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Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies

Authors Kalnins, Wilschanski M

Received 19 October 2011

Accepted for publication 10 January 2012

Published 20 June 2012 Volume 2012:6 Pages 151—161

DOI https://doi.org/10.2147/DDDT.S9258

Review by Single-blind

Peer reviewer comments 4


Daina Kalnins,1 Michael Wilschanski2

1Clinical Dietetics, Respiratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada; 2Pediatric Gastroenterology Unit, Hadassah University Hospitals, Jerusalem, Israel

Abstract: Poor clinical outcomes in cystic fibrosis are often associated with undernutrition. Normal growth and development should be achieved in cystic fibrosis, and nutritional counseling is paramount at all ages. Prevention and early detection of growth failure is the key to successful nutritional intervention. The advance in nutritional management is certainly one factor that has contributed to the improved survival in recent decades. This review outlines the major nutritional parameters in the management of the patient with cystic fibrosis, including recent advances in pancreatic enzyme replacement therapy and fat-soluble vitamin therapy. There are sections on complicated clinical situations which directly affect nutrition, for example, before and after lung transplantation, cystic fibrosis-related diabetes, and bone health.

Keywords: cystic fibrosis, nutrition, fat-soluble vitamins, pancreatic enzymes

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