesophageal reflux disease

IL:

Interleukin

MI:

Meconium ileus

NO:

Nitric oxide

SIBO:

Small intestinal bacterial overgrowth

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Author’s contribution

Prof. Dr. Kris De Boeck and Prof. Dr. Peter Witters contributed to the critical revision. All authors contributed to the literature search, critical evaluation of the information and creation of the review.

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Authors and Affiliations

  1. Universitaire Ziekenhuizen Leuven, Leuven, Vlaams-Brabant, Belgium

    Stephanie Demeyer

  2. Department of Pediatrics, University Hospital Gasthuisberg, Herestraat 49, Leuven, 3000, Belgium

    Stephanie Demeyer, Kris De Boeck & Peter Witters

  3. Department of Pharmocology, University Hospital Gasthuisberg, Herestraat 49, Leuven, 3000, Belgium

    Katrien Cosaert

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Demeyer, S., De Boeck, K., Witters, P. et al. Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr 175, 881–894 (2016). https://doi.org/10.1007/s00431-016-2719-5

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