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Beyond pancreatic insufficiency and liver disease in cystic fibrosis.
Eur J Pediatr 2016; 175(7):881-94EJ

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.

Authors+Show Affiliations

Universitaire Ziekenhuizen Leuven, Leuven, Vlaams-Brabant, Belgium. stephanie.demeyer@uzleuven.be. Department of Pediatrics, University Hospital Gasthuisberg, Herestraat 49, Leuven, 3000, Belgium. stephanie.demeyer@uzleuven.be.Department of Pediatrics, University Hospital Gasthuisberg, Herestraat 49, Leuven, 3000, Belgium.Department of Pediatrics, University Hospital Gasthuisberg, Herestraat 49, Leuven, 3000, Belgium.Department of Pharmocology, University Hospital Gasthuisberg, Herestraat 49, Leuven, 3000, Belgium.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

27055450

Citation

Demeyer, Stephanie, et al. "Beyond Pancreatic Insufficiency and Liver Disease in Cystic Fibrosis." European Journal of Pediatrics, vol. 175, no. 7, 2016, pp. 881-94.
Demeyer S, De Boeck K, Witters P, et al. Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr. 2016;175(7):881-94.
Demeyer, S., De Boeck, K., Witters, P., & Cosaert, K. (2016). Beyond pancreatic insufficiency and liver disease in cystic fibrosis. European Journal of Pediatrics, 175(7), pp. 881-94. doi:10.1007/s00431-016-2719-5.
Demeyer S, et al. Beyond Pancreatic Insufficiency and Liver Disease in Cystic Fibrosis. Eur J Pediatr. 2016;175(7):881-94. PubMed PMID: 27055450.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Beyond pancreatic insufficiency and liver disease in cystic fibrosis. AU - Demeyer,Stephanie, AU - De Boeck,Kris, AU - Witters,Peter, AU - Cosaert,Katrien, Y1 - 2016/04/07/ PY - 2016/01/19/received PY - 2016/03/22/accepted PY - 2016/03/17/revised PY - 2016/4/9/entrez PY - 2016/4/9/pubmed PY - 2017/8/2/medline KW - Cystic fibrosis KW - Diagnosis and treatment KW - Gastrointestinal complications KW - Symptoms SP - 881 EP - 94 JF - European journal of pediatrics JO - Eur. J. Pediatr. VL - 175 IS - 7 N2 - UNLABELLED: 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. SN - 1432-1076 UR - https://www.unboundmedicine.com/medline/citation/27055450/Beyond_pancreatic_insufficiency_and_liver_disease_in_cystic_fibrosis L2 - https://dx.doi.org/10.1007/s00431-016-2719-5 DB - PRIME DP - Unbound Medicine ER -