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European Journal of Pediatrics

Erschienen in:

07.04.2016 | Review

Beyond pancreatic insufficiency and liver disease in cystic fibrosis

verfasst von: Stephanie Demeyer, Kris De Boeck, Peter Witters, Katrien Cosaert

Erschienen in: European Journal of Pediatrics | Ausgabe 7/2016

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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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Titel: Beyond pancreatic insufficiency and liver disease in cystic fibrosis
verfasst von: Stephanie Demeyer
Kris De Boeck
Peter Witters
Katrien Cosaert
Publikationsdatum: 07.04.2016
Verlag: Springer Berlin Heidelberg
Erschienen in: European Journal of Pediatrics / Ausgabe 7/2016
Print ISSN: 0340-6199
Elektronische ISSN: 1432-1076
DOI: https://doi.org/10.1007/s00431-016-2719-5

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Beyond pancreatic insufficiency and liver disease in cystic fibrosis

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